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Sample records for mucinous cystic neoplasm

  1. [A case of mucinous cystic neoplasm of the liver].

    PubMed

    Tatsumi, Ryoji; Amizuka, Hisato; Matsubara, Yu; Yoshizaki, Koji; Sakamoto, Jun; Sato, Ryu; Kimura, Keisuke; Nishimori, Hiroyuki; Ohta, Tomoyuki

    2015-07-01

    We present a case of resected mucinous cystic neoplasm of the liver in a 71-year-old woman admitted to our hospital with epigastric discomfort. Abdominal ultrasonography and computed tomography revealed a multi-locular cystic tumor measuring 35 mm in diameter in segment IV of the liver. Left hepatic lobectomy was performed based on the diagnosis of mucinous cystic neoplasm of the liver; subsequent histology revealed that the tumor was multi-locular, cystic, and lined with a single layer of columnar epithelium with low-grade atypia and was associated with a typical ovarian-like stroma. There was no evidence (imaging or histological) to support communication of the cyst with the intrahepatic bile duct, despite modest bile deposition being observed in the cystic wall. The definitive diagnosis was mucinous cystic neoplasm with low-grade intrahepatic epithelial neoplasia. PMID:26155869

  2. Mucinous cystic neoplasms of the mesentery: a case report and review of the literature

    PubMed Central

    Metaxas, Georgios; Tangalos, Athanasios; Pappa, Polyxeni; Papageorgiou, Irene

    2009-01-01

    Background Mucinous cystic neoplasms arise in the ovary and various extra-ovarian sites. While their pathogenesis remains conjectural, their similarities suggest a common pathway of development. There have been rare reports involving the mesentery as a primary tumour site. Case presentation A cystic mass of uncertain origin was demonstrated radiologically in a 22 year old female with chronic abdominal pain. At laparotomy, the mass was fixed within the colonic mesentery. Histology demonstrated a benign mucinous cystadenoma. Methods and results We review the literature on mucinous cystic neoplasms of the mesentery and report on the pathogenesis, biologic behavior, diagnosis and treatment of similar extra-ovarian tumors. We propose an updated classification of mesenteric cysts and cystic tumors. Conclusion Mucinous cystic neoplasms of the mesentery present almost exclusively in women and must be considered in the differential diagnosis of mesenteric tumors. Only full histological examination of a mucinous cystic neoplasm can exclude a borderline or malignant component. An updated classification of mesenteric cysts and cystic tumors is proposed. PMID:19454018

  3. Overweight increases the risk of malignancy in patients with pancreatic mucinous cystic neoplasms.

    PubMed

    Chang, Yu-Ting; Tien, Yu-Wen; Jeng, Yung-Ming; Yang, Ching-Yao; Liang, Po-Chin; Wong, Jau-Min; Chang, Ming-Chu

    2015-05-01

    Distinguishing between benign and malignant pancreatic cysts remains a clinical challenge. The aim of this study was to investigate the influence of body mass index (BMI) and preoperative clinical and cyst features, as described by the International Consensus Guidelines, on malignancy in patients with pancreatic mucinous cystic neoplasms (PMCNs).A retrospective cohort study was performed on patients with PMCNs who underwent surgical resection between January 1994 and June 2014. Preoperative BMI, clinical demographic data, cystic features, tumor markers, and surgical pathology results were analyzed. Predictors of malignancy were determined by univariate and multivariate analysis using logistic regression.One hundred sixty-four cases of PMCNs, including 106 intraductal papillary mucinous neoplasms (IPMNs) and 58 mucinous cystic neoplasms (MCNs), were analyzed. On univariate analysis, older age (P = 0.008), male sex (P = 0.007), high-risk stigmata (P = 0.007), diabetes mellitus (DM; P = 0.008), and BMI >25 (P < 0.001) were associated with malignancy. Multivariate analysis found that BMI >25 (odds ratio, 3.99; 95% confidence interval: 1.60-10) was an independent predictor of malignancy. In subgroup analysis, BMI >25 was an independent predictor of malignancy in IPMNs but not in MCNs.Overweight patients with IPMNs have a higher risk of malignancy and should be followed closely or undergo resection. The operative strategy for PMCNs should consider cyst-related and patient-related risk factors. PMID:25997051

  4. Overweight Increases the Risk of Malignancy in Patients with Pancreatic Mucinous Cystic Neoplasms

    PubMed Central

    Chang, Yu-Ting; Tien, Yu-Wen; Jeng, Yung-Ming; Yang, Ching-Yao; Liang, Po-Chin; Wong, Jau-Min; Chang, Ming-Chu

    2015-01-01

    Abstract Distinguishing between benign and malignant pancreatic cysts remains a clinical challenge. The aim of this study was to investigate the influence of body mass index (BMI) and preoperative clinical and cyst features, as described by the International Consensus Guidelines, on malignancy in patients with pancreatic mucinous cystic neoplasms (PMCNs). A retrospective cohort study was performed on patients with PMCNs who underwent surgical resection between January 1994 and June 2014. Preoperative BMI, clinical demographic data, cystic features, tumor markers, and surgical pathology results were analyzed. Predictors of malignancy were determined by univariate and multivariate analysis using logistic regression. One hundred sixty-four cases of PMCNs, including 106 intraductal papillary mucinous neoplasms (IPMNs) and 58 mucinous cystic neoplasms (MCNs), were analyzed. On univariate analysis, older age (P = 0.008), male sex (P = 0.007), high-risk stigmata (P = 0.007), diabetes mellitus (DM; P = 0.008), and BMI >25 (P < 0.001) were associated with malignancy. Multivariate analysis found that BMI >25 (odds ratio, 3.99; 95% confidence interval: 1.60–10) was an independent predictor of malignancy. In subgroup analysis, BMI >25 was an independent predictor of malignancy in IPMNs but not in MCNs. Overweight patients with IPMNs have a higher risk of malignancy and should be followed closely or undergo resection. The operative strategy for PMCNs should consider cyst-related and patient-related risk factors. PMID:25997051

  5. Cytopathological Analysis of Cyst Fluid Enhances Diagnostic Accuracy of Mucinous Pancreatic Cystic Neoplasms

    PubMed Central

    Utomo, Wesley K.; Braat, Henri; Bruno, Marco J.; van Eijck, Casper H.J.; Koerkamp, Bas Groot; Krak, Nanda C.; van de Vreede, Adriaan; Fuhler, Gwenny M.; Peppelenbosch, Maikel P.; Biermann, Katharina

    2015-01-01

    Abstract Widespread use of cross-sectional imaging and increasing age of the general population has increased the number of detected pancreatic cystic lesions. However, several pathological entities with a variety in malignant potential have to be discriminated to allow clinical decision making. Discrimination between mucinous pancreatic cystic neoplasms (PCNs) and nonmucinous pancreatic lesions is the primary step in the clinical work-up, as malignant transformation is mostly associated with mucinous PCN. We performed a retrospective analysis of all resected PCN in our tertiary center from 2000 to 2014, to evaluate preoperative diagnostic performance and the results of implementation of the consensus guidelines over time. This was followed by a prospective cohort study of patients with an undefined pancreatic cyst, where the added value of cytopathological mucin evaluation to carcinoembryonic antigen (CEA) in cyst fluid for the discrimination of mucinous PCN and nonmucinous cysts was investigated. Retrospective analysis showed 115 patients operated for a PCN, with a correct preoperative classification in 96.2% of the patients. High-grade dysplasia or invasive carcinoma was observed in only 32.3% of mucinous PCN. In our prospective cohort (n = 71), 57.7% of patients were classified as having a mucinous PCN. CEA ≥192 ng/mL had an accuracy of 63.4%, and cytopathological mucin evaluation an accuracy of 73.0%. Combining these 2 tests further improved diagnostic accuracy of a mucinous PCN to 76.8%. CEA level and mucin evaluation were not predictive of the degree of dysplasia. These findings show that adding cytopathology to cyst fluid biochemistry improves discrimination between mucinous PCN and nonmucinous cysts.

  6. Mucinous Cystic Neoplasm of the Liver Masquerading as an Echinococcal Cyst: Radiologic-pathologic Differential of Complex Cystic Liver Lesions

    PubMed Central

    Jeong, Daniel; Jiang, Kun; Anaya, Daniel A.

    2016-01-01

    Although simple liver cysts are common, complex cystic liver lesions are infrequent and represent a diagnostic and therapeutic challenge. The differential diagnosis of complex cystic liver lesions can be grouped into neoplastic, infectious or inflammatory, and miscellaneous pathologic entities. Clinicians should remember to consider mucinous cystic neoplasm and echinococcal cysts in the differential, which are uncommon etiologies for liver lesions but may expose unique challenges. We present a case of a 49-year-old female who was referred for evaluation of a new complex cystic liver lesion. The following brief review describes how radiologic imaging and pathologic testing can help distinguish between the broad spectrum of diseases that may produce cystic liver lesions. PMID:27195178

  7. Glandular neoplasms of the urachus: a report of 55 cases emphasizing mucinous cystic tumors with proposed classification.

    PubMed

    Amin, Mahul B; Smith, Steven C; Eble, John N; Rao, Priya; Choi, William W L; Tamboli, Pheroze; Young, Robert H

    2014-08-01

    Published experience remains limited for glandular neoplasms of the urachus, especially mucinous cystic tumors. We reviewed 55 glandular urachal neoplasms to evaluate their clinical features and histopathologic spectrum and to devise a classification system for the mucinous cystic forms. Within the 55 cases studied, we observed 2 groups with differing clinical, gross, and histopathologic features. The first group, invasive, noncystic adenocarcinomas (n=24), had clinicopathologic features in accord with the known spectrum of urachal adenocarcinoma (mean age 50 y, female:male ratio 1.7, with recurrence or death from disease in 9/16 cases over a 45 mo mean follow-up). The second group, mucinous cystic tumors (n=31), morphologically resembled mucinous cystic tumors of the ovary and appeared classifiable by the same approach (mean age 47 y, female:male ratio 1.4) and included mucinous cystadenoma (n=4), mucinous cystic tumor of low malignant potential (n=22, including 2 cases with intraepithelial carcinoma), and mucinous cystadenocarcinoma with microscopic (n=4) or frank invasion (n=1). Follow-up information was available for 13 patients with mucinous cystic tumors (mean 41 mo); we observed no local recurrence or distant metastasis. This experience suggests that there is a distinct group of glandular, cystic tumors of the urachus that is classifiable in a manner similar to ovarian neoplasms and that has a favorable prognosis after complete excision. As with cystic neoplasms of other organs, rigorous sampling is recommended to identify potentially small foci of carcinoma that could be missed by inadequate sampling. Accordingly, classification based on methods other than complete surgical excision may be hazardous. PMID:25025366

  8. Anaplastic Carcinoma Arising in a Mucinous Cystic Neoplasm Masquerading as Pancreatic Pseudocyst.

    PubMed

    Aldaoud, Najla; Joudeh, Amani; Al-Momen, Sami; Alnahawi, Mamdouh; Al-Abbadi, Mousa A

    2016-06-01

    Mucinous cystic neoplasms (MCN) of the pancreas can vary from benign to premalignant and malignant. Preoperative diagnosis is essential to offer the patient appropriate treatment. Occasionally these cases may harbor anaplastic carcinoma while clinically masquerade as a pseudocyst. Here in, we report an unusual case of a 37-year old female presented with recurrent abdominal pain that was suspected clinically and by imaging studies to have a pseudocyst. EUS-FNA with internal drainage of the cyst was performed. Cytological evaluation of the cyst fluid showed numerous inflammatory cells composed mainly of many neutrophils admixed with macrophages reminiscent of the usual pseudocyst content but there were scattered rare dyscohesive malignant cells which were highly pleomorphic with multinucleation. Immunostains on the cell block showed immunoreactivity of these cells including the multinucleated cells for Cam 5.2 and AE1/AE3 and focally for Ber-Ep4, Moc -31, and CA19-9. The subsequent resection confirmed the presence of anaplastic (undifferentiated) carcinoma (AC) arising in a MCN of the pancreas. Diagn. Cytopathol. 2016;44:538-542. © 2016 Wiley Periodicals, Inc. PMID:27028547

  9. Clinicopathologic Characteristics of 29 Invasive Carcinomas Arising in 178 Pancreatic Mucinous Cystic Neoplasms With Ovarian-type Stroma

    PubMed Central

    Jang, Kee-Taek; Park, Sang Mo; Basturk, Olca; Bagci, Pelin; Bandyopadhyay, Sudeshna; Stelow, Edward B.; Walters, Dustin M.; Choi, Dong Wook; Choi, Seoung Ho; Heo, Jin Seok; Sarmiento, Juan M.; Reid, Michelle D.; Adsay, Volkan

    2015-01-01

    Information on the clinicopathologic characteristics of invasive carcinomas arising from mucinous cystic neoplasms (MCNs) is limited, because in many early studies they were lumped and analyzed together with noninvasive MCNs. Even more importantly, many of the largest prior studies did not require ovarian-type stroma (OTS) for diagnosis. We analyzed 178 MCNs, all strictly defined by the presence of OTS, 98% of which occurred in perimenopausal women (mean age, 47 y) and arose in the distal pancreas. Twenty-nine (16%) patients had associated invasive carcinoma, and all were female with a mean age of 53. Invasion was far more common in tumors with grossly visible intracystic papillary nodule formation ≥ 1.0 cm (79.3% vs. 8.7%, P = 0.000) as well as in larger tumors (mean cyst size: 9.4 vs. 5.4 cm, P = 0.006); only 4/29 (14%) invasive carcinomas occurred in tumors that were < 5 cm; however, none were < 3 cm. Increased serum CA19-9 level (> 37 U/L) was also more common in the invasive tumors (64% vs. 23%, P = 0.011). Most invasive carcinomas (79%) were of tubular type, and the remainder (5 cases) were mostly undifferentiated carcinoma (2, with osteoclast-like giant cells), except for 1 with papillary features. Interestingly, there were no colloid carcinomas; 2 patients had nodal metastasis at the time of diagnosis, and both died of disease at 10 and 35 months, respectively. While noninvasive MCNs had an excellent prognosis (100% at 5 y), tumors with invasion often had an aggressive clinical course with 3- and 5-year survival rates of 44% and 26%, respectively (P = 0.000). The pT2 (> 2 cm) invasive tumors had a worse prognosis than pTl (≤ 2 cm) tumors (P = 0.000), albeit 3 patients with T1a (< 0.5 cm) disease also died of disease. In conclusion, invasive carcinomas are seen in 16% of MCNs and are mostly of tubular (pancreatobiliary) type; colloid carcinoma is not seen in MCNs. Serum CA19-9 is often higher in invasive carcinomas, and invasion is typically seen in OTS

  10. Immunohistochemical analysis of steroidogenic enzymes in ovarian-type stroma of pancreatic mucinous cystic neoplasms: Comparative study of subepithelial stromal cells in intraductal papillary mucinous neoplasms of the pancreas.

    PubMed

    Ishida, Kazuyuki; Sasano, Hironobu; Moriya, Takuya; Takahashi, Yayoi; Sugimoto, Ryo; Mue, Yoshiharu; Murakami, Keigo; Fujishima, Fumiyoshi; Nakamura, Yasuhiro; Morikawa, Takanori; Motoi, Fuyuhiko; Suzuki, Takashi; Unno, Michiaki; Sugai, Tamotsu

    2016-05-01

    Mucinous cystic neoplasms (MCNs) are generally defined as cyst-forming epithelial neoplasms that arise in the pancreas and harbor characteristic ovarian-type stroma beneath the epithelium. In this study, we compared the immunoreactivity of steroid-related factors in these subepithelial stromal cells in MCNs to those in intraductal papillary mucinous neoplasms (IPMNs) to further characterize this unique MCN ovarian-type stroma through evaluation of sex steroid biosynthesis. Twenty MCNs and twenty IPMNs were examined. Immunoreactivity of steroid hormone receptors, including estrogen receptor (ERα and ERβ), progesterone receptor (PR, PR-A, and PR-B), and androgen receptor (AR), was more frequently detected in MCN ovarian-type stromal cells than in IPMN stromal cells (P < 0.01). The H-scores (mean ± SD) of steroidogenic factor (SF)-1 were also significantly higher in MCNs (112.3 ± 33.1) than in IPMNs (0.9 ± 1.2) (P < 0.01). The steroidogenic enzymes cytochrome P450 cholesterol side-chain cleavage enzyme (P450scc), cytochrome P450 17 alpha-hydroxylase (P450c17) and 3β-hydroxysteroid dehydrogenase (3β-HSD) showed immunoreactivity in 9/20 (45.0 %), 15/20 (75.0 %) and 13/20 (65.0 %), respectively, of ovarian-type stroma from MCN cases. These results demonstrate that the ovarian-type stroma of MCNs can express steroidogenic enzymes. Thus, the ovarian-type stroma of MCNs can produce sex steroids that may also act on these cells. PMID:27060902

  11. Whole-exome sequencing identified the genetic origin of a mucinous neoplasm in a mature cystic teratoma.

    PubMed

    Choi, Youn Jin; Lee, Sung Hak; Kim, Min Sung; Jung, Seung-Hyun; Hur, Soo Young; Chung, Yeun-Jun; Lee, Sug Hyung

    2016-06-01

    Mucinous tumour arising from a mature cystic teratoma associated with pseudomyxoma peritonei (PMP) is a rare disease and its tissue origin is not easy to specify by conventional histological and immunohistochemical analyses. To identify the origin of a secondary tumour arising from a mature teratoma, we performed whole-exome sequencing of a PMP secondary to a primary ovarian mucinous tumour. The mucinous tumour was CK20 (+), CK7 (-) and CDX2 (+). Its genome harboured 28 somatic non-silent mutations (27 missense and 1 nonsense) that included eight putative driver gene mutations catalogued in COSMIC database (KRAS, GNAS, ZBTB38, ENAM, HTR5A, BAI1, ADAMTS8 and RASA3). KRAS mutation as well as mutations in genes that antagonise RAS signalling (RASA3 and ADAMTS8) suggest that alterations in RAS signalling may play a role in its development. More importantly, the concurrent KRAS and GNAS hotspot mutations, and CK20 (+), CK7 (-) and CDX2 (+) expression strongly indicated its appendiceal origin. Our results indicate that next-generation sequencing combined with histological and immunohistochemical analyses may be a better strategy than the conventional analyses alone to identify the origin of a secondary tumour arising from a mature teratoma. Also, the data suggest that a PMP secondary to a primary ovarian mucinous tumour genome arising in the teratoma may recapitulate the mutational features of appendiceal mucinous tumours. PMID:27114374

  12. Premalignant cystic neoplasms of the pancreas.

    PubMed

    Dudeja, Vikas; Allen, Peter J

    2015-02-01

    Due to increasing utilization of cross-sectional imaging, asymptomatic pancreatic cysts are frequently being diagnosed. Many of these cysts have premalignant potential and offer a unique opportunity for cancer prevention. Mucinous cystic neoplasm and intraductal papillary mucinous neoplasm are the major premalignant cystic neoplasms of pancreas. The prediction of the risk of malignancy (incidental and future risk of malignant transformation) and balancing the risks of watchful waiting with that of operative management with associated mortality and morbidity is the key to the management of these lesions. We review the literature that has contributed to the development of our approach to the management of these cystic neoplasms. We provide an overview of the key features used in diagnosis and in predicting malignancy. Particular attention is given to the natural history and management decision making. PMID:25726053

  13. Cystic Neoplasms of the Exocrine Pancreas

    PubMed Central

    Ribaux, C.; Schnyder, P.

    1990-01-01

    Cystic neoplasms of the pancreas are rare and their diagnosis and treatment can be difficult. This report details 7 patients who had histologically proven serous cystadenoma4, mucinous cystadenoma2 and cystadeno carcinoma1. Computed tomography and sonography allowed excellent preoperative assessment but to attempt a distinction between the histological variants may be hazardous. Two tumours were only autopsy findings and 5 patients underwent laparotomy. It is confirmed that potentially malignant mucinous cystadenomas and cytadenocarcinomas should be resected whenever possible; serous cystadenomas are always benign and should therefore be resected only when the diagnosis is doubtful or if they cause symptoms. PMID:2278913

  14. Prolapse into the bile duct and expansive growth is characteristic behavior of mucinous cystic neoplasm of the liver: report of two cases and review of the literature.

    PubMed

    Takano, Yuichi; Nagahama, Masatsugu; Yamamura, Eiichi; Maruoka, Naotaka; Mizukami, Hiroki; Tanaka, Jun-ichi; Ohike, Nobuyuki; Takahashi, Hiroshi

    2015-06-01

    Mucinous cystic neoplasm of the liver (MCN-L) is a very rare tumor whose detailed behavior is still unknown. We describe two cases of MCN-L that exhibited extremely interesting growth patterns, and discuss the characteristics of MCN-Ls. Both cases exhibited MCN-L that originated from the left hepatic lobe (Segment 4) and then prolapsed into the left hepatic duct and common bile duct, resulting in obstructive jaundice due to expansive growth. Endoscopic retrograde cholangiopancreatographies showed the characteristic oval-shaped filling defects in the bile ducts. Endoscopic ultrasound and intraductal ultrasound were useful for differentiating the tumors from stones, since multiple septal formations were observed inside the tumors. A literature search revealed that, over the past 10 years, 15 cases of MCN-L (biliary cystadenomas with ovarian-like stroma) that showed expansive growth in the bile duct had been reported. Prolapse into the bile duct and expansive growth appear to be characteristic behavior of MCN-L. In the future, additional data on more cases needs to be collected to further elucidate MCN-L pathophysiology. PMID:25951998

  15. Intraductal Papillary Mucinous Neoplasm of Pancreas

    PubMed Central

    Machado, Norman Oneil; al Qadhi, Hani; al Wahibi, Khalifa

    2015-01-01

    Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are neoplasms that are characterized by ductal dilation, intraductal papillary growth, and thick mucus secretion. This relatively recently defined pathology is evolving in terms of its etiopathogenesis, clinical features, diagnosis, management, and treatment guidelines. A PubMed database search was performed. All the relevant abstracts in English language were reviewed and the articles in which cases of IPMN could be identified were further scrutinized. Information of IPMN was derived, and duplication of information in several articles and those with areas of persisting uncertainties were excluded. The recent consensus guidelines were examined. The reported incidence of malignancy varies from 57% to 92% in the main duct-IPMN (MD-IPMN) and from 6% to 46% in the branch duct-IPMN (BD-IPMN). The features of high-risk malignant lesions that raise concern include obstructive jaundice in a patient with a cystic lesion in the pancreatic head, the findings on radiological imaging of a mass lesion of >30 mm, enhanced solid component, and the main pancreatic duct (MPD) of size ≥10 mm; while duct size 5-9 mm and cyst size <3 mm are considered as “worrisome features.” Magnetic resonance imaging (MRI) and endoscopic ultrasound (EUS) are primary investigations in diagnosing and following up on these patients. The role of pancreatoscopy and the analysis of aspirated cystic fluid for cytology and DNA analysis is still to be established. In general, resection is recommended for most MD-IPMN, mixed variant, and symptomatic BD-IPMN. The 5-year survival of patients after surgical resection for noninvasive IPMN is reported to be at 77-100%, while for those with invasive carcinoma, it is significantly lower at 27-60%. The follow-up of these patients could vary from 6 months to 1 year and would depend on the risk stratification for invasive malignancy and the pathology of the resected specimen. The understanding of

  16. Cystic micropapillary neoplasm of peribiliary glands with concomitant perihilar cholangiocarcinoma.

    PubMed

    Uchida, Tsuneyuki; Yamamoto, Yusuke; Ito, Takaaki; Okamura, Yukiyasu; Sugiura, Teiichi; Uesaka, Katsuhiko; Nakanuma, Yasuni

    2016-02-21

    We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diameter in the right hepatic hilum and a cystic lesion around the common hepatic duct. Under a diagnosis of perihilar cholangiocarcinoma, right hepatectomy with caudate lobectomy and bile duct resection were performed. Pathological examination revealed perihilar cholangiocarcinoma mainly involving the right hepatic duct. The cystic lesion was multilocular and covered by columnar lining epithelia exhibiting increased proliferative activity and p53 nuclear expression; it also contained foci of micropapillary and glandular proliferation. Therefore, the lesion was diagnosed as a cystic micropapillary neoplasm of peribiliary glands and resembled flat branch-type intraductal papillary mucinous neoplasm of the pancreas. Histological examination showed the lesion was discontinuous with the perihilar cholangiocarcinoma. Immunohistochemistry showed the cystic neoplasm was strongly positive for MUC6 and that the cholangiocarcinoma was strongly positive for MUC5AC and S100P. These results suggest these two lesions have different origins. This case warrants further study on whether this type of neoplasm is associated with concomitant cholangiocarcinoma as observed in pancreatic intraductal papillary mucinous neoplasm with concomitant pancreatic duct adenocarcinoma. PMID:26900302

  17. Cystic micropapillary neoplasm of peribiliary glands with concomitant perihilar cholangiocarcinoma

    PubMed Central

    Uchida, Tsuneyuki; Yamamoto, Yusuke; Ito, Takaaki; Okamura, Yukiyasu; Sugiura, Teiichi; Uesaka, Katsuhiko; Nakanuma, Yasuni

    2016-01-01

    We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diameter in the right hepatic hilum and a cystic lesion around the common hepatic duct. Under a diagnosis of perihilar cholangiocarcinoma, right hepatectomy with caudate lobectomy and bile duct resection were performed. Pathological examination revealed perihilar cholangiocarcinoma mainly involving the right hepatic duct. The cystic lesion was multilocular and covered by columnar lining epithelia exhibiting increased proliferative activity and p53 nuclear expression; it also contained foci of micropapillary and glandular proliferation. Therefore, the lesion was diagnosed as a cystic micropapillary neoplasm of peribiliary glands and resembled flat branch-type intraductal papillary mucinous neoplasm of the pancreas. Histological examination showed the lesion was discontinuous with the perihilar cholangiocarcinoma. Immunohistochemistry showed the cystic neoplasm was strongly positive for MUC6 and that the cholangiocarcinoma was strongly positive for MUC5AC and S100P. These results suggest these two lesions have different origins. This case warrants further study on whether this type of neoplasm is associated with concomitant cholangiocarcinoma as observed in pancreatic intraductal papillary mucinous neoplasm with concomitant pancreatic duct adenocarcinoma. PMID:26900302

  18. Pancreatic cystic neoplasms: Review of current knowledge, diagnostic challenges, and management options

    PubMed Central

    Jana, Tanima; Shroff, Jennifer; Bhutani, Manoop S.

    2015-01-01

    Pancreatic cystic lesions are being detected with increasing frequency, largely due to advances in cross-sectional imaging. The most common neoplasms include serous cystadenomas, mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, solid pseudopapillary neoplasms, and cystic pancreatic endocrine neoplasms. Computed tomography (CT), magnetic resonance imaging (MRI), and endoscopic ultrasound (EUS) are currently used as imaging modalities. EUS-guided fine needle aspiration has proved to be a useful diagnostic tool, and enables an assessment of tumor markers, cytology, chemistries, and DNA analysis. Here, we review the current literature on pancreatic cystic neoplasms, including classification, diagnosis, treatment, and recommendations for surveillance. Data for this manuscript was acquired via searching the literature from inception to December 2014 on PubMed and Ovid MEDLINE. PMID:25821410

  19. MRI findings of intraductal papillary mucinous neoplasms (IPMNs).

    PubMed

    Lana, Silvia; Vallara, Manuela; Bono, Nicola Emanuele; Russo, Giuseppe; Artioli, Giulia; Capretti, Giovanni; Paladini, Ilaria; Pesce, Antonella; Ruggirello, Margherita; Barbalace, Sandro; Mostardi, Maurizio

    2016-01-01

    Cystic lesions of the pancreas are relatively frequent imaging findings due to the improvement of imaging technologies. They may be secondary to both benign and malignant disease processes and their prevalence increases with age. In most cases, these lesions are detected incidentally by computed tomography and magnetic resonance imaging (MRI) performed for other reasons. Intraductal papillary mucinous neoplasms (IPMNs) represent 25% of the cystic neoplasms, morphologically classified into "main pancreatic duct IPMN" (MPD-IPMN), "side branches IPMN" (SB-IPMN) and mixed forms. Magnetic Resonance Cholangiopancreatography (MRCP) is a multiparametricity not invasive radiological technique that doesn't use ionizing radiation or organ iodinized contrast agents; it allows an accurate characterization of the lesions (number and size of cystic lesions, internal features of a cyst, ducts dilation, communication with main pancreatic duct) that is important to guide the differential diagnosis and establish a correct follow-up. International guidelines consider IPMN of MPD and mixed forms to be an indication for surgery, while clinical and radiological follow-up is indicated in asymptomatic patients with SB-IPMN, especially when lesions are < 2,5-3 cm in diameter and there are no mural nodules or dilation of MPD. PMID:27467864

  20. Subcellular localization of KL-6 mucin in intraductal papillary mucinous neoplasm of the pancreas.

    PubMed

    Inagaki, Yoshinori; Seyama, Yasuji; Hasegawa, Kiyoshi; Tang, Wei; Kokudo, Norihiro

    2014-08-01

    This study aimed to clarify the expression profile of KL-6 mucin in intraductal papillary mucinous neoplasm (IPMN) and its relation to tumor malignancy. Expression of KL-6 mucin in 38 IPMNs (intraductal papillary mucinous adenoma (IPMA), 24 cases; minimally invasive intraductal papillary mucinous carcinoma (MI-IPMC), 8 cases; invasive carcinoma originating from IPMC (IC-IPMC), 6 cases) and 66 pancreatic ductal adenocarcinomas (PDACs) was evaluated immunohistochemically. IC-IPMCs and MI-IPMCs had positive staining of KL-6 mucin whereas 58% of IPMAs tested negative. Subcellular localization of KL-6 mucin varied among IPMNs whereas all of the PDAC had positive expression in the circumferential membrane and cytoplasm of cancer cells. IC-IPMCs and MI-IPMCs had a higher frequency of circumferential membrane and cytoplasmic localization of KL-6 mucin than did IPMAs. These results suggest that localization of KL-6 mucin could be used to predict the malignancy of IPMN. PMID:25047009

  1. Intraductal Papillary Mucinous Neoplasm of the Pancreas: An Update

    PubMed Central

    Xiao, Shu-Yuan

    2012-01-01

    Intraductal papillary mucinous neoplasm (IPMN) is a cystic tumor of the pancreas. The etiology is unknown, but increasing evidence suggests the involvement of several tumorigenesis pathways, including an association with hereditary syndromes. IPMN occurs more commonly in men, with the mean age at diagnosis between 64 and 67 years old. At the time of diagnosis, it may be benign, with or without dysplasia, or frankly malignant with an invasive carcinoma. Tumors arising from the main pancreatic duct are termed main-duct IPMNs, those involving the branch ducts, branch-duct IPMNs. In general, small branch-duct IPMNs are benign, particularly in asymptomatic patients, and can be safely followed. In contrast, main-duct tumors should be surgically resected and examined carefully for an invasive component. In the absence of invasion, patient's survival is excellent, from 94 to 100%. For patients with an IPMN-associated invasive carcinoma, the prognosis overall is better than those with a de novo pancreatic ductal adenocarcinoma, with a 5-year survival of 40% to 60% in some series. However, no survival advantage can be demonstrated if the invasive component in an IPMN patient is that of the conventional tubular type (versus mucinous carcinoma). Several histomorphologic variants are recognized, although the clinical significance of this “subtyping” is not well defined. PMID:24278753

  2. Myxoma of the upper leg originating from an appendiceal mucinous neoplasm: A case report

    PubMed Central

    LI, JUN; ZHANG, XUE-YAN; WANG, BIN; CAO, QING-YONG

    2015-01-01

    Myxoma is rare in the upper leg. The current study presents the case of a large tumor in the right upper leg. The tumor extended to pelvic cavity and was found to be connected with a cystic and solid neoplasm that was adjacent to the ascending colon in the right lower quadrant. The large tumor of the upper leg had existed for 15 years and had ulcerated through the skin 4 days prior to admittance. Palliative surgery was performed, with histological findings of a myxoma. Since appendiceal mucinous neoplasms may invade organizations outside of the mucous layer of the appendix and cause secondary peritoneal myxoma, this myxoma of the upper leg probably originated from an appendiceal mucinous neoplasm. PMID:26622844

  3. Frequent GNAS mutations in low-grade appendiceal mucinous neoplasms

    PubMed Central

    Nishikawa, G; Sekine, S; Ogawa, R; Matsubara, A; Mori, T; Taniguchi, H; Kushima, R; Hiraoka, N; Tsuta, K; Tsuda, H; Kanai, Y

    2013-01-01

    Background: The molecular basis for the development of appendiceal mucinous tumours, which can be a cause of pseudomyxoma peritonei, remains largely unknown. Methods: Thirty-five appendiceal mucinous neoplasms were analysed for GNAS and KRAS mutations. A functional analysis of mutant GNAS was performed using a colorectal cancer cell line. Results: A mutational analysis identified activating GNAS mutations in 16 of 32 low-grade appendiceal mucinous neoplasms (LAMNs) but in none of three mucinous adenocarcinomas (MACs). KRAS mutations were found in 30 LAMNs and in all MACs. We additionally analysed a total of 186 extra-appendiceal mucinous tumours and found that GNAS mutations were highly prevalent in intraductal papillary mucinous tumours of the pancreas (88%) but were rare or absent in mucinous tumours of the colorectum, ovary, lung and breast (0–9%). The prevalence of KRAS mutations was quite variable among the tumours. The introduction of the mutant GNAS into a colorectal cancer cell line markedly induced MUC2 and MUC5AC expression, but did not promote cell growth either in vitro or in vivo. Conclusion: Activating GNAS mutations are a frequent and characteristic genetic abnormality of LAMN. Mutant GNAS might play a direct role in the prominent mucin production that is a hallmark of LAMN. PMID:23403822

  4. Cytopathologic Diagnosis of Oncocytic Type Intraductal Papillary Mucinous Neoplasm: Criteria and Clinical Implications of Accurate Diagnosis

    PubMed Central

    Reid, Michelle D.; Stallworth, Christina R.; Lewis, Melinda M.; Akkas, Gizem; Memis, Bahar; Basturk, Olca; Adsay, Volkan

    2016-01-01

    BACKGROUND Cytologic findings of pancreatic oncocytic-type intraductal papillary mucinous neoplasms (IPMNs)/intraductal oncocytic papillary neoplasms (IOPNs) are largely unknown. METHODS Five IOPNs encountered by the authors were analyzed. RESULTS Four IOPNs were located in the pancreatic head, and 1 was located in the pancreatic body/tail in 2 men and 3 women ages 56 to 84 years (mean age, 66 years). Radiologic diagnoses included pancreatic ductal adenocarcinoma (PDAC) in 2 patients, invasive cancer associated with IPMN in 1 patient, IPMN versus mucinous cystic neoplasm in 1 patient, and cystic mass in 1 patient. Cytologic findings included: hypercellular smears (4 of 5 cases) containing well formed clusters of oncocytic cells (5 of 5 cases) with prominent, slightly eccentric nucleoli (4 of 5 cases), predominantly arranged in sheets/papillary units (5 of 5 cases), with punched-out intercytoplasmic spaces (4 of 5 cases), and with occasional 3-dimensional groups and focal necrosis (3 of 5 cases). The intracytoplasmic mucin and thick extracellular mucin typical of other IPMNs were observed only in 2 cases and were very limited. The mean size on resection was 4.5 cm. Invasion was observed in 3 cases (0.1, 0.3, and 2.0 cm) of tubular-type IPMN. Initial cytologic evaluation was performed by the authors in 4 of 5 cases, which were diagnosed as IOPN (n = 3) and IPMN versus cystic PDAC (n = 1). One case was initially misdiagnosed as PDAC and, on resection, proved to be noninvasive IOPN. CONCLUSIONS Cytologic features of IOPNs are classical, similar to their histologic counterparts, and differ significantly from other IPMN subtypes. Because of their highly complex appearance, they are often radiologically misdiagnosed as PDAC; thus, failure to recognize their characteristic features on fine-needle aspiration may lead to inappropriate treatment. Patients with IOPN have an incomparably better prognosis than patients with ordinary PDAC, even when their neoplasms are invasive

  5. Prevalence, Diagnosis and Management of Pancreatic Cystic Neoplasms: Current Status and Future Directions

    PubMed Central

    Farrell, James J.

    2015-01-01

    Cystic neoplasms of the pancreas are found with increasing prevalence, especially in elderly asymptomatic individuals. Although the overall risk of malignancy is very low, the presence of these pancreatic cysts is associated with a large degree of anxiety and further medical investigation due to concerns about malignancy. This review discusses the different cystic neoplasms of the pancreas and reports diagnostic strategies based on clinical features and imaging data. Surgical and nonsurgical management of the most common cystic neoplasms, based on the recently revised Sendai guidelines, is also discussed, with special reference to intraductal papillary mucinous neoplasm (IPMN; particularly the branch duct variant), which is the lesion most frequently identified incidentally. IPMN pathology, its risk for development into pancreatic ductal adenocarcinoma, the pros and cons of current guidelines for management, and the potential role of endoscopic ultrasound in determining cancer risk are discussed. Finally, surgical treatment, strategies for surveillance of pancreatic cysts, and possible future directions are discussed. PMID:26343068

  6. Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases.

    PubMed

    Misdraji, Joseph; Yantiss, Rhonda K; Graeme-Cook, Fiona M; Balis, Ulysses J; Young, Robert H

    2003-08-01

    The classification of appendiceal mucinous tumors is controversial and terminology used for them inconsistent, particularly when they lack overtly malignant features but are associated with extra-appendiceal spread. We reviewed 107 appendiceal mucinous neoplasms and classified them as low-grade appendiceal mucinous neoplasm (LAMN) (n = 88), mucinous adenocarcinomas (MACAs) (n = 16), or discordant (n = 3) based on architectural and cytologic features. LAMNs were characterized by a villous or flat proliferation of mucinous epithelium with low-grade atypia. Thirty-nine tumors were confined to the appendix, but 49 had extra-appendiceal tumor spread, including 39 with peritoneal tumor characterized by mucin pools harboring low-grade mucinous epithelium, usually dissecting in a hyalinized stroma. Eight of the 16 MACAs lacked destructive invasion of the appendiceal wall and eight showed an infiltrative pattern of invasion. Extra-appendiceal tumor spread was present in 12 MACAs (four peritoneum, seven peritoneum and ovaries; one ovaries only). In MACAs with an infiltrative pattern, peritoneal tumor consisted of glands and single cells in a desmoplastic stroma. The peritoneal tumor in the remaining cases consisted of mucin pools that contained mucinous epithelium with high-grade atypia and, in some cases, increased cellularity compared with that seen in peritoneal spread in cases of LAMN. Three cases were classified as discordant because the appendiceal tumors were LAMNs but the peritoneal tumors were high-grade. Follow-up was available for 49 LAMNs, 15 MACAs, and 2 discordant cases. None of the patients with LAMNs confined to the appendix experienced recurrence (median follow-up 6 years). LAMNs with extra-appendiceal spread were associated with 3-, 5-, and 10-year survival rates of 100%, 86%, and 45%, respectively. Patients with MACA had 3- and 5-year survival rates of 90% and 44%, respectively (p = 0.04). The bulk of peritoneal disease correlated with prognosis among

  7. Diagnosis and Treatment of Mucinous Appendiceal Neoplasm Presented as Acute Appendicitis

    PubMed Central

    Kehagias, Ioannis; Markopoulos, Georgios; Papandreou, Thanasis; Kraniotis, Pantelis

    2016-01-01

    Appendiceal mucocele is a rare cause of acute abdomen. Mucinous appendiceal neoplasms represent 0.2–0.7% of all appendix specimens. The aim of this study is to report a case of a mucinous appendiceal neoplasm presented as acute appendicitis, discussing the clinical and surgical approach in the emergency setting. A 72-year-old female patient was admitted to the emergency department with a clinical examination indicative of acute abdomen. The patient underwent abdominal computed tomography scan which revealed a cystic lesion in the right iliac fossa measuring 8.3 × 5.2 × 4.1 cm, with calcified walls, and a mean density indicative of high protein content. The patient was taken to the operating room and a right hemicolectomy was performed. The postoperative course was unremarkable. The histopathological examination revealed a low-grade mucinous appendiceal neoplasm with negative regional lymph nodes. Ultrasound and CT are useful in diagnosing appendiceal mucocele and synchronous cancers in the emergency setting. The initial operation should include appendectomy and resection of the appendicular mesenteric fat along with any fluid collection for cytologic examination. During urgent appendectomy it is important to consider every mucocele as malignant in order to avoid iatrogenic perforation causing pseudomyxoma peritonei. Although laparotomy is recommended, the laparoscopic approach is not contraindicated. PMID:27066284

  8. Diagnosis and Treatment of Mucinous Appendiceal Neoplasm Presented as Acute Appendicitis.

    PubMed

    Kehagias, Ioannis; Zygomalas, Apollon; Markopoulos, Georgios; Papandreou, Thanasis; Kraniotis, Pantelis

    2016-01-01

    Appendiceal mucocele is a rare cause of acute abdomen. Mucinous appendiceal neoplasms represent 0.2-0.7% of all appendix specimens. The aim of this study is to report a case of a mucinous appendiceal neoplasm presented as acute appendicitis, discussing the clinical and surgical approach in the emergency setting. A 72-year-old female patient was admitted to the emergency department with a clinical examination indicative of acute abdomen. The patient underwent abdominal computed tomography scan which revealed a cystic lesion in the right iliac fossa measuring 8.3 × 5.2 × 4.1 cm, with calcified walls, and a mean density indicative of high protein content. The patient was taken to the operating room and a right hemicolectomy was performed. The postoperative course was unremarkable. The histopathological examination revealed a low-grade mucinous appendiceal neoplasm with negative regional lymph nodes. Ultrasound and CT are useful in diagnosing appendiceal mucocele and synchronous cancers in the emergency setting. The initial operation should include appendectomy and resection of the appendicular mesenteric fat along with any fluid collection for cytologic examination. During urgent appendectomy it is important to consider every mucocele as malignant in order to avoid iatrogenic perforation causing pseudomyxoma peritonei. Although laparotomy is recommended, the laparoscopic approach is not contraindicated. PMID:27066284

  9. Cystic renal neoplasms and renal neoplasms associated with cystic renal diseases in adults: cross-sectional imaging findings.

    PubMed

    Katabathina, Venkata S; Garg, Deepak; Prasad, Srinivasa R; Vikram, Raghu

    2012-01-01

    Cystic renal neoplasms in adults are a heterogeneous group of tumors with characteristic histogenesis, pathological findings, and variable biological profiles. They include disparate entities that are either biologically benign (lymphangioma, cystic nephroma, and mixed epithelial and stromal tumor) or malignant (cystic renal cell carcinoma, multilocular cystic renal cell carcinoma, and primary renal synovial sarcoma). Renal cystic diseases are characterized by cystic changes of the kidneys due to hereditary, developmental, or acquired etiology. Cystic renal diseases such as acquired cystic kidney disease, von Hippel-Lindau disease, and tuberous sclerosis are associated with the development of a wide spectrum of benign and malignant renal neoplasms. Most cystic renal tumors and cystic disease-associated renal neoplasms show characteristic cross-sectional imaging findings that permit accurate diagnosis. In addition, cross-sectional imaging is pivotal in the follow-up and surveillance of adult cystic tumors of the kidney. PMID:23192202

  10. Cystic mucinous adenocarcinoma of the lung: a case report

    PubMed Central

    2011-01-01

    Mucinous cystic tumors of the lung are uncommon, the preoperative pathologic diagnosis is difficult and their biological behavior is still controversial. We report the case of a patient with a clinically benign cystic lesion that post-operatively showed to be consistent with an invasive adenocarcinoma arising in a mucinous cystadenoma of the lung, We underline the difficulty of the clinical pre-operative diagnosis of this cystic neoplasia radiologically mimicking a hydatid cyst, and we report the negative TTF1 immunostaining potentially misleading in the differential diagnosis with metastatic mucinous carcinomas. Finallly, we evidence the presence of a pre-existing mucinous benign lesion suggesting early and complete resection of benign appearing lung cysts because they can undergo malignant transformation if left untreated or they can already harbor foci of invasive carcinoma at the time of the presentation. Even if a good prognosis, better than in other lung carcinomas, with no recurrrence or metastasis after complete surgical exicision, has been reported for cystic mucinous cystoadenocarcinomas, the follow-up showed an aggressive biological behaviour, with the early onset of metastasis, in keeping with P53 positive immunostaining and high Ki-67 proliferation index. PMID:21970610

  11. Molecular pathology of intraductal papillary mucinous neoplasms of the pancreas

    PubMed Central

    Paini, Marina; Crippa, Stefano; Partelli, Stefano; Scopelliti, Filippo; Tamburrino, Domenico; Baldoni, Andrea; Falconi, Massimo

    2014-01-01

    Since the first description of intraductal papillary mucinous neoplasms (IPMNs) of the pancreas in the eighties, their identification has dramatically increased in the last decades, hand to hand with the improvements in diagnostic imaging and sampling techniques for the study of pancreatic diseases. However, the heterogeneity of IPMNs and their malignant potential make difficult the management of these lesions. The objective of this review is to identify the molecular characteristics of IPMNs in order to recognize potential markers for the discrimination of more aggressive IPMNs requiring surgical resection from benign IPMNs that could be observed. We briefly summarize recent research findings on the genetics and epigenetics of intraductal papillary mucinous neoplasms, identifying some genes, molecular mechanisms and cellular signaling pathways correlated to the pathogenesis of IPMNs and their progression to malignancy. The knowledge of molecular biology of IPMNs has impressively developed over the last few years. A great amount of genes functioning as oncogenes or tumor suppressor genes have been identified, in pancreatic juice or in blood or in the samples from the pancreatic resections, but further researches are required to use these informations for clinical intent, in order to better define the natural history of these diseases and to improve their management. PMID:25110429

  12. Intracystic papillary neoplasm with an associated mucinous adenocarcinoma arising in Rokitansky-Aschoff sinus of the gallbladder.

    PubMed

    Sato, Ryuichiro; Ando, Toshinori; Tateno, Hiroo; Rikiyama, Toshiki; Furukawa, Toru; Ebina, Nobuo

    2016-12-01

    Intraepithelial neoplasias are preinvasive neoplastic lesions found throughout in the digestive system, and when such lesions are discovered in the gallbladder, they are referred to as intracystic papillary neoplasm (ICPN). In the gallbladder, mucinous adenocarcinoma is a rare histologic phenotype, and adenocarcinomas involving Rokitansky-Aschoff (RA) sinuses are uncommon, which were indeed found in a case reported here. A 64-year-old male presenting with upper abdominal pain demonstrated a spherical mass protruding outward from the gallbladder fundus in imaging studies. Laparoscopic cholecystectomy was performed, and the resected specimen revealed a subserosal cystic mass with a small communication with the gallbladder lumen. The cystic mass contained a gelatinous material without solid component. Histologically, the mass was consisted of subserosal cysts lined by atypical columnar mucinous epithelium with micropapillary growth and nuclear stratification. The neoplastic transformation was more pronounced toward the serosal side of the lesion where disruption of the cyst wall, intrastromal mucin lakes, and invasion of the neoplastic cells into surrounding stroma were observed. The epithelium was of intestinal lineage, which was supported by the positive immunoreactivity against CDX2 and MUC2. The cystic spaces were communicated with surrounding RA sinuses, which indicated that the tumor arose in the sinus. The pathological diagnosis was ICPN, intestinal type, with an associated mucinous adenocarcinoma arising in RA sinus. PMID:27316722

  13. CYSTIC FIBROSIS: AN INHERITED DISEASE AFFECTING MUCIN-PRODUCING ORGANS

    PubMed Central

    Ehre, Camille; Ridley, Caroline; Thornton, David J

    2014-01-01

    Our current understanding of cystic fibrosis (CF) has revealed that the biophysical properties of mucus play a considerable role in the pathogenesis of the disease in view of the fact that most mucus-producing organs are affected in CF patients. In this review, we discuss the potential causal relationship between altered cystic fibrosis transmembrane conductance regulator (CFTR) function and the production of mucus with abnormal biophysical properties in the intestine and lungs, highlighting what has been learned from cell cultures and animal models that mimic CF pathogenesis. A similar cascade of events, including mucus obstruction, infection and inflammation, is common to all epithelia affected by impaired surface hydration. Hence, the main structural components of mucus, namely the polymeric, gel-forming mucins, are critical to the onset of the disease. Defective CFTR leads to epithelial surface dehydration, altered pH/electrolyte composition and mucin concentration. Further, it can influence mucin transition from the intracellular to extracellular environment, potentially resulting in aberrant mucus gel formation. While defective HCO3− production has long been identified as a feature of CF, it has only recently been considered as a key player in the transition phase of mucins. We conclude by examining the influence of mucins on the biophysical properties of CF sputum and discuss existing and novel therapies aimed at removing mucus from the lungs. PMID:24685676

  14. Coexistence of mature cystic teratoma and adenocarcinoma in situ within atypical proliferative mucinous tumour of ovary--a case report of 35-year-old woman.

    PubMed

    Wincewicz, A; Lewitowicz, P; Adamczyk-Gruszka, O; Sulkowski, S; Kanczuga-Koda, L; Koda, M

    2015-01-01

    Combined ovarian tumors are found in common pathologic practice due to amazing potential of ovarian tissue to copy almost every tissue of human body and imitate many neoplasms of various other organs in a very flexible way. A multicystic tumor is presented in this case report of 35-year-old woman. It consisted of a cyst with sebum and hair and cavities with papillomatous projections and mucus. The ovarian tumor was diagnosed a mature cystic teratoma presenting mainly as dermoid cyst and mucinous adenocarcinoma in situ, arising within atypical proliferative mucinous tumor. This report demonstrates how histoformative properties are reflected in ovarian tumorigenesis. Such a stunning histoformativity makes ovaries the possible site of primary origin for malignant tumors that mimic extra ovarian differentiation. In the authors' point of view, the diagnosis of primary ovarian mucinous tumor within cystic teratoma is firm, whenever simultaneous extraovarian involvement by mucinous neoplasm is excluded. PMID:26050362

  15. Effects of calcium on intestinal mucin: implications for cystic fibrosis.

    PubMed

    Forstner, J F; Forstner, G G

    1976-06-01

    A major feature of the disease cystic fibrosis is the excessive concentration of mucus within ducts and glands of mucous-producing organs. Some mucous secretions also show an elevation in calcium concentration. Using purified rat intestinal goblet cell mucin as a model mucin, we have investigated the effect of millimolar additions (1-25 mM) of CaCl2 on the physical properties of the mucin. Isotonicity of incubation media was preserved in order to mimic in vivo conditions. CaCl2 (8-15mM) caused a 15-33% decrease in viscosity, no change in electrophoretic mobility in acrylamide gels, and a 20-30% decrease in solubility of the mucin. Solubility changes were reversed by the addition of EDTA (20 mM) to incubations. Insolubility was also produced in incubations of mucin with a mixture of soluble intestinal contents (NaCl washings). These findings strongly suggest that the mucin became smaller and more dense as calcium was added, a process most probably achieved by loss of intramolecular water. PMID:1272637

  16. Adjuvant Chemoradiotherapy After Pancreatic Resection for Invasive Carcinoma Associated With Intraductal Papillary Mucinous Neoplasm of the Pancreas

    SciTech Connect

    Swartz, Michael J.; Hsu, Charles C.; Pawlik, Timothy M.; Winter, Jordan; Hruban, Ralph H.; Guler, Mehmet; Schulick, Richard D.; Cameron, John L.; Laheru, Daniel A.; Wolfgang, Christopher L.; Herman, Joseph M.

    2010-03-01

    Purpose: Intraductal papillary mucinous neoplasms are mucin-producing cystic neoplasms of the pancreas. One-third are associated with invasive carcinoma. We examined the benefit of adjuvant chemoradiotherapy (CRT) for this cohort. Methods and Materials: Patients who had undergone pancreatic resection at Johns Hopkins Hospital between 1999 and 2004 were reviewed. Of these patients, 83 with a resected pancreatic mass were found to have an intraductal papillary mucinous neoplasm with invasive carcinoma, 70 of whom met inclusion criteria for the present analysis. Results: The median age at surgery was 68 years. The median tumor size was 3.3 cm, and invasive carcinoma was present at the margin in 16% of the patients. Of the 70 patients, 50% had metastases to the lymph nodes and 64% had Stage II disease. The median survival was 28.0 months, and 2- and 5-year survival rate was 57% and 45%, respectively. Of the 70 patients, 40 had undergone adjuvant CRT. Those receiving CRT were more likely to have lymph node metastases, perineural invasion, and Stage II-III disease. The 2-year survival rate after surgery with vs. without CRT was 55.8% vs. 59.3%, respectively (p = NS). Patients with lymph node metastases or positive surgical margins benefited significantly from CRT (p = .047 and p = .042, respectively). On multivariate analysis, adjuvant CRT was associated with improved survival, with a relative risk of 0.43 (95% confidence interval, 0.19-0.95; p = .044) after adjusting for major confounders. Conclusion: Adjuvant CRT conferred a 57% decrease in the relative risk of mortality after pancreaticoduodenectomy for intraductal papillary mucinous neoplasms with an associated invasive component after adjusting for major confounders. Patients with lymph node metastases or positive margins appeared to particularly benefit from CRT after definitive surgery.

  17. Appendiceal mucinous neoplasms: an uncertain nosological entity. Report of a case

    PubMed Central

    AGRUSA, A.; ROMANO, G.; GALIA, M.; CUCINELLA, G.; SORCE, V.; DI BUONO, G.; GULOTTA, L.; AGNELLO, F.; AMATO, G.; GULOTTA, G.

    2016-01-01

    Introduction Appendiceal mucocele is a relatively rare condition characterized by progressive dilation of the appendix caused by intra-luminal accumulation of mucoid substance. Its incidence is 0.07 – 0,63% of all appendectomies performed. Case report We report the case of a 70-year-old man who came to our observation with gravative pain in right lower abdominal region. A computed tomography abdominal scan revealed a cystic/tubular structure like an appendicular mass with wall enhancement but without calcifications suggestive of a mucocele. Into peritoneal cavity we found profuse mucinous material with a 1,5 cm size parietal nodule. We also identified a free perforation of the cecum with consensual spillage of gelatinous material mimicking a pseudomyxoma peritonei. We decided to perform a right hemicolectomy with excision of peritoneal lesion. Discussion The controversy in the pathologic terminology can give rise to a clinical dilemma in terms of the management and follow-up plans. For mucosal hyperplasia and cystadenoma simple appendectomy is curative. Only in case of large base of implantation it may be necessary the resection of the ileum and caecum or right hemicolectomy. In case of mucinous cystoadenocarcinoma authors perform a right hemicolectomy. Conclusion Appendiceal mucinous neoplasms are different pathological entities. The correct surgical management depends on size and location of lesion. A preoperative diagnosis is obviously needed in order to perform the correct treatment. CT abdominal scan is the better diagnostic tool, but different authors show their inability to reach a preoperative diagnosis in the larger majority of cases. PMID:27381696

  18. Refractory Jaundice From Intraductal Papillary Mucinous Neoplasm Treated With Cholangioscopy-Guided Radiofrequency Ablation.

    PubMed

    Brown, Nicholas G; Camilo, Joel; McCarter, Martin; Shah, Raj J

    2016-04-01

    Intraductal papillary mucinous neoplasms (IPMNs) are epithelial neoplasms treated with surgical resection when appropriate. We present a 79-year-old man with jandice refractory to endoscopic stenting. Biliary radiofrequency ablation (RFA) with cholangioscopy was used as palliation of obstructive jaundice due to a mucin-producing pancreatic IPMN with fistulous biliary communication. Clinical improvement permitted surgery, and he returned to pre-illness status at 17 months. The use of cholangioscopy in the setting of mucinous filling defects can guide over-the-wire RFA for palliation and may be a bridge to surgery. PMID:27144205

  19. Refractory Jaundice From Intraductal Papillary Mucinous Neoplasm Treated With Cholangioscopy-Guided Radiofrequency Ablation

    PubMed Central

    Brown, Nicholas G.; Camilo, Joel; McCarter, Martin

    2016-01-01

    Intraductal papillary mucinous neoplasms (IPMNs) are epithelial neoplasms treated with surgical resection when appropriate. We present a 79-year-old man with jandice refractory to endoscopic stenting. Biliary radiofrequency ablation (RFA) with cholangioscopy was used as palliation of obstructive jaundice due to a mucin-producing pancreatic IPMN with fistulous biliary communication. Clinical improvement permitted surgery, and he returned to pre-illness status at 17 months. The use of cholangioscopy in the setting of mucinous filling defects can guide over-the-wire RFA for palliation and may be a bridge to surgery. PMID:27144205

  20. CFTR, Mucins, and Mucus Obstruction in Cystic Fibrosis

    PubMed Central

    Kreda, Silvia M.; Davis, C. William; Rose, Mary Callaghan

    2012-01-01

    Mucus pathology in cystic fibrosis (CF) has been known for as long as the disease has been recognized and is sometimes called mucoviscidosis. The disease is marked by mucus hyperproduction and plugging in many organs, which are usually most fatal in the airways of CF patients, once the problem of meconium ileus at birth is resolved. After the CF gene, CFTR, was cloned and its protein product identified as a cAMP-regulated Cl− channel, causal mechanisms underlying the strong mucus phenotype of the disease became obscure. Here we focus on mucin genes and polymeric mucin glycoproteins, examining their regulation and potential relationships to a dysfunctional cystic fibrosis transmembrane conductance regulator (CFTR). Detailed examination of CFTR expression in organs and different cell types indicates that changes in CFTR expression do not always correlate with the severity of CF disease or mucus accumulation. Thus, the mucus hyperproduction that typifies CF does not appear to be a direct cause of a defective CFTR but, rather, to be a downstream consequence. In organs like the lung, up-regulation of mucin gene expression by inflammation results from chronic infection; however, in other instances and organs, the inflammation may have a non-infectious origin. The mucus plugging phenotype of the β-subunit of the epithelial Na+ channel (βENaC)-overexpressing mouse is proving to be an archetypal example of this kind of inflammation, with a dehydrated airway surface/concentrated mucus gel apparently providing the inflammatory stimulus. Data indicate that the luminal HCO3 − deficiency recently described for CF epithelia may also provide such a stimulus, perhaps by causing a mal-maturation of mucins as they are released onto luminal surfaces. In any event, the path between CFTR dysfunction and mucus hyperproduction has proven tortuous, and its unraveling continues to offer its own twists and turns, along with fascinating glimpses into biology. PMID:22951447

  1. Incidental Finding of a Rare Urachal Pathology: Urachal Mucinous Cystic Tumour of Low Malignant Potential

    PubMed Central

    Wang, Luke L.; Liddell, Heath; Tanny, Sharman Tan; Norris, Briony; Appu, Sree; Pan, David

    2016-01-01

    Urachal mucinous cystic tumours are rare pathological findings with only 23 previously reported cases in the literature. We present the case of a 54-year-old man with an incidentally found urachal mucinous cystic tumour laparoscopically excised. With its known potential to cause pseudomyxoma peritonei, complete surgical excision is important. Long-term cystoscopic and radiological surveillance is also required. PMID:26881171

  2. Villous Tumor of the Urinary Bladder Resembling Low-grade Mucinous Neoplasm of the Appendix

    PubMed Central

    Ito, Ayako; Sakura, Yuma; Sugimoto, Mikio; Kakehi, Yoshiyuki; Kuroda, Naoto

    2016-01-01

    Mucinous neoplasms of the urinary tract are very rare. We present a 63-year-old-women who had a sessile papillary villous tumor in urinary bladder. Although transurethral resection of the bladder tumor (TURBT) was performed, the villous tumor repetitively recurred and gradually spread to the entire surface of bladder lumen. Histopathologic and immunohistochemical examination showed that the lesion was very similar to low-grade mucinous neoplasm arising in appendix vermiformis. There are no reports on appendiceal metaplasia of urinary bladder mucosa. In this case, we describe this unprecedented neoplasm as “villous tumor of the urinary bladder resembling low-grade mucinous neoplasm of the appendix.” PMID:27169015

  3. [Mucinous ovarian neoplasms. Prognostically mostly excellent, infrequently a wolf in sheep's clothing].

    PubMed

    Lax, S; Staebler, A

    2014-07-01

    Mucinous ovarian neoplasms represent the second largest group of epithelial ovarian tumors after serous neoplasms, of which benign cystadenomas constitute more than 80 %. Mucinous cystadenomas and carcinomas cannot be distinguished by the clinical features or the mean age of onset of the disease. They typically occur unilaterally, are confined to the adnexae (FIGO stage I) and clinically present with non-specific abdominal symptoms or are diagnosed by chance. The mean age of disease onset is around 50 years old. The prognosis is excellent. Implants, peritoneal metastases and bilateral occurrence of ovarian mucinous neoplasms should lead to the suspicion of metastasis particularly from a gastrointestinal tumor. Neither microinvasion defined as a maximum extent of invasion of 5 mm, nor intraepithelial carcinoma characterized by high grade atypia without invasion, affect the prognosis of mucinous borderline tumors. Mucinous carcinomas typically show confluent glandular, expansile growth that leads to a labyrinth-like pattern. A destructive infiltrative or nodular growth pattern, however, should lead to the consideration of metastasis. Mural nodules that may reveal a spindle cell sarcomatous or anaplastic carcinomatous pattern occur infrequently in mucinous and do not affect the prognosis. Pax8 positivity is indicative of a primary ovarian neoplasm. In this case, however, mucinous tumors associated with teratomas may show the colonic immunoreaction pattern (CK7-/CK20+/CDX2+). The rare mucinous tumors with endocervical differentiation are now designated as seromucinous tumors and consist of two or more distinct cell types, are frequently associated with endometriosis and seem to show a molecular genetic relationship to endometrioid neoplasms. PMID:24962632

  4. Molecular Genetic Analysis of Ovarian Brenner Tumors and Associated Mucinous Epithelial Neoplasms: High Variant Concordance and Identification of Mutually Exclusive RAS Driver Mutations and MYC Amplification.

    PubMed

    Tafe, Laura J; Muller, Kristen E; Ananda, Guruprasad; Mitchell, Talia; Spotlow, Vanessa; Patterson, Sara E; Tsongalis, Gregory J; Mockus, Susan M

    2016-03-01

    Benign ovarian Brenner tumors often are associated with mucinous cystic neoplasms, which are hypothesized to share a histogenic origin and progression, however, supporting molecular characterization is limited. Our goal was to identify molecular mechanisms linking these tumors. DNA from six Brenner tumors with paired mucinous tumors, two Brenner tumors not associated with a mucinous neoplasm, and two atypical proliferative (borderline) Brenner tumors was extracted from formalin-fixed, paraffin-embedded tumor samples and sequenced using a 358-gene next-generation sequencing assay. Variant calls were compared within tumor groups to assess somatic mutation profiles. There was high concordance of the variants between paired samples (40% to 75%; P < 0.0001). Four of the six tumor pairs showed KRAS hotspot driver mutations specifically in the mucinous tumor. In the two paired samples that lacked KRAS mutations, MYC amplification was detected in both of the mucinous and the Brenner components; MYC amplification also was detected in a third Brenner tumor. Five of the Brenner tumors had no reportable potential driver alterations. The two atypical proliferative (borderline) Brenner tumors both had RAS mutations. The high degree of coordinate variants between paired Brenner and mucinous tumors supports a shared origin or progression. Differences observed in affected genes and pathways, particularly involving RAS and MYC, may point to molecular drivers of a divergent phenotype and progression of these tumors. PMID:26797085

  5. Endoscopic ultrasound guided radiofrequency ablation, for pancreatic cystic neoplasms and neuroendocrine tumors

    PubMed Central

    Pai, Madhava; Habib, Nagy; Senturk, Hakan; Lakhtakia, Sundeep; Reddy, Nageshwar; Cicinnati, Vito R; Kaba, Iyad; Beckebaum, Susanne; Drymousis, Panagiotis; Kahaleh, Michel; Brugge, William

    2015-01-01

    AIM: To outline the feasibility, safety, adverse events and early results of endoscopic ultrasound (EUS)-radiofrequency ablation (RFA) in pancreatic neoplasms using a novel probe. METHODS: This is a multi-center, pilot safety feasibility study. The intervention described was radiofrequency ablation (RF) which was applied with an innovative monopolar RF probe (1.2 mm Habib EUS-RFA catheter) placed through a 19 or 22 gauge fine needle aspiration (FNA) needle once FNA was performed in patients with a tumor in the head of the pancreas. The Habib™ EUS-RFA is a 1 Fr wire (0.33 mm, 0.013”) with a working length of 190 cm, which can be inserted through the biopsy channel of an echoendoscope. RF power is applied to the electrode at the end of the wire to coagulate tissue in the liver and pancreas. RESULTS: Eight patients [median age of 65 (range 27-82) years; 7 female and 1 male] were recruited in a prospective multicenter trial. Six had a pancreatic cystic neoplasm (four a mucinous cyst, one had intraductal papillary mucinous neoplasm and one a microcystic adenoma) and two had a neuroendocrine tumors (NET) in the head of pancreas. The mean size of the cystic neoplasm and NET were 36.5 mm (SD ± 17.9 mm) and 27.5 mm (SD ± 17.7 mm) respectively. The EUS-RFA was successfully completed in all cases. Among the 6 patients with a cystic neoplasm, post procedure imaging in 3-6 mo showed complete resolution of the cysts in 2 cases, whilst in three more there was a 48.4% reduction [mean pre RF 38.8 mm (SD ± 21.7 mm) vs mean post RF 20 mm (SD ± 17.1 mm)] in size. In regards to the NET patients, there was a change in vascularity and central necrosis after EUS-RFA. No major complications were observed within 48 h of the procedure. Two patients had mild abdominal pain that resolved within 3 d. CONCLUSION: EUS-RFA of pancreatic neoplasms with a novel monopolar RF probe was well tolerated in all cases. Our preliminary data suggest that the procedure is straightforward and safe. The

  6. Non-pancreatic retroperitoneal mucinous neoplasms and a discussion of the differential diagnosis

    PubMed Central

    Cobb, Camilla; Raza, Anwar Sultana

    2016-01-01

    Retroperitoneal mucinous neoplasms are common and have a wide range of differential diagnoses to consider. It is of utmost importance to maintain a multidisciplinary approach when evaluating these lesions. Clinical history, surgical impression and radiographic studies should be obtained and used in conjunction with morphology and immunohistochemistry to help guide the pathologist to the correct diagnosis. We recently encountered a case of a 51-year-old man who presented with an 11.5 cm left-sided retroperitoneal mucinous neoplasm. This lesion was initially diagnosed as a low-grade mucinous carcinoma of probable pancreatic origin at an outside facility, and he was subsequently treated with chemotherapy. One year later he presented to our institution and underwent retroperitoneal en bloc resection of the 7-cm residual multiloculated mucinous neoplasm. When discussed at tumor board additional medical history was obtained, and review of the patient’s chart revealed a remote history of left orchiectomy for a mixed malignant germ cell tumor and metastatic embryonal carcinoma in 2 of 34 retroperitoneal/para-aortic lymph nodes. With no clinical evidence of tumor in the pancreas or extension from it, the predominately para-aortic location of the tumor favors a mucinous carcinoma arising from a rest of mature metastatic/proliferating teratoma that persisted after chemotherapy. This case illustrates the importance of a thorough history, however remote, and correlation with imaging in the development of differential diagnoses, as well as, the need to consider non-pancreaticobiliary sources of retroperitoneal mucinous tumors. PMID:27034817

  7. Cyst infection of intraductal papillary mucinous neoplasms of the pancreas: management of a rare complication: report of 2 cases.

    PubMed

    Watanabe, Kenji; Karasaki, Hidenori; Mizukami, Yusuke; Kawamoto, Toru; Kono, Toru; Imai, Koji; Einama, Takahiro; Taniguchi, Masahiko; Kohgo, Yutaka; Furukawa, Hiroyuki

    2014-04-01

    The purpose of this study was to describe the cyst infection of intraductal papillary mucinous neoplasm in 2 patients. The patients were 62- and 74-year-old men. The initial symptom was acute febrile abdominal pain. Laboratory tests revealed severe infection (C-reactive protein concentrations were 23.3 µg/mL in patient 1 and 22.3 µg/mL in patient 2) and multilocular cystic masses (the diameters were 70 mm in patient 1 and 50 mm in patient 2) at the pancreatic head that involved peripancreatic vessels were demonstrated by computed tomography. Laboratory and radiographic findings were markedly improved by endoscopic transpapillary drainage. The enteric bacteria were detected in the drainage specimens. Curative resection was achieved, and histological findings indicated a carcinoma in situ in patient 1 and an invasive carcinoma in patient 2. Neither hyperamylasemia nor histological fat necrosis, frequently observed in acute pancreatitis, was evident. Both patients were free from recurrence after surgery (17 months in patient 1, and 18 months in patient 2). Cyst infection is an unknown complication of intraductal papillary mucinous neoplasm. Transpapillary drainage is highly recommended as an initial intervention. It is difficult to distinguish between cyst infection and unresectable invasive carcinoma with imaging modalities; however, surgical intervention after drainage may contribute to long-term survival. PMID:24622083

  8. Current perspectives on pancreatic serous cystic neoplasms: Diagnosis, management and beyond

    PubMed Central

    Zhang, Xiao-Peng; Yu, Zhong-Xun; Zhao, Yu-Pei; Dai, Meng-Hua

    2016-01-01

    Pancreatic cystic neoplasms have been increasingly recognized recently. Comprising about 16% of all resected pancreatic cystic neoplasms, serous cystic neoplasms are uncommon benign lesions that are usually asymptomatic and found incidentally. Despite overall low risk of malignancy, these pancreatic cysts still generate anxiety, leading to intensive medical investigations with considerable financial cost to health care systems. This review discusses the general background of serous cystic neoplasms, including epidemiology and clinical characteristics, and provides an updated overview of diagnostic approaches based on clinical features, relevant imaging studies and new findings that are being discovered pertaining to diagnostic evaluation. We also concisely discuss and propose management strategies for better quality of life. PMID:27022447

  9. Intraductal Papillary Mucinous Neoplasm of the Pancreas: Current State of the Art and Ongoing Controversies.

    PubMed

    Fong, Zhi Ven; Ferrone, Cristina R; Lillemoe, Keith D; Fernández-Del Castillo, Carlos

    2016-05-01

    With the widespread use and advances in radiographic imaging, Intraductal Papillary Mucinous Neoplasms (IPMNs) of the pancreas are identified with increasing frequency. Although many studies have addressed its biology and treatment, true understanding of its natural history continues to elude us. Its malignant potential places careproviders in a clinical dilemma of balancing the morbidity of pancreatectomy against the risk of malignant transformation while under continuous surveillance. Recently, there have been conflicting data published in the literature, generating more uncertainty in the field. In this article, we critically analyze the contrasting consensus guidelines from the International Association of Pancreatology and the American Gastroenterology Association, and address lingering questions and controversies. We also synthesize newly published data in the context of current standard of care, and provide a comprehensive review and recommendations for the clinical diagnosis, treatment, and follow-up strategy in the management of patients with Intraductal Papillary Mucinous Neoplasms. PMID:26727096

  10. An evaluation of the morphologic features of low-grade mucinous neoplasms of the appendix metastatic in the ovary, and comparison with primary ovarian mucinous tumors.

    PubMed

    Stewart, Colin J R; Ardakani, Nima M; Doherty, Dorota A; Young, Robert H

    2014-01-01

    It may be difficult to distinguish ovarian involvement by a low-grade appendiceal mucinous neoplasm (LAMN) from a primary gastrointestinal-type primary borderline (proliferative) ovarian tumor (IBMT) or an ovarian mucinous tumor arising within a teratoma, particularly when the latter is associated with mucinous ascites/pseudomyxoma peritonei. We noted that LAMNs involving the ovaries show 2 distinctive histologic features, "scalloped" glands and subepthelial stromal clefts, whereas IBMTs more often are associated with reactive cellular stroma and histiocyte aggregates (mucin granulomas). The frequency of these features was investigated in 18 LAMNs (16 with pseudomyxoma peritonei), 18 primary IBMTs, and 6 teratoma-associated mucinous tumors (selected on the basis of associated pseudomyxoma peritonei). Scalloped glands and subepithelial clefts were identified in 17 and 16 LAMNs, respectively, and in 3 and 7 IBMTs, respectively. Conversely, reactive stroma and histiocyte aggregates were present in 2 and 0 LAMNs, respectively, and in 11 and 10 IBMTs, respectively. LAMNs were often bilateral (12/18 cases) and they more frequently showed mucin dissection of the ovarian stroma and tall mucin-rich (hypermucinous) epithelial cells compared with IBMTs. Our findings suggest that scalloped glands, subepithelial clefts, cellular stroma, and histiocyte aggregates may be useful additional morphologic parameters to help distinguish these tumor types. However, teratoma-associated mucinous neoplasms can show identical histologic features to those of LAMNs involving the ovary, and therefore accurate diagnosis of such cases requires careful macroscopic and microscopic examinations of the ovaries together with complete histologic assessment of the appendix. PMID:24300528

  11. Recommendations in the management of epithelial appendiceal neoplasms and peritoneal dissemination from mucinous tumours (pseudomyxoma peritonei).

    PubMed

    Barrios, P; Losa, F; Gonzalez-Moreno, S; Rojo, A; Gómez-Portilla, A; Bretcha-Boix, P; Ramos, I; Torres-Melero, J; Salazar, R; Benavides, M; Massuti, T; Aranda, E

    2016-05-01

    The epithelial appendiceal neoplasms are uncommon and are usually detected as an unexpected surgical finding. The general surgeon should be aware of the diversity of its clinical manifestations and biological behaviors along with the significance of the surgical treatment on the progression of the illness and the prognosis of the patients. The operative findings and, especially, tumor histology, determine the type of surgery. Intestinal histologic subtype behaves and should be treated similarly to the right colon neoplasms; while mucinous tumors, often discordant between histology and its aggressiveness, can be treated with a simple appendectomy or require complex oncological surgeries. Mucinous tumors are often associated with the presence of mucin or tumor implants in the abdominal cavity, being the clinical syndrome known as pseudomyxoma peritonei (PMP). PMP tends to present an indolent but deadly evolution and requires a multimodal approach as a single treatment with curative potential: complete cytoreductive surgery plus hyperthermic Intra-peritoneal chemotherapy (CCRS + HIPEC) now considered the standard of care in this pathology. The general surgeon should be aware of the governing principles of the treatment of appendiceal neoplasms with or without peritoneal dissemination, know the therapeutic frontiers in every situation (avoiding unnecessary or counterproductive surgeries) and sending early these patients to specialised centres in the radical management of malignant diseases of the peritoneum in the conditions and with the necessary information to facilitate a possible radical treatment. PMID:26489426

  12. Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure

    PubMed Central

    Henderson, Ashley G.; Ehre, Camille; Button, Brian; Abdullah, Lubna H.; Cai, Li-Heng; Leigh, Margaret W.; DeMaria, Genevieve C.; Matsui, Hiro; Donaldson, Scott H.; Davis, C. William; Sheehan, John K.; Boucher, Richard C.; Kesimer, Mehmet

    2014-01-01

    The pathogenesis of mucoinfective lung disease in cystic fibrosis (CF) patients likely involves poor mucus clearance. A recent model of mucus clearance predicts that mucus flow depends on the relative mucin concentration of the mucus layer compared with that of the periciliary layer; however, mucin concentrations have been difficult to measure in CF secretions. Here, we have shown that the concentration of mucin in CF sputum is low when measured by immunologically based techniques, and mass spectrometric analyses of CF mucins revealed mucin cleavage at antibody recognition sites. Using physical size exclusion chromatography/differential refractometry (SEC/dRI) techniques, we determined that mucin concentrations in CF secretions were higher than those in normal secretions. Measurements of partial osmotic pressures revealed that the partial osmotic pressure of CF sputum and the retained mucus in excised CF lungs were substantially greater than the partial osmotic pressure of normal secretions. Our data reveal that mucin concentration cannot be accurately measured immunologically in proteolytically active CF secretions; mucins are hyperconcentrated in CF secretions; and CF secretion osmotic pressures predict mucus layer–dependent osmotic compression of the periciliary liquid layer in CF lungs. Consequently, mucin hypersecretion likely produces mucus stasis, which contributes to key infectious and inflammatory components of CF lung disease. PMID:24892808

  13. Managing incidental pancreatic cystic neoplasms with integrated molecular pathology is a cost-effective strategy

    PubMed Central

    Das, Ananya; Brugge, William; Mishra, Girish; Smith, Dennis M.; Sachdev, Mankanwal; Ellsworth, Eric

    2015-01-01

    Background and study aims: Current guidelines recommend using endoscopic ultrasound (EUS), carcinoembryonic antigen (CEA) testing and cytology to manage incidental pancreatic cystic neoplasms (PCN); however, studies suggest a strategy including integrated molecular pathology (IMP) of cyst fluid may further aid in predicting risk of malignancy. Here, we evaluate several strategies for diagnosing and managing asymptomatic PCN using healthcare economic modeling. Patients and methods: A third-party-payer perspective Markov decision model examined four management strategies in a hypothetical cohort of 1000 asymptomatic patients incidentally found to have a 3 cm solitary pancreatic cystic lesion. Strategy I used cross-sectional imaging, recommended surgery only if symptoms or risk factors emerged. Strategy II considered patients for resection without initial EUS. Strategy III (EUS + CEA + Cytology) referred only those with mucinous cysts (CEA > 192 ng/mL) for resection. Strategy IV implemented IMP; a commercially available panel provided a “Benign,” “Mucinous,” or “Aggressive” classification based on the level of mutational change in cyst fluid. “Benign” and “Mucinous” patients were followed with surveillance; “Aggressive” patients were referred for resection. Quality-adjusted life-years (QALY), relative risk with 95 %CI, Number Needed to Treat (NNT), and incremental cost-effectiveness ratios were calculated. Results: Strategy IV provided the greatest increase in QALY at nearly identical cost to the cheapest approach, Strategy I. Relative risk of malignancy compared to the current standard of care and nearest competing strategy, Strategy III, was 0.18 (95 %CI 0.06 – 0.53) with an NNT of 56 (95 %CI 34 – 120). Conclusions: Use of IMP was the most cost-effective strategy, supporting its routine clinical use. PMID:26528505

  14. Intraductal papillary mucinous neoplasm of the bile duct with gastric and duodenal fistulas

    PubMed Central

    Hong, Man Yong; Yu, Dong Wook; Hong, Seung Goun

    2014-01-01

    Intraductal papillary mucinous neoplasm (IPMN) of the bile duct is still rare and not yet understood despite of its increased incidence and similar clinicopathologic characteristics compared with IPMN of the pancreas. The fistula formation into other organs can occur in IPMN, especially the pancreatic type. To our knowledge, only two cases of IPMN of the bile duct with a choledochoduodenal fistula were reported and we have recently experienced a case of IPMN of the bile duct penetrating into two neighboring organs of the stomach and duodenum presenting with abdominal pain and jaundice. Endoscopy showed thick mucin extruding from two openings of the fistulas. Endoscopic suction of thick mucin using direct peroral cholangioscopy with ultra-slim endoscope through choledochoduodenal fistula was very difficult and ineffective because of very thick mucin and next endoscopic suction through the stent after prior insertion of biliary metal stent into choledochogastric fistula also failed. Pathologic specimen obtained from the proximal portion of the choledochogastric fistula near left intrahepatic bile duct through the metal stent showed a low grade adenoma. The patient declined the surgical treatment due to her old age and her abdominal pain with jaundice was improved after percutaneous transhepatic biliary drainage with the irrigation of N-acetylcysteine three times daily for 10 d. PMID:25031793

  15. Distinct patterns of peritumoral histological findings in subtypes of intraductal papillary mucinous neoplasms of the pancreas.

    PubMed

    Saito, Michihiro; Imada, Hiroki; Suzuki, Tsukasa; Sata, Naohiro; Yasuda, Yoshikazu; Maetani, Iruru; Fukushima, Noriyoshi

    2015-10-01

    Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are primary pancreatic neoplasms that can act as precursors to invasive adenocarcinoma of the pancreas. The peritumoral stroma has been increasingly recognized to play an important role in many types of tumors. Therefore, to investigate the clinicopathological significance of the peritumoral stroma in IPMNs, we examined the histological features of the peritumoral stroma in each subtype and histological grade of IPMNs. Eighty-two patients with IPMN, who underwent surgical resection, were reviewed clinicopathologically. Intraductal papillary mucinous neoplasms (86 lesions in total) were histologically subclassified into gastric (n = 51), intestinal (n = 22), pancreatobiliary (n = 11), and oncocytic (n = 2) subtypes. Peritumoral histological features between the gastric and intestinal subtypes were evaluated by each histological grade. The results showed that subepithelial edema and inflammatory cell infiltration were more commonly observed in the gastric subtype (74% and 79%, respectively) than in the intestinal subtype (12% and 25%, respectively) of low-grade IPMNs. On the other hand, mucus lakes were more commonly observed in the intestinal subtype (100%) than in the gastric subtype (0%) of high-grade IPMNs. In addition, pancreatobiliary subtype IPMNs tended to exhibit acute inflammation such as neutrophil predominance. This study showed that peritumoral histological features differed among subtypes of IPMNs from low-grade tumors. These differences suggest the possibility that each subtype of IPMNs has a distinct mechanism from an early stage of tumor progression, which is reflected in the properties of the peritumoral stroma. PMID:26265194

  16. Pancreatic lipoma with a solid nodule mimicking invasion from adjoining intraductal papillary mucinous neoplasm.

    PubMed

    Namiki, Yoko; Maeda, Eriko; Gonoi, Wataru; Akamatsu, Nobuhisa; Ikemura, Masako; Ohtomo, Kuni

    2016-06-01

    A 74-year-old man was referred to our hospital for a mass in the pancreatic head found during screening chest computed tomography. Contrast computed tomography showed a 5-cm multicystic mass with an irregular border containing a solid component showing contrast enhancement. Caudal to this mass, a 5-cm solid mass of fat density with a nodular soft-tissue component was found. Cytology of the aspirated pancreatic fluid revealed malignant cells, and surgery was performed for suspected intraductal papillary mucinous carcinoma. Pathologic analysis of the resected specimen revealed a collision tumor of intraductal papillary mucinous neoplasm (IPMN) with high-grade dysplasia and pancreatic lipoma. The soft-tissue component within the lipoma was a nodule consisting of pancreatic tissue with inflammatory infiltration and hyalinization and was not associated with IPMN invasion. PMID:27257448

  17. K-Ras and cyclooxygenase-2 coactivation augments intraductal papillary mucinous neoplasm and Notch1 mimicking human pancreas lesions

    PubMed Central

    Chiblak, Sara; Steinbauer, Brigitte; Pohl-Arnold, Andrea; Kucher, Dagmar; Abdollahi, Amir; Schwager, Christian; Höft, Birgit; Esposito, Irene; Müller-Decker, Karin

    2016-01-01

    Mutational activation of K-Ras is an initiating event of pancreatic ductal adenocarcinomas (PDAC) that may develop either from pancreatic intraepithelial neoplasia (PanIN) or intraductal papillary mucinous neoplasms (IPMN). Cyclooxygenase-2 (COX-2)-derived prostaglandin E2 (PGE2) is causally related to pancreatic carcinogenesis. Here, we deciphered the impact of COX-2, a key modulator of inflammation, in concert with active mutant K-RasG12D on tumor burden and gene expression signature using compound mutant mouse lines. Concomitant activation of COX-2 and K-RasG12D accelerated the progression of pancreatic intraepithelial lesions predominantly with a cystic papillary phenotype resembling human IPMN. Transcriptomes derived from laser capture microdissected preneoplastic lesions of single and compound mutants revealed a signature that was significantly enriched in Notch1 signaling components. In vitro, Notch1 signaling was COX-2-dependent. In line with these findings, human IPMN stratified into intestinal, gastric and pancreatobillary types displayed Notch1 immunosignals with high prevalence, especially in the gastric lesions. In conclusion, a yet unknown link between activated Ras, protumorigenic COX-2 and Notch1 in IPMN onset was unraveled. PMID:27381829

  18. K-Ras and cyclooxygenase-2 coactivation augments intraductal papillary mucinous neoplasm and Notch1 mimicking human pancreas lesions.

    PubMed

    Chiblak, Sara; Steinbauer, Brigitte; Pohl-Arnold, Andrea; Kucher, Dagmar; Abdollahi, Amir; Schwager, Christian; Höft, Birgit; Esposito, Irene; Müller-Decker, Karin

    2016-01-01

    Mutational activation of K-Ras is an initiating event of pancreatic ductal adenocarcinomas (PDAC) that may develop either from pancreatic intraepithelial neoplasia (PanIN) or intraductal papillary mucinous neoplasms (IPMN). Cyclooxygenase-2 (COX-2)-derived prostaglandin E2 (PGE2) is causally related to pancreatic carcinogenesis. Here, we deciphered the impact of COX-2, a key modulator of inflammation, in concert with active mutant K-Ras(G12D) on tumor burden and gene expression signature using compound mutant mouse lines. Concomitant activation of COX-2 and K-Ras(G12D) accelerated the progression of pancreatic intraepithelial lesions predominantly with a cystic papillary phenotype resembling human IPMN. Transcriptomes derived from laser capture microdissected preneoplastic lesions of single and compound mutants revealed a signature that was significantly enriched in Notch1 signaling components. In vitro, Notch1 signaling was COX-2-dependent. In line with these findings, human IPMN stratified into intestinal, gastric and pancreatobillary types displayed Notch1 immunosignals with high prevalence, especially in the gastric lesions. In conclusion, a yet unknown link between activated Ras, protumorigenic COX-2 and Notch1 in IPMN onset was unraveled. PMID:27381829

  19. Characterization of intraductal papillary neoplasm of bile duct with respect to histopathologic similarities to pancreatic intraductal papillary mucinous neoplasm.

    PubMed

    Nakanuma, Yasuni; Kakuda, Yuko; Uesaka, Katsuhiko; Miyata, Takashi; Yamamoto, Yusuke; Fukumura, Yuki; Sato, Yasunori; Sasaki, Motoko; Harada, Kenichi; Takase, Masaru

    2016-05-01

    Intraductal papillary neoplasm of bile duct (IPNB) is a papillary tumor covered by well-differentiated neoplastic epithelium with fine fibrovascular cores in the dilated bile ducts. It reportedly shows similarities to intraductal papillary mucinous neoplasm of pancreas (IPMN), to various degrees. Herein, IPNB was pathologically analyzed by classifying 52 cases into 4 groups based on the histopathologic similarities to IPMN: group A (identical to IPMN, 19 cases), group B (similar to but slightly different from IPMN, 18 cases), group C (vaguely similar to IPMN, 5 cases), and group D (different from IPMN, 10 cases). In group A, intrahepatic and perihilar regions were mainly affected, most cases were of low/intermediate or high grade without invasion, and gastric type was the most common phenotype, followed by oncocytic and intestinal types. In groups C and D, perihilar and distal bile ducts were affected, almost all cases were of high grade with invasion, and most of them were of intestinal and pancreatobiliary phenotypes. Most group B cases were of intestinal phenotype, and all were of high grade with or without invasion. In conclusion, these 4 groups of IPNB showed unique pathologic features and behaviors. Group A cases were less aggressive and shared many features with IPMN, whereas group C and D cases were more aggressive and mainly found in perihilar and distal bile ducts. Group B resembling IPMN was intermediate between them. This classification may be useful in clinical practice and holds promise for a novel approach to analyze IPNB tumorigenesis. PMID:27067788

  20. Evidence and Role for Bacterial Mucin Degradation in Cystic Fibrosis Airway Disease

    PubMed Central

    Flynn, Jeffrey M.; Niccum, David; Dunitz, Jordan M.

    2016-01-01

    Chronic lung infections in cystic fibrosis (CF) patients are composed of complex microbial communities that incite persistent inflammation and airway damage. Despite the high density of bacteria that colonize the lower airways, nutrient sources that sustain bacterial growth in vivo, and how those nutrients are derived, are not well characterized. In this study, we examined the possibility that mucins serve as an important carbon reservoir for the CF lung microbiota. While Pseudomonas aeruginosa was unable to efficiently utilize mucins in isolation, we found that anaerobic, mucin-fermenting bacteria could stimulate the robust growth of CF pathogens when provided intact mucins as a sole carbon source. 16S rRNA sequencing and enrichment culturing of sputum also identified that mucin-degrading anaerobes are ubiquitous in the airways of CF patients. The collective fermentative metabolism of these mucin-degrading communities in vitro generated amino acids and short chain fatty acids (propionate and acetate) during growth on mucin, and the same metabolites were also found in abundance within expectorated sputum. The significance of these findings was supported by in vivo P. aeruginosa gene expression, which revealed a heightened expression of genes required for the catabolism of propionate. Given that propionate is exclusively derived from bacterial fermentation, these data provide evidence for an important role of mucin fermenting bacteria in the carbon flux of the lower airways. More specifically, microorganisms typically defined as commensals may contribute to airway disease by degrading mucins, in turn providing nutrients for pathogens otherwise unable to efficiently obtain carbon in the lung. PMID:27548479

  1. Evidence and Role for Bacterial Mucin Degradation in Cystic Fibrosis Airway Disease.

    PubMed

    Flynn, Jeffrey M; Niccum, David; Dunitz, Jordan M; Hunter, Ryan C

    2016-08-01

    Chronic lung infections in cystic fibrosis (CF) patients are composed of complex microbial communities that incite persistent inflammation and airway damage. Despite the high density of bacteria that colonize the lower airways, nutrient sources that sustain bacterial growth in vivo, and how those nutrients are derived, are not well characterized. In this study, we examined the possibility that mucins serve as an important carbon reservoir for the CF lung microbiota. While Pseudomonas aeruginosa was unable to efficiently utilize mucins in isolation, we found that anaerobic, mucin-fermenting bacteria could stimulate the robust growth of CF pathogens when provided intact mucins as a sole carbon source. 16S rRNA sequencing and enrichment culturing of sputum also identified that mucin-degrading anaerobes are ubiquitous in the airways of CF patients. The collective fermentative metabolism of these mucin-degrading communities in vitro generated amino acids and short chain fatty acids (propionate and acetate) during growth on mucin, and the same metabolites were also found in abundance within expectorated sputum. The significance of these findings was supported by in vivo P. aeruginosa gene expression, which revealed a heightened expression of genes required for the catabolism of propionate. Given that propionate is exclusively derived from bacterial fermentation, these data provide evidence for an important role of mucin fermenting bacteria in the carbon flux of the lower airways. More specifically, microorganisms typically defined as commensals may contribute to airway disease by degrading mucins, in turn providing nutrients for pathogens otherwise unable to efficiently obtain carbon in the lung. PMID:27548479

  2. Gd-EOB-DTPA-enhanced magnetic resonance imaging for bile duct intraductal papillary mucinous neoplasms

    PubMed Central

    Ying, Shi-Hong; Teng, Xiao-Dong; Wang, Zhao-Ming; Wang, Qi-Dong; Zhao, Yi-Lei; Chen, Feng; Xiao, Wen-Bo

    2015-01-01

    AIM: To investigate gadolinium-ethoxybenzyl-diethylenetriamine-pentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) of intraductal papillary mucinous neoplasms of the bile duct (IPMN-B). METHODS: The imaging findings of five cases of IPMN-B which were pathologically confirmed at our hospital between March 2012 and May 2013 were retrospectively analyzed. Three of these cases were diagnosed by duodenal endoscopy and biopsy pathology, and two cases were diagnosed by surgical pathology. All five patients underwent enhanced and non-enhanced computed tomography (CT), magnetic resonance cholangiopancreatography, and Gd-EOB-DTPA-enhanced MRI; one case underwent both Gd-EOB-DTPA-enhanced MRI and positron emission tomography-CT. The clinical data and imaging results for these cases were compared and are presented. RESULTS: Conventional imaging showed diffuse dilatation of bile ducts and multiple intraductal polypoid and papillary neoplasms or serrated changes along the bile ducts. In two cases, Gd-EOB-DTPA-enhanced MRI revealed dilated biliary ducts and intraductal tumors, as well as filling defects caused by mucin in the dilated bile ducts in the hepatobiliary phase. Gd-EOB-DTPA-enhanced MRI in one case clearly showed a low-signal tumor in the hepatobiliary phase, similar to what was seen by positron emission tomography-CT. In two patients, routine inspection was unable to discern whether the lesions were inflammation or tumors. However, Gd-EOB-DTPA-enhanced MRI revealed a pattern of gradual enhancement during the hepatobiliary phase, and the signal intensity of the lesions was lower than the surrounding liver parenchyma, suggesting tissue inflammation in both cases, which were confirmed by surgical pathology. CONCLUSION: Gd-EOB-DTPA-enhanced MRI reveals the intraductal mucin component of IPMN-B in some cases and the extent of tumor infiltration beyond the bile ducts in invasive cases. PMID:26167082

  3. Ovarian mucinous tumors arising from mature cystic teratomas--a molecular genetic approach for understanding the cellular origin.

    PubMed

    Fujii, Kaho; Yamashita, Yoriko; Yamamoto, Toshimichi; Takahashi, Koji; Hashimoto, Katsunori; Miyata, Tomoko; Kawai, Kumi; Kikkawa, Fumitaka; Toyokuni, Shinya; Nagasaka, Tetsuro

    2014-04-01

    Mucinous tumors of the ovary are frequently associated with mature cystic teratomas, and it has been speculated that the mucinous tumors arise from teratoma components. The cellular origins of mature cystic teratomas are believed to be post-meiotic ovarian germ cells, and the analysis of microsatellite markers such as short tandem repeats is suitable for determining the cellular origin of tumors. In this study, we analyzed 3 ovarian mature cystic teratomas, all of which were associated with simultaneous ovarian mucinous tumors within the same ovary. Two of the 3 mucinous tumors were intestinal-type and the other was endocervical type. A laser capture microdissection technique was used to separate the epithelial component of the mucinous tumor, the components of the mature cystic teratoma, and control ovarian somatic tissue. Using short tandem repeat analysis based on 6 markers (D20S480, D6S2439, D6S1056, D9S1118, D4S2639, and D17S1290), we could distinguish the germ cell (homozygous) or somatic (heterozygous) origin of a given component in each sample. The epithelial components of the intestinal-type mucinous tumors in cases 1 and 2 were homozygous, and the epithelial component in case 3 (endocervical type) was heterozygous. All teratomatous components were homozygous, and the control components were heterozygous. In addition, we analyzed 3 mature cystic teratomas without mucinous tumors, and all 3 were homozygous in the tumor component. Our data suggest that the origin of mucinous tumors in the ovary may differ among histological subtypes, and intestinal-type mucinous tumors may arise from mature cystic teratomas, although endocervical-type mucinous tumors may not. PMID:24485845

  4. Detection of Hot-Spot Mutations in Circulating Cell-Free DNA From Patients With Intraductal Papillary Mucinous Neoplasms of the Pancreas.

    PubMed

    Berger, Andreas W; Schwerdel, Daniel; Costa, Ivan G; Hackert, Thilo; Strobel, Oliver; Lam, Sandra; Barth, Thomas F; Schröppel, Bernd; Meining, Alexander; Büchler, Markus W; Zenke, Martin; Hermann, Patrick C; Seufferlein, Thomas; Kleger, Alexander

    2016-08-01

    Intraductal papillary mucinous neoplasms (IPMNs) are the most frequent cystic pancreatic tumors. Little is known about their molecular alterations, but mutations in GNAS have been reported to promote IPMN formation. A tumor-derived fraction of circulating cell-free DNA (cfDNA), isolated from blood samples, contains many of the same mutations as the primary tumor, and could be a tool for noninvasive disease monitoring. We found that the total amount of cfDNA can discriminate between individuals without pancreatic lesions (controls) and patients with Fukuoka-negative branch-duct IPMN or pancreatic cancer. Furthermore, we detected GNAS mutations in cfDNA from patients with IPMN, but not in patients with serous cystadenoma or controls. Analyses of cfDNA might therefore be used in the diagnosis of patients with IPMN or in monitoring disease progression. PMID:27343369

  5. Heterogeneity of mucus glycoproteins from cystic fibrotic sputum. Are there different families of mucins?

    PubMed Central

    Thornton, D J; Sheehan, J K; Carlstedt, I

    1991-01-01

    High-Mr mucin glycopeptides prepared from sputum of an individual with cystic fibrosis (CF) were studied by ion-exchange h.p.l.c. The glycopeptides were heterogeneous and a number of partially resolved populations were identified. Whole mucins from the gel phase were separated into four fractions by isopycnic density-gradient centrifugation in CsCl, and high-Mr glycopeptides from these fractions were examined by ion-exchange h.p.l.c. The acidic nature of the high-Mr glycopeptides increased with increasing buoyant density of the intact mucins, and a periodate-Schiff (PAS)-rich and an extremely high-iron diamine (HID)-reactive component were present in the lowest and highest density fractions respectively. The various glycopeptide populations were identified in different proportions in mucins from four other individuals with CF. CF sputum thus seems to contain distinct mucin populations containing different oligosaccharide clusters corresponding to these high-Mr glycopeptides. PMID:2064606

  6. Current roles of endoscopy in the management of intraductal papillary mucinous neoplasm of the pancreas.

    PubMed

    Tanaka, Masao

    2015-05-01

    Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is characterized by intraductal papillary proliferation of mucin-producing epithelial cells that exhibit various degrees of dysplasia. IPMN is classified into four histological subtypes (gastric, intestinal, pancreatobiliary, and oncocytic) according to its histomorphological and immunohistochemical characteristics. Endoscopic retrograde cholangiopancreatography plays a crucial role in the evaluation of these features of IPMN. Endoscopic ultrasonography (EUS) has proven to be more sensitive than computed tomography or magnetic resonance imaging for early detection of malignancy. The present review addresses the current roles of endoscopy and related techniques in the management of IPMN. The particular focus is on diagnosing IPMN and malignancy within IPMN, detecting pancreatic cancer concomitant with IPMN, differentiating the epithelial subtypes of IPMN, determining the optimal strategy for the management of branch duct IPMN, and discussing innovative endoscopic technology related to IPMN. The disadvantages of endoscopic examinations of IPMN and different attitudes toward EUS-guided fine-needle aspiration for IPMN between Japan (negative) and other countries (active) are also discussed. PMID:25588761

  7. Validation of international consensus guideline 2012 for intraductal papillary mucinous neoplasm of pancreas

    PubMed Central

    Han, Dong Hyeon; Lee, Huisong; Park, Jin Young; Kwon, Wooil; Heo, Jin Seok; Choi, Seong Ho

    2016-01-01

    Purpose Intraductal papillary mucinous neoplasm (IPMN) has variable malignant potential ranging from premalignant intraductal lesions to malignant neoplasms with invasive carcinoma. To help physicians managing patients with IPMN, International consensus guidelines was made in 2006 and revised in 2012. This study was designed to evaluate the clinical usefulness of guidelines and to validate. Methods From October 1996 to December 2011, we retrospectively reviewed the data of 230 patients who underwent pancreatic resection for IPMN. Univariate and multivariable analyses were used to identify significant predictors of malignancy in IPMN. Results Of the 230 patients, 62 patients (27%) were diagnosed with invasive carcinoma. Jaundice (P < 0.001; 95% confidence interval [CI], 3.086–40.010) main pancreatic duct diameter equal to or greater than 10 mm (P < 0.001; 95% CI, 1.723–6.673) and also abdominal pain (P < 0.001; 95% CI, 4.363–22.600) show statistical significance in univariate and multivariate analysis. "High-risk stigmata" was statistical powerful predictors of malignancy than "worrisome features". International consensus guidelines 2012 had improvement on specificity but deterioration of sensitivity. Conclusion Revised guidelines seemed to bring about an improvement of weak side of Sendai criteria. Abdominal pain, jaundice, main pancreas duct greater than 10 mm can be clinical variables to predict malignancy. PMID:26942155

  8. Global Genomic Analysis of Intraductal Papillary Mucinous Neoplasms of the Pancreas Reveals Significant Molecular Differences Compared to Ductal Adenocarcinoma

    PubMed Central

    Fritz, Stefan; Fernandez-del Castillo, Carlos; Mino-Kenudson, Mari; Crippa, Stefano; Deshpande, Vikram; Lauwers, Gregory Y.; Warshaw, Andrew L.; Thayer, Sarah P.; Iafrate, A. John

    2013-01-01

    Objective To determine whether intraductal papillary mucinous neoplasms of the pancreas (IPMNs) have a different genetic background compared with ductal adenocarcinoma (PDAC). Summary Background Data The biologic and clinical behavior of IPMNs and IPMN-associated adenocarcinomas is different from PDAC in having a less aggressive tumor growth and significantly improved survival. Up to date, the molecular mechanisms underlying the clinical behavior of IPMNs are incompletely understood. Methods 128 cystic pancreatic lesions were prospectively identified during the course of 2 years. From the corresponding surgical specimens, 57 IPMNs were separated and subdivided by histologic criteria into those with low-grade dysplasia, moderate dysplasia, high-grade dysplasia, and invasive cancer. Twenty specimens were suitable for DNA isolation and subsequent performance of array CGH. Results While none of the IPMNs with low-grade dysplasia displayed detectable chromosomal aberrations, IPMNs with moderate and high-grade dysplasia showed frequent copy number alterations. Commonly lost regions were located on chromosome 5q, 6q, 10q, 11q, 13q, 18q, and 22q. The incidence of loss of chromosome 5q, 6q, and 11q was significantly higher in IPMNs with high-grade dysplasia or invasion compared with PDAC. Ten of 13 IPMNs with moderate dysplasia or malignancy had loss of part or all of chromosome 6q, with a minimal deleted region between linear positions 78.0 and 130.0. Conclusions This study is the first to use array CGH to characterize IPMNs. Recurrent cytogenetic alterations were identified and were different than those described in PDAC. Array CGH may help distinguish between these 2 entities and give insight into the differences in their biology and prognosis. PMID:19247032

  9. Biochemical characterization of the component parts of intestinal mucin from patients with cystic fibrosis.

    PubMed Central

    Mantle, M; Forstner, G G; Forstner, J F

    1984-01-01

    Previous studies have shown that human small-intestinal mucin consists of high-Mr glycoproteins and a smaller S-S-bonded protein of 118 kDa. The major antigenic determinants of the mucin were associated with the large glycoproteins, but depended for stability on intact disulphide bonds, and were destroyed by digestion with Pronase. In the present study we isolated and analysed the component parts of mucin from patients with cystic fibrosis with special attention being paid to the peptide constituents. After reduction with 0.2 M-beta-mercaptoethanol [5 min, 100 degrees C in 1% SDS (sodium dodecyl sulphate)], the large glycoproteins and smaller peptide with an apparent molecular size of 118 kDa were separated by equilibrium density-gradient centrifugation in CsCl, Sepharose 4B chromatography or preparative SDS/polyacrylamide-gel electrophoresis. The large glycoproteins contained about 70% of the protein of the native mucin. Digestion with Pronase resulted in a further loss of 'naked' protein (10% of the native mucin protein) from the C-terminal end of the glycoprotein peptide core, and left behind highly glycosylated proteins comprised mainly (70 mol%) of threonine, serine and proline. The 118 kDa component, which contained about 30% of the native mucin protein, consisted mainly of aspartic acid, serine, glutamic acid and glycine (40 mol%), plus threonine, proline, alanine, valine and leucine (35 mol%). Together with the 'naked' protein segment, the 118 kDa component contained most of the cysteine residues of the native mucin. Surprisingly, the peptide also contained carbohydrate (less than or equal to 5% of the native mucin carbohydrate but 50% by weight of the 118 kDa component), which included 9 mol% mannose, suggesting the presence of N-linked oligosaccharides. The peptide exhibited strong non-covalent interactions with the high-Mr glycoproteins and a tendency to self-aggregate in the absence of dissociating agents. Our findings therefore suggest that native

  10. Host mucin glycosylation plays a role in bacterial adhesion in lungs of individuals with cystic fibrosis.

    PubMed

    Venkatakrishnan, Vignesh; Packer, Nicolle H; Thaysen-Andersen, Morten

    2013-10-01

    Malfunction of the cell surface glycoprotein, cystic fibrosis transmembrane conductance regulator, is the molecular hallmark of cystic fibrosis (CF), causing salt imbalance across the lung epithelium and biochemical and biophysical alterations of the mucous secretion and airway surfaces. Abnormal glycosylation of both secreted and membrane-tethered airway mucins in CF hosts are reported by a substantial body of literature and correlates with bacterial infection and inflammation in CF airways, features that are linked to the CF pathology. It is established that Pseudomonas aeruginosa and other CF-typic bacteria use the altered host mucin glycosylation as receptors for adhesion by dedicated lectins and adhesins recognizing an array of the aberrantly expressed glycan determinants. This review aims to describe the aberrant mucin glycosylation phenotype observed in CF airways relative to the non-CF equivalent by summarizing the wealth of literature on this topic. The possible causes and effects of altered glycosylation in the respiratory system are discussed. Specific attention is given to the adhesion mechanisms of the opportunistic P. aeruginosa, which utilizes the molecular alterations of the lung to gain access to the normally sterile airways. Finally, the emerging glycosylation-based therapeutics that show promising potential for reducing bacterial infection in individuals with CF by molecular mimicry mechanisms are discussed. PMID:24138697

  11. Transcriptional Activation of Mucin by Pseudomonas aeruginosa Lipopolysaccharide in the Pathogenesis of Cystic Fibrosis Lung Disease

    NASA Astrophysics Data System (ADS)

    Li, Jian-Dong; Dohrman, Austin F.; Gallup, Marianne; Miyata, Susumu; Gum, James R.; Kim, Young S.; Nadel, Jay A.; Prince, Alice; Basbaum, Carol B.

    1997-02-01

    An unresolved question in cystic fibrosis (CF) research is how mutations of the CF transmembrane conductance regulator, a CI ion channel, cause airway mucus obstruction leading to fatal lung disease. Recent evidence has linked the CF transmembrane conductance regulator mutation to the onset and persistence of Pseudomonas aeruginosa infection in the airways, and here we provide evidence directly linking P. aeruginosa infection to mucus overproduction. We show that P. aeruginosa lipopolysaccharide profoundly upregulates transcription of the mucin gene MUC 2 in epithelial cells via inducible enhancer elements and that this effect is blocked by the tyrosine kinase inhibitors genistein and tyrphostin AG 126. These findings improve our understanding of CF pathogenesis and suggest that the attenuation of mucin production by lipopolysaccharide antagonists and tyrosine kinase inhibitors could reduce morbidity and mortality in this disease.

  12. Sub-branch and mixed-type intraductal papillary mucinous neoplasms of the pancreas: 2 case reports.

    PubMed

    Ozmen, Zafer; Aktas, Fatma; Okan, İsmail; Özsoy, Zeki; Arıcı, Akgül

    2016-03-01

    Intraductal papillary mucinous neoplasia (IPMN) is one of the cystic neoplasias of the pancreas. The imaging findings provide that these tumors are differentiated from the other cystic lesions of the pancreas, especially from the chronic pancreatitis, where the treatment protocol is completely different. Therefore, the correct diagnosis and classification of the IPMN ensures that the patient receives the correct approach and the appropriate surgery, if necessary. The purpose of this study is to emphasize the imaging findings of the different types of the IPMN and the changes in the management protocol of the patients according to these radiological findings. PMID:26973726

  13. A case of pancreatic heterotopy of duodenal wall, intraductal papillary mucinous tumor and intraepithelial neoplasm of pancreas, papillary carcinoma of kidney in a single patient.

    PubMed

    Nobili, Cinzia; Franciosi, Claudio; Degrate, Luca; Caprotti, Roberto; Romano, Fabrizio; Perego, Elisa; Trezzi, Rosangela; Leone, Biagio Eugenio; Uggeri, Franco

    2006-01-01

    We report a case of the contemporaneous presence of two histologically different pancreatic neoplasms, one renal cancer and one embryogenic duodenal anomaly in a single patient. A 66-year-old man underwent ultrasound examination because of urinary disorders; a solid neoformation within the inferior pole of the left kidney was observed. Computed tomography confirmed the renal lesion, but also a heterogeneous mass within the pancreatic head appeared without bile ducts dilatation. Abdominal magnetic resonance revealed a multiloculated cystic component of the pancreatic mass. A second CT scan confirmed the renal and biliary findings, but it revealed a modest enlargement of the pancreatic asymptomatic mass. A resection of the left kidney inferior pole and a pylorus-preserving pancreaticoduodenectomy were performed. Histopathologic analysis of the surgical specimen revealed mild differentiated papillary renal carcinoma, intraductal papillary mucinous adenoma of the pancreatic head, foci of intraepithelial pancreatic neoplasm and pancreatic heterotopy of duodenal muscular and submucosal layers. The coexistence of several primaries and anomalies in one patient led us to suppose a genetic predisposition to different lesions, even in the absence of known familial genetic syndromes. The study of such cases may help to improve the investigation of molecular correlations and etiological factors of different solid tumors. Nowadays, surgery is the only effective cure. PMID:17168444

  14. [A case of intussusception caused by low-grade appendiceal mucinous neoplasm with high serum levels of carcinoembryonic antigen resected by laparoscopy-assisted colectomy].

    PubMed

    Mitsuoka, Akito; Kuwabara, Hiroshi; Watanabe, Shuichi; Liu, Bo; Nakamura, Hiroshi; Sanada, Takahiro; Koike, Morio; Goseki, Narihide

    2013-11-01

    The patient was a 43-year-old woman who visited our hospital with a primary complaint of intermittent abdominal pain. Upper gastrointestinal endoscopy revealed mild gastritis. The patient was treated with oral drugs, and the course was followed. Subsequently, the abdominal pain aggravated, and computed tomography (CT) was performed, in which right colic intussusception with a 4 cm cystic mass in the advanced portion was noted. Reduction by enema was attempted on the same day; however, a residual tumorous lesion was present in the ileocecal region. The patient was diagnosed with a cecal submucosal tumor with intussusception, and therefore, laparoscopic surgery was performed. Intussusception of the entire appendix in the ascending colon was noted, for which laparoscopic ileocecal resection was performed. In the excised specimen, a cystic lesion comprising the appendix filled with mucus was present. The postoperative recovery was favorable, and the patient was discharged on the seventh postoperative day. The lesion was histopathologically diagnosed as low-grade appendiceal mucinous neoplasm. PMID:24393974

  15. Current roles of endoscopy in the management of intraductal papillary mucinous neoplasm of the pancreas

    PubMed Central

    2015-01-01

    Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is characterized by intraductal papillary proliferation of mucin‐producing epithelial cells that exhibit various degrees of dysplasia. IPMN is classified into four histological subtypes (gastric, intestinal, pancreatobiliary, and oncocytic) according to its histomorphological and immunohistochemical characteristics. Endoscopic retrograde cholangiopancreatography plays a crucial role in the evaluation of these features of IPMN. Endoscopic ultrasonography (EUS) has proven to be more sensitive than computed tomography or magnetic resonance imaging for early detection of malignancy. The present review addresses the current roles of endoscopy and related techniques in the management of IPMN. The particular focus is on diagnosing IPMN and malignancy within IPMN, detecting pancreatic cancer concomitant with IPMN, differentiating the epithelial subtypes of IPMN, determining the optimal strategy for the management of branch duct IPMN, and discussing innovative endoscopic technology related to IPMN. The disadvantages of endoscopic examinations of IPMN and different attitudes toward EUS‐guided fine‐needle aspiration for IPMN between Japan (negative) and other countries (active) are also discussed. PMID:25588761

  16. Immunobiology and immunosurveillance in patients with intraductal papillary mucinous neoplasms (IPMNs), premalignant precursors of pancreatic adenocarcinomas.

    PubMed

    Beatty, Pamela L; van der Geest, Rick; Hashash, Jana G; Kimura, Takashi; Gutkin, Dmitriy; Brand, Randall E; Finn, Olivera J

    2016-07-01

    Premalignant lesions for many cancers have been identified, and efforts are currently directed toward identification of antigens expressed on these lesions that would provide suitable targets for vaccines for cancer prevention. Intraductal papillary mucinous neoplasms (IPMNs) are premalignant pancreatic cysts of which a subset has the potential to progress to cancer. Currently, there are no validated predictive markers for progression to malignancy. We hypothesized that the presence or absence of immune surveillance of these lesions would be one such factor. Here we show that the tumor antigen MUC1, which is abnormally expressed on pancreatic cancer and is a target for cancer immunosurveillance, is also abnormally expressed on premalignant IPMN. We show that some IPMN patients make MUC1-specific IgG. Moreover, we show evidence of CD4 and CD8 T cell infiltration into IPMN areas of high dysplasia suggesting an ongoing immune response within the lesions. We also found, however, increased levels of circulating myeloid-derived suppressor cells (MDSCs) and regulatory T cells (Tregs) in some IPMN patients as well as evidence of T cell exhaustion. Further studies correlating immunosurveillance or immunosuppression with IPMN progression to malignancy will help define the immune response as a biomarker of risk, leading potentially to a vaccine to boost spontaneous immunity and prevent progression to cancer. PMID:27106024

  17. The chromatin regulator Brg1 suppresses formation of intraductal papillary mucinous neoplasm and pancreatic ductal adenocarcinoma

    PubMed Central

    von Figura, Guido; Fukuda, Akihisa; Roy, Nilotpal; Liku, Muluye E.; Morris, John P.; Kim, Grace E.; Russ, Holger A.; Firpo, Matthew A.; Mulvihill, Sean J.; Dawson, David W.; Ferrer, Jorge; Mueller, William F.; Busch, Anke; Hertel, Klemens J.; Hebrok, Matthias

    2015-01-01

    Pancreatic ductal adenocarcinoma (PDA) develops through distinct precursor lesions, including pancreatic intraepithelial neoplasia (PanIN) and intraductal papillary mucinous neoplasia (IPMN). However, genetic features resulting in IPMN-associated PDA (IPMN–PDA) versus PanIN-associated PDA (PanIN-PDA) are largely unknown. Here we find that loss of Brg1, a core subunit of SWI/SNF chromatin remodelling complexes, cooperates with oncogenic Kras to form cystic neoplastic lesions that resemble human IPMN and progress to PDA. Although Brg1-null IPMN–PDA develops rapidly, it possesses a distinct transcriptional profile compared with PanIN-PDA driven by mutant Kras and hemizygous p53 deletion. IPMN–PDA also is less lethal, mirroring prognostic trends in PDA patients. In addition, Brg1 deletion inhibits Kras-dependent PanIN development from adult acinar cells, but promotes Kras-driven preneoplastic transformation in adult duct cells. Therefore, this study implicates Brg1 as a determinant of context-dependent Kras-driven pancreatic tumorigenesis and suggests that chromatin remodelling may underlie the development of distinct PDA subsets. PMID:24561622

  18. Proteomic Mucin Profiling for the Identification of Cystic Precursors of Pancreatic Cancer

    PubMed Central

    2014-01-01

    Background Pancreatic cystic lesions (PCLs) are increasingly frequent radiological incidentalomas, with a considerable proportion representing precursors of pancreatic cancer. Better diagnostic tools are required for patients to benefit from this development. Methods To evaluate whether cyst fluid mucin expression could predict malignant potential and/or transformation in PCLs, a proteomic method was devised and prospectively evaluated in consecutive patients referred to our tertiary center for endoscopic ultrasound-guided aspiration of cystic lesions from May 2007 through November 2008 (discovery cohort) and from December 2008 through October 2012 (validation cohort). Cytology and cyst fluid carcinoembryonic antigen (CEA; premalignancy > 192ng/mL, malignancy > 1000ng/mL) were routinely analyzed, and samples were further processed as follows: one-dimensional gel electrophoresis, excision of high-mass areas, tryptic digestion and nano-liquid chromatography–tandem mass spectrometry, with peptide identification by Mascot software and an in-house mucin database. All diagnostic evaluations were blinded to proteomics results. Histology was required to confirm the presence/absence of malignant transformation. All statistical tests were two-sided. Results Proteomic mucin profiling proved statistically significantly more accurate (97.5%; 95% confidence interval [CI] = 90.3% to 99.6%) than cytology (71.4%; 95% CI = 59.8% to 80.9%; P < .001) and cyst fluid CEA (78.0%; 95% CI = 65.0% to 87.3%; P < .001) in identifying the 37 (out of 79; 46.8%) lesions with malignant potential (ie, premalignant or malignant tumors). The accuracy of proteomics was nearly identical (96.6% vs 98.0%) between the discovery (n = 29) and validation (n = 50) cohorts. Furthermore, mucin profiling predicted malignant transformation, present in 16 out of 29 (discovery cohort: 9, validation cohort: 20) lesions with available histology, with 89.7% accuracy (95% CI = 71.5% to 97.3%) (for the validation

  19. Value of apparent diffusion coefficient for predicting malignancy of intraductal papillary mucinous neoplasms of the pancreas

    PubMed Central

    Zhang, Lei; Rao, Sheng-Xiang; Xu, Xue-Feng; Wang, Dan-Song; Jin, Da-Yong; Zeng, Meng-Su

    2016-01-01

    PURPOSE We aimed to explore the potential value of the whole tumor apparent diffusion coefficient (ADC) for discriminating between benign and malignant intraductal papillary mucinous neoplasms (IPMNs) of the pancreas. METHODS Forty-two patients underwent 1.5 T magnetic resonance imaging that included diffusion-weighted imaging (DWI, b=0.500 s/mm2). The mean, minimum, and maximum ADC values were measured for the whole tumor. The differences between benign and malignant IPMNs were calculated for the mean ADC, ADC-min, and ADC-max values. Receiver operating characteristics (ROC) analysis was conducted to evaluate their potential diagnostic performance. RESULTS Fifteen of 25 benign IPMNs demonstrated low or iso-signal intensity on DWI with a b value of 500 s/mm2 compared with normal pancreatic parenchyma, whereas all malignant IPMNs demonstrated high signal intensity. The mean value of ADC was significantly higher in benign IPMNs compared with malignant IPMNs (3.39×10−3 mm2/s vs. 2.39×10−3 mm2/s, P < 0.001), with an area under the ROC curve (AUC) of 0.92 (95% confidence interval [CI], 0.79–0.98). The ADC-min value of malignant IPMNs was also significantly lower than that of benign IPMNs (1.24×10−3 mm2/s vs. 2.58×10−3 mm2/s, P < 0.001), with an AUC of 0.94 (95% CI, 0.82–0.99). No marked difference was found between benign and malignant IPMNs for the ADC-max value (3.89×10−3 mm2/s vs. 3.78×10−3 mm2/s, P = 0.299). CONCLUSION Lower mean and minimum ADC values of the whole tumor might be potential predictors of malignant IPMNs of the pancreas. PMID:27283593

  20. MIB-1 labeling index, Ki-67, is an indicator of invasive intraductal papillary mucinous neoplasm

    PubMed Central

    Shimura, Tatsuo; Kofunato, Yasuhide; Okada, Ryo; Yashima, Rei; Okada, Koji; Araki, Kenichiro; Hosouchi, Yasuo; Kuwano, Hiroyuki; Takenoshita, Seiichi

    2016-01-01

    Despite strict criteria for the observation of intraductal papillary mucinous neoplasm (IPMN), it remains difficult to distinguish invasive IPMN from non-invasive IPMN. The aim of the present study was to identify an indicator of invasive IPMN. The present study retrospectively evaluated 53 patients (28 with non-invasive and 25 with invasive IPMN) who underwent resection of IPMN, and examined the usefulness of the MIB-1 labeling index as an indicator of invasive IPMN. The MIB-1 labeling indexes in patients with invasive IPMN were significantly higher compared with those with non-invasive IPMN (P<0.001). A receiver operating characteristic curve revealed that the area under the curve was 0.822. These results suggested that a cut-off level for the MIB-1 labeling index should be set to 15.5% to distinguish invasive from non-invasive IPMN. A multivariate analysis using a logistic regression model revealed the MIB-1 labeling index (hazard ratio, 18.692; 95% confidential interval, 4.171–83.760; P<0.001) and the existence of mural nodules (hazard ratio, 6.187, 95% confidential interval, 1.039–36.861; P=0.045) were predictive factors for invasive IPMN. However, no statistically significant differences were observed between patients with a lower MIB-1 labeling index and patients with a higher MIB-1 labeling index (P=0.798). The MIB-1 labeling index must be considered as a candidate for the classification of IPMN. PMID:27446570

  1. Cystic acantholytic dyskeratosis of the vulva: An unusual presentation of a follicular adnexal neoplasm

    PubMed Central

    Torres, Kara Melissa Tiangco; Junkins-Hopkins, Jacqueline M.

    2016-01-01

    Acantholytic dyskeratosis (AD) is a histologic pattern seen in Darier's disease or dyskeratosis follicularis, warty dyskeratoma, and transient AD. This pattern is characterized by suprabasilar clefting, acantholysis, and formation of corps ronds and grains. We present a case of AD that is unique based on its genital location and cystic architecture. A 53-year-old woman presented with an otherwise asymptomatic cyst on her left vulva of uncertain duration. On microscopic examination, there were fragments of cystic epithelium with areas of hypergranulosis, acantholysis, corps ronds, and corps grains formation. These features are felt by the authors to be a unique presentation of a follicular adnexal neoplasm. PMID:27559500

  2. [Intraductal papillary mucinous neoplasia: which findings support observation?].

    PubMed

    Mayerle, J; Kraft, M; Menges, P; Simon, P; Ringel, J; Partecke, L I; Heidecke, C D; Lerch, M M

    2012-02-01

    On abdominal CT scans asymptomatic cystic lesions of the pancreas are accidentally detected in 1-2% of patients. Congenital cysts and pancreatic pseudocysts account for two thirds of these lesions. Pancreatic pseudocysts are a frequent complication of acute and chronic pancreatitis. Among resected cystic neoplasms serous cystic adenoma accounts for 30%, mucinous cystic neoplasms for 45% and intraductal papillary mucinous neoplasms for 25%. The diagnosis of a cystic pancreatic lesion is usually made by diagnostic imaging. Symptomatic lesions require definitive therapeutic treatment after appropriate diagnostic work-up. In the diagnosis of asymptomatic cystic lesions several factors are important, among them whether the cyst is connected to the pancreatic duct (as in IPMN and pseudocysts), the size of lesion (for treatment indications) and whether nodules form in the wall of the cyst (a sign of potential malignancy). EUS-guided fine needle aspiration of the cyst fluid adds to the discrimination between benign, premalignant and malignant cystic lesions. Measuring lipase activity, CEA, viscosity and mucin as well as cytology can help in differentiating cystic lesions. An algorithm is discussed for the differential diagnosis and for selection of the appropriate treatment for pancreatic cystic lesions, most of which never require surgery. PMID:22271054

  3. A case of undifferentiated carcinoma of the pancreas mimicking main-duct intraductal papillary mucinous neoplasm (IPMN).

    PubMed

    Kawai, Yuichi; Nakamichi, Rei; Kamata, Noriko; Miyake, Hideo; Fujino, Masahiko; Itoh, Shigeki

    2015-03-01

    We report here a rare case of undifferentiated carcinoma of the pancreas mimicking main-duct intraductal papillary mucinous neoplasm. In an 80-year-old woman, an approximately 8-mm papillary mass was incidentally detected at the downstream edge of a dilatated main pancreatic duct lumen on CT and MRI. Main pancreatic duct dilatation in the pancreatic body and tail and parenchymal atrophy were observed in the upstream of the mass. Histopathologically, the tumor protruded into the downstream edge of the dilatated main pancreatic duct lumen in the pancreatic body. The tumor cells had highly atypical nuclei and abundant polymorphic structures, and showed positive staining for granulocyte colony-stimulating factor, which led to the diagnosis of undifferentiated carcinoma. A total of 13 cases of undifferentiated carcinoma with intraductal tumor growth have been reported to date. The case report by Bergmann et al. has been the smallest in histopathological specimen, and the present case is the smallest in size detected by radiological images. Since early undifferentiated carcinoma of the pancreas can resemble those of main-duct intraductal papillary mucinous neoplasm in cross-sectional images, we have to consider undifferentiated carcinoma in the differential diagnosis of the solitary and papillary mass with low contrast enhancement in early phase in the main pancreatic duct. PMID:25526684

  4. Cystic lesions of the pancreas

    PubMed Central

    Karoumpalis, Ioannis; Christodoulou, Dimitrios K.

    2016-01-01

    Different types of benign or malignant cystic lesions can be observed in the pancreas. Pancreatic cystic lesions are classified under pathology terms into simple retention cysts, pseudocysts and cystic neoplasms. Mucinous cystic neoplasm is a frequent type of cystic neoplasm and has a malignant potential. Serous cystadenoma follows in frequency and is usually benign. Intraductal papillary mucinous neoplasms are the most commonly resected cystic pancreatic neoplasms characterized by dilated segments of the main pancreatic duct and/or side branches, the wall of which is covered by mucus secreting cells. These neoplasms can occupy the pancreatic head or any part of the organ. Solid pseudopapillary tumor is rare, has a low tendency for malignancy, and is usually located in the pancreatic body or tail. Endoscopic ultrasound with the use of fine-needle aspiration and cytology permits discrimination of those lesions. In this review, the main characteristics of those lesions are presented, as well as recommendations regarding their follow up and management according to recent guidelines. PMID:27065727

  5. Cystic lesions of the pancreas.

    PubMed

    Karoumpalis, Ioannis; Christodoulou, Dimitrios K

    2016-01-01

    Different types of benign or malignant cystic lesions can be observed in the pancreas. Pancreatic cystic lesions are classified under pathology terms into simple retention cysts, pseudocysts and cystic neoplasms. Mucinous cystic neoplasm is a frequent type of cystic neoplasm and has a malignant potential. Serous cystadenoma follows in frequency and is usually benign. Intraductal papillary mucinous neoplasms are the most commonly resected cystic pancreatic neoplasms characterized by dilated segments of the main pancreatic duct and/or side branches, the wall of which is covered by mucus secreting cells. These neoplasms can occupy the pancreatic head or any part of the organ. Solid pseudopapillary tumor is rare, has a low tendency for malignancy, and is usually located in the pancreatic body or tail. Endoscopic ultrasound with the use of fine-needle aspiration and cytology permits discrimination of those lesions. In this review, the main characteristics of those lesions are presented, as well as recommendations regarding their follow up and management according to recent guidelines. PMID:27065727

  6. GNAS(R201H) and Kras(G12D) cooperate to promote murine pancreatic tumorigenesis recapitulating human intraductal papillary mucinous neoplasm.

    PubMed

    Taki, K; Ohmuraya, M; Tanji, E; Komatsu, H; Hashimoto, D; Semba, K; Araki, K; Kawaguchi, Y; Baba, H; Furukawa, T

    2016-05-01

    Intraductal papillary mucinous neoplasm (IPMN), the most common pancreatic cystic neoplasm, is known to progress to invasive ductal adenocarcinoma. IPMNs commonly harbor activating somatic mutations in GNAS and KRAS, primarily GNAS(R201H) and KRAS(G12D). GNAS encodes the stimulatory G-protein α subunit (Gsα) that mediates a stimulatory signal to adenylyl cyclase to produce cyclic adenosine monophosphate (cAMP), subsequently activating cAMP-dependent protein kinase A. The GNAS(R201H) mutation results in constitutive activation of Gsα. To study the potential role of GNAS in pancreatic tumorigenesis in vivo, we generated lines of transgenic mice in which the transgene consisted of Lox-STOP-Lox (LSL)-GNAS(R201H) under the control of the CAG promoter (Tg(CAG-LSL-GNAS)). These mice were crossed with pancreatic transcription factor 1a (Ptf1a)-Cre mice (Ptf1a(Cre/+)), generating Tg(CAG-LSL-GNAS);Ptf1a(Cre/+) mice. This mouse line showed elevated cAMP levels, small dilated tubular complex formation, loss of acinar cells and fibrosis in the pancreas; however, no macroscopic tumorigenesis was apparent by 2 months of age. We then crossed Tg(CAG-LSL-GNAS);Ptf1a(Cre/+) mice with LSL-Kras(G12D) mice, generating Tg(CAG-LSL-GNAS);LSL-Kras(G12D);Ptf1a(Cre/+) mice. We used these mice to investigate a possible cooperative effect of GNAS(R201H) and Kras(G12D) in pancreatic tumorigenesis. Within 5 weeks, Tg(CAG-LSL-GNAS);LSL-Kras(G12D);Ptf1a(Cre/+) mice developed a cystic tumor consisting of marked dilated ducts lined with papillary dysplastic epithelia in the pancreas, which closely mimicked the human IPMN. Our data strongly suggest that activating mutations in GNAS and Kras cooperatively promote murine pancreatic tumorigenesis, which recapitulates IPMN. Our mouse model may serve as a unique in vivo platform to find biomarkers and effective drugs for diseases associated with GNAS mutations. PMID:26257060

  7. Uncommon presentations of common pancreatic neoplasms: a pictorial essay.

    PubMed

    D'Onofrio, Mirko; De Robertis, Riccardo; Capelli, Paola; Tinazzi Martini, Paolo; Crosara, Stefano; Gobbo, Stefano; Butturini, Giovanni; Salvia, Roberto; Barbi, Emilio; Girelli, Roberto; Bassi, Claudio; Pederzoli, Paolo

    2015-08-01

    Pancreatic neoplasms are a wide group of solid and cystic lesions with different and often characteristic imaging features, clinical presentations, and management. Among solid tumors, ductal adenocarcinoma is the most common: it arises from exocrine pancreas, comprises about 90% of all pancreatic neoplasms, and generally has a bad prognosis; its therapeutic management must be multidisciplinary, involving surgeons, oncologists, gastroenterologists, radiologists, and radiotherapists. The second most common solid pancreatic neoplasms are neuroendocrine tumors: they can be divided into functioning or non-functioning and present different degrees of malignancy. Cystic pancreatic neoplasms comprise serous neoplasms, which are almost always benign, mucinous cystic neoplasms and intraductal papillary mucinous neoplasms, which can vary from benign to frankly malignant lesions, and solid pseudopapillary tumors. Other pancreatic neoplasms, such as lymphoma, metastases, or pancreatoblastoma, are rarely seen in clinical practice and have different and sometimes controversial managements. Rare clinical presentations and imaging appearance of the most common pancreatic neoplasms, both solid and cystic, are more frequently seen and clinically relevant than rare pancreatic tumors; their pathologic and radiologic appearances must be known to improve their management. The purpose of this paper is to present some rare or uncommon clinical and radiological presentations of common pancreatic neoplasms providing examples of multi-modality imaging approach with pathologic correlations, thus describing the histopathological bases that can explain the peculiar imaging features, in order to avoid relevant misdiagnosis and to improve lesion management. PMID:25772002

  8. Colloid (mucinous non-cystic) carcinoma of the pancreas: A case report

    PubMed Central

    GAO, YANG; ZHU, YA-YUN; YUAN, ZHOU

    2015-01-01

    Colloid carcinoma (CC) of the pancreas, also known as mucinous non-cystic carcinoma, is a rare histological variant of pancreatic cancer. The present study reports the case of a 65-year-old male with a pancreatic head tumor 4.6×3.1 cm in size. The tumor had not invaded the superior mesenteric or celiac arteries. Laboratory data showed elevated alkaline phosphatase, γ-gluytamyl transpeptidase and cancer antigen 19-9 (CA19-9) levels. The patient underwent a pancreaticoduodenectomy (PD), and the pathology revealed CC extending to the submucosa of the duodenum, with mild atypical hyperplasia of the pancreatic duct. The patient was negative for circulating tumor cells, indicating a good prognosis. The CA19-9 concentration decreased to within the normal range following surgery. The present study reports a rare case of CC of the pancreas presenting with obstructive jaundice, in which a PD procedure was performed. Primary surgical treatment with curative intent is the optimal management and this subtype of pancreatic cancer is associated with a better prognosis compared with pancreatic ductal adenocarcinoma. PMID:26722311

  9. Expression of MUC5AC and MUC5B mucins in normal and cystic fibrosis lung.

    PubMed

    Groneberg, D A; Eynott, P R; Oates, T; Lim, S; Wu, R; Carlstedt, I; Nicholson, A G; Chung, K F

    2002-02-01

    Hypersecretion of airway mucus is a characteristic feature of chronic airway diseases like cystic fibrosis (CF) and leads via impairment of the muco-ciliary clearance and bacterial superinfection to respiratory failure. The major components of the mucus matrix forming family of mucins in the airways are MUC5AC and MUC5B. To investigate the expression of these glycoproteins in CF, immunohistochemistry was carried out on trachea, bronchi and peripheral lung obtained from CF patients and compared to normal lung tissues. MUC5AC immunohistochemistry demonstrated signals in goblet cells of the epithelial lining. Also, goblet cells inside glandular secretory ducts revealed MUC5AC-positive staining. In comparison to those from normal subjects, CF sections were characterized by inflammatory changes and goblet cell hyperplasia, resulting in increased numbers of MUC5AC-positive cells. Immunohistochemical staining for MUC5B showed abundant staining of submucosal glands and epithelial goblet cells. Inside the glands, the immunoreactivity was restricted to glandular mucous cells. MUC5AC and MUC5B are expressed in the same histological pattern in CF compared to normal tissues with an increase of MUC5AC-positive cells due to goblet cell hyper- and metaplasia. PMID:11860173

  10. Cystic nephroma/mixed epithelial stromal tumor: a benign neoplasm with potential for recurrence.

    PubMed

    Sun, Belinda L; Abern, Michael; Garzon, Steven; Setty, Suman

    2015-05-01

    Cystic nephroma (CN) is a rare, benign, renal neoplasm composed of epithelial and stromal elements. Only about 200 cases have been reported since 1892 and recurrence has rarely been observed. We report a 32-year-old Hispanic woman, with a history of a right, complex cystic, renal mass treated by robotic decortication 2 years ago, who presented with flank pain, hematuria, and recurrent urinary tract infection. A magnetic resonance imaging study showed a 3.4-cm multicystic lesion with thickened septa and enhancement at the right kidney. The partial nephrectomy specimen revealed a well-circumscribed, multicystic tumor abutting the renal pelvis, with thick septa and smooth walls, filled with clear fluid. Microscopic examination showed variably sized cysts lined by cuboidal epithelium with focal hobnailing, without significant cytologic atypia and mitosis. The epithelial lining was positive for CK19, high molecular weight cytokeratin, and α-methylacyl-CoA racemase suggesting a primitive tubular epithelial phenotype. Primitive glomeruli-like structures were also present. The ovarian-like stroma was condensed around the cysts and was variably cellular with areas of muscle differentiation and thick-walled vessels. The stroma was positive for desmin, estrogen receptor, progesterone receptor, and CD10. We suggest that CN represents a variable mixture of epithelial and stromal elements, immature glomerular, tubular, muscle, and vascular elements, which may be present in variable proportions creating a spectrum of lesions previously described as CN and mixed epithelial and stromal tumors (MEST). This case emphasizes that CN/MEST clinically/radiologically mimics other cystic renal neoplasms, especially cystic renal cell carcinoma and tubulocystic carcinoma, necessitating histopathological examination and immunohistochemial studies for definitive diagnosis. Additionally, CN has the tendency to recur when not completely excised initially. PMID:25525149

  11. Tracking the Clonal Evolution of Adenosquamous Carcinoma, a Rare Variant of Intraductal Papillary Mucinous Neoplasm of the Pancreas.

    PubMed

    Matsuzaka, Suguru; Karasaki, Hidenori; Ono, Yusuke; Ogata, Munehiko; Oikawa, Kensuke; Tamakawa, Susumu; Chiba, Shin-Ichi; Muraki, Miho; Yokochi, Tomoki; Funakoshi, Hiroshi; Kono, Toru; Nagashima, Kazuo; Mizukami, Yusuke

    2016-07-01

    Adenosquamous carcinoma (ASC) is an uncommon variant of pancreatic neoplasm. We sought to trace the mode of tumor progression using specimens of ASC associated with intraductal papillary mucinous neoplasm (IPMN) of the pancreas. A resected specimen of the primary pancreatic ASC, developed in a 72-year-old man, was subjected to mutation profiling using amplicon-targeted sequencing and digital polymerase chain reaction. DNA was isolated from each histological compartment including noninvasive IPMN, squamous cell carcinoma (SCC), and adenocarcinoma (AC). Histologically, an IPMN with a large mural nodule was identified. The invasive tumor predominantly consisted of SCC, and a smaller AC was found around the lesion. Squamous metaplasias were sporadically distributed within benign IPMNs. Mutation alleles KRAS and GNAS were identified in all specimens of IPMN including the areas of squamous metaplasia. In addition, these mutations were found in SCC and AC. Clear transition from flat/low-papillary IPMN to SCC indicated a potent invasion front, and the SCC compartment was genetically unique, because the area has a higher frequency of mutation KRAS. The invasive tumors with distinct histological appearances shared the form of noninvasive IPMN as a common precursor, rather than de novo cancer, suggesting the significance of a genetic profiling scheme of tumors associated with IPMN. PMID:27295533

  12. Single-incision laparoscopic cecectomy for low-grade appendiceal mucinous neoplasm after laparoscopic rectectomy

    PubMed Central

    Fujino, Shiki; Miyoshi, Norikatsu; Noura, Shingo; Shingai, Tatsushi; Tomita, Yasuhiko; Ohue, Masayuki; Yano, Masahiko

    2014-01-01

    In this case report, we discuss single-incision laparoscopic cecectomy for low-grade appendiceal neoplasm after laparoscopic anterior resection for rectal cancer. The optimal surgical therapy for low-grade appendiceal neoplasm is controversial; currently, the options include appendectomy, cecectomy, right hemicolectomy, and open or laparoscopic surgery. Due to the risk of pseudomyxoma peritonei, complete resection without rupture is necessary. We have encountered 5 cases of low-grade appendiceal neoplasm and all 5 patients had no lymph node metastasis. We chose the appendectomy or cecectomy without lymph node dissection if preoperative imaging studies did not suspect malignancy. In the present case, we performed cecectomy without lymph node dissection by single-incision laparoscopic surgery (SILS), which is reported to be a reduced port surgery associated with decreased invasiveness and patient stress compared with conventional laparoscopic surgery. We are confident that SILS is a feasible alternative to traditional surgical procedures for borderline tumors, such as low-grade appendiceal neoplasms. PMID:24868331

  13. Spread of Intraductal Papillary Neoplasm Arising from the Cystic Duct of the Biliary Tree

    PubMed Central

    Kadowaki, Yoshihiko; Yokota, Yuki; Komoto, Satoshi; Kubota, Nobuhito; Okamoto, Takahiro; Ishido, Nobuhiro; Okino, Tsuyoshi

    2016-01-01

    Intraductal papillary neoplasm of the bile duct (IPNB) is a variant type of the bile duct carcinoma characterized by intraductal growth. IPNB is also recognized as a precursor of invasive carcinoma. We describe herein an extremely rare case of IPNB arising from the cystic duct. A 68-year-old man was admitted to our hospital for investigation of epigastralgia and abnormal levels of biliary tract enzyme. Computed tomography and magnetic resonance imaging showed a mass lesion spreading from the cystic duct to the upper-middle bile duct. Endoscopic retrograde cholangiography demonstrated diffuse duct dilation with a grossly visible intraductal mass and amorphous blobs, suggesting the presence of mucobilia or scattered tumors. We performed extrahepatic bile duct resection with lymphadenectomy. Macroscopically, a friable papillary tumor originated from the cystic duct grows intraluminally into the bile duct. Pathologically, the tumor was found to be intramucosal adenocarcinoma spreading to the whole extrahepatic bile duct, which was compatible with IPNB. We should discuss the features and progression processes of IPNB through this precious case.

  14. Immunohistochemistry in the Diagnosis of Mucinous Neoplasms Involving the Ovary: The Added Value of SATB2 and Biomarker Discovery Through Protein Expression Database Mining.

    PubMed

    Strickland, Sarah; Wasserman, Jason K; Giassi, Ana; Djordjevic, Bojana; Parra-Herran, Carlos

    2016-05-01

    Immunohistochemistry is frequently used to identify ovarian mucinous neoplasms as primary or metastatic; however, there is significant overlap in expression patterns. We compared traditional markers (CK7, CK20, CDX2, PAX8, estrogen receptor, β-catenin, MUC1, MUC2, and MUC5AC) to 2 novel proteins identified through mining of the Human Protein Atlas expression database: SATB2 and POF1B. The study cohort included 49 primary gastrointestinal (GI) mucinous adenocarcinomas (19 colorectal, 15 gastric, 15 pancreatobiliary), 60 primary ovarian mucinous neoplasms (19 cystadenomas, 21 borderline tumors, 20 adenocarcinomas), and 19 metastatic carcinomas to the ovary (14 lower and 5 upper GI primaries). Immunohistochemistry was performed on tissue microarrays, scored and interpreted as negative (absent or focal/weak) or positive. Metastatic tumors were frequently unilateral (42.8% of tumors from lower and 40% of tumors from upper tract) and ≥10 cm (85.7% of tumors from lower and 80% of tumors from upper tract). CK7 was positive in 88.5% upper GI and 88.3% primary ovarian compared with 24.3% lower GI neoplasms. CK20 and CDX2 were positive in 84.8% and 100% of lower GI tumors, respectively; however, expression was also common in upper GI (CK20 42.8%, CDX2 50%) and primary ovarian neoplasms (CK20 65.7%, CDX2 38.3%). Conversely, SATB2 was more specific for lower GI origin, being positive in 78.8% lower GI but only 11.5% upper GI and 1.7% primary ovarian neoplasms. PAX8 expression was common in primary ovarian neoplasms (75% of all neoplasms, 65% of carcinomas); only 1 (1.5%) GI tumor was positive. MUC2 and β-catenin were frequently positive in lower GI tumors (96.9% and 51.5%, respectively). Estrogen receptor expression was only seen in primary ovarian neoplasms (13.3%). Nuclear premature ovarian failure 1B (POF1B) expression was seen in malignant tumors regardless of their origin. A panel including CK7, SATB2, and PAX8 separated primary from secondary GI neoplasms with up to

  15. Surgical treatment of mucin-producing cholangiocarcinoma arising from intraductal papillary neoplasm of the intrahepatic bile duct: a report of 2 cases

    PubMed Central

    Baterdene, Namsrai; Lee, Jong-Wook; Jung, Min-Jae; Shin, Heeji; Seo, Hye Kyoung; Kim, Myeong-Hwan; Lee, Sung-Koo

    2016-01-01

    Intraductal papillary neoplasms of the bile duct (IPNB) leads to malignant transformation and mucin production. Herein, we presented two cases of mucin-producing IPNB with obstructive jaundice who underwent resection of the intrahepatic lesions and bypass hepaticojejunostomy. The first case was a 69 year-old male patient with 5-year follow up for gallstone disease. Imaging studies showed mucin-secreting IPNB mainly in the hepatic segment III bile duct (B3) and multiple intrahepatic duct stones for which, segment III resection, intrahepatic stone removal, end-to-side choledochojejunostomy and B3 hepaticojejunostomy were conducted. The second case was a 74 year-old female patient with 11-year follow up for gallstone disease. Imaging studies showed mucin-producing IPNB with dilatation of the segment IV duct (B4) and mural nodules for which, segment IV resection, partial resection of the diaphragm and central hepaticojejunostomy were conducted. Both patients recovered uneventfully from surgery. These cases highlight that in patients with IPNB, abundant production of highly viscous mucin inducing obstructive jaundice may be associated with malignant transformation. PMID:27621752

  16. Osteopathic Approach to the Diagnosis of Appendiceal Mucinous Cystadenocarcinoma Mimicking Primary Ovarian Malignant Neoplasm.

    PubMed

    Martingano, Daniel; Gurm, Hashroop; Oliff, Andrew; Martingano, Francis X; Aglialoro, George

    2016-07-01

    The fifth leading cause of cancer-related deaths among women in the United States is ovarian cancer. An estimated 21,980 new cases and 14,270 estimated deaths occurred nationwide in 2014. More than two-thirds of cases of ovarian cancer are diagnosed at stage III or IV when the peritoneal cavity or other organs are affected. Primary appendiceal malignant neoplasms may mimic advanced-stage ovarian cancer and can be misdiagnosed because of its presentation as a palpable adnexal mass. The authors describe a 42-year-old woman who was admitted to the department of obstetrics and gynecology to receive treatment for presumed advanced-stage ovarian cancer. She subsequently received a diagnosis of primary pseudomyxoma peritonei metastatic to the ovaries, mimicking a primary ovarian cancer by osteopathic structural examination findings, serum tumor markers, surgical exploration, and histopathologic confirmation. PMID:27367953

  17. Solid tumors of the pancreas can put on a mask through cystic change

    PubMed Central

    2011-01-01

    Background Solid pancreatic tumors such as pancreatic ductal adenocarcinoma (PDAC), solid pseudopapillary tumor (SPT), and pancreatic endocrine tumor (PET) may occasionally manifest as cystic lesions. In this study, we have put together our accumulated experience with cystic manifestations of various solid tumors of the pancreas. Methods From 2000 to 2006, 376 patients with pancreatic solid tumor resections were reviewed. Ten (2.66%) of these tumors appeared on radiological imaging studies as cystic lesions. We performed a retrospective review of medical records and pathologic findings of these 10 cases. Results Of the ten cases in which solid tumors of the pancreas manifested as cystic lesions, six were PDAC with cystic degeneration, two were SPT undergone complete cystic change, one was cystic PET, and one was a cystic schwannoma. The mean tumor size of the cystic portion in PDAC was 7.3 cm, and three patients were diagnosed as 'pseudocyst' with or without cancer. Two SPT were found incidentally in young women and were diagnosed as other cystic neoplasms. One cystic endocrine tumor was preoperatively suspected as intraductal papillary mucinous neoplasm or mucinous cystic neoplasm. Conclusions Cystic changes of pancreas solid tumors are extremely rare. However, the possibility of cystic manifestation of pancreas solid tumors should be kept in mind. PMID:21771323

  18. Trends in malignant intraductal papillary mucinous neoplasm in US adults from 1990 to 2010: a SEER database analysis

    PubMed Central

    McCarty, Thomas R.; Njei, Basile

    2016-01-01

    Background: Intraductal papillary mucinous neoplasms (IPMNs) are precancerous lesions with a well-described adenoma-carcinoma sequence. Although the risk of malignant transformation has been well studied, data on trends in long-term survival and important prognostic factors associated with survival in malignant IPMN are lacking. Methods: The Surveillance, Epidemiology, and End Results (SEER) database was queried to identify patients with confirmed malignant IPMN based upon pathologic diagnosis or radiographic evidence concerning for malignant potential. Median survival and age-adjusted incidence were calculated. Cox proportional hazard regression was used to determine independent mortality factors. Results: Based upon the SEER database query, 2651 patients were diagnosed with malignant IPMN between 1990 and 2010. The age-adjusted incidence of IPMN in 1990 was 0.361 per 100 000 persons (95% confidence interval [CI]: 0.285–0.451) with a steady decline observed through 2010 (0.135 per 100 000 persons, 95% CI: 0.098–0.186). A total of 564 patients (21.3%) underwent a surgical procedure, though the number of patients who underwent surgery from 1990 to 2010 also decreased (1990–1995, n = 132 to 2006–2010, n = 96, respectively). The overall median survival was 4 months and remained relatively stable from 1990 to 2010. Performance of surgery (HR: 0.45, 95% CI: 0.40–0.53, P < 0.001) was associated with a decreased risk of death. Conclusion: A significant decrease in the incidence of malignant IPMN was seen from 1990 to 2010. There was also no improvement observed in long-term survival. The small percentage of eligible cases receiving surgical treatment suggests that there is room for further improvement in survival, with increased utilization of surgery. PMID:26818977

  19. Stromal Fibrosis and Expression of Matricellular Proteins Correlate With Histological Grade of Intraductal Papillary Mucinous Neoplasm of the Pancreas

    PubMed Central

    Kakizaki, Yasuharu; Makino, Naohiko; Tozawa, Tomohiro; Honda, Teiichiro; Matsuda, Akiko; Ikeda, Yushi; Ito, Miho; Saito, Yoshihiko; Kimura, Wataru; Ueno, Yoshiyuki

    2016-01-01

    Objective The aim of the study was to clarify the correlation between the microenvironmental factors and histological grade in intraductal papillary mucinous neoplasm (IPMN). Methods We investigated 65 IPMNs resected at Yamagata University Hospital between 2000 and 2011, and all cases were categorized to low-inter (including low- and intermediate-grade dysplasia) and high-inv (including high-grade dysplasia and IPMN with an associated invasive carcinoma) groups. We compared between the 2 groups pathologically with regard to fibrosis and the expression of alpha-smooth muscle actin (α-SMA), periostin, and galectin-1 in the periductal stroma of IPMN. Results There were 41 low-inter and 24 high-inv. The subtype was categorized as 22 main duct type (MD-IPMN) and 43 branch duct type (BD-IPMN). The degree of fibrosis and the expression of α-SMA, periostin, and galectin-1 were significantly higher in high-inv than in low-inter within BD-IPMNs. Multivariate logistic regression analysis indicated that high expression of α-SMA (odds ratio, 13.802; 95% confidence interval, 1.108–171.893; P = 0.0414) was a significant independent related factor of high-inv in BD-IPMN. Conclusions Stromal fibrosis and expression of α-SMA, periostin, and galectin-1 are more marked in high-inv than in low-inter within BD-IPMNs, and they could become new markers for determining the indications for surgery in BD-IPMN. PMID:26967452

  20. Determining the natural history of pancreatic cystic neoplasms: a Manitoban cohort study

    PubMed Central

    Broughton, Jon; Lipschitz, Jeremy; Cantor, Michael; Moffatt, Dana; Abdoh, Ahmed; McKay, Andrew

    2016-01-01

    Background Most pancreatic cystic neoplasms (PCN) are thought to harbor a low malignant potential. This historical cohort study attempts to describe the natural history of these lesions in a provincial cohort, to assess the safety of non-surgical management. Pathological diagnosis of malignancy was the primary outcome measure of interest. Methods All adult patients (age 18+) with PCN seen between 2000 and 2012 by the two main institutions in Manitoba were included in this study. PCN were graded as high and low risk, which dictated initial treatment plan (surgery or observation). Predictors of initial surgical treatment, delayed surgery in the observation group and the clinical/radiological predictors of malignancy were determined. Results 497 patients were included in this study. 43 (8.7%) high-risk lesions underwent initial surgery, with 13 (30.2%) cases of malignancy discovered. 450 (90.5%) low-risk cysts were observed for a median of 17.3 months (range: 0.00–142.3). 29 (6.4%) cases of delayed surgery occurred, with malignancy discovered in five (17.2%). Conclusions This study supports current selection criteria for management of PCNs. Due to the low incidence of malignancy in low-risk PCN, it appears that long-term observation is safe and should be the treatment modality of choice in the absence of high-risk features. PMID:27037209

  1. Malignant Transformation of a Mature Cystic Ovarian Teratoma into Thyroid Carcinoma, Mucinous Adenocarcinoma, and Strumal Carcinoid: A Case Report and Literature Review

    PubMed Central

    Hinshaw, Hilary D.; Smith, Ashlee L.; Desouki, Mohamed Mokhtar; Olawaiye, Alexander B.

    2012-01-01

    Malignant transformation of a mature cystic teratoma (MCT) is an infrequent, often asymptomatic event. We report the first example of a struma ovarii with a focus of follicular variant of papillary thyroid carcinoma (a), mucinous adenocarcinoma (b), and strumal carcinoid tumor (c)—all three arising in one mature cystic teratoma of the ovary. From our reviews, we found limited data to guide management when these malignant foci occur within an MCT. Consideration should be given to thyroidectomy followed by total-body scanning and serum studies for foci of thyroid carcinoma and adjuvant therapy with thyroidectomy and radioablation if residual disease is identified (a). Additionally, extrapolating from data for mucinous adenocarcinomas, consideration could be given to adjuvant chemotherapy after appropriate staging (b). Strumal carcinoid tumors should be treated as tumors of low malignant potential. Observation is appropriate if after complete staging, no invasive implants are noted (c). PMID:23029627

  2. MRI, enhanced CT, and FDG PET/CT in primary retroperitoneal mucinous cystadenocarcinoma.

    PubMed

    Dong, Aisheng; Zhai, Zhijun; Wang, Yang; Zuo, Changjing

    2015-01-01

    Primary retroperitoneal mucinous cystadenocarcinoma (PRMC) is an extremely rare neoplasm. We present a case of PRMC with MRI, enhanced CT, and FDG PET/CT findings. Abdominal MRI showed a cystic lesion in the retroperitoneum with a mural nodule. The mural nodule showed progressive enhancement on enhanced CT and intense FDG uptake on early PET/CT with increased SUVmax on delayed PET/CT. Laparoscopy was performed. Retroperitoneal mucinous cystadenocarcinoma was confirmed histopathologically. Metastasis from gastrointestinal tract or ovary was excluded. This case indicates, although rare, PRMC should be considered when a hypermetabolic retroperitoneal cystic lesion with bilateral normal ovaries is found on FDG PET/CT. PMID:24445275

  3. Magnetic resonance imaging in the detection of pancreatic neoplasms.

    PubMed

    Zhong, Liang

    2007-08-01

    Recently, with the rapid scanning time and improved image quality, outstanding advances in magnetic resonance (MR) methods have resulted in an increase in the use of MRI for patients with a variety of pancreatic neoplasms. MR multi-imaging protocol, which includes MR cross-sectional imaging, MR cholangiopancreatography and dynamic contrast-enhanced MR angiography, integrates the advantages of various special imaging techniques. The non-invasive all-in-one MR multi-imaging techniques may provide the comprehensive information needed for the preoperative diagnosis and evaluation of pancreatic neoplasms. Pancreatic neoplasms include primary tumors and pancreatic metastases. Primary tumors of the pancreas may be mainly classified as ductal adenocarcinomas, cystic tumors and islet cell tumors (ICT). Pancreatic adenocarcinomas can be diagnosed in a MRI study depending on direct evidence or both direct and indirect evidence. The combined MRI features of a focal pancreatic mass, pancreatic duct dilatation and parenchymal atrophy are highly suggestive of a ductal adenocarcinoma. Most cystic neoplasms of the pancreas are either microcystic adenomas or mucinous cystic neoplasms. Intraductal papillary mucinous tumors are the uncommon low-grade malignancy of the pancreatic duct. ICT are rare neoplasms arising from neuroendocrine cells in the pancreas or the periampullary region. ICT are classified as functioning and non-functioning. The most frequent tumors to metastasize to the pancreas are cancers of the breast, lung, kidney and melanoma. The majority of metastases present as large solitary masses with well-defined margins. PMID:17650223

  4. Mucinous adenocarcinoma of the renal pelvis with pseudomyxoma peritonei.

    PubMed

    Shah, Vinaya B; Amonkar, Gayathri P; Deshpande, Jaya R; Bhalekar, Hemant

    2008-01-01

    Mucinous adenocarcinoma of the renal pelvis is an extremely rare tumor with very few case reports in literature. Pseudomyxoma peritonei is an uncommon condition characterized by the presence of mucinous gelatinous material in the peritoneal cavity. It occurs secondary to primary mucinous neoplasms of particularly the appendix and the ovary. We present a case of a 35-year-old female who had a history of dull aching pain in the right flank since one and a half years. Upon ultrasonography (USG) and computerized tomography (CT) scan, there was a large cystic mass measuring 15x15x12 cm, extending into the right lumbar region. Grossly, the entire kidney was converted into a cystic mass measuring 15x15x12 cm containing gelatinous mucinous material weighing 1 kg. Histologically, the tumor was composed of simple and complex glandular acini together with a superficial resemblance to colonic mucosa with abundant extracellular mucin. Thus, a diagnosis of mucinous adenocarcinoma of the renal pelvis leading to pseudomyxoma peritonei was made. PMID:19008588

  5. Difficulty in management of intraductal papillary mucinous neoplasm-associated pancreatobiliary fistulas and the role of “pig-nose” appearance and intraductal ultrasonography in diagnosis

    PubMed Central

    Koizumi, Mitsuhito; Kumagi, Teru; Kuroda, Taira; Azemoto, Nobuaki; Yamanishi, Hirofumi; Ohno, Yoshinori; Yokota, Tomoyuki; Ochi, Hironori; Tange, Kazuhiro; Ikeda, Yoshiou; Hiasa, Yoichi

    2016-01-01

    Pancreatobiliary fistulas associated with intraductal papillary mucinous neoplasms (IPMN) often develop obstructive jaundice and cholangitis; thus, early diagnosis is important. However, computed tomography and cholangiography, the current methods for detecting pancreatobiliary fistulas, are not always effective. We previously reported a case of IPMN-associated pancreatobiliary fistula and proposed a potential new diagnostic marker: the “pig-nose” appearance of the duodenal papilla, which results from dilated pancreatic and bile ducts and can be visualized via endoscopy. In this study, we report another three cases of IPMN-associated pancreatobiliary fistulas detected by a different technology, intraductal ultrasonography (IDUS). As with our previously reported case, we confirmed the utility of the “pig-nose” appearance and IDUS in the diagnosis of IPMN-associated pancreatobiliary fistulas. In addition, we found it difficult to manage biliary obstruction that resulted from the flow of mucinous material through pancreatobiliary fistulas. The obstruction was treated with endoscopic nasal biliary drainage (ENBD), but this was not always successful. In two of our cases, additional treatment with a large diameter fully covered metal stent failed to improve jaundice. Therefore, we conclude that standard endoscopic stenting may not be effective, and that alternative endoscopic methods or surgery may be necessary. PMID:27092326

  6. Difficulty in management of intraductal papillary mucinous neoplasm-associated pancreatobiliary fistulas and the role of "pig-nose" appearance and intraductal ultrasonography in diagnosis.

    PubMed

    Koizumi, Mitsuhito; Kumagi, Teru; Kuroda, Taira; Azemoto, Nobuaki; Yamanishi, Hirofumi; Ohno, Yoshinori; Yokota, Tomoyuki; Ochi, Hironori; Tange, Kazuhiro; Ikeda, Yoshiou; Hiasa, Yoichi

    2016-04-01

    Pancreatobiliary fistulas associated with intraductal papillary mucinous neoplasms (IPMN) often develop obstructive jaundice and cholangitis; thus, early diagnosis is important. However, computed tomography and cholangiography, the current methods for detecting pancreatobiliary fistulas, are not always effective. We previously reported a case of IPMN-associated pancreatobiliary fistula and proposed a potential new diagnostic marker: the "pig-nose" appearance of the duodenal papilla, which results from dilated pancreatic and bile ducts and can be visualized via endoscopy. In this study, we report another three cases of IPMN-associated pancreatobiliary fistulas detected by a different technology, intraductal ultrasonography (IDUS). As with our previously reported case, we confirmed the utility of the "pig-nose" appearance and IDUS in the diagnosis of IPMN-associated pancreatobiliary fistulas. In addition, we found it difficult to manage biliary obstruction that resulted from the flow of mucinous material through pancreatobiliary fistulas. The obstruction was treated with endoscopic nasal biliary drainage (ENBD), but this was not always successful. In two of our cases, additional treatment with a large diameter fully covered metal stent failed to improve jaundice. Therefore, we conclude that standard endoscopic stenting may not be effective, and that alternative endoscopic methods or surgery may be necessary. PMID:27092326

  7. Advanced Mucinous Adenocarcinoma Arising from a Mature Cystic Teratoma: A Case Report and Literature Review

    PubMed Central

    Miyasaka, Aki; Nishikawa, Tadaaki; Kozawa, Eito; Yasuda, Masanori; Fujiwara, Keiichi; Hasegawa, Kosei

    2016-01-01

    Purpose To describe the postoperative progressive course of advanced-stage adenocarcinoma arising from a mature cystic teratoma (MCT) and review the literature regarding this disease. Methods A 59-year-old woman visited our hospital with an abdominal mass. Laparotomy showed enlargement of the left ovary and dissemination throughout the abdominal and pelvic cavities. The diagnosis was FIGO stage IIIB adenocarcinoma arising from a MCT. We report this case in detail with a review of the literature. Results A literature search yielded 9 cases of stage III adenocarcinoma with malignant transformation. Six of these 9 patients died within 12 months after diagnosis. Of the 8 patients who underwent postoperative chemotherapy, 3 survived for over 39 months. The review indicates that prognosis of adenocarcinoma is as poor as that for squamous cell carcinoma arising from a MCT. Conclusions In general, as with this case, prognosis of advanced adenocarcinoma associated with a MCT is poor. However, we should be aware that not all patients are resistant to chemotherapy. PMID:27462234

  8. Cystic tumor of the liver without ovarian-like stroma or bile duct communication: two case reports and a review of the literature.

    PubMed

    Kishida, Norihiro; Shinoda, Masahiro; Masugi, Yohei; Itano, Osamu; Fujii-Nishimura, Yoko; Ueno, Akihisa; Kitago, Minoru; Hibi, Taizo; Abe, Yuta; Yagi, Hiroshi; Tanimoto, Akihiro; Tanabe, Minoru; Sakamaoto, Michiie; Kitagawa, Yuko

    2014-01-01

    We report two cases of cystic neoplasm of the liver with mucinous epithelium in which both ovarian-like stroma and bile duct communication were absent. The first case was a 41-year-old woman. She underwent right trisegmentectomy due to a multilocular cystic lesion, 15 cm in diameter, with papillary nodular components in the medial segment and right lobe. Histologically, arborizing papillae were seen in the papillary lesion. The constituent neoplastic cells had sufficient cytoarchitectural atypia to be classified as high-grade dysplasia. The second case was a 60-year-old woman. She underwent left lobectomy due to a unilocular cystic lesion, 17 cm in diameter, in the left lobe. Histologically, the cyst wall was lined by low columnar epithelia with slight cellular atypia. In both cases, neither ovarian-like stroma nor bile duct communications were found throughout the resected specimen. According to the most recent World Health Organization (WHO) classification in 2010, cystic tumors of the liver with mucinous epithelium are classified as mucinous cystic neoplasms when ovarian-like stromata are found, and as intraductal papillary neoplasm of bile duct when bile duct communication exists. Therefore, we diagnosed the cystic tumors as 'biliary cystadenoma' according to the past WHO classification scheme from 2000. We believe that the combined absence of both ovarian-like stroma and bile duct communication is possible in mucinous cystic tumors of the liver. Herein, we have described the clinicopathologic features of the two cases and reviewed past cases in the literature. PMID:25047921

  9. [Cystic lymphangioma of the transverse mesocolon simulating neoplasm of the pancreatic tail].

    PubMed

    Zago, A; Tosi, D; Portuese, D; Rosa, G

    1997-01-01

    Mesenteric cysts are uncommon lesions interesting surgeons above all for frequently difficulties in the preoperatory diagnosis. The role of radiology is to demonstrate the nature of these abdominal masses, but only with surgery we can establish a definitive histologic diagnosis. The authors report a case of a lynphangioma of the transverso mesocolon, miming a pancreatic neoplasm. PMID:9235871

  10. Intraductal Oncocytic Papillary Neoplasms of the Pancreas.

    PubMed

    Kallen, Michael E; Naini, Bita V

    2016-09-01

    Intraductal oncocytic papillary neoplasms (IOPNs) are cystic neoplasms with intraductal growth and complex papillae composed of oncocytic cells. IOPNs have been reported both in the pancreas and biliary tree, and are most likely closely related in these 2 locations. In the pancreas, these rare tumors are now considered 1 of the 4 histologic subtypes of intraductal papillary mucinous neoplasm (IPMN). Significant differences in histology, immunophenotype, and molecular genetics have been reported between IOPNs and other IPMN subtypes. However, there are limited data regarding the clinical behavior and prognosis of IOPNs in comparison to other subtypes of IPMN. We review features of pancreatic IOPNs and discuss the differential diagnosis of other intraductal lesions in the pancreas. PMID:27575268

  11. A Primary Retroperitoneal Mucinous Tumor

    PubMed Central

    Heelan Gladden, Alicia A.; Wohlauer, Max; McManus, Martine C.; Gajdos, Csaba

    2015-01-01

    A twenty-five-year-old female presented with a large retroperitoneal mass. Workup included history and physical exam, imaging, biopsy, colonoscopy, and gynecologic exam. After surgical resection, the mass was determined to be a primary retroperitoneal mucinous tumor (PRMT). Clinically and histologically, these tumors are similar pancreatic and ovarian mucinous neoplasms. PRMTs are rare and few case reports have been published. PRMTs are divided into mucinous cystadenomas, mucinous borderline tumors of low malignant potential, and mucinous carcinoma. These tumors have malignant potential so resection is indicated and in some cases adjuvant chemotherapy and/or surveillance imaging. PMID:25874152

  12. Primary Retroperitoneal Mucinous Cystadenoma

    PubMed Central

    Han, Weon Cheol

    2016-01-01

    Mucinous cystadenomas and cystadenocarcinomas of the ovary are clinically and histopathologically well-established common tumors. However, primary retroperitoneal mucinous cystic tumors are extremely rare, and although their histopathogenesis is still uncertain, several theories have been proposed. Most authors suggest that they develop through mucinous metaplasia in a preexisting mesothelium-lined cyst. An accurate preoperative diagnosis of these tumors is difficult because no effective diagnostic measures have been established. Delay in diagnosis and treatment of this tumor may be fatal for the patient because of complications such as rupture, infection, and malignant transformation. We describe the case of a 31-year-old woman with abdominal pain and a palpable mass. Computed tomography of the abdomen revealed a retroperitoneal cystic mass, which was resected successfully through laparoscopy. Histopathological examination of the resected mass confirmed the diagnosis of a primary retroperitoneal mucinous cystadenoma. The patient was discharged on postoperative day 5 without any complications. PMID:26962534

  13. Intestinal mucins from normal subjects and patients with cystic fibrosis. Variable contents of the disulphide-bound 118 kDa glycoprotein and different reactivities with an anti-(118 kDa glycoprotein) antibody.

    PubMed Central

    Mantle, M; Stewart, G

    1989-01-01

    1. A specific antibody was developed against the disulphide-bound 118 kDa glycoprotein of human intestinal mucin and used to establish an e.l.i.s.a. Fourteen purified mucins [eight normal (N) and six cystic fibrosis (CF)] had the same affinity for the antibody in the e.l.i.s.a., but their relative immunoreactivities varied widely (approx. 100,000-fold). In general, CF mucins were more antigenic than N mucins. 2. Variations (approx. 10-fold) were detected in the 118 kDa glycoprotein content of both N and CF mucins (assessed from Coomassie Blue-stained polyacrylamide gels), but these did not appear to be responsible for the differences in mucin immunoreactivity. 3. Variations (approx. 6-fold) were also observed in the size of the 118 kDa peak produced by N and CF mucins on Western blots. These were mostly due to differences in the 118 kDa glycoprotein content of mucins, although a small proportion resulted from changes in the number of antigenic determinants within individual 118 kDa glycoproteins. 4. After concanavalin A affinity chromatography of four reduced mucins (two N and two CF), purified 118 kDa glycoprotein was recovered in the bound fractions from the column, specifically eluted by methyl alpha-mannoside. 5. The amounts of 118 kDa glycoprotein isolated from the four mucins varied as predicted from the size of their 118 kDa bands on Coomassie Blue-stained gels. 6. Three 118 kDa glycoproteins (one N and two CF) showed almost identical reactivity in the e.l.i.s.a.; the fourth had fewer antigenic determinants. 7. Since differences in 118 kDa glycoprotein content and in the number of antigenic determinants within the 118 kDa glycoprotein did not account for variations in the reactivity of native mucins in the e.l.i.s.a., it appeared that accessibility of the 118 kDa glycoprotein to antibody binding may be critical in determining mucin immunoreactivity. This suggests that the three-dimensional conformation of CF mucins may differ from that of N mucins, leading

  14. Eosinophilic, Solid, and Cystic Renal Cell Carcinoma: Clinicopathologic Study of 16 Unique, Sporadic Neoplasms Occurring in Women.

    PubMed

    Trpkov, Kiril; Hes, Ondrej; Bonert, Michael; Lopez, Jose I; Bonsib, Stephen M; Nesi, Gabriella; Comperat, Eva; Sibony, Mathilde; Berney, Daniel M; Martinek, Petr; Bulimbasic, Stela; Suster, Saul; Sangoi, Ankur; Yilmaz, Asli; Higgins, John P; Zhou, Ming; Gill, Anthony J; Przybycin, Christopher G; Magi-Galluzzi, Cristina; McKenney, Jesse K

    2016-01-01

    A unique renal neoplasm characterized by eosinophilic cytoplasm and solid and cystic growth was recently reported in patients with tuberous sclerosis complex (TSC). We searched multiple institutional archives and consult files in an attempt to identify a sporadic counterpart. We identified 16 morphologically identical cases, all in women, without clinical features of TSC. The median age was 57 years (range, 31 to 75 y). Macroscopically, tumors were tan and had a solid and macrocystic (12) or only solid appearance (4). Average tumor size was 50 mm (median, 38.5 mm; range, 15 to 135 mm). Microscopically, the tumors showed solid areas admixed with variably sized macrocysts and microcysts that were lined by cells with a pronounced hobnail arrangement. The cells had voluminous eosinophilic cytoplasm with prominent granular cytoplasmic stippling and round to oval nuclei with prominent nucleoli. Scattered histiocytes and lymphocytes were invariably present. Thirteen of 16 patients were stage pT1; 2 were pT2, and 1 was pT3a. The cells demonstrated a distinct immunoprofile: nuclear PAX8 expression, predominant CK20-positive/CK7-negative phenotype, patchy AMACR staining, but no CD117 reactivity. Thirteen of 14 patients with follow-up were alive and without disease progression after 2 to 138 months (mean: 53 mo; median: 37.5 mo); 1 patient died of other causes. Although similar to a subset of renal cell carcinomas (RCCs) seen in TSC, we propose that sporadic "eosinophilic, solid, and cystic RCC," which occurs predominantly in female individuals and is characterized by distinct morphologic features, predominant CK20-positive/CK7-negative immunophenotype, and indolent behavior, represents a novel subtype of RCC. PMID:26414221

  15. Accuracy of 2012 International Consensus Guidelines for the prediction of malignancy of branch-duct intraductal papillary mucinous neoplasms of the pancreas

    PubMed Central

    Robles, Enrique Pérez-Cuadrado; Cros, Jérôme; Vullierme, Marie-Pierre; Rebours, Vinciane; Sauvanet, Alain; Aubert, Alain; Dokmak, Safi; Lévy, Philippe; Ruszniewski, Philippe

    2016-01-01

    Objective To determine accuracy of 2012 International Consensus Guidelines (ICG) predicting malignancy in a surgical cohort of branch-duct intraductal papillary mucinous neoplasms (BD-IPMN). Methods This study included all consecutive patients with final pathological diagnosis of pure BD-IPMN resected between 2006 and 2014 at Beaujon Hospital. Neoplasms were classified as malignant in presence of high-grade dysplasia (HGD) or invasive carcinoma. Medical, pathological, and radiological data were retrospectively recorded. Results One hundred and twenty patients (65 males, mean age: 57.9 ± 10.8 years) were included. Malignant BD-IPMN accounted for 30% (HGD: 18%, invasive: 12%). Thickened cyst walls (odds ratio (OR): 3.058, 95% confidence interval (CI 95%): 1.102–8.484, p = 0.032), main duct diameter 5–9 mm (OR: 3.395, CI 95%: 1.349–8.543, p = 0.007), and mural nodule (OR: 3.802, CI 95%: 1.156–12.511, p = 0.028) were independently associated with malignancy in multivariate analysis. Among the 89 patients (74%) who underwent surgical resection with ICG criteria, the malignancy rate was 38%, compared with 6% in the 31 ICG-negative group. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy for malignancy of having at least one ICG criteria were 94%, 34%, 38%, 94%, and 53%, respectively. Patients with malignant tumors had more ICG criteria than those with benign lesions (2.06 ± 0.98 vs. 0.99 ± 0.95, p < 0.001). Conclusions 2012 ICG criteria are useful to manage BD-IPMN permitting not to miss a malignant form (NPV of 94%), but frequently point out unnecessary surgery (PPV of 38%). Malignancy rate increases with the number of ICG criteria. In patients with only one criterion, additional criteria would be necessary.

  16. Plexiform Angiomyxoid Myofibroblastic Tumor (PAMT) of Stomach with Synchronous Bilateral Cystic Ovarian Neoplasms, a Rare Case Presentation.

    PubMed

    Dixit, Jagannath Dattatreya; Sharief, Shiraz Ahamed; Goyal, Manish Kumar; Khan, Sameeha; Kauser, Lubna

    2016-03-01

    Plexiform Angiomyxoid Myofibroblastic Tumor (PAMT) is a recently identified mesenchymal tumor of the stomach, which was first described in the year 2007 and was added in the 2010 WHO classification of tumors of the digestive system World J Gastroenterol 16(6): 2835-2840, 2010. It closely resembles with other gastric tumors but distinctly varies in clinical management as well as the histopathology. We had a 51 year, female patient, laborer by profession with low socio economic status, who had abdominal pain with vomiting since 6 months. She had similar complaints 3 years ago for which she was evaluated and presumed to have Carcinoma Stomach and underwent laparotomy which ended up only with Gastro- Jejunal anastomosis. She was admitted at our institution. Endoscopy revealed antral bulge with central area ulceration and biopsy was taken which was not confirmatory for malignancy. CT images showed heterogeneous mass with necrotic changes arising from the duodenum favored the diagnosis of perigastric neoplasm. PET CT was done, 8.4 × 5 × 6.1 cm exophytic mass in the pyloric region of stomach with solid and cystic components causing significant gastric outlet obstruction. She underwent exploratory laparotomy and complete excision of mass with achievement of R0 clearance. Histopathology was reported as Plexiform angiomyxoid myofibroblastic tumor (PAMT). PMID:27065688

  17. Intraductal papillary mucinous neoplasm (IPMN) with high-grade dysplasia is a risk factor for the subsequent development of pancreatic ductal adenocarcinoma☆

    PubMed Central

    Rezaee, Neda; Barbon, Carlotta; Zaki, Ahmed; He, Jin; Salman, Bulent; Hruban, Ralph H.; Cameron, John L.; Herman, Joseph M.; Ahuja, Nita; Lennon, Anne Marie; Weiss, Matthew J.; Wood, Laura D.; Wolfgang, Christopher L.

    2015-01-01

    Background Non-invasive intraductal papillary mucinous neoplasm (IPMN) with high-grade dysplasia and IPMN-associated invasive pancreatic ductal adenocarcinoma (PDAC) are frequently included under the term “malignancy”. The goal of this study is to clarify the difference between these two entities. Methods From 1996 to 2013, data of 616 patients who underwent pancreatic resection for an IPMN were reviewed. Results The median overall survival for patients with IPMN with high-grade dysplasia (92 months) was similar to survival for patients with IPMN with low/intermediate-grade dysplasia (118 months, p = 0.081), and superior to that of patients with IPMN-associated PDAC (29 months, p < 0.001). IPMN-associated PDAC had lymph node metastasis in 53%, perineural invasion in 58%, and vascular invasion in 33%. In contrast, no lymph node metastasis, perineural or vascular invasion was observed with high-grade dysplasia. None of the patients with IPMN with high-grade dysplasia developed recurrence outside the remnant pancreas. In stark contrast 58% of patients with IPMN-associated PDAC recurred outside the remnant pancreas. The rate of progression within the remnant pancreas was significant in patients with IPMN with high-grade (24%) and with low/intermediate dysplasia (22%, p = 0.816). Conclusion Non-invasive IPMN with high-grade dysplasia should not be considered a malignant entity. Compared to patients with IPMN with low/intermediate-grade dysplasia, those with high-grade dysplasia have an increased risk of subsequent development of PDAC in the remnant pancreas. PMID:27017163

  18. Deep Sequencing of Cancer-Related Genes Revealed GNAS Mutations to Be Associated with Intraductal Papillary Mucinous Neoplasms and Its Main Pancreatic Duct Dilation

    PubMed Central

    Takano, Shinichi; Fukasawa, Mitsuharu; Maekawa, Shinya; Kadokura, Makoto; Miura, Mika; Shindo, Hiroko; Takahashi, Ei; Sato, Tadashi; Enomoto, Nobuyuki

    2014-01-01

    Background To clarify the genetic mutations associated with intraductal papillary mucinous neoplasms (IPMN) and IPMN-related pancreatic tumours, we conducted cancer-related gene profiling analyses using pure pancreatic juice and resected pancreatic tissues. Methods Pure pancreatic juice was collected from 152 patients [nine with a normal pancreas, 22 with chronic pancreatitis (CP), 39 with pancreatic ductal adenocarcinoma (PDAC), and 82 with IPMN], and resected tissues from the pancreas were collected from 48 patients (six IPMNs and 42 PDACs). The extracted DNA was amplified by multiplexed polymerase chain reaction (PCR) targeting 46 cancer-related genes containing 739 mutational hotspots. The mutations were analysed using a semiconductor-based DNA sequencer. Results Among the 46 cancer-related genes, KRAS and GNAS mutations were most frequently detected in both PDAC and IPMN cases. In pure pancreatic juice, GNAS mutations were detected in 7.7% of PDAC cases and 41.5% of IPMN cases (p<0.001 vs. others). All PDAC cases with GNAS mutations (n = 3) were accompanied by IPMN. Multivariate analysis revealed that GNAS mutations in IPMN cases were associated with dilated main pancreatic ducts (MPD, p = 0.016), while no statistically independent associations with clinical variables were observed for KRAS mutations. In the resected pancreatic tissues, GNAS mutations were detected in 50% of PDAC cases concomitant with IPMN, 33.3% of PDAC cases derived from IPMN, and 66.7% of IPMN cases, while no GNAS mutations were detected in cases of PDAC without IPMN. Conclusions The GNAS mutation was specifically found in the cases with IPMN and it was speculated that some PDACs might be influenced by the concomitant but separately-located IPMN in their pathogenic mechanism. Furthermore, the GNAS mutation was significantly associated with MPD dilatation in IPMN cases, suggesting its role in mucus hypersecretion. PMID:24897499

  19. Mucin overproduction in chronic inflammatory lung disease

    PubMed Central

    Hauber, Hans-Peter; Foley, Susan C; Hamid, Qutayba

    2006-01-01

    Mucus overproduction and hypersecretion are commonly observed in chronic inflammatory lung disease. Mucins are gel-forming glycoproteins that can be stimulated by a variety of mediators. The present review addresses the mechanisms involved in the upregulation of secreted mucins. Mucin induction by neutrophil elastase, bacteria, cytokines, growth factors, smoke and cystic fibrosis transmembrane conductance regulator malfunction are also discussed. PMID:16983448

  20. Mucinous adenocarcinoma of the intestinal type arising from mature cystic teratoma of the ovary: a rare case report and review of the literature

    PubMed Central

    2012-01-01

    Background Mature cystic teratomas (MCTs) are the most common germ cell tumors of the ovary. Malignant tranformation occurs in 1-2% of these neoplasms. Although most of the malignancies arising from MCTs are squamous cell carcinomas, adenocarcinoma of the gastrointestinal type is extremery rare. We herein present a case of adenocarcinoma of the intestinal type arising from a MCT. Case A 49-year-old female underwent surgery for a left ovarian tumor. The histology of the cyst walls revealed a MCT with a few hair shafts and a squamous layer, while another part of the tumor showed adenocarcinoma of the intestinal type. Five years after surgery, she is alive without disease. PMID:23216975

  1. Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype

    PubMed Central

    Gustafsson, Jenny K.; Ermund, Anna; Ambort, Daniel; Johansson, Malin E.V.; Nilsson, Harriet E.; Thorell, Kaisa; Hebert, Hans; Sjövall, Henrik

    2012-01-01

    Cystic fibrosis (CF) is caused by a nonfunctional chloride and bicarbonate ion channel (CF transmembrane regulator [CFTR]), but the link to the phenomenon of stagnant mucus is not well understood. Mice lacking functional CFTR (CftrΔ508) have no lung phenotype but show similar ileal problems to humans. We show that the ileal mucosa in CF have a mucus that adhered to the epithelium, was denser, and was less penetrable than that of wild-type mice. The properties of the ileal mucus of CF mice were normalized by secretion into a high concentration sodium bicarbonate buffer (∼100 mM). In addition, bicarbonate added to already formed CF mucus almost completely restored the mucus properties. This knowledge may provide novel therapeutic options for CF. PMID:22711878

  2. Structure and interactions of human respiratory mucin

    NASA Astrophysics Data System (ADS)

    Purdy, Kirstin; Sheehan, John; Rubinstein, Michael; Wong, Gerard

    2006-03-01

    Human respiratory mucin plays a crucial role in the pathology of Cystic Fibrosis lung infections. Mucin is a flexible, linear polyelectrolyte, characterized by its many charged oligo-carbohydrate side chains that give it its bottle-brush structure. The macroscopic properties of a mucin suspension are known to change drastically with changes in ion concentration and solution pH, but little is known about the effect of these variables on individual mucin structure. We present preliminary results on the structural response of individual human respiratory mucin molecules to variations in concentration of ions of different valences via small angle x-ray diffraction.

  3. Giant cell tumor of the pancreas arising in the ovarian-like stroma of a mucinous cystadenocarcinoma.

    PubMed

    Bergman, S; Medeiros, L J; Radr, T; Mangham, D C; Lewandrowski, K B

    1995-08-01

    We describe a malignant mucinous cystic neoplasm of the pancreas with ovarian-like stroma within which an osteoclast-like giant cell rich tumor arose. This rare tumor had a unique immunohistochemical profile with the giant cells staining for vimentin, leukocyte common antigen, and the monocyte/macrophage marker CD68, whereas the mucinous epithelium stained for epithelial membrane antigen and cytokeratin. The immunohistochemical findings are consistent with two lines of differentiation, one epithelial and the other suggesting mesenchymal differentiation of the giant cell tumor with an immunophenotype similar to giant cell tumor of bone. The coexistence of these two rare tumors suggests that they are histogenetically related. The findings of a giant cell tumor arising in the ovarian stroma indicates that the stroma of mucinous tumors is not always an innocuous component of the tumor. PMID:7594774

  4. Low Rectal Cancer Study (MERCURY II)

    ClinicalTrials.gov

    2016-03-11

    Adenocarcinoma; Adenocarcinoma, Mucinous; Carcinoma; Neoplasms, Glandular and Epithelial; Neoplasms by Histologic Type; Neoplasms; Neoplasms, Cystic, Mucinous, and Serous; Colorectal Neoplasms; Intestinal Neoplasms; Gastrointestinal Neoplasms; Digestive System Neoplasms; Neoplasms by Site; Digestive System Diseases; Gastrointestinal Diseases; Intestinal Diseases; Rectal Diseases

  5. Molecular, Pathologic and MRI Investigation of the Prognostic and Redictive Importance of Extramural Venous Invasion in Rectal Cancer (MARVEL) Trial

    ClinicalTrials.gov

    2013-11-26

    Adenocarcinoma; Rectal Diseases; Colorectal Neoplasms; Adenocarcinoma, Mucinous; Carcinoma; Neoplasms, Glandular and Epithelial; Neoplasms by Histologic Type; Neoplasms; Neoplasms, Cystic, Mucinous, and Serous; Intestinal Neoplasms; Gastrointestinal Neoplasms; Digestive System Neoplasms; Neoplasms by Site; Digestive System Diseases; Gastrointestinal Diseases; Intestinal Diseases

  6. Updates in the Pathologic Diagnosis and Classification of Epithelial Neoplasms of Urachal Origin.

    PubMed

    Paner, Gladell P; Lopez-Beltran, Antonio; Sirohi, Deepika; Amin, Mahul B

    2016-03-01

    Since the publication of the World Health Organization "blue book" in 2004, several recent studies have provided new insights on the pathologic aspects of urachal neoplasms. The proposed updates include modified criteria for the diagnosis of urachal carcinoma. A uniform nomenclature for cystic tumors was lacking, and it is recommended that urachal mucinous cystic tumors should be separated and classified in a manner similar to ovarian mucinous neoplasms. The spectrum includes mucinous cystadenoma, mucinous cystic tumor of low malignant potential, mucinous cystic tumor of low malignant potential with intraepithelial carcinoma, and microscopically or frankly invasive mucinous cystadenocarcinoma, with 65% of cystic tumors classified as mucinous cystic tumor of low malignant potential. Most importantly, it has been shown that progression-free survival of noninvasive mucinous cystic tumors is significantly better than noncystic invasive adenocarcinoma. This development, along with prior descriptions of urachal villous adenoma, has also reaffirmed the occurrence of benign tumors of urachal epithelial origin. For noncystic (usual) invasive adenocarcinomas, the traditionally described histologic subtypes of enteric, mucinous, signet ring cell, not otherwise specified, and mixed remain appropriate, with 50% of tumors classified as mucinous subtype. Although this subtyping is helpful in diagnosis and differential diagnosis, the clinical significance of subtyping adenocarcinoma is still uncertain. Rare nonglandular morphologies such as urothelial, squamous, and neuroendocrine carcinoma in urachal carcinomas have been described in detail with proposals for their own set of diagnostic criteria. These criteria are based on unique features of urachal nonglandular carcinomas. Among the immunomarkers studied, only β-catenin and CK7 may be of help in the distinction of urachal from colorectal adenocarcinoma. Awareness of the expression profile of immunomarkers such as CDX2, P504S

  7. Primary salivary duct carcinoma of the lung, mucin-rich variant.

    PubMed

    Fishbein, Gregory A; Grimes, Brandon S; Xian, Rena R; Lee, Jay M; Barjaktarevic, Igor; Xu, Haodong

    2016-01-01

    Primary salivary gland-type lung cancer is a heterogeneous group of neoplasms arising from the seromucinous glands of the respiratory tract. Histopathologically, they are identical to salivary gland neoplasms of the head and neck. While mucoepidermoid carcinoma and adenoid cystic carcinoma are overwhelmingly the most common subtypes found in the lung, reports of uncommon subtypes can be found in the literature. We report a case of a 73-year-old woman with primary lung salivary duct carcinoma, mucin-rich variant--an exceedingly rare subtype of an already rare malignant salivary-type neoplasm. One case of primary lung salivary duct carcinoma has been reported in the literature; however, the mucin-rich variant has never been described in the lung. Furthermore, the tumor in our case bears a rare BRAF G464V mutation. To our knowledge, this is the first reported case of a BRAF G464V mutation detected in a salivary duct carcinoma or any other salivary-type neoplasm. PMID:26527521

  8. The value of KRAS mutation testing with CEA for the diagnosis of pancreatic mucinous cysts

    PubMed Central

    Kadayifci, Abdurrahman; Al-Haddad, Mohammad; Atar, Mustafa; Dewitt, John M.; Forcione, David G.; Sherman, Stuart; Casey, Brenna W.; Fernandez-del Castillo, Carlos; Schmidt, C. Max; Pitman, Martha B.; Brugge, William R.

    2016-01-01

    Background and aims: Pancreatic cyst fluid (PCF) CEA has been shown to be the most accurate preoperative test for detection of cystic mucinous neoplasms (CMNs). This study aimed to assess the added value of PCF KRAS mutational analysis to CEA for diagnosis of CMNs. Patients and methods: This is a retrospective study of prospectively collected endoscopic ultrasonography (EUS) fine-needle aspiration (FNA) data. KRAS mutation was determined by direct sequencing or equivalent methods. Cysts were classified histologically (surgical cohort) or by clinical (EUS or FNA) findings (clinical cohort). Performance characteristics of KRAS, CEA and their combination for detection of a cystic mucinous neoplasm (CMN) and malignancy were calculated. Results: The study cohort consisted of 943 patients: 147 in the surgical cohort and 796 in the clinical cohort. Overall, KRAS and CEA each had high specificity (100 % and 93.2 %), but low sensitivity (48.3 % and 56.3 %) for the diagnosis of a CMN. The positivity of KRAS or CEA increased the diagnostic accuracy (80.8 %) and AUC (0.84) significantly compared to KRAS (65.3 % and 0.74) or CEA (65.8 % and 0.74) alone, but only in the clinical cohort (P < 0.0001 for both). KRAS mutation was significantly more frequent in malignant CMNs compared to histologically confirmed non-malignant CMNs (73 % vs. 37 %, P = 0.001). The negative predictive value of KRAS mutation was 77.6 % in differentiating non-malignant cysts. Conclusions: The detection of a KRAS mutation in PCF is a highly specific test for mucinous cysts. It outperforms CEA for sensitivity in mucinous cyst diagnosis, but the data does not support its routine use. PMID:27092317

  9. Mucinous Balls Tangled With Mesothelial Cells and MUC2-Positive Cancer Cells in the Ascites of Pseudomyxoma Peritonei.

    PubMed

    Akashi, Shizuka; Kuwabara, Hiroko; Yasuda, Emi; Akutagawa, Hiroshi; Takeshita, Atsushi; Kurisu, Yoshitaka; Egashira, Yutaro; Hirose, Yoshinobu; Yuki, Masako; Tsuda, Yasuhiro; Hayashi, Michihiro

    2016-07-01

    Pseudomyxoma peritonei (PMP) is characterized by extensive mucinous ascites following rupture of mucinous neoplasms of an intra-abdominal origin, and contain secreted gel-forming mucins such as MUC2 and MUC5AC. We encountered a 66-year-old Japanese man complaining of abdominal distension. Ascites at the site was gelatinous upon gross examination, and needle aspirate smears showed histiocytes and many mucinous balls wrapped in spindle cells, which were positive for vimentin, pan-cytokeratin, and podoplanin. The cell block showed several adenocarcinoma clusters, which were positive for MUC2, MUC5AC, CK20, and CDX-2, and negative for CK7. From these findings, a diagnosis of PMP arising from colon cancer was indicated. Cytoreductive surgery was performed, and the cystic diverticulum was found to be infiltrated by tumor cells in the sigmoid colon that caused PMP. Mucinous balls surrounded by mesothelial cells and MUC2-positive adenocarcinoma cells are useful clues in the diagnosis of PMP. Diagn. Cytopathol. 2016;44:628-631. © 2016 Wiley Periodicals, Inc. PMID:27079739

  10. Salivary mucins in host defense and disease prevention.

    PubMed

    Frenkel, Erica Shapiro; Ribbeck, Katharina

    2015-01-01

    Mucus forms a protective coating on wet epithelial surfaces throughout the body that houses the microbiota and plays a key role in host defense. Mucins, the primary structural components of mucus that creates its viscoelastic properties, are critical components of the gel layer that protect against invading pathogens. Altered mucin production has been implicated in diseases such as ulcerative colitis, asthma, and cystic fibrosis, which highlights the importance of mucins in maintaining homeostasis. Different types of mucins exist throughout the body in various locations such as the gastrointestinal tract, lungs, and female genital tract, but this review will focus on mucins in the oral cavity. Salivary mucin structure, localization within the oral cavity, and defense mechanisms will be discussed. These concepts will then be applied to present what is known about the protective function of mucins in oral diseases such as HIV/AIDS, oral candidiasis, and dental caries. PMID:26701274

  11. Salivary mucins in host defense and disease prevention

    PubMed Central

    Frenkel, Erica Shapiro; Ribbeck, Katharina

    2015-01-01

    Mucus forms a protective coating on wet epithelial surfaces throughout the body that houses the microbiota and plays a key role in host defense. Mucins, the primary structural components of mucus that creates its viscoelastic properties, are critical components of the gel layer that protect against invading pathogens. Altered mucin production has been implicated in diseases such as ulcerative colitis, asthma, and cystic fibrosis, which highlights the importance of mucins in maintaining homeostasis. Different types of mucins exist throughout the body in various locations such as the gastrointestinal tract, lungs, and female genital tract, but this review will focus on mucins in the oral cavity. Salivary mucin structure, localization within the oral cavity, and defense mechanisms will be discussed. These concepts will then be applied to present what is known about the protective function of mucins in oral diseases such as HIV/AIDS, oral candidiasis, and dental caries. PMID:26701274

  12. Renal cystic disease

    SciTech Connect

    Hartman, D.S.

    1988-01-01

    The book begins with an overview of renal cystic disease and a presentation of simple renal cysts. Subsequent chapters cover cystic disease in association with renal neoplasms and medullary sponge kidney. The chapters addressing autosomal-dominant and autosomal-recessive polycystic kidney disease discuss and differentiate the infantile and adult forms of the disease. There are also separate discussions of medullary cystic disease, multicystic dysplastic kidney, and cysts of the renarenal sinus.

  13. Cross-species immunoreactivity of airway mucin as revealed by monoclonal antibodies directed against mucins from human, hamster, and rat.

    PubMed

    Shin, C Y; Lee, W J; Kim, D J; Park, C S; Choi, E Y; Ko, K H

    2000-10-01

    Airway mucin plays crucial role in host-defense and has been implicated in pathophysiology of various airway diseases including asthma and cystic fibrosis. The analysis of airway mucin has been hampered mostly by the lack of specific and efficient methods for the detection of mucin. Recent production of antibodies against airway mucin from several species and also the development of immunoassay procedures make it more efficient to study the airway mucin. However, the cross-species immunoreactivity of antibodies against airway mucin has not been clearly demonstrated and this prompted us to investigate the cross-species immunoreactivity of monoclonal antibodies against human (HM02), hamster (HTA), and rat airway mucin (RT03), which is three most widely used species in the study of mucin. All the monoclonal antibodies (MAbs) used in this study is IgM isotype and recognizes N-acetyl-galactosamine-linked carbohydrate core or backbone portion of airway mucin. In enzyme-linked immunoadsorbent assay (ELISA), Western blot, immunoprecipitation, and immunohistochemical staining experiments, it was demonstrated that human and hamster airway mucin showed strong cross-species immunoreactivity. However, rat airway mucin did not show any cross-species immunoreactivity against human and hamster airway mucin. Endotoxin-induced secretory cell metaplasia and hence the increase in mucin release from hamster airway mucin could be detected with antibodies against hamster and human airway mucin in vivo and in vitro. However, the same increase from rat airway could only be detected with antibody against rat airway mucin but not with antibodies against human and hamster airway mucin. In addition, the increase in mucin release from asthmatic patients could be detected with antibodies against human and hamster airway mucin but not with the antibody against rat airway mucin. The data from the present study implicates that the carbohydrate chain of human and hamster airway mucin, but not that

  14. 64-Slice spiral computed tomography and three-dimensional reconstruction in the diagnosis of cystic pancreatic tumors

    PubMed Central

    WEN, ZHAOXIA; YAO, FENGQING; WANG, YUXING

    2016-01-01

    The present study aimed to describe the characteristics of cystic pancreatic tumors using computed tomography (CT) and to evaluate the diagnostic accuracy (DA) of post-imaging three-dimensional (3D) reconstruction. Clinical and imaging data, including multi-slice spiral CT scans, enhanced scans and multi-faceted reconstruction, from 30 patients with pathologically confirmed cystic pancreatic tumors diagnosed at the Linyi People's Hospital between August 2008 and June 2014 were retrospectively analyzed. Following the injection of Ultravist® 300 contrast agent, arterial, portal venous and parenchymal phase scans were obtained at 28, 60 and 150 sec, respectively, and 3D reconstructions of the CT images were generated. The average age of the patients was 38.4 years (range, 16–77 years), and the cohort included 5 males and 25 females (ratio, 1:5). The patients included 8 cases of mucinous cystadenoma (DA), 80%]; 9 cases of cystadenocarcinoma (DA, 84%); 6 cases of serous cystadenoma (DA, 100%); 3 cases of solid pseudopapillary tumor (DA, 100%); and 4 cases of intraductal papillary mucinous neoplasm (DA, 100%). 3D reconstructions of CT images were generated and, in the 4 cases of intraductal papillary mucinous neoplasm, the tumor was connected to the main pancreatic duct and multiple mural nodules were detected in one of these cases. The DA of the 3D-reconstructed images of cystic pancreatic tumors was 89.3%. The 64-slice spiral CT and 3D-reconstructed CT images facilitated the visualization of cystic pancreatic tumor characteristics, in particular the connections between the tumor and the main pancreatic duct. In conclusion, the 3D reconstruction of multi-slice CT data may provide an important source of information for the surgical team, in combination with the available clinical data. PMID:27073473

  15. Regulation of Airway Mucin Gene Expression

    PubMed Central

    Thai, Philip; Loukoianov, Artem; Wachi, Shinichiro; Wu, Reen

    2015-01-01

    Mucins are important components that exert a variety of functions in cell-cell interaction, epidermal growth factor receptor signaling, and airways protection. In the conducting airways of the lungs, mucins are the major contributor to the viscoelastic property of mucous secretion, which is the major barrier to trapping inhaled microbial organism, particulates, and oxidative pollutants. The homeostasis of mucin production is an important feature in conducting airways for the maintenance of mucociliary function. Aberrant mucin secretion and accumulation in airway lumen are clinical hallmarks associated with various lung diseases, such as asthma, chronic obstructive pulmonary disease, cystic fibrosis, emphysema, and lung cancer. Among 20 known mucin genes identified, 11 of them have been verified at either the mRNA and/or protein level in airways. The regulation of mucin genes is complicated, as are the mediators and signaling pathways. This review summarizes the current view on the mediators, the signaling pathways, and the transcriptional units that are involved in the regulation of airway mucin gene expression. In addition, we also point out essential features of epigenetic mechanisms for the regulation of these genes. PMID:17961085

  16. Identification of Differentially Expressed miRNAs in Appendiceal Mucinous Cystadenocarcinoma from Mucinous Cystadenoma

    PubMed Central

    Wu, Richard Licheng; Ali, Shadan; Sarkar, Fazlul H; Beydoun, Rafic

    2016-01-01

    samples compared to the mucinous cystadenoma. These data suggest that the miRNA expression in mucinous appendiceal neoplasm may help to supplement the morphological evaluation in distinguishing benign from malignant tumors. PMID:26807210

  17. Assessment of Intracellular Mucin Content In Vivo

    PubMed Central

    Piccotti, Lucia; Dickey, Burton F.

    2013-01-01

    Airway mucus presents a first line of defense against inhaled materials. It also, however, is a significant pathological contributor to chronic lung diseases such as asthma, cystic fibrosis, and chronic obstructive pulmonary disease. Thus, gaining a better understanding of the mechanisms of mucus production and secretion is an important goal for improving respiratory health. Mucins, the chief glycoprotein components of airway mucus, are very large polymeric glycoproteins, and measuring their production and secretion in experimental animals present significant technical challenges. Over the past several years, we have developed assays for accurately quantifying mucin production and secretion using histological and biochemical assays. These methods are described here. PMID:22259143

  18. Epidural Cystic Spinal Meningioma

    PubMed Central

    Zhang, Ji; Chen, Zheng-he; Wang, Zi-feng; Sun, Peng; Jin, Jie-tian; Zhang, Xiang-heng; Zhao, Yi-ying; Wang, Jian; Mou, Yong-gao; Chen, Zhong-ping

    2016-01-01

    Abstract Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor. Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable. PMID:26986119

  19. Krukenberg carcinoma metastasized from stomach resembling mucinous cystadenocarcinoma of the ovary.

    PubMed

    Doğanay, Melike; Topçu, Hasan Onur; Kokanali, Mahmut Kuntay; Güzel, Ali Irfan; Oskovi, Asli; Akbay, Serap; Cavkaytar, Sabri

    2015-01-01

    The ovaries are common site of metastasis in a variety of primary neoplasms. Multiple tumors such as breast, lung, and pancreas have been reported to metastasize to the ovary, however; the colon and stomach are the most common primary cancer sites that of ovarian metastasis. An ovarian mass mostly originates from its self-tissue, but sometimes it can be a metastasis of a gastrointestinal system tumor. Such cases are often misdiagnosed as primary ovarian cancers. A 42-year-old woman was admitted to our hospital with pelvic pain. She had a history of her complaints for two months. Bilateral large ovarian mass was detected in transvaginal ultrasound. Laparotomy was performed, the pathologist suggested inspection of the stomach after the frozen section analysis; therefore, an irregular mass on the stomach was detected. The general surgeon was attended to the operation, and an inoperative stomach tumor was reported by the general surgeon. After that due to the partial obstruction of jejunum, a gastrojejunostomy was performed. It is in fact difficult to distinguish between metastatic mucinous carcinomas and primary mucinous carcinomas of the ovary, due to the similar appearance of as cystic tumors on gross examination. The clinicians should be aware of the likely concomitant gastrointestinal system tumor when a large and bilaterally mass was detected on physical examination. This case also reminds that a systemic examination is necessary even if the large ovarian tumors suspicious of primary malignancy were noticed. PMID:26259386

  20. Myelodysplastic/ Myeloproliferative Neoplasms Treatment

    MedlinePlus

    ... Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Myelodysplastic/ ...

  1. Chronic Myeloproliferative Neoplasms Treatment

    MedlinePlus

    ... Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Chronic Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Chronic ...

  2. Cystic Fibrosis

    MedlinePlus

    ... and Diseases > Lung Disease Lookup > Cystic Fibrosis Cystic Fibrosis Cystic Fibrosis (CF) is an inherited disease that ... quality of life has improved. Learn About Cystic Fibrosis Cystic fibrosis is a genetic (inherited) condition that ...

  3. Physical Properties of the Glycoprotein Mucin

    NASA Astrophysics Data System (ADS)

    Matthews, Garrett; Davis, William; Superfine, Richard; Boucher, Richard

    2003-03-01

    Epithelial cell surfaces are covered by a protective gel known as mucus. The physiological function of this gel depends on its rheological properties, and these properties are largely derived from the secreted glycoprotein mucin. The genetic disease Cystic Fibrosis (CF) is characterized by the adhesion of thick, viscous mucus on these tissues. In the lungs, this results in the interruption of mucus transport thus compromising the first line of defense against pathogens in these tissues. In order to restore the flow of tracheobronchial mucus out of the body, knowledge of the molecular and physical properties of mucin and mucin solutions would be greatly beneficial. The present model for these molecules is that of a long linear strand consisting of highly glycosylated regions linked by cystein-rich globular regions. It is thought that the globular regions may interact either through intermolecular disulfide bonds or through hydrophobic interactions. It has also been speculated that the glycosylated regions may have lectin-like interactions. In the present work, single mucin molecules were imaged at high resolution using atomic force microscopy (AFM). Phase mode imaging was used to map the interactions between functionalized AFM tips and the molecular topography. Additionally, using force-distance curves with the AFM, the adhesion between mucin bound tips and cell surface glycocalyx and glycocalyx-like model surfaces, was measured. And, finally, the viscoelastic properties of mucin solutions were measured using the recently developed technique, single particle tracking microrheology. A model is being developed that will incorporate the properties of mucins beginning at the single molecule and ending with the bulk viscoelastic properties.

  4. Periampullary Diverticula Misdiagnosed as Cystic Pancreatic Lesions: A Review of 3 Cases

    PubMed Central

    Ng, Chee Hui; Lee, Chau Hung

    2016-01-01

    Case series Patient: Female, 67 • Male, 69 • Female, 65 Final Diagnosis: Periampullary diverticulum Symptoms: — Medication: — Clinical Procedure: Magnetic Resonance Imaging Specialty: Radiology Objective: Diagnostic/therapeutic accidents Background: Cystic lesions on the pancreatic head can mimic fluid-filled duodenal or periampullary diverticula. We reviewed a series of cases in which periampullary diverticula were misdiagnosed as cystic pancreatic lesions. Case Report: Case 1. A Chinese woman presented to the surgical outpatient clinic for intermittent upper abdominal discomfort. Contrast-enhanced MRI of the abdomen revealed a cystic-appearing lesion in the region of the pancreatic head, which was reported as a cystic pancreatic lesion. A follow-up scan showed this lesion to be filled with fluid, gas, and debris, suggestive of a periampullary diverticulum. Review of a prior CT scan confirmed a periampullary diverticulum. Case 2. A Chinese man with a history of chronic hepatitis B infection underwent an MRI of the liver, which revealed a cystic-appearing lesion in the region of the pancreatic head, reported as a cystadenoma or pseudocyst. The patient underwent an endoscopic ultrasound. A large periampullary diverticulum was discovered but there was no pancreatic head lesion. Case 3. A Chinese woman with a history total hysterectomy and bilateral salpingo-oophorectomy for ovarian malignancy underwent an MRI of the abdomen and pelvis. A cystic-appearing lesion was found in the region of the pancreatic head, which was reported as a cystadenoma or intraductal papillary mucinous neoplasm. Follow-up magnetic resonance cholangiopancreatography showed a signal void within, suggestive of gas within a periampullary diverticulum. Review of a prior CT scan showed a periampullary diverticulum. Conclusions: Periampullary diverticula, when fluid-filled, can be confused with cystic lesions in the pancreatic head. Radiologists should be aware of this potential pitfall

  5. Ovarian mucinous tumor with malignant mural nodules: dedifferentiation or collision?

    PubMed

    Desouki, Mohamed M; Khabele, Dineo; Crispens, Marta A; Fadare, Oluwole

    2015-01-01

    Ovarian mucinous tumors with mural nodules are rare surface epithelial-stromal tumors. The mural nodules are divergent neoplasms that may be benign or malignant. The latter may be in the form of a sarcoma, carcinosarcoma, anaplastic carcinoma, or a variety of other recognized histotypes of carcinoma, which raises the question of whether malignant mural nodules represent a form of dedifferentiation in ovarian mucinous tumors or whether they represent collision tumors. We recently reported the K-RAS gene mutation status in a case of ovarian mucinous adenocarcinoma with mural nodule of high-grade sarcoma. The mucinous and sarcomatous components revealed a mutation in codon 12 of the K-RAS gene of a different nucleotide substitution, indicating that these 2 tumor components were different clones of the same tumor. Herein, we are reporting another case of a 20-yr-old woman who presented with 22 cm pelvic mass, omental caking, and ascites. A diagnosis of invasive mucinous carcinoma with mural nodules of anaplastic carcinoma was rendered. K-RAS gene mutation studies revealed p.G12V, c.35G>T mutation in the 2 components of the tumor, which is the most common mutation reported in mucinous tumors of the ovary. The fact that sarcomatous or anaplastic carcinomatous mural nodules in ovarian mucinous tumors display the same K-RAS mutations as their underlying mucinous neoplasms provides supportive evidence that at least some malignant mural nodules represent a form of dedifferentiation in ovarian mucinous tumors, rather than a collision of 2 divergent tumor types. PMID:25473748

  6. Primary ovarian neuroendocrine tumor arising in association with a mature cystic teratoma: A case report.

    PubMed

    Orsi, Nicolas M; Menon, Mini

    2016-08-01

    Primary ovarian carcinoid tumors are exceptionally rare entities accounting for approximately 0.1% of all ovarian neoplasms. This report describes a primary ovarian neuroendocrine tumor arising in association with a mature cystic teratoma in a 65 year-old woman. Macroscopically, the unilateral adnexal tumor was composed of cystic, solid and mucinous elements which resolved into a dual component lesion histologically. The majority of the tumor displayed an organoid architecture with mild to moderate pleomorphism and no discernible mitotic activity, while approximately 10% consisted of sheets and groups of cells with highly pleomorphic nuclei, necrosis and occasional mitoses. Features of a mature cystic teratoma were seen very focally. Immunohistochemistry revealed strong, diffuse positivity for CD56 and synaptophysin. Chromogranin immunonegativity was noted and there was an absence of nuclear β-catenin accumulation. Ki-67 index was 10-12%. Although there is no established diagnostic framework for primary ovarian carcinoid tumors, this case was diagnosed as a well-differentiated neuroendocrine tumor, Grade 2 (intermediate grade), arising in association with a mature cystic teratoma/dermoid cyst. This case highlights the need to develop ovarian diagnostic criteria in this area. PMID:27508272

  7. Intestinal-Type Adenocarcinoma Arising in a Mature Cystic Teratoma of the Ovary.

    PubMed

    Clark, Mary E; Will, Micah D

    2016-07-01

    Here, we present a rare case of intestinal type adenocarcinoma arising in mature cystic teratoma (MCT) and review all previously reported similar cases with emphasis on the immunohistochemical characteristics of prior cases. Nine prior cases of intestinal type adenocarcinoma arising in MCT have been previously reported. Two of the prior cases as well as this case have been associated with CA19-9 elevation. CK20 is consistently strongly positive and CK7 is usually negative in cases of intestinal type adenocarcinoma arising in MCT. This contrasts with mucinous tumors of the ovary which are known to be usually CK7 strongly positive and inconsistently CK20 positive. The pattern of strong CK20 staining and often negative or weak CK7 staining is common to mucinous neoplasms arising in MCT. These findings suggest that the less common subset of primary mucinous ovarian tumors that have strong CK20 staining and partial or negative CK7 staining may represent a group of tumors with germ cell origin rather than the more common surface epithelial origin. PMID:26937866

  8. Elevated Erythropoietin and Multicystic Neoplasm of the Pancreas

    PubMed Central

    Nai, Qiang; Regeti, Kalyani; Arshed, Sabrina; Hossain, Mohammed Amzad; Zhang, Ping; Luo, Hongxiu; Singh, Shilpi; Mathew, Teena; Islam, Mohammed; Sen, Shuvendu; Yousif, Abdalla M.; Duhl, Jozsef

    2015-01-01

    Cystic lesions of the pancreas are more frequently recognized due to the widespread use of improved imaging techniques. There are a variety of pancreatic cystic lesions with different clinical presentations and malignant potentials, and their management depends on the type of the cysts. Although the early recognition of a cystic neoplasm with malignant potential provides an opportunity of early surgical treatment, the precise diagnosis of the cystic neoplasm can be a challenge, largely due to the lack of reliable biomarkers of malignant transformation. We report a case of a large, multicystic neoplasm within the body and tail of the pancreas complicated by elevated erythropoietin, which is likely related to the malignant transformation of the pancreatic neoplasm. PMID:25873882

  9. Elevated erythropoietin and multicystic neoplasm of the pancreas.

    PubMed

    Nai, Qiang; Regeti, Kalyani; Arshed, Sabrina; Hossain, Mohammed Amzad; Zhang, Ping; Luo, Hongxiu; Singh, Shilpi; Mathew, Teena; Islam, Mohammed; Sen, Shuvendu; Yousif, Abdalla M; Duhl, Jozsef

    2015-01-01

    Cystic lesions of the pancreas are more frequently recognized due to the widespread use of improved imaging techniques. There are a variety of pancreatic cystic lesions with different clinical presentations and malignant potentials, and their management depends on the type of the cysts. Although the early recognition of a cystic neoplasm with malignant potential provides an opportunity of early surgical treatment, the precise diagnosis of the cystic neoplasm can be a challenge, largely due to the lack of reliable biomarkers of malignant transformation. We report a case of a large, multicystic neoplasm within the body and tail of the pancreas complicated by elevated erythropoietin, which is likely related to the malignant transformation of the pancreatic neoplasm. PMID:25873882

  10. Optimum Timing for Surgery After Pre-operative Radiotherapy 6 vs 12 Weeks

    ClinicalTrials.gov

    2015-06-22

    Adenocarcinoma of the Rectum; Adenocarcinoma; Adenocarcinoma, Mucinous; Carcinoma; Neoplasms, Glandular and Epithelial; Neoplasms by Histologic Type; Neoplasms; Neoplasms, Cystic, Mucinous, and Serous; Colorectal Neoplasms; Intestinal Neoplasms; Gastrointestinal Neoplasms; Digestive System Neoplasms; Neoplasms by Site; Digestive System Diseases; Gastrointestinal Diseases; Intestinal Diseases; Rectal Diseases

  11. S100P is a useful marker for differentiation of ovarian mucinous tumors.

    PubMed

    Umezaki, Y; Ito, M; Nakashima, M; Mihara, Y; Naruke, Y; Kurohama, H; Yatsunami, N; Yasuhi, I

    2015-01-01

    The S100P protein stimulates cell proliferation and survival, thereby contributing to tumor progression. The purpose of this study was to evaluate S100P expression in the three subtypes of mucinous cystic tumors, cystadenomas, borderline tumors, and adenocarcinomas. The authors examined nuclear S100P expression in 60 mucinous ovarian tumor specimens, including 24 specimens of mucinous cystadenoma, 15 of borderline tumors, and 21 of adenocarcinomas. Immunohistochemistry revealed S100P expression followed one of three patterns: (1) Expressed in most nuclei of mucinous epithelial cells, (2) sporadic (spotted or patchy) expression, or (3) absent or rarely expressed in the nuclei of mucinous epithelial cells. Most adenomas showed the first expression pattern, and borderline tumors often showed a patchy expression pattern. Adenocarcinomas generally demonstrated absence of S100P expression. These data suggest that S100P is a useful histological marker to differentiate between benign, borderline, and malignant mucinous tumors of the ovary. PMID:26050349

  12. The value of SATB2 in the differential diagnosis of intestinal-type mucinous tumors of the ovary: primary vs metastatic.

    PubMed

    Perez Montiel, Delia; Arispe Angulo, Karen; Cantú-de León, David; Bornstein Quevedo, Leticia; Chanona Vilchis, José; Herrera Montalvo, Luis

    2015-08-01

    Primary mucinous adenocarcinomas of the ovary are a diagnostic challenge because their histologic and immunohistochemical features usually overlap with metastatic tumors. SATB2 is a recently identified protein with restricted expression in the glandular cells lining the lower gastrointestinal tract. The aim of this study is to examine the differential expression of SATB2 in primary and metastatic tumors of the ovary. Mucinous ovarian tumors of intestinal type were retrieved from the pathology files of the Instituto Nacional de Cancerología de México. A double reading of the hematoxylin and eosin-stained slides was performed to confirm the diagnosis, and a detailed review of the clinical chart was performed to define the primary origin of the tumor (ovarian vs metastatic). Immunohistochemical staining for CK20, CDX2, and SATB2 was performed and evaluated by 2 gynecopathologists. A total of 106 mucinous tumors were identified, 26 of which were considered to be metastatic, and 80 of which were primary ovarian tumors. All of the primary tumors that were not associated with cystic teratomas were negative for SATB2, and the 4 that were associated with a teratoma were positive for SATB2. All 20 of the metastatic tumors of the colon and appendix were positive for CK20, and 4 were positive for CK7. In addition, all 20 of these tumors were positive for SATB2, and 19 were positive for CDX2. SATB2 appears to be a useful marker for the diagnosis of primary vs metastatic mucinous intestinal-type neoplasms and is highly sensitive in detecting lower gastrointestinal tract metastasis. PMID:26059401

  13. Folliculosebaceous Cystic Hamartoma With Spindle Cell Lipoma-Like Stromal Features

    PubMed Central

    Skupsky, Hadas; Cassarino, David

    2015-01-01

    Abstract: Folliculosebaceous cystic hamartoma is a distinctive cutaneous hamartoma composed of follicular, sebaceous, and mesenchymal components. The lesions are most commonly found on the face and scalp of young adults, with approximately 30% occurring in the nasal or paranasal regions of the face. The clinical differential diagnoses are extensive and include epidermoid cyst, dermal nevus, soft fibroma, and adnexal tumors including sebaceous neoplasms. Here, the authors present a case of a 24-year-old man who presented for evaluation of an asymptomatic growth on the nose, which had slowly enlarged over 9 years. On examination, there was a 0.6 cm dome-shaped flesh-colored papule on the nasal bridge. The clinical differential included dermatofibroma versus intradermal nevus. A shave biopsy was performed, and histological examination of the sections showed a proliferation of multiple enlarged and irregular-appearing sebaceous glands attached to a cystic follicular structure. The associated dermal mesenchymal component consisted of numerous mature-appearing adipocytes associated with a fibromyxoid stroma, prominent collections of mucin, and bundles of ropey collagen resembling a spindle cell lipoma. This combination of a folliculosebaceous cystic hamartoma with a spindle cell lipoma-like mesenchymal proliferation is unusual and has not been previously reported. PMID:26588344

  14. A procedure for Alcian blue staining of mucins on polyvinylidene difluoride membranes.

    PubMed

    Dong, Weijie; Matsuno, Yu-ki; Kameyama, Akihiko

    2012-10-16

    The isolation and characterization of mucins are critically important for obtaining insight into the molecular pathology of various diseases, including cancers and cystic fibrosis. Recently, we developed a novel membrane electrophoretic method, supported molecular matrix electrophoresis (SMME), which separates mucins on a polyvinylidene difluoride (PVDF) membrane impregnated with a hydrophilic polymer. Alcian blue staining is widely used to visualize mucopolysaccharides and acidic mucins on both blotted membranes and SMME membranes; however, this method cannot be used to stain mucins with a low acidic glycan content. Meanwhile, periodic acid-Schiff staining can selectively visualize glycoproteins, including mucins, but is incompatible with glycan analysis, which is indispensable for mucin characterizations. Here we describe a novel staining method, designated succinylation-Alcian blue staining, for visualizing mucins on a PVDF membrane. This method can visualize mucins regardless of the acidic residue content and shows a sensitivity 2-fold higher than that of Pro-Q Emerald 488, a fluorescent periodate Schiff-base stain. Furthermore, we demonstrate the compatibility of this novel staining procedure with glycan analysis using porcine gastric mucin as a model mucin. PMID:22950532

  15. Solid and papillary epithelial neoplasm of the pancreas

    SciTech Connect

    Friedman, A.C.; Lichtenstein, J.E.; Fishman, E.K.; Oertel, J.E.; Dachman, A.H.; Siegelman, S.S.

    1985-02-01

    Solid and papillary epithelial neoplasm of the pancreas is an uncommon low grade malignant tumor histologically distinct from the usual ductal adenocarcinoma and amenable to cure by surgical excision. It tends to occur in black women in their second or third decade of life and has often been misclassified as nonfunctional islet cell tumor or as cystadenoma or cystadenocarcinoma. Twelve cases were reviewed. Sonography and CT of solid and pipillary epithelial neoplasms depict a well-demarcated mass that can be solid, mixed cystic and solid, or largely cystic. The radiologic appearance is dependent on the maintenance of the integrity of the neoplasm versus the extent of retrogressive changes that have occurred.

  16. Abnormal fucosylation of ileal mucus in cystic fibrosis: I. A histochemical study using peroxidase labelled lectins.

    PubMed Central

    Thiru, S; Devereux, G; King, A

    1990-01-01

    Peroxidase conjugated lectins were used to analyse the glycoproteins of small intestinal mucins in normal infants and those with cystic fibrosis to ascertain whether there are any detectable histochemical differences in saccharide composition. A significant decrease in Lotus tetragonolobus (LTG) binding fucose was shown in normal small intestinal mucin starting around 36 weeks' gestation with total absence of staining at term and beyond. In contrast, the age matched patients with cystic fibrosis showed persistent and intense LTG binding of fucose. These results provide the first clear histochemical evidence that cystic fibrosis mucin is abnormal and confirm the findings of previous biochemical studies. Images PMID:2266173

  17. FTIR Study On The Secondary Structure Of Mucin From Mucinous Cystadenoma Of The Ovary

    NASA Astrophysics Data System (ADS)

    Shen, Keng; Wu, Paochen; Zhou, Weij in; Liu, Fuan; Guo, Hai; Wu, Jinguang

    1989-12-01

    The mucinous cystadenoma, a common benign neoplasm of the ovary, may sometime bring about a fatal outcome known as pseudomyxoma peritonei which is characterized by massive accumulation of mucinous substance in the peritoneal cavity, resulting in extensive adhesions, chronic progressive intestinal obstruction and finally death of the patient. Surgical approach to this condition proves to be a palliative procedure. Repeated operation can only remove part of the geletinous material and reaccumulation of mucus within 1-2 years after the initial surgery is almost a rule. In view of the benign histologic nature of the disease, chemotherapy, either systemic or intraperitoneal, and radiotherapy are generally ineffective in arresting the progression of the pathologic process and preventing the reaccumulation of mucus. Therefore, the only hope lies on the introduction into the peritoneal cavity some agents which may dissolve the accumulated mucin, relieve the intestinal obstruction, and consequently, prolong and even save the life f the patient. Based on this conception, sporadic articles by a few authors(1,2)ap-peared in the literature reporting their clinical experience with different mucolytic agents. However, some blindness would inevitably be involved in such investigations due to the lack of a comprehensive understanding of the chemical structures of the substance. The purpose of the present paper is to report our preliminary results of study of the secondary structures of mucin secreted by this special type of tumor.

  18. VAMP8 is a vesicle SNARE that regulates mucin secretion in airway goblet cells.

    PubMed

    Jones, Lisa C; Moussa, Lama; Fulcher, M Leslie; Zhu, Yunxiang; Hudson, Elizabeth J; O'Neal, Wanda K; Randell, Scott H; Lazarowski, Eduardo R; Boucher, Richard C; Kreda, Silvia M

    2012-02-01

    Mucin secretion is an innate defence mechanism, which is noxiously upregulated in obstructive lung diseases (e.g. chronic obstructive pulmonary disease (COPD), cystic fibrosis and asthma). Mucin granule exocytosis is regulated by specific protein complexes, but the SNARE exocytotic core has not been defined in airway goblet cells. In this study, we identify VAMP8 as one of the SNAREs regulating mucin granule exocytosis. VAMP8 mRNA was present in human airway and lung epithelial cells, and deep-sequencing and expression analyses of airway epithelial cells revealed that VAMP8 transcripts were expressed at 10 times higher levels than other VAMP mRNAs. In human airway epithelial cell cultures and freshly excised tissues, VAMP8 immunolocalised mainly to goblet cell mucin granules. The function of VAMP8 in airway mucin secretion was tested by RNA interference techniques. Both VAMP8 short interfering RNAs (siRNAs) and short hairpin RNAs (shRNAs) reduced mucin secretion induced by PAR agonists, neutrophil elastase and ATP in two airway epithelial cell culture models. Notably, basal (non-agonist elicited) mucin secretion was also reduced in these experiments. VAMP8 knockdown was also effective in decreasing mucin secretion in airway epithelial cell cultures with induced mucous metaplasia/mucin hypersecretion. Unlike VAMP8 silencing, knockdown of VAMP2 or VAMP3 did not affect mucin secretion. Importantly, in VAMP8 knock-out (KO) mice with IL-13-induced mucous metaplasia, mucin content in the bronchoalveolar lavage (BAL) and ATP-stimulated mucin secretion in the trachea were reduced compared to WT-matched littermates. Our data indicate that VAMP8 is an essential SNARE in airway mucin granule exocytosis. Reduction of VAMP8 activity/expression may provide a novel therapeutic target to ameliorate airway mucus obstruction in lung diseases. PMID:22144578

  19. Mucin-drugs interaction: The case of theophylline, prednisolone and cephalexin.

    PubMed

    Pontremoli, Carlotta; Barbero, Nadia; Viscardi, Guido; Visentin, Sonja

    2015-10-15

    The binding of mucin with three commercially available drugs (theophylline, cephalexin and prednisolone) belonging to different pharmaceutical classes was investigated. The studied drugs are normally used to treat the symptomatology of cystic fibrosis. The interaction between drugs and mucin has been investigated using fluorescence and UV-Vis absorption spectroscopy; quenching mechanism, binding constants, binding sites, thermodynamic parameters and binding distance of the interaction were obtained. PMID:26422788

  20. A huge ovarian mucinous cystadenoma causing virilization, preterm labor, and persistent supine hypotensive syndrome during pregnancy

    PubMed Central

    Acar, Canan; Temizkan, Osman; Ozagari, Aysim; Gozukara, Ilay; Akyol, Atif

    2016-01-01

    Mucinous cystadenoma (MC) of the ovary is an unilateral, multilocular cystic benign epithelial tumor. Supposed to be hormone responsive, MC reaches huge sizes during pregnancy. Aortocaval compression is common during pregnancy, especially when the pregnant woman is in the supine position. However, the compression recovers with a change in position. The authors report the first case of a huge mucinous cystadenoma of the ovary complicating pregnancy and causing virilization, premature labor, and persistent supine hypotensive syndrome. PMID:27547742

  1. Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable

    MedlinePlus

    ... Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Myelodysplastic/ ...

  2. Mucinous tumors of the ovary: current thoughts on diagnosis and management.

    PubMed

    Brown, Jubilee; Frumovitz, Michael

    2014-06-01

    Mucinous tumors of the ovary represent a spectrum of neoplastic disorders, including benign mucinous cystadenoma, pseudomyxoma peritonei, mucinous tumors of low malignant potential (borderline), and invasive mucinous ovarian carcinoma. These tumors are related closely to each other and are distinct from other histologic subtypes of epithelial ovarian neoplasms from a clinical, histologic, and molecular standpoint. A continuum appears to be present from benign to borderline to malignant, which is different from other types of epithelial ovarian cancer. Mutational profiles are also distinct, as KRAS mutations are common, but p53 and BRCA mutations are infrequent. These characteristics lead to specific biologic behavior and guide both clinical management and research efforts in patients with mucinous ovarian tumors. PMID:24777667

  3. Clonality analysis of combined Brenner and mucinous tumours of the ovary reveals their monoclonal origin.

    PubMed

    Wang, Yihong; Wu, Ren-chin; Shwartz, Lauren Ende; Haley, Lisa; Lin, Ming-tse; Shih, Ie-ming; Kurman, Robert J

    2015-10-01

    The derivation of ovarian intestinal-type mucinous tumours is not well established. Some are derived from teratomas but the origin of the majority is not clear. It has been recently proposed that the non-germ cell group may be derived from Brenner tumours, as the association of a mucinous tumour with a Brenner tumour is frequently observed. In order to explore the histogenesis of these neoplasms, we undertook a clonality analysis of the two components of ten combined Brenner and mucinous tumours using a human androgen receptor gene (HUMARA) assay. All eight informative cases of ten showed a concordant X-chromosome inactivation pattern between the two tumour components, indicative of a shared clonal origin (p = 0.0039). Microsatellite genotyping in five of the combined tumours displayed an identical heterozygous pattern with paired Fallopian tube tissue, indicative of a somatic cell origin. In addition, paired box protein 8, a highly sensitive Müllerian epithelial marker, was not detected by immunohistochemistry in either tumour component in any of the ten tumours, suggesting that this subset of mucinous tumours does not originate from Müllerian-derived epithelium. In conclusion, this study demonstrates that in combined mucinous and Brenner tumours, there is a shared clonal relationship between the two different tumour components and suggests that some pure mucinous tumours may develop from a Brenner tumour in which the Brenner tumour component becomes compressed and obliterated by an expanding mucinous neoplasm. PMID:26095692

  4. Mucin-Based Vaccines

    NASA Astrophysics Data System (ADS)

    Richardson, Jonathan P.; MacMillan, Derek

    Mucins are heavily O-glycosylated cell surface and secreted glycoproteins . In addition to orchestrating cell-extracellular matrix and cell-cell interactions in healthy organisms mucins are also the major carriers of altered glycosylation in carcinomas. Tumor-associated antigens displayed by cancer cells comprise oligosaccharide and glycopeptide motifs not encountered in the same locale or at the same frequency in healthy cells, and potentially confer a selective advantage to the tumor. Frequently tumor-associated antigens are under-glycosylated and prematurely sialylated, and it is these relatively simple saccharide and glycopeptide structures that have been targeted to serve as drug candidates in most cases. A major goal is to assemble glycopeptide vaccine candidates based on partial mucin sequences and displaying tumor-associated antigens that can mount a potent immunological tumor-specific response when, in reality, the tumor has already coerced the immune system into a state of co-existence.

  5. [Cutaneous neoplasms].

    PubMed

    Dummer, R; Beyeler, M; Morcinek, J; Burg, G

    2003-09-01

    The skin is the organ most commonly affected by malignancies. Various cancers of the skin show a dramatic increase in incidence over the last decades. Epithelial skin tumors are most frequently, e.g., basal cell carcinoma and the squamous cell carcinoma with its precursors, the actinic keratoses. Melanoma, which is extremely difficult to treat in advanced tumor stages, is dreaded. Besides that, there are other epithelial malignant diseases, e.g. Morbus Bowen and adnexal tumors originating from the skin appendices. Mesenchymal malignant neoplasias such as Morbus Kaposi, angiosarcomas and other dermal sarcomas, are rare. Since the majority of malignant neoplasms is removable and curable by a simple surgical intervention, the knowledge of the different skin tumors is essential for non-dermatologist. PMID:14526630

  6. Recently described neoplasms of the sinonasal tract.

    PubMed

    Bishop, Justin A

    2016-03-01

    Surgical pathology of the sinonasal region (i.e., nasal cavity and the paranasal sinuses) is notoriously difficult, due in part to the remarkable diversity of neoplasms that may be encountered in this area. In addition, a number of neoplasms have been only recently described in the sinonasal tract, further compounding the difficulty for pathologists who are not yet familiar with them. This manuscript will review the clinicopathologic features of some of the recently described sinonasal tumor types: NUT midline carcinoma, HPV-related carcinoma with adenoid cystic-like features, SMARCB1 (INI-1) deficient sinonasal carcinoma, biphenotypic sinonasal sarcoma, and adamantinoma-like Ewing family tumor. PMID:26776744

  7. Effect of reserpine on the histochemical and biochemical properties of rat intestinal mucin

    SciTech Connect

    Forstner, J.; Roomi, N.; Khorasani, R.; Kuhns, W.; Forstner, G. )

    1991-04-01

    Biochemical and histochemical parameters of intestinal mucins were examined in control and reserpine-treated rats. An assay for intestinal mucin sulfotransferase was developed and the activity shown to increase 3.4 times over control levels in rats given intraperitonal reserpine (0.5 mg/kg body wt) daily for 7 days. Histochemical staining of intestinal sections revealed an increase in sulfomucins in goblet cells of reserpine-treated rats. The effects were prominent as early as 1 day following injection, particularly in the distal third of the small intestine, and during the next 6 days these changes spread progressively to the middle and proximal thirds. After 3 days of treatment mucins were purified from each intestinal segment and compared to control mucins with respect to composition and (35S)NaSO{sub 4} incorporation. Although individual amino acid and carbohydrate molar ratios were unchanged, the total carbohydrate and sulfate content of mucins in treated animals was elevated (two to three times above control) in the middle and distal thirds of the intestine. In vivo ({sup 35}S)SO{sub 4} incorporation into these mucins was also proportionaltely elevated, and was targetted to O-linked oligosaccharide side chains. These findings are consistent with an action of reserpine causing an increased production of mucin which is enriched in glycoprotein components bearing sulfated oligosaccharide chains. The relevance of these findings to the production of hypersulfated and hyperglycosylated mucins in cystic fibrosis is discussed.

  8. Mucinous ovarian tumors associated with mucinous adenocarcinomas of the cervix. A clinicopathological analysis of 16 cases.

    PubMed

    Young, R H; Scully, R E

    1988-01-01

    Sixteen cases of mucinous adenocarcinoma of the cervix that were associated with a mucinous tumor of one or both ovaries are reported. The patients ranged from 25 to 70 (average, 44) years of age; two of them had the Peutz-Jeghers syndrome. Eight patients complained of abdominal swelling; most of the remainder had symptoms of uterine origin. Twelve patients had bilateral and four had unilateral ovarian tumors, which were typically large and cystic. Microscopic examination of most of the ovarian tumors revealed various combinations of benign-appearing, borderline, and carcinomatous mucinous epithelium within the same specimen. Most of the cervical tumors were deeply invasive; 10 of them were of the adenoma malignum type. Although there were varying degrees of uncertainty in individual cases, consideration of several features including the extent and distribution of disease in the abdomen, the comparative histology of the tumors, and the pattern of ovarian involvement suggested that 10 of the ovarian tumors were independent primary tumors, three were metastatic from the cervix, and in three cases the ovaries contained both primary and metastatic tumors. PMID:2840404

  9. Cystic Fibrosis

    MedlinePlus

    ... for the Public » Health Topics » Cystic Fibrosis Explore Cystic Fibrosis What Is... Other Names Causes Who Is at Risk Signs & Symptoms Diagnosis Treatments Living With Clinical Trials Links Related Topics Bronchiectasis ...

  10. Cystic fibrosis

    MedlinePlus

    ... page: //medlineplus.gov/ency/article/000107.htm Cystic fibrosis To use the sharing features on this page, please enable JavaScript. Cystic fibrosis is a disease that causes thick, sticky mucus ...

  11. Detecting, visualising, and quantifying mucins.

    PubMed

    Harrop, Ceri A; Thornton, David J; McGuckin, Michael A

    2012-01-01

    The extreme size, extensive glycosylation, and gel-forming nature of mucins make them a challenge to work with, and methodologies for the detection of mucins must take into consideration these features to ensure that one obtains both accurate and meaningful results. In understanding and appreciating the nature of mucins, this affords the researcher a valuable toolkit which can be used to full advantage in detecting, quantifying, and visualising mucins. The employment of a combinatorial approach to mucin detection, using antibody, chemical, and lectin detection methods, allows important information to be gleaned regarding the size, extent of glycosylation, specific mucin species, and distribution of mucins within a given sample. In this chapter, the researcher is guided through considerations into the structure of mucins and how this both affects the detection of mucins and can be used to full advantage. Techniques including ELISA, dot/slot blotting, and Western blotting, use of lectins and antibodies in mucin detection on membranes as well as immunohistochemistry and immunofluorescence on both tissues and cells grown on Transwell™ inserts are described. Notes along with each section advice the researcher on best practice and describe any associated limitations of a particular technique from which the researcher can further develop a particular protocol. PMID:22259129

  12. Childhood ovarian neoplasms in Ibadan, South-western Nigeria

    PubMed Central

    Ajani, Mustapha Akanji; Aramide, Kolawole Olanrewaju; Ajani, Tinuade Adesola; Salami, Ayodeji A.; Okolo, Clement Abu

    2016-01-01

    Background: Childhood ovarian neoplasms are very rare. Little information is available on the relative pattern and frequency of these tumors in Nigerian children. Earlier study done in Ibadan involved ovarian neoplasms in children and adolescents. The aim of this study is to review cases of ovarian neoplasms in children <15 years over a 22½ years period. Materials and Methods: This was a retrospective study. Twenty-four cases of childhood ovarian neoplasms were seen in patients <15 years of age. These cases were classified using the current World Health Organization histological classification of ovarian tumors. Results: Childhood ovarian neoplasms accounted for 2.8% of all cases of ovarian tumors seen in this period. Fourteen (58.3%) cases of childhood ovarian neoplasms were benign, and 10 (41.7%) were malignant. Mature cystic teratoma occurring in 13 (54.2%) was the most common childhood ovarian neoplasm and was most prevalent between 10 and 14 years of age. Burkitt lymphoma was the most common 4 (40%) malignant childhood ovarian tumor and prevalent between 5 and 14 years of age. Conclusion: Mature cystic teratoma remains the single most common childhood ovarian neoplasms, and Burkitt's lymphoma is the most malignant childhood ovarian tumor in Ibadan, South-western Nigeria. PMID:27397956

  13. Epidural Cystic Spinal Meningioma: A Case Report.

    PubMed

    Zhang, Ji; Chen, Zheng-He; Wang, Zi-Feng; Sun, Peng; Jin, Jie-Tian; Zhang, Xiang-Heng; Zhao, Yi-Ying; Wang, Jian; Mou, Yong-Gao; Chen, Zhong-Ping

    2016-03-01

    Cystic spinal meningioma (CSM) is an uncommon meningioma variant. Extradural CSMs are particularly rare and difficult to distinguish from other intraaxial tumors. This study presents a case of a 36-year-old woman with intraspinal extradual CSM at the thoracolumbar spine. She experienced persistent weakness, progressive numbness, and sensory disturbance in the right lower limb. Magnetic resonance imaging (MRI) of the patient revealed an irregular cystic mass at the thoracic 11 to lumbar 3 levels dorsally. This case was misdiagnosed as other neoplasms prior to surgery because of the atypical radiographic features and location of the tumor.Extradural CSMs should be considered in the differential diagnosis of intraspinal extradural cystic neoplasms. Complete removal of cystic wall provides an optimal outcome, rendering the lesion curable. PMID:26986119

  14. A case of MUC5AC-positive intraductal neoplasm of the pancreas classified as an intraductal tubulopapillary neoplasm?

    PubMed

    Muraki, Takashi; Uehara, Takeshi; Sano, Kenji; Oota, Hiroyoshi; Yoshizawa, Akihiko; Asaka, Shiho; Tateishi, Ayako; Otsuki, Toshiaki; Shingu, Kunihiko; Matoba, Hisanori; Kobayashi, Shota; Ichimata, Shojiro; Watanabe, Takayuki; Itou, Tetsuya; Tanaka, Eiji

    2015-12-01

    This report describes a unique case of intraductal tubulopapillary neoplasm (ITPN) of the pancreas in order to clarify its oncogenesis and more precisely classify pancreatic intraductal neoplasms. A 74-year-old man visited our institution for follow-up of acute pancreatitis. Imaging examinations revealed a hypovascular intraductal mass in the head of the pancreas with progressive dilation of the pancreatic duct, atrophy of the pancreatic parenchyma, and a non-mucinous appearance. A pancreatoduodenectomy was performed to identify this pancreatic intraductal neoplasm. Macroscopically, the tumor was a solid nodular mass with no visibly secreted mucin obstructing the dilated ducts. Histologically, it had a homogeneous appearance with nodules of back-to-back tubular glands and occasional papillary elements, and there were no apparent transitions to areas with less marked cytoarchitectural atypia. Although the intraductal neoplastic growth corresponded to an ITPN, immunohistochemical staining revealed partial positivity for MUC5AC, for which ITPNs are characteristically negative. Somatic mutations in KRAS, GNAS, BRAF, and PIK3CA were not detected. A loss of MUC5AC expression and mutations in KRAS and GNAS are key elements in the diagnosis of ITPN. Thus, it was difficult to distinguish the present case as a pancreatobiliary-type (PB-type) intraductal papillary mucinous neoplasm (IPMN) or a phenotypic variant of ITPN. As it is possible that some cases of PB-type IPMN and ITPN overlap, the precise classification of these rare lesions may require re-evaluation. PMID:26586167

  15. Salivary Mucin 19 Glycoproteins

    PubMed Central

    Culp, David J.; Robinson, Bently; Cash, Melanie N.; Bhattacharyya, Indraneel; Stewart, Carol; Cuadra-Saenz, Giancarlo

    2015-01-01

    Saliva functions in innate immunity of the oral cavity, protecting against demineralization of teeth (i.e. dental caries), a highly prevalent infectious disease associated with Streptococcus mutans, a pathogen also linked to endocarditis and atheromatous plaques. Gel-forming mucins are a major constituent of saliva. Because Muc19 is the dominant salivary gel-forming mucin in mice, we studied Muc19−/− mice for changes in innate immune functions of saliva in interactions with S. mutans. When challenged with S. mutans and a cariogenic diet, total smooth and sulcal surface lesions are more than 2- and 1.6-fold higher in Muc19−/− mice compared with wild type, whereas the severity of lesions are up to 6- and 10-fold higher, respectively. Furthermore, the oral microbiota of Muc19−/− mice display higher levels of indigenous streptococci. Results emphasize the importance of a single salivary constituent in the innate immune functions of saliva. In vitro studies of S. mutans and Muc19 interactions (i.e. adherence, aggregation, and biofilm formation) demonstrate Muc19 poorly aggregates S. mutans. Nonetheless, aggregation is enhanced upon adding Muc19 to saliva from Muc19−/− mice, indicating Muc19 assists in bacterial clearance through formation of heterotypic complexes with salivary constituents that bind S. mutans, thus representing a novel innate immune function for salivary gel-forming mucins. In humans, expression of salivary MUC19 is unclear. We find MUC19 transcripts in salivary glands of seven subjects and demonstrate MUC19 glycoproteins in glandular mucous cells and saliva. Similarities and differences between mice and humans in the expression and functions of salivary gel-forming mucins are discussed. PMID:25512380

  16. Characterization of pig colonic mucins.

    PubMed Central

    Fogg, F J; Hutton, D A; Jumel, K; Pearson, J P; Harding, S E; Allen, A

    1996-01-01

    Pig colonic mucins isolated from the adherent mucus gel in the presence of proteinase inhibitors were solubilized by homogenization and the component mucins fractionated by CsC1 density-gradient centrifugation. Polymeric and reduced pig colonic mucin were both largely excluded on Sepharose CL-2B, papain-digested colonic mucin was included. The M(r) values of polymeric, reduced and digested mucins were 5.5 x 10(6), 2.1 x 10(6) and 0.6 x 10(6) respectively. This suggests that pig colonic mucin is comprised of 2-3 subunits, each subunit containing 3-4 glycosylated regions. The intrinsic viscosities of polymeric, reduced and digested mucin were 240 ml.g-1, 100 ml.g-1 and 20 ml.g-1 respectively. Polymeric pig colonic mucin comprised 16% protein per mg of glycoprotein and was rich in serine, threonine and proline (43% of total amino acids). There were approx. 150 disulphide bridges and 53 free thiol groups per mucin polymer. A seventh of the protein content was lost on reduction. This protein was particularly rich in proline and the hydrophobic amino acids. Papain-digested pig colonic mucin contained 11% protein per mg of glycoprotein and was rich in serine, threonine, glutamate and aspartate. All types of amino acids with the exception of aspartate were lost on digestion. The amino acid analysis of the proteolytically digested regions of pig colonic mucin are markedly different to the tandem repeat regions of the human mucin genes shown to be expressed in the colon. PMID:8670173

  17. GATA3 Expression in Normal Skin and in Benign and Malignant Epidermal and Cutaneous Adnexal Neoplasms

    PubMed Central

    de Peralta-Venturina, Mariza N.; Balzer, Bonnie L.; Frishberg, David P.

    2015-01-01

    Abstract: Initial investigations reported GATA3 to be a sensitive and relatively specific marker for mammary and urothelial carcinomas. Recently, GATA3 expression has been described in several other epithelial tumors. However, there has been only limited investigation of GATA3 expression in cutaneous epithelial tumors. The objective of this study was to examine the immunohistochemical expression of GATA3 in a wide variety of cutaneous epithelial neoplasms. GATA3 expression was evaluated in 99 benign and 63 malignant cutaneous epithelial tumors. GATA3 was consistently and usually strongly expressed in clear cell acanthoma, trichofolliculoma, trichoepithelioma, trichilemmoma, sebaceous adenoma, sebaceoma, apocrine hidrocystoma, apocrine tubular papillary adenoma, hidradenoma papilliferum, and syringocystadenoma papilliferum. Hidradenomas exhibited variable positive staining. Most poromas, syringomas, chondroid syringomas, cylindromas, and spiradenomas were negative or only focally and weakly positive. Focal staining was present in all pilomatrixomas. Thirteen of 14 basal cell carcinomas, 21 of 24 squamous carcinomas, and all 6 sebaceous carcinomas exhibited positive staining. The 1 apocrine carcinoma, both mucinous carcinomas, and 2 of 3 microcystic adnexal carcinomas also exhibited positive staining, whereas the 1 eccrine porocarcinoma and the 1 adenoid cystic carcinoma were negative. One of 11 Merkel cell carcinomas exhibited focal weak staining. Our findings demonstrate that GATA3 is expressed in a wide variety of benign and malignant cutaneous epithelial neoplasms. In addition to carcinomas of breast and urothelial origin and other more recently described GATA3-positive tumors, the differential diagnosis of a metastatic tumor of unknown primary origin that expresses GATA3 should also include a carcinoma of cutaneous epithelial origin. PMID:26595821

  18. REG4 Is Highly Expressed in Mucinous Ovarian Cancer: A Potential Novel Serum Biomarker.

    PubMed

    Lehtinen, Laura; Vesterkvist, Pia; Roering, Pia; Korpela, Taina; Hattara, Liisa; Kaipio, Katja; Mpindi, John-Patrick; Hynninen, Johanna; Auranen, Annika; Davidson, Ben; Haglund, Caj; Iljin, Kristiina; Grenman, Seija; Siitari, Harri; Carpen, Olli

    2016-01-01

    Preoperative diagnostics of ovarian neoplasms rely on ultrasound imaging and the serum biomarkers CA125 and HE4. However, these markers may be elevated in non-neoplastic conditions and may fail to identify most non-serous epithelial cancer subtypes. The objective of this study was to identify histotype-specific serum biomarkers for mucinous ovarian cancer. The candidate genes with mucinous histotype specific expression profile were identified from publicly available gene-expression databases and further in silico data mining was performed utilizing the MediSapiens database. Candidate biomarker validation was done using qRT-PCR, western blotting and immunohistochemical staining of tumor tissue microarrays. The expression level of the candidate gene in serum was compared to the serum CA125 and HE4 levels in a patient cohort of prospectively collected advanced ovarian cancer. Database searches identified REG4 as a potential biomarker with specificity for the mucinous ovarian cancer subtype. The specific expression within epithelial ovarian tumors was further confirmed by mRNA analysis. Immunohistochemical staining of ovarian tumor tissue arrays showed distinctive cytoplasmic expression pattern only in mucinous carcinomas and suggested differential expression between benign and malignant mucinous neoplasms. Finally, an ELISA based serum biomarker assay demonstrated increased expression only in patients with mucinous ovarian cancer. This study identifies REG4 as a potential serum biomarker for histotype-specific detection of mucinous ovarian cancer and suggests serum REG4 measurement as a non-invasive diagnostic tool for postoperative follow-up of patients with mucinous ovarian cancer. PMID:26981633

  19. REG4 Is Highly Expressed in Mucinous Ovarian Cancer: A Potential Novel Serum Biomarker

    PubMed Central

    Lehtinen, Laura; Vesterkvist, Pia; Roering, Pia; Korpela, Taina; Hattara, Liisa; Kaipio, Katja; Mpindi, John-Patrick; Hynninen, Johanna; Auranen, Annika; Davidson, Ben; Haglund, Caj; Iljin, Kristiina; Grenman, Seija; Siitari, Harri; Carpen, Olli

    2016-01-01

    Preoperative diagnostics of ovarian neoplasms rely on ultrasound imaging and the serum biomarkers CA125 and HE4. However, these markers may be elevated in non-neoplastic conditions and may fail to identify most non-serous epithelial cancer subtypes. The objective of this study was to identify histotype-specific serum biomarkers for mucinous ovarian cancer. The candidate genes with mucinous histotype specific expression profile were identified from publicly available gene-expression databases and further in silico data mining was performed utilizing the MediSapiens database. Candidate biomarker validation was done using qRT-PCR, western blotting and immunohistochemical staining of tumor tissue microarrays. The expression level of the candidate gene in serum was compared to the serum CA125 and HE4 levels in a patient cohort of prospectively collected advanced ovarian cancer. Database searches identified REG4 as a potential biomarker with specificity for the mucinous ovarian cancer subtype. The specific expression within epithelial ovarian tumors was further confirmed by mRNA analysis. Immunohistochemical staining of ovarian tumor tissue arrays showed distinctive cytoplasmic expression pattern only in mucinous carcinomas and suggested differential expression between benign and malignant mucinous neoplasms. Finally, an ELISA based serum biomarker assay demonstrated increased expression only in patients with mucinous ovarian cancer. This study identifies REG4 as a potential serum biomarker for histotype-specific detection of mucinous ovarian cancer and suggests serum REG4 measurement as a non-invasive diagnostic tool for postoperative follow-up of patients with mucinous ovarian cancer. PMID:26981633

  20. Mucolytics in cystic fibrosis.

    PubMed

    Henke, Markus O; Ratjen, Felix

    2007-03-01

    Mucus accumulation in the lower airways is a key feature of cystic fibrosis (CF) lung disease. The major component of mucus in CF is not mucin derived from mucus producing cells but rather pus that includes viscous material such as polymerized DNA derived from degraded neutrophils. This has important implications for mucolytic therapy aiming to improve mucus clearance from the airways, since degradation of mucin may not be a suitable treatment strategy. In addition, thinning of secretions may not always be beneficial, since it may negatively affect certain aspects of mucus transport such as cough clearance. While inhaled N-acetylcysteine has been used as a mucolytic drug in CF for decades, there is little evidence that it has any beneficial effect. Dornase alfa has been shown to reduce pulmonary exacerbations and improve lung function and is currently the only mucolytic agent with proven efficacy in CF. Newer agents targeting other components of CF mucus, such as filamentous actin, are currently in development. Ultimately, drugs that are mucokinetic, which preserve viscoelasticity, rather than mucolytic may prove to be beneficial for CF lung disease in the future. PMID:17419975

  1. A case of Krukenberg carcinoma metastasized from colon cancer resembling mucinous cystadenocarcinoma of the ovary.

    PubMed

    Shiono, Saori; Saito, Tsuyoshi; Fujii, Hiroaki; Arakawa, Atsushi; Nakamura, Takanori; Yao, Takashi

    2014-01-01

    We report a case of a 44-year-old woman with bilateral ovarian carcinoma that had metastasized from the colon and mimicked primary mucinous cystadenocarcinoma. Macroscopically, both ovarian tumors were large, multiloculated cystic masses with abundant mucinous content. Histologically, they were lined with mucinous epithelium with mild to moderate nuclear atypia and showed stromal invasion and surface involvement. At first, the tumors were diagnosed as bilateral primary ovarian mucinous cystadenocarcinomas. However, three months after surgery, a large villous tumor was discovered in the ascending colon by colonoscopic examination and was surgically resected. Histologically, the colonic tumor was a villous adenomatous tumor with invasive components of mucinous adenocarcinoma composed of well-differentiated adenocarcinoma and exhibited abundant extracellular mucin production. As a villous adenomatous component was present in the mucosal area, the colonic tumor was considered a primary tumor. Therefore, the original diagnosis of bilateral ovarian tumors was revised for consistent with metastasis from the colon carcinoma, in line with the findings of immunohistochemistry and loss of heterozygosity analysis. This case highlights the importance of considering the possibility of metastatic tumors from the gastrointestinal tract in the diagnosis of mucinous ovarian tumors. PMID:24427362

  2. Giant cystic pheochromocytoma: A silent entity

    PubMed Central

    Gupta, Amit; Bains, Lovenish; Agarwal, Manish Kumar; Gupta, Renu

    2016-01-01

    Pheochromocytoma is a catecholamine secreting tumor that originate from chromaffin cells. Usually, it is solid neoplasm of the adrenal medulla, however cystic pheochromocytoma is a rare neuro-endocrine tumour that is frequently asymptomatic and often diagnosed incidentally on imaging or intra-operatively. Only a few cases of cystic pheochromocytomas have been reported in the world literature. We present a case of giant cystic pheochromocytoma in a 65 years old lady who presented with a large retroperitoneal lump, which is probably the world's third largest pheochromocytoma as per the available indexed literature. PMID:27453669

  3. Heterogeneity of airways mucus: variations in the amounts and glycoforms of the major oligomeric mucins MUC5AC and MUC5B.

    PubMed Central

    Kirkham, Sara; Sheehan, John K; Knight, David; Richardson, Paul S; Thornton, David J

    2002-01-01

    Respiratory mucus contains a mixture of gel-forming mucins but the functional significance of these different mucin species is unknown. To help gain a better understanding of mucus in airways we therefore need to ascertain the concentration of each of the gel-forming mucins within respiratory secretions. Thus the aim of this study was to determine the amounts of specific gel-forming mucins directly from solubilized secretions of the airways and purified mucin preparations. We investigated the feasibility of using direct-binding ELISA employing mucin-specific antisera but were unable to obtain reliable data owing to interference with the immobilization of the mucins on the assay surface by 6 M urea and high levels of non-mucin proteins. We therefore developed an alternative approach based on quantitative Western blotting after agarose-gel electrophoresis, which was not subject to these problems. Here we demonstrate that this procedure provides reliable and reproducible data and have employed it to determine the amounts of the MUC2, MUC5AC and MUC5B mucins in saline-induced sputa from healthy airways and spontaneous sputa from asthmatic airways. Additionally we have used this procedure to analyse these glycoproteins in mucin preparations purified from cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) mucus. Our findings indicate that MUC5AC and MUC5B are the major oligomeric mucins and that airways mucus contains variable amounts of these glycoproteins. By contrast, the MUC2 mucin comprised, at most, only 2.5% of the weight of the gel-forming mucins, indicating that MUC2 is a minor component in sputum. Finally, we show that the amounts and glycosylated variants of the MUC5AC and MUC5B mucins can be altered significantly in diseased airways with, for instance, an increase in the low-charge form of the MUC5B mucin in CF and COPD mucus. PMID:11802783

  4. Actions of adenosine A1 and A2 receptor antagonists on CFTR antibody-inhibited β-adrenergic mucin secretion response

    PubMed Central

    Pereira, M M C; Lloyd Mills, C; Dormer, R L; McPherson, M A

    1998-01-01

    The cystic fibrosis gene protein, the cystic fibrosis transmembrane conductance regulator (CFTR) acts as a chloride channel and is a key regulator of mucin secretion. The mechanism by which 3-isobutyl-1-methylxanthine (IBMX) corrects the defect in CFTR mediated β-adrenergic stimulation of mucin secretion has not been determined. The present study has investigated the actions of adenosine A1 and A2 receptor antagonists to determine whether ability to stimulate mucin secretion correlates with correction of CFTR antibody inhibited β-adrenergic response and whether excessive cyclic AMP rise is required.CFTR antibodies were introduced into living rat submandibular acini by hypotonic swelling. Following recovery, mucin secretion in response to isoproterenol was measured.The adenosine A1 receptor antagonist, 8 cyclopentyltheophylline (CPT) was a less potent stimulator of mucin secretion than was the A2 receptor antagonist dimethylpropargylxanthine (DMPX). A concentration of CPT close to the Ki for A1 receptor antagonism (10 nM) did not stimulate mucin secretion.DMPX, although a potent stimulator of mucin secretion, did not correct CFTR antibody inhibited mucin secretion.CPT corrected defective CFTR antibody inhibited mucin secretion at a high (1 mM) concentration, suggesting a mechanism other than adenosine receptor antagonism.DMPX potentiated the isoproterenol induced cyclic AMP rise, whereas CPT did not.Correction of the defective CFTR mucin secretion response did not correlate with ability to stimulate mucin secretion and did not require potentiation of β-adrenergic induced increases in cyclic AMP. This affords real promise for the development of a selective drug treatment for cystic fibrosis. PMID:9831904

  5. Primary mucinous adenocarcinoma of the scalp: A case report and literature review

    PubMed Central

    Beteddini, Osama S. Al; Sheikh, Salwa; Shareefi, Faisal; Shahab, Rana

    2015-01-01

    Introduction Primary mucinous adenocarcinoma or mucinous eccrine carcinoma of the skin is a rare malignant neoplasm showing predilection to the head and neck. Distinguishing between these primary neoplasms and the more frequent metastatic mucinous deposits on the skin from primaries in the breast and gastrointestinal tract constitutes a diagnostic dilemma. Presentation of case We report a case of a 61-year-old lady who presented with a slow-growing, painless scalp nodule. Upon excision, it was diagnosed as “primary mucinous adenocarcinoma”. An extensive work-up in search for another primary tumour failed to show a primary malignancy elsewhere and the diagnosis of a primary eccrine mucinous adenocarcinoma of the skin was rendered. Discussion A review of the literature on this entity is presented, discussing diagnostic challenges and therapeutic options that of interest to surgeons, pathologists and dermatologists. Conclusion These tumours are indolent and low-grade, with a tendency for local, sometimes multiple, recurrences. Proper patient counselling and follow-up are important in treatment. Sound collaboration between clinicians and pathologists, for good therapeutic results, is of utmost importance. PMID:25708135

  6. [Mucinous adenocarcinoma of the appendix. Report of a case].

    PubMed

    Wolniczak, Isabella; Cáceres Del Águila, Alonso; Santillana Callirgos, Juan Alberto

    2016-01-01

    Mucinous adenocarcinoma of the appendix is a rare neoplasm with an incidence rate of 0.08% of all malignancies. The diagnosis is usually made by biopsy because its clinical presentation may mimic other diseases of structures located in the right lower quadrant. Currently, the treatment is still controversial, being surgery the best option. This report describes a patient with a history of appendectomy 27 years ago that is hospitalized for a painful mass in the lower abdomen associated with carcinoembryonic antigen of 138 ng/dl. PMID:27409095

  7. SATB2 Expression Distinguishes Ovarian Metastases of Colorectal and Appendiceal Origin From Primary Ovarian Tumors of Mucinous or Endometrioid Type.

    PubMed

    Moh, Michelle; Krings, Gregor; Ates, Deniz; Aysal, Anil; Kim, Grace E; Rabban, Joseph T

    2016-03-01

    The primary origin of some ovarian mucinous tumors may be challenging to determine, because some metastases of extraovarian origin may exhibit gross, microscopic, and immunohistochemical features that are shared by some primary ovarian mucinous tumors. Metastases of primary colorectal, appendiceal, gastric, pancreatic, and endocervical adenocarcinomas may simulate primary ovarian mucinous cystadenoma, mucinous borderline tumor, or mucinous adenocarcinoma. Recently, immunohistochemical expression of SATB2, a transcriptional regulator involved in osteoblastic and neuronal differentiation, has been shown to be a highly sensitive marker of normal colorectal epithelium and of colorectal adenocarcinoma. SATB2 expression has not been reported in normal epithelium of the female reproductive tract. Therefore, we hypothesized that SATB2 may be of value in distinguishing ovarian metastases of colorectal adenocarcinoma from primary ovarian mucinous tumors and from primary ovarian endometrioid tumors. Among primary ovarian tumors, SATB2 staining was observed in 0/22 mucinous cystadenomas that lacked a component of mature teratoma, 4/12 mucinous cystadenomas with mature teratoma, 1/60 mucinous borderline tumors, 0/17 mucinous adenocarcinomas, 0/3 endometrioid borderline tumors, and 0/72 endometrioid adenocarcinomas. Among ovarian metastases, SATB2 staining was observed in 24/32 (75%) colorectal adenocarcinomas; 8/10 (80%) low-grade appendiceal mucinous neoplasms; and 4/4 (100%) high-grade appendiceal adenocarcinomas. No SATB2 staining was observed in any ovarian metastasis of pancreatic, gastric, gallbladder, or endocervical origin. Evaluation of primary extraovarian tumors showed the highest incidences of SATB2 staining among primary colorectal adenocarcinomas (71%), primary appendiceal low-grade mucinous neoplasms (100%), and primary appendiceal high-grade adenocarcinomas (100%). Similar to their metastatic counterparts, none of the primary pancreatic or gastric

  8. Ossifying cystic odontogenic and Schneiderian choristoma of the orbit.

    PubMed

    Mudhar, Hardeep Singh; Nurrudin, Murtuza

    2014-02-01

    A 12-year-old girl presented with a left infraorbital lesion, causing upward globe displacement. Imaging confirmed a mass between the globe and the orbital floor. The lesion was removed via a sub-ciliary approach and histology revealed a a mature tooth along with a periodontal ligament, oral-type mucinous glands and bone. Six years later a mass recurred at exactly the same site and on this occasion, revealed cysts containing mucin and lined by Schneiderian type epithelium. A rather complex combination of a tooth, lamellar bone, mucinous oral type glands and Schneiderian cystic epithelium is highly unusual and we have called the lesion "ossifying cystic odontogenic and Schneiderian choristoma of the orbit." PMID:24171664

  9. Pathology of Mucinous Appendiceal Tumors and Pseudomyxoma Peritonei.

    PubMed

    Ramaswamy, Veena

    2016-06-01

    Neoplasms of the appendix are rare, but because of their unusual presentation and unpredictable biologic behavior, it is important to diagnose them correctly. Mucinous tumors account for 58 % of malignant tumors of appendix in SEER database and the remaining are carcinoids. The mucinous appendiceal tumors have a potential to spread to the peritoneum and viscera in the form of gelatinous material with or without neoplastic cells resulting in Pseudomyxoma peritonei. (PMP) PMP is a clinical entity that has a unique biological behavior and can arise from seemingly benign tumors to frankly malignant ones. Several classifications exist for PMP of which Ronnet's classification has been the most popular. In 2010, the WHO proposed a 2 tier classification that classified PMP as either low grade or high grade based on the presence of mucin, cytological and architectural features. According to this classification when the underlying cause for PMP is an appendiceal tumor it is always a mucinous adenocarcinoma rather than a mucocoele or adenoma and these terms should no longer be used. This system of classification helps in predicting the behavior of the tumor and proper treatment strategies. The understanding of the pathogenesis of the disease has also improved with identification of newer biomarkers and molecular genetic alterations. IHC markers CK 20, CDX2 and MUC2 are found to be positive in these tumors in addition to KRAS mutation and loss of heterozygosity in some gene loci. Proper histopathologic classification and predicting the tumor behavior requires a close interaction between the pathologist and the surgeon. The use of the combined modality treatment of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) has led to a 5-year survival ranging from 62.5 % to 100 % for low grade, and 0 %-65 % for high grade disease. This article focuses on the etiopathogenesis, clinical behavior, diagnosis and classification of mucinous tumors of the

  10. Sarcoma-like mural nodule in a borderline mucinous tumor of the ovary: A rare entity.

    PubMed

    Ghosh, Prithwijit; Saha, Kaushik; Bhowmik, Sourav

    2014-10-01

    Sarcoma-like mural nodule (SLMN) is a very uncommon and misleading benign entity which may be associated with benign, borderline or malignant mucinous neoplasm of the ovary. It should be distinguished from other malignant mural nodules with sarcoma, carcinosarcoma or anaplastic carcinoma for proper management. We report a rare case of SLMN in a borderline mucinous tumor of the ovary in a 30-year-old lady. In spite of having confusing histopathological features the final diagnosis was made depending on the younger age of the patient, well circumscription of the nodule, absence of vascular invasion and immunohistochemical profile. PMID:25540570

  11. Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1--Benign Tumors: From the Radiologic Pathology Archives.

    PubMed

    Manning, Maria A; Srivastava, Amogh; Paal, Edina E; Gould, Charles F; Mortele, Koenraad J

    2016-01-01

    Solid and cystic pancreatic neoplasms are being recognized more frequently with increasing utilization and spatial resolution of modern imaging techniques. In addition to the more common primary pancreatic solid (ductal adenocarcinoma) and cystic neoplasms of epithelial origin, nonepithelial neoplasms of the pancreas may appear as well-defined solid or cystic neoplasms. Most of these lesions have characteristic imaging features, such as a well-defined border, which allows differentiation from ductal adenocarcinoma. Solid masses include neurofibroma, ganglioneuroma, leiomyoma, lipoma, and perivascular epithelioid cell tumor (PEComa). Schwannomas and desmoid tumors can be solid or cystic. Cystic tumors include mature cystic teratoma and lymphangioma. Lipoma, PEComa, and mature cystic teratoma can contain fat, and ganglioneuroma and mature cystic teratoma may contain calcification. Although these unusual benign neoplasms are rare, the radiologist should at least consider them in the differential diagnosis of well-defined lesions of the pancreas. The goal of this comprehensive review is to improve understanding of these rare primary pancreatic mesenchymal tumors. PMID:26761535

  12. Advanced Stage Mucinous Adenocarcinoma of the Ovary is both Rare and Highly Lethal: A Gynecologic Oncology Group Study

    PubMed Central

    Zaino, Richard J.; Brady, Mark F.; Lele, Subodh M.; Michael, Helen; Greer, Benjamin; Bookman, Michael A.

    2010-01-01

    Background Primary mucinous adenocarcinomas of the ovary are uncommon and their biologic behavior uncertain. Retrospective studies suggest that many mucinous carcinomas diagnosed as primary to the ovary were actually metastatic from another site. A prospective randomized trial provided an opportunity to estimate the frequency of mucinous tumors, diagnostic reproducibility, and clinical outcomes. Methods A phase III trial enrolled 4000 women with stage III or IV ovarian carcinoma, treated by surgical staging and debulking, with randomization to one of five chemotherapeutic arms. Slides and pathology reports classified as primary mucinous carcinoma were reviewed independently by three pathologists. Cases were re-classified as primary or metastatic to the ovary according to two methods. Overall survival (OS) of reclassified groups was compared with each other and with that of patients with serous carcinomas. Results Forty-four cases were classified as mucinous adenocarcinoma at review. Using either method, only about one third were interpreted by the three reviewers as primary mucinous carcinomas. Reproducibility of interpretations among the reviewers was high with unanimity of opinion in 30 of the 44 (68%) cases. The median survival (MS) did not differ significantly between the groups interpreted as primary or metastatic, but the OS was significantly less than that for women with serous carcinoma (14 vs 42 months, p<0.001). Conclusion Advanced stage mucinous carcinoma of the ovary is very rare and is associated with poor OS. Many mucinous adenocarcinomas that are diagnosed as primary ovarian neoplasms appear to be metastatic to the ovary. PMID:20862744

  13. Cystic Lesions in Autoimmune Pancreatitis.

    PubMed

    Gompertz, Macarena; Morales, Claudia; Aldana, Hernán; Castillo, Jaime; Berger, Zoltán

    2015-01-01

    Autoimmune pancreatitis (AIP) can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered after corticosteroid therapy. One year later, he presented with a recurrent episode of AIP with elevated IgG4 levels, accompanied by the appearance of multiple intrapancreatic cystic lesions. All but 1 of these cysts disappeared after steroid treatment, but the remaining cyst in the pancreatic head was even somewhat larger 1 year later. Pancreatoduodenectomy was finally performed. Histology showed the wall of the cystic lesion to be fibrotic; the surrounding pancreatic tissue presented fibrosis, atrophy and lymphoplasmacytic infiltration by IgG4-positive cells, without malignant elements. Our case illustrates the rare possibility that cystic lesions can be part of AIP. These pseudocysts appear in the pancreatic segments involved in the autoimmune disease and can be a consequence of the local inflammation or related to ductal strictures. Steroid treatment should be initiated, after which these cysts can completely disappear with recovery from AIP. Surgical intervention may be necessary in some exceptional cases. PMID:26675058

  14. Immunophenotype and K-RAS mutation in mucinous ovarian adenocarcinoma with mural nodule of high-grade sarcoma: case report.

    PubMed

    Desouki, Mohamed M; Fadare, Oluwole; Kanbour, Anisa; Kanbour-Shakir, Amal

    2014-03-01

    Ovarian mucinous tumors with mural nodules are rare. The mural nodules are microscopically divergent neoplasms of varying sizes that may be benign (eg, sarcoma-like and carcinosarcoma-like), or malignant (eg, anaplastic carcinoma and sarcoma). The K-RAS gene mutation in ovarian mucinous neoplasms with mural nodules has not been previously reported. This is a case report of a 25-year-old female diagnosed with ovarian invasive mucinous adenocarcinoma with mural nodule of high-grade sarcoma. The mucinous tumor component demonstrated a K-RAS codon 12/13 mutation (p.G12V, c.35 G>T), whereas the sarcomatous component demonstrated a K-RAS codon 12/13 mutation (p.G12D, c.35 G>A). Although both tumor components revealed a mutation in codon 12 of K-RAS, they were of different nucleotide substitutions, indicating that these 2 tumor components were of different clonal origins. However, the fact that the 2 mutations identified in the tumor components are the most common mutations reported in mucinous tumors of the ovary, raises the possibility that sarcomatous mural nodules simply represent a form of dedifferentiation in mucinous tumors. PMID:24487474

  15. General Information about Chronic Myeloproliferative Neoplasms

    MedlinePlus

    ... Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Chronic Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Chronic ...

  16. Treatment Options for Myelodysplastic/Myeloproliferative Neoplasms

    MedlinePlus

    ... Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Myelodysplastic/ ...

  17. Treatment Option Overview (Myelodysplastic/Myeloproliferative Neoplasms)

    MedlinePlus

    ... Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Myelodysplastic/ ...

  18. Treatment Option Overview (Chronic Myeloproliferative Neoplasms)

    MedlinePlus

    ... Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Chronic Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Chronic ...

  19. Treatment Options for Chronic Myeloproliferative Neoplasms

    MedlinePlus

    ... Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Chronic Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Chronic ...

  20. General Information about Myelodysplastic/Myeloproliferative Neoplasms

    MedlinePlus

    ... Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment (PDQ®)–Patient Version General Information About Myelodysplastic/ ...

  1. Diagnostic Approach to Eosinophilic Renal Neoplasms

    PubMed Central

    Kryvenko, Oleksandr N.; Jorda, Merce; Argani, Pedram; Epstein, Jonathan I.

    2015-01-01

    Context Eosinophilic renal neoplasms include a spectrum of solid and papillary tumors ranging from indolent benign oncocytoma to highly aggressive malignancies. Recognition of the correct nature of the tumor, especially in biopsy specimens, is paramount for patient management. Objective To review the diagnostic approach to eosinophilic renal neoplasms with light microscopy and ancillary techniques. Data Sources Review of the published literature and personal experience. Conclusions The following tumors are in the differential diagnosis of oncocytic renal cell neoplasm: oncocytoma, chromophobe renal cell carcinoma (RCC), hybrid tumor, tubulocystic carcinoma, papillary RCC, clear cell RCC with predominant eosinophilic cell morphology, follicular thyroid-like RCC, hereditary leiomyomatosis–associated RCC, acquired cystic disease–associated RCC, rhabdoid RCC, microphthalmia transcription factor translocation RCC, epithelioid angiomyolipoma, and unclassified RCC. In low-grade nonpapillary eosinophilic neoplasms, distinction between oncocytoma and low-grade RCC mostly rests on histomorphology; however, cytokeratin 7 immunostain may be helpful. In high-grade nonpapillary lesions, there is more of a role for ancillary techniques, including immunohistochemistry for cytokeratin 7, CA9, CD10, racemase, HMB45, and Melan-A. In papillary eosinophilic neoplasms, it is important to distinguish sporadic type 2 papillary RCC from microphthalmia transcription factor translocation and hereditary leiomyomatosis–associated RCC. Histologic and cytologic features along with immunohistochemistry and fluorescence in situ hybridization tests for TFE3 (Xp11.2) and TFEB [t(6;11)] are reliable confirmatory tests. Eosinophilic epithelial neoplasms with architecture, cytology, and/or immunoprofile not qualifying for either of the established types of RCC should be classified as unclassified eosinophilic RCC and arbitrarily assigned a grade (low or high). PMID:25357116

  2. Cystic fibrosis

    MedlinePlus

    ... or three times each week. Swimming, jogging, and cycling are good options. Clearing or bringing up mucus ... cannot be prevented. Screening those with a family history of the disease may detect the cystic fibrosis ...

  3. Cystic fibrosis - resources

    MedlinePlus

    Resources - cystic fibrosis ... The following organizations are good resources for information on cystic fibrosis : Cystic Fibrosis Foundation -- www.cff.org March of Dimes -- www.marchofdimes.org/baby/cystic-fibrosis-and- ...

  4. Retroperitoneal mucinous cystadenoma of the appendix mimicking hydatid cyst: A case report

    PubMed Central

    Sikar, Hasan Ediz; Çetin, Kenan; Gündoğan, Ersin; Gündoğan, Gökçen Alinak; Kaptanoğlu, Levent

    2016-01-01

    Appendiceal mucocele is a cystic dilatation of the appendix due to abnormal appendiceal mucinous secretion. Cystadenoma of the appendix is one of the most common causes and is encountered in 0.6% of all appendectomy specimens. The diagnosis may be difficult due to the asymptomatic nature of the disease; pain in the right lower quadrant may be the only symptom. Complex ovarian cyst, urolithiasis or cystic hydatid disease of the liver have been reported as mimicking appendiceal mucocele in the literature. In this study, we present a case of mucinous cystadenoma of the appendix mimicking retroperitoneal hydatid cyst in a 59-year-old woman. The patient was treated with laparoscopic appendectomy with partial resection of the caecum following laparoscopic exploration.

  5. Transmembrane mucins as novel therapeutic targets

    PubMed Central

    Constantinou, Pamela E; Danysh, Brian P; Dharmaraj, Neeraja; Carson, Daniel D

    2011-01-01

    Membrane-tethered mucin glycoproteins are abundantly expressed at the apical surfaces of simple epithelia, where they play important roles in lubricating and protecting tissues from pathogens and enzymatic attack. Notable examples of these mucins are MUC1, MUC4 and MUC16 (also known as cancer antigen 125). In adenocarcinomas, apical mucin restriction is lost and overall expression is often highly increased. High-level mucin expression protects tumors from killing by the host immune system, as well as by chemotherapeutic agents, and affords protection from apoptosis. Mucin expression can increase as the result of gene duplication and/or in response to hormones, cytokines and growth factors prevalent in the tumor milieu. Rises in the normally low levels of mucin fragments in serum have been used as markers of disease, such as tumor burden, for many years. Currently, several approaches are being examined that target mucins for immunization or nanomedicine using mucin-specific antibodies. PMID:22201009

  6. KRAS mutation positive mucinous adenocarcinoma originating in mature ovarian teratoma: case report and review of literature.

    PubMed

    Hershkovitz, Dov; Vlodavsky, Euvgeni; Simon, Einav; Ben-Izhak, Ofer

    2013-12-01

    Mature ovarian teratomas rarely undergo transformation into malignancy. Carcinomas, mostly squamous cell carcinoma, are the most common malignancy arising in mature cystic teratoma. In the present report we describe a 13-year-old girl who developed a large mass in her ovary. Fine needle biopsy identified intestinal type mucinous adenocarcinoma, which was also identified in the full surgical specimen. Extensive sampling of the surgical specimen also identified areas of mature cystic teratoma. Interestingly, molecular analysis of DNA extracted from various components of the lesion identified KRAS mutation in the carcinoma, borderline mucinous tumor and benign intestinal-type epithelium but not in the epidermal component of the teratoma. To the best of our knowledge this is the first report of KRAS mutation in mucinous carcinoma originating in mature cystic teratoma. We discuss the importance of extensive tissue sampling to differentiate between carcinoma originating in teratoma and metastatic colorectal carcinoma to the ovary. Additionally, the identification of KRAS mutation in the morphologically benign intestinal-type epithelium indicated that it is an early event in the carcinogenic sequence and that the molecular pathway of carcinogenesis in teratoma is similar to that in the carcinogenic process of somatic tissue. PMID:24422958

  7. Cystic Change in Pleomorphic Adenoma: A Rare Finding and a Diagnostic Dilemma

    PubMed Central

    Khetrapal, Shaan; Jetley, Sujata; Hassan, Mohd. Jaseem

    2015-01-01

    Pleomorphic adenoma forms the majority of salivary gland neoplasms. Cystic change in pleomorphic adenomas is a diagnostic dilemma and can mimic mucoepidermoid carcinoma, mucocele or carcinoma ex pleomorphic adenoma and squamous cell carcinoma. Hereby we report this interesting and rare case of cystic pleomorphic adenoma in a 32-year-old male. PMID:26675071

  8. Cystic Change in Pleomorphic Adenoma: A Rare Finding and a Diagnostic Dilemma.

    PubMed

    Khetrapal, Shaan; Jetley, Sujata; Hassan, Mohd Jaseem; Jairajpuri, Zeeba

    2015-11-01

    Pleomorphic adenoma forms the majority of salivary gland neoplasms. Cystic change in pleomorphic adenomas is a diagnostic dilemma and can mimic mucoepidermoid carcinoma, mucocele or carcinoma ex pleomorphic adenoma and squamous cell carcinoma. Hereby we report this interesting and rare case of cystic pleomorphic adenoma in a 32-year-old male. PMID:26675071

  9. Diffuse Cystic Lung Disease. Part I.

    PubMed

    Gupta, Nishant; Vassallo, Robert; Wikenheiser-Brokamp, Kathryn A; McCormack, Francis X

    2015-06-15

    The diffuse cystic lung diseases (DCLDs) are a group of pathophysiologically heterogenous processes that are characterized by the presence of multiple spherical or irregularly shaped, thin-walled, air-filled spaces within the pulmonary parenchyma. Although the mechanisms of cyst formation remain incompletely defined for all DCLDs, in most cases lung remodeling associated with inflammatory or infiltrative processes results in displacement, destruction, or replacement of alveolar septa, distal airways, and small vessels within the secondary lobules of the lung. The DCLDs can be broadly classified according to underlying etiology as those caused by low-grade or high-grade metastasizing neoplasms, polyclonal or monoclonal lymphoproliferative disorders, infections, interstitial lung diseases, smoking, and congenital or developmental defects. In the first of a two-part series, we present an overview of the cystic lung diseases caused by neoplasms, infections, smoking-related diseases, and interstitial lung diseases, with a focus on lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis. PMID:25906089

  10. Cystic Fibrosis

    MedlinePlus

    Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. CF causes your mucus to be thick and sticky. The mucus clogs the lungs, causing breathing problems ...

  11. Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects

    PubMed Central

    2014-01-01

    Pseudomyxoma peritonei (PMP, ORPHA26790) is a clinical syndrome characterized by progressive dissemination of mucinous tumors and mucinous ascites in the abdomen and pelvis. PMP is a rare disease with an estimated incidence of 1–2 out of a million. Clinically, PMP usually presents with a variety of unspecific signs and symptoms, including abdominal pain and distention, ascites or even bowel obstruction. It is also diagnosed incidentally at surgical or non-surgical investigations of the abdominopelvic viscera. PMP is a neoplastic disease originating from a primary mucinous tumor of the appendix with a distinctive pattern of the peritoneal spread. Computed tomography and histopathology are the most reliable diagnostic modalities. The differential diagnosis of the disease includes secondary peritoneal carcinomatoses and some rare peritoneal conditions. Optimal elimination of mucin and the mucin-secreting tumor comprises the current standard of care for PMP offered in specialized centers as visceral resections and peritonectomy combined with intraperitoneal chemotherapy. This multidisciplinary approach has reportedly provided a median survival rate of 16.3 years, a median progression-free survival rate of 8.2 years and 10- and 15-year survival rates of 63% and 59%, respectively. Despite its indolent, bland nature as a neoplasm, PMP is a debilitating condition that severely impacts quality of life. It tends to be diagnosed at advanced stages and frequently recurs after treatment. Being ignored in research, however, PMP remains a challenging, enigmatic entity. Clinicopathological features of the PMP syndrome and its morbid complications closely correspond with the multifocal distribution of the secreted mucin collections and mucin-secreting implants. Novel strategies are thus required to facilitate macroscopic, as well as microscopic, elimination of mucin and its source as the key components of the disease. In this regard, MUC2, MUC5AC and MUC5B have been found as

  12. Adenoid Cystic Carcinoma of the Buccal Mucosa: A Case Report with Review of Literature

    PubMed Central

    S, Vidyalakshmi; R, Aravindhan

    2014-01-01

    Minor salivary gland neoplasms of the buccal mucosa are relatively uncommon. Adenoid cystic carcinoma (ACC), a well-defined entity, occurs most of the times in the parotid, submandibular glands and palate, as far as the intraoral site is concerned. Adenoid cystic carcinoma tends to have an indolent, extended clinical course with wide local infiltration and late distant metastases. We are presenting a case of an adenoid cystic carcinoma of the buccal mucosa in a 48-year-old female patient. PMID:24783155

  13. Excess Secretion of Gel-Forming Mucins and Associated Innate Defense Proteins with Defective Mucin Un-Packaging Underpin Gallbladder Mucocele Formation in Dogs.

    PubMed

    Kesimer, Mehmet; Cullen, John; Cao, Rui; Radicioni, Giorgia; Mathews, Kyle G; Seiler, Gabriela; Gookin, Jody L

    2015-01-01

    Mucosal protection of the gallbladder is vital yet we know very little about the mechanisms involved. In domestic dogs, an emergent syndrome referred to as gallbladder mucocele formation is characterized by excessive secretion of abnormal mucus that results in obstruction and rupture of the gallbladder. The cause of gallbladder mucocele formation is unknown. In these first mechanistic studies of this disease, we investigated normal and mucocele-forming dog gallbladders to determine the source, identity, biophysical properties, and protein associates of the culprit mucins with aim to identify causes for abnormal mucus behavior. We established that mucocele formation involves an adoptive excess secretion of gel forming mucins with abnormal properties by the gallbladder epithelium. The mucus is characterized by a disproportionally significant increase in Muc5ac relative to Muc5b, defective mucin un-packaging, and mucin-interacting innate defense proteins that are capable of dramatically altering the physical and functional properties of mucus. These findings provide an explanation for abnormal mucus behavior and based on similarity to mucus observed in the airways of people with cystic fibrosis, suggest that abnormal mechanisms for maintenance of gallbladder epithelial hydration may be an instigating factor for mucocele formation in dogs. PMID:26414376

  14. Excess Secretion of Gel-Forming Mucins and Associated Innate Defense Proteins with Defective Mucin Un-Packaging Underpin Gallbladder Mucocele Formation in Dogs

    PubMed Central

    Kesimer, Mehmet; Cullen, John; Cao, Rui; Radicioni, Giorgia; Mathews, Kyle G.; Seiler, Gabriela; Gookin, Jody L.

    2015-01-01

    Mucosal protection of the gallbladder is vital yet we know very little about the mechanisms involved. In domestic dogs, an emergent syndrome referred to as gallbladder mucocele formation is characterized by excessive secretion of abnormal mucus that results in obstruction and rupture of the gallbladder. The cause of gallbladder mucocele formation is unknown. In these first mechanistic studies of this disease, we investigated normal and mucocele-forming dog gallbladders to determine the source, identity, biophysical properties, and protein associates of the culprit mucins with aim to identify causes for abnormal mucus behavior. We established that mucocele formation involves an adoptive excess secretion of gel forming mucins with abnormal properties by the gallbladder epithelium. The mucus is characterized by a disproportionally significant increase in Muc5ac relative to Muc5b, defective mucin un-packaging, and mucin-interacting innate defense proteins that are capable of dramatically altering the physical and functional properties of mucus. These findings provide an explanation for abnormal mucus behavior and based on similarity to mucus observed in the airways of people with cystic fibrosis, suggest that abnormal mechanisms for maintenance of gallbladder epithelial hydration may be an instigating factor for mucocele formation in dogs. PMID:26414376

  15. Cysts and cystic-appearing lesions of the knee: A pictorial essay

    PubMed Central

    Telischak, Nicholas A; Wu, Jim S; Eisenberg, Ronald L

    2014-01-01

    Cysts and cystic-appearing lesions around the knee are common and can be divided into true cysts (synovial cysts, bursae, ganglia, and meniscal cysts) and lesions that mimic cysts (hematomas, seromas, abscesses, vascular lesions, and neoplasms). The specific anatomic location of the cystic lesion often permits the correct diagnosis. In difficult cases, identifying a cystic mass in an atypical location and/or visualizing internal solid contrast enhancement on magnetic resonance imaging (MRI) should raise concern for a neoplasm and the need for further evaluation and intervention. PMID:25024531

  16. Surgery for Malignant Sublingual and Minor Salivary Gland Neoplasms.

    PubMed

    Bradley, Patrick J; Ferris, Robert L

    2016-01-01

    Malignant sublingual gland neoplasms are rare, early-stage neoplasms presenting as painless non-ulcerated masses in the antero-lateral floor of the mouth. The majority of patients present with advanced disease, with symptoms of pain or anaesthesia of the tongue. Malignant minor salivary gland neoplasms are more common, the majority (>80%) of which present in the oral cavity, most frequently in the palatal area, as painless masses or as obstructive symptoms in the head and neck region. The most frequent pathologies are adenoid cystic carcinoma and mucoepidermoid carcinoma (>85%), with the majority presenting at an advanced stage (III/IV). Wide tumour-free surgical margin excision is the treatment of choice, followed by radiotherapy, after discussion of the multidisciplinary head and neck cancer tumour board. Improvements in survival and quality of life have been achieved since the introduction of endoscopic and robotic surgeries for many minor salivary gland malignancies. PMID:27092950

  17. Cystic Fibrosis Research

    MedlinePlus

    ... turn Javascript on. Feature: Steady Advances Against Cystic Fibrosis Cystic Fibrosis Research Past Issues / Fall 2012 Table of Contents "Remarkable strides in cystic fibrosis research over the past two decades have culminated ...

  18. Cystic renal tumors: new entities and novel concepts.

    PubMed

    Moch, Holger

    2010-05-01

    Cystic renal neoplasms and renal epithelial stromal tumors are diagnostically challenging and represent some novel tumor entities. In this article, clinical and pathologic features of established and novel entities are discussed. Predominantly cystic renal tumors include cystic nephroma/mixed epithelial and stromal tumor, synovial sarcoma, and multilocular cystic renal cell carcinoma. These entities are own tumor entities of the 2004 WHO classification of renal tumors. Tubulocystic carcinoma and acquired cystic disease-associated renal cell carcinoma are neoplasms with an intrinsically cystic growth pattern. Both tumor types should be included in a future WHO classification as novel entities owing to their characteristic features. Cysts and clear cell renal cell carcinoma frequently coexist within the kidneys of patients with von Hippel-Lindau disease. Sporadic clear cell renal cell carcinomas often contain cysts, usually as a minor component. Some clear cell renal cell carcinomas have prominent cysts, and multilocular cystic renal cell carcinoma is composed almost exclusively of cysts. Recent molecular findings suggest that clear cell renal cancer may develop through cyst-dependent and cyst-independent molecular pathways. PMID:20418675

  19. Rare condition of needle tract seeding after EUS-guided FNA for intraductal papillary mucinous carcinoma

    PubMed Central

    Yamabe, Akane; Irisawa, Atsushi; Shibukawa, Goro; Hoshi, Koki; Fujisawa, Mariko; Igarashi, Ryo; Sato, Ai; Maki, Takumi; Hojo, Hiroshi

    2016-01-01

    Background and study aims: We report on a 75-year-old man who was admitted due to pancreatic cystic lesion accompanied by a solid mass with liver metastasis. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) was performed on the solid mass, and pathological findings revealed the lesion to be an adenocarcinoma diagnosed as intraductal papillary mucinous carcinoma (IPMC). Approximately 3 months after, a cystic subepithelial lesion appeared in the posterior gastric wall where the EUS-FNA had been performed. We performed EUS-FNA again, which revealed that the cystic mass was IPMC with pathology similar to the original lesion. This is a rare case demonstrating needle tract seeding of EUS-FNA for IPMC.

  20. Pancreatic Cystic Fluid Reference Set — EDRN Public Portal

    Cancer.gov

    The proposed reference set will be designed to evaluate biomarkers for the following applications aimed at determining whether a patient would benefit from surgical resection of their cystic lesion: 1. Distinguish between pancreatic cysts that have high-malignant potential and cysts that have low or no malignant potential at time of endoscopic ultrasound evaluation. a. By analysis of blood b. By analysis of pancreatic cystic fluid 2. Distinguish between cysts that have any malignant potential (high and low) and cysts that have no malignant potential (benign lesions) at time of endoscopic ultrasound evaluation. a. By analysis of blood b. By analysis of pancreatic cystic fluid 3. Distinguish between a cyst that is mucinous (malignant potential) or non-mucinous (no malignant potential) at time of endoscopic ultrasound evaluation. a. By analysis of blood b. By analysis of pancreatic cystic fluid 4. Distinguish between pancreatic cysts that have high-malignant potential and cysts that have low or no malignant potential at time of evaluation in surgery clinic. a. By analysis of blood 5. Distinguish between cysts that have any malignant potential (high and low) and cysts that have no malignant potential (benign lesions) at time of evaluation in surgery clinic. a. By analysis of blood 6. Distinguish between a cyst that is mucinous (malignant potential) or non-mucinous (no malignant potential) at time of evaluation in surgery clinic. a. By analysis of blood If successful in meeting these afore-mentioned objectives, these results can then be used in the development of future studies at identifying the need for additional evaluation (i.e., endoscopic ultrasound with fine needle aspiration) of an incidental cyst

  1. Ocular Surface Membrane-Associated Mucins.

    PubMed

    Ablamowicz, Anna F; Nichols, Jason J

    2016-07-01

    Ocular surface epithelial cells produce and secrete mucins that form a hydrophilic barrier for protection and lubrication of the eye. This barrier, the glycocalyx, is formed by high molecular weight heavily glycosylated membrane-associated mucins (MAMs) that include MUC1, MUC4, and MUC16. These mucins extend into the tear film from the anterior surfaces of the conjunctiva and cornea, and, through interactions with galectin-3, prevent penetrance of pathogens into the eye. Due primarily to the glycosylation of the mucins, the glycocalyx also creates less friction during blinking and enables the tear film to maintain wetting of the eye. The secretory mucins include soluble MUC7 and gel-forming MUC5AC. These mucins, particularly MUC5AC, assist with removal of debris from the tear film and contribute to the hydrophilicity of the tear film. While new methodologies and cell culture models have expanded our understanding of mucin structure and function on the ocular surface, there is still a paucity of studies characterizing the glycosylation of MAMs on a normal ocular surface and a diseased ocular surface. Although studies have shown alterations in mucin production and expression in dry eye diseases, the relationship between changes in mucins and functional consequences is unclear. This review focuses on comparing what is known about MAMs in wet-surfaced epithelia of the body to what has been studied on the eye. PMID:27154035

  2. Adenoid Cystic Carcinoma of Accessory Parotid Gland: A Case Report.

    PubMed

    Das, Somdipto; Nayak, Umanath K; Buggavetti, Rahul; Sekhar, Shobana

    2016-05-01

    The accessory parotid gland is salivary gland tissue separated from the main gland at a variable distance. This gland is histologically similar to the main gland, but has a higher incidence of malignant neoplasms than the main gland. Regarding the various malignant neoplasms, studies have shown higher incidences of mucoepidermoid carcinoma, with less than 2% being adenoid cystic carcinoma. We present a case of swelling in the midcheek region that, after clinical examination, was diagnosed as a case of neoplasm of the accessory parotid gland. On the basis of auxiliary investigations including intraoperative frozen section, it was concluded that it was adenoid cystic carcinoma, grade I, and after wide surgical resection, the tumor was removed without undergoing superficial parotidectomy. The patient received postoperative radiotherapy (RT) and was followed for 14 months without any recurrence or substantial facial asymmetry. PMID:26851989

  3. [A Case of Mucinous Cystadenocarcinoma of the Retroperitoneum Supposed to Be Derived from Ectopic Ovarian Tissue].

    PubMed

    Inoue, Hirohide; Takagawa, Ryo; Matsuki, Hiroki; Horii, Nobutoshi; Suwa, Hirokazu; Suzuki, Yoshihiro; Ota, Mitsuyoshi; Kunisaki, Chikara; Tamagawa, Hiroshi; Sawazaki, Sho; Yukawa, Norio; Rino, Yasushi; Masuda, Munetaka

    2015-10-01

    Most tumors arising in the retroperitoneum are non-epithelial, and epithelial tumors are very rare. We report a case of mucinous cystadenocarcinoma of the retroperitoneum supposed to be derived from ectopic ovarian tissue. A 56-year-old woman was admitted to the hospital because of abdominal distention, and an abdominal CT scan revealed a cystic tumor of the right lower retroperitoneum. We had been observing the patient for 10 years for this lesion, but at this point, the tumor showed an increase in size and nodules had appeared inside of the cyst. It was presumed to be malignant, and so the decision was made to perform resection. Upon laparotomy, a large cystic tumor was found in the right iliac fossa displacing the ascending colon and the cecum medially. The cyst had no connection with any surrounding structures. The tumor was a simple large cyst, measuring 9×7 cm, with a protruding papillary tumor inside. The inner space of the cyst was filled with mucinous fluid. Histologically, the tumor was diagnosed as a mucinous cystadenocarcinoma. The patient has currently been undergoing follow-up for 7 months with no evidence of recurrence. PMID:26489567

  4. Mucinous Variant of Follicular Carcinoma of the Thyroid Gland: Case Report and Review of the Literature.

    PubMed

    Squillaci, Salvatore; Pitino, Antonio; Spairani, Cinzia; Ferrari, Mauro; Carlon, Eugenio; Cosimi, Maria Fabia

    2016-04-01

    The rare reports of mucinous variant of follicular carcinoma of the thyroid gland have not provided enough evidence to support the recognition of these tumors as a distinct clinicopathologic entity or to understand their etiopathogenesis. We report the fourth case of mucinous variant of follicular carcinoma displaying a minimally invasive tumor with diffuse expression of thyroglobulin, TTF-1, CD56, PAX-8, cytokeratins 7 and 19, in the absence of monoclonal carcinoembryonic antigen (CEA), cytokeratin 20, chromogranin, HBME-1, P63 expression, and BRAF gene mutation, in a 51-year-old woman who is alive without signs of disease 13 months after total thyroidectomy, bilateral neck dissection, and radioactive iodine. Herein, fine-needle aspiration cytology disclosed "worrisome" cytologic features consisting of large epithelial cells arranged in clusters or singularly, with high nucleocytoplasmic ratio, nuclear grooves and evident nucleoli which were shared by those of mucin-producing papillary thyroid carcinoma. Therefore, knowledge of the cytological and histopathological spectrum of this lesion is important to avoid misdiagnosis. The morphologic clues leading to the correct diagnosis of mucinous variant of follicular neoplasm have been correlated with the data of the literature, and the differential diagnosis is briefly discussed. PMID:26582770

  5. Mucin Binding Reduces Colistin Antimicrobial Activity

    PubMed Central

    Huang, Johnny X.; Blaskovich, Mark A. T.; Pelingon, Ruby; Ramu, Soumya; Kavanagh, Angela; Elliott, Alysha G.; Butler, Mark S.

    2015-01-01

    Colistin has found increasing use in treating drug-resistant bacterial lung infections, but potential interactions with pulmonary biomolecules have not been investigated. We postulated that colistin, like aminoglycoside antibiotics, may bind to secretory mucin in sputum or epithelial mucin that lines airways, reducing free drug levels. To test this hypothesis, we measured binding of colistin and other antibiotics to porcine mucin, a family of densely glycosylated proteins used as a surrogate for human sputum and airway mucin. Antibiotics were incubated in dialysis tubing with or without mucin, and concentrations of unbound antibiotics able to penetrate the dialysis tubing were measured over time using liquid chromatography-tandem mass spectrometry (LC-MS/MS). The percentage of antibiotic measured in the dialysate after 4 h in the presence of mucin, relative to the amount without mucin, was 15% for colistin, 16% for polymyxin B, 19% for tobramycin, 52% for ciprofloxacin, and 78% for daptomycin. Antibiotics with the strongest mucin binding had an overall polybasic positive charge, whereas those with comparatively little binding were less basic. When comparing MICs measured with or without added mucin, colistin and polymyxin B showed >100-fold increases in MICs for multiple Gram-negative bacteria. Preclinical evaluation of mucin binding should become a standard procedure when considering the potential pulmonary use of new or existing antibiotics, particularly those with a polybasic overall charge. In the airways, mucin binding may reduce the antibacterial efficacy of inhaled or intravenously administered colistin, and the presence of sub-MIC effective antibiotic concentrations could result in the development of antibiotic resistance. PMID:26169405

  6. Mucin Dynamics in Intestinal Bacterial Infection

    PubMed Central

    Lindén, Sara K.; Florin, Timothy H. J.; McGuckin, Michael A.

    2008-01-01

    Background Bacterial gastroenteritis causes morbidity and mortality in humans worldwide. Murine Citrobacter rodentium infection is a model for gastroenteritis caused by the human pathogens enteropathogenic Escherichia coli and enterohaemorrhagic E. coli. Mucin glycoproteins are the main component of the first barrier that bacteria encounter in the intestinal tract. Methodology/Principal Findings Using Immunohistochemistry, we investigated intestinal expression of mucins (Alcian blue/PAS, Muc1, Muc2, Muc4, Muc5AC, Muc13 and Muc3/17) in healthy and C. rodentium infected mice. The majority of the C. rodentium infected mice developed systemic infection and colitis in the mid and distal colon by day 12. C. rodentium bound to the major secreted mucin, Muc2, in vitro, and high numbers of bacteria were found in secreted MUC2 in infected animals in vivo, indicating that mucins may limit bacterial access to the epithelial surface. In the small intestine, caecum and proximal colon, the mucin expression was similar in infected and non-infected animals. In the distal colonic epithelium, all secreted and cell surface mucins decreased with the exception of the Muc1 cell surface mucin which increased after infection (p<0.05). Similarly, during human infection Salmonella St Paul, Campylobacter jejuni and Clostridium difficile induced MUC1 in the colon. Conclusion Major changes in both the cell-surface and secreted mucins occur in response to intestinal infection. PMID:19088856

  7. [Pancreatic neuroendocrine neoplasms].

    PubMed

    Beiderwellen, K; Sabet, A; Lauenstein, T C; Lahner, H; Poeppel, T D

    2016-04-01

    Pancreatic neuroendocrine neoplasms (NEN) account for 1-2% of all pancreatic neoplasms and represent a rare differential diagnosis. While some pancreatic NEN are hormonally active and exhibit endocrine activity associated with characteristic symptoms, the majority are hormonally inactive. Imaging techniques such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) or as combined PET/CT play a crucial role in the initial diagnosis, therapy planning and control. Endoscopic ultrasound (EUS) and multiphase CT represent the reference methods for localization of the primary pancreatic tumor. Particularly in the evaluation of small liver lesions MRI is the method of choice. Somatostatin receptor scintigraphy and somatostatin receptor PET/CT are of particular value for whole body staging and special aspects of further therapy planning. PMID:27003413

  8. Challenge in preoperative diagnosis of retroperitoneal mucinous cyst in a pediatric patient

    PubMed Central

    Zhang, Deying; Zhang, Yan; Liu, Xing; Zhu, Jin; Feng, Chuan; Yang, Chunjiang; Wu, Shengde; Liu, Junhong; Hua, Yi; Liu, Feng; Zhang, Nan; Zhang, Yuanyuan; He, Dawei; Lin, Tao; Wei, Guanghui

    2015-01-01

    Mucinous cystic lesions of the retroperitoneum can be either neoplastic or non-neoplastic. It is very important to make a correct diagnosis, or at least, an accurate classification, to proceed with an optimal treatment strategy. In spite of advantage of ultrasound and X-ray image examinations, it is still a challenge to make differential diagnosis of retroperitoneal mucinous cyst from gangliocytoma because both tumors have similar density under the image assessment. In this article, we reported an asymptomatic 8-year-old boy with multiple bronchogenic cysts in both lung and adrenal area on the left side, the latter was considered to be a gangliocytoma preoperatively by ultrasound and computed tomography, but confirmed as bronchogenic cyst by histopathology post laparoscopic resection. The differential diagnosis, imaging features and treatment of bronchogenic cyst are discussed and the relative literatures are reviewed. PMID:26770607

  9. Mycoplasma pneumoniae Modulates STAT3-STAT6/EGFR-FOXA2 Signaling To Induce Overexpression of Airway Mucins

    PubMed Central

    Hao, Yonghua; Kuang, Zhizhou; Jing, Jia; Miao, Jinfeng; Mei, Li Yu; Lee, Ryan J.; Kim, Susie; Choe, Shawn; Krause, Duncan C.

    2014-01-01

    Aberrant mucin secretion and accumulation in the airway lumen are clinical hallmarks associated with various lung diseases such as asthma, chronic obstructive pulmonary disease, and cystic fibrosis. Mycoplasma pneumoniae, long appreciated as one of the triggers of acute exacerbations of chronic pulmonary diseases, has recently been reported to promote excessive mucus secretion. However, the mechanism of mucin overproduction induced by M. pneumoniae remains unclear. This study aimed to determine the mechanism by which M. pneumoniae induces mucus hypersecretion by using M. pneumoniae infection of mouse lungs, human primary bronchial epithelial (NHBE) cells cultured at the air-liquid interface, and the conventionally cultured airway epithelial NCI-H292 cell line. We demonstrated that M. pneumoniae induced the expression of mucins MUC5AC and MUC5B by activating the STAT6-STAT3 and epidermal growth factor receptor (EGFR) signal pathways, which in turn downregulated FOXA2, a transcriptional repressor of mucin biosynthesis. The upstream stimuli of these pathways, including interleukin-4 (IL-4), IL-6, and IL-13, increased dramatically upon exposure to M. pneumoniae. Inhibition of the STAT6, STAT3, and EGFR signaling pathways significantly restored the expression of FOXA2 and attenuated the expression of airway mucins MUC5AC and MUC5B. Collectively, these studies demonstrated that M. pneumoniae induces airway mucus hypersecretion by modulating the STAT/EGFR-FOXA2 signaling pathways. PMID:25287927

  10. Small-Molecule Inhibition of GCNT3 Disrupts Mucin Biosynthesis and Malignant Cellular Behaviors in Pancreatic Cancer.

    PubMed

    Rao, Chinthalapally V; Janakiram, Naveena B; Madka, Venkateshwar; Kumar, Gaurav; Scott, Edgar J; Pathuri, Gopal; Bryant, Taylor; Kutche, Hannah; Zhang, Yuting; Biddick, Laura; Gali, Hariprasad; Zhao, Yan D; Lightfoot, Stan; Mohammed, Altaf

    2016-04-01

    Pancreatic cancer is an aggressive neoplasm with almost uniform lethality and a 5-year survival rate of 7%. Several overexpressed mucins that impede drug delivery to pancreatic tumors have been therapeutically targeted, but enzymes involved in mucin biosynthesis have yet to be preclinically evaluated as potential targets. We used survival data from human patients with pancreatic cancer, next-generation sequencing of genetically engineered Kras-driven mouse pancreatic tumors and human pancreatic cancer cells to identify the novel core mucin-synthesizing enzyme GCNT3 (core 2 β-1,6 N-acetylglucosaminyltransferase). In mouse pancreatic cancer tumors, GCNT3 upregulation (103-fold; P < 0.0001) was correlated with increased expression of mucins (5 to 87-fold; P < 0.04-0.0003). Aberrant GCNT3 expression was also associated with increased mucin production, aggressive tumorigenesis, and reduced patient survival, and CRISPR-mediated knockout of GCNT3 in pancreatic cancer cells reduced proliferation and spheroid formation. Using in silico small molecular docking simulation approaches, we identified talniflumate as a novel inhibitor that selectively binds to GCNT3. In particular, docking predictions suggested that three notable hydrogen bonds between talniflumate and GCNT3 contribute to a docking affinity of -8.3 kcal/mol. Furthermore, talniflumate alone and in combination with low-dose gefitinib reduced GCNT3 expression, leading to the disrupted production of mucins in vivo and in vitro Collectively, our findings suggest that targeting mucin biosynthesis through GCNT3 may improve drug responsiveness, warranting further development and investigation in preclinical models of pancreatic tumorigenesis. Cancer Res; 76(7); 1965-74. ©2016 AACR. PMID:26880801

  11. Mucins in pancreatic cancer and its microenvironment

    PubMed Central

    Kaur, Sukhwinder; Kumar, Sushil; Momi, Navneet; Sasson, Aaron R.; Batra, Surinder K.

    2014-01-01

    Pancreatic cancer remains a lethal malignancy with poor prognosis owing to therapeutic resistance, frequent recurrence and the absence of treatment strategies that specifically target the tumour and its supporting stroma. Deregulated cell-surface proteins drive neoplastic transformations and are envisioned to mediate crosstalk between the tumour and its microenvironment. Emerging studies have elaborated on the role of mucins in diverse biological functions, including enhanced tumorigenicity, invasiveness, metastasis and drug resistance through their characteristic O-linked and N-linked oligosaccharides (glycans), extended structures and unique domains. Multiple mucin domains differentially interact and regulate different components of the tumour microenvironment. This Review discusses: the expression pattern of various mucins in the pancreas under healthy, inflammatory, and cancerous conditions; the context-dependent attributes of mucins that differ under healthy and pathological conditions; the contribution of the tumour microenvironment in pancreatic cancer development and/or progression; diagnostic and/or prognostic efficacy of mucins; and mucin-based therapeutic strategies. Overall, this information should help to delineate the intricacies of pancreatic cancer by exploring the family of mucins, which, through various mechanisms in both tumour cells and the microenvironment, worsen disease outcome. PMID:23856888

  12. Biomimetic oral mucin from polymer micelle networks

    NASA Astrophysics Data System (ADS)

    Authimoolam, Sundar Prasanth

    Mucin networks are formed by the complexation of bottlebrush-like mucin glycoprotein with other small molecule glycoproteins. These glycoproteins create nanoscale strands that then arrange into a nanoporous mesh. These networks play an important role in ensuring surface hydration, lubricity and barrier protection. In order to understand the functional behavior in mucin networks, it is important to decouple their chemical and physical effects responsible for generating the fundamental property-function relationship. To achieve this goal, we propose to develop a synthetic biomimetic mucin using a layer-by-layer (LBL) deposition approach. In this work, a hierarchical 3-dimensional structures resembling natural mucin networks was generated using affinity-based interactions on synthetic and biological surfaces. Unlike conventional polyelectrolyte-based LBL methods, pre-assembled biotin-functionalized filamentous (worm-like) micelles was utilized as the network building block, which from complementary additions of streptavidin generated synthetic networks of desired thickness. The biomimetic nature in those synthetic networks are studied by evaluating its structural and bio-functional properties. Structurally, synthetic networks formed a nanoporous mesh. The networks demonstrated excellent surface hydration property and were able capable of microbial capture. Those functional properties are akin to that of natural mucin networks. Further, the role of synthetic mucin as a drug delivery vehicle, capable of providing localized and tunable release was demonstrated. By incorporating antibacterial curcumin drug loading within synthetic networks, bacterial growth inhibition was also demonstrated. Thus, such bioactive interfaces can serve as a model for independently characterizing mucin network properties and through its role as a drug carrier vehicle it presents exciting future opportunities for localized drug delivery, in regenerative applications and as bio

  13. Learning about Cystic Fibrosis

    MedlinePlus

    ... order to digest food. Cystic Fibrosis: A Single Gene Disease Mutations in a single gene - the Cystic ... the defective gene, or correcting the defective protein. Gene Therapy Research Offers Promise of a Cure for ...

  14. What Causes Cystic Fibrosis?

    MedlinePlus

    ... What Causes Cystic Fibrosis? A defect in the CFTR gene causes cystic fibrosis (CF). This gene makes ... and very salty sweat. Research suggests that the CFTR protein also affects the body in other ways. ...

  15. Diffuse Cystic Lung Diseases: Diagnostic Considerations.

    PubMed

    Xu, Kai-Feng; Feng, Ruie; Cui, Han; Tian, Xinlun; Wang, Hanping; Zhao, Jing; Huang, Hui; Zhang, Weihong; Lo, Bee Hong

    2016-06-01

    Diffuse cystic lung disease (DCLD) is a group of heterogeneous diseases that present as diffuse cystic changes in the lung on computed tomography of the chest. Most DCLD diseases are rare, although they might resemble common diseases such as emphysema and bronchiectasis. Main causes of DCLD include lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, pulmonary Langerhans cell histiocytosis, lymphoid interstitial pneumonia, amyloidosis, light-chain deposition disease, Sjögren syndrome, and primary or metastatic neoplasm. We discuss clinical factors that are helpful in the differential diagnosis of DCLDsuch as sex and age, symptoms and signs, extrapulmonary presentations, cigarette smoking, and family history. Investigations for DCLD include high-resolution computed tomography, biochemical and histopathological studies, genetic tests, pulmonary function tests, and bronchoscopic and video-assisted thoracoscopic biopsies. A proposed diagnostic algorithm would enhance ease of diagnosing most cases of DCLD. PMID:27231867

  16. Cystic fibrosis transmembrane conductance regulator protein expression in the male excretory duct system during development.

    PubMed

    Marcorelles, Pascale; Gillet, Danièle; Friocourt, Gaëlle; Ledé, Françoise; Samaison, Laura; Huguen, Geneviève; Ferec, Claude

    2012-03-01

    Sterility due to bilateral destruction in utero or in early infancy resulting in congenital absence of the vas deferens is the rule in male patients with cystic fibrosis. To understand the developmental pattern of this anomaly, the microscopic morphology of the male excretory system was analyzed during development and the expression of the cystic fibrosis transmembrane conductance regulator protein was explored by immunohistochemistry. We observed that cystic fibrosis fetuses had no excretory ducts agenesis or obstruction until 22 weeks of gestation. However, a focal inflammatory pattern and mucinous plugs in the oldest cystic fibrosis case suggested a disruptive mechanism. Immunolabeling of cytoplasmic epithelial cystic fibrosis transmembrane conductance regulator protein was demonstrated in all cystic fibrosis and control cases with a similar pattern of expression of the protein between age-matched controls and cystic fibrosis cases. At midgestation, an apical intensification appeared in both cystic fibrosis and control cases and was stable during the remainder of fetal life. No gradient of intensity could be detected between the different segments of the excretory tract. These findings are different from those reported in adults. The absence of any morphologic anomaly until 22 weeks of gestation, the focal destruction of the epithelial structures during the second trimester, and the chronological pattern of expression of cystic fibrosis transmembrane conductance regulator are of interest for a better understanding of the pathophysiology of this disease. PMID:21840567

  17. Mucinous Urothelial Carcinoma of the Renal Pelvis

    PubMed Central

    Behzatğlu, Kemal; Boyaci, Ceren; Okçu, Oğuzhan; Hacihasanoğlu, Ezgi; Çakir, Yasemin; Darakçi, Seher

    2014-01-01

    Urothelial carcinoma with abundant myxoid stroma is a newly-described and extremely rare entity. Since only very few cases have been reported, there is no consensus on its nomenclature. Microscopic examination revealed invasive urothelial carcinoma with widespread low-grade noninvasive areas. There were focal invasive areas in the neighborhood of the renal parenchyma. Malignant urothelial tumor/cell groups localized in the stroma had abundant myxoid/mucinous background in the invasive areas. The cytoplasm of the tumoral cells was more eosinophilic in these areas and the cells formed small groups and cords. Histochemically, PAS and Alcian Blue were positive in the cytoplasm of the tumoral cells and in the stroma while negative in the non-mucinous areas. Immunohistochemically, the tumoral cells of the mucinous invasive areas diffusely expressed MUC1 and MUC2. We discuss the origin of the mucinous/myxoid stroma, the tumor’s nature and its nomenclature with histochemical and immunohistochemical features. PMID:25568745

  18. Pancreatic Cystic Fluid Reference Set — EDRN Public Portal

    Cancer.gov

    The proposed reference set will be designed to evaluate biomarkers for the following applications aimed at determining whether a patient would benefit from surgical resection of their cystic lesion: 1.   Distinguish between pancreatic cysts that have high-malignant potential and cysts that have low or no malignant potential at time of endoscopic ultrasound evaluation. a.   By analysis of blood b.   By analysis of pancreatic cystic fluid 2.   Distinguish between cysts that have any malignant potential (high and low) and cysts that have no malignant potential (benign lesions) at time of endoscopic ultrasound evaluation. a.   By analysis of blood b.   By analysis of pancreatic cystic fluid 3.   Distinguish between a cyst that is mucinous (malignant potential) or non-mucinous (no malignant potential) at time of endoscopic ultrasound evaluation. a.   By analysis of blood b.   By analysis of pancreatic cystic fluid 4.   Distinguish between pancreatic cysts that have high-malignant potential and cysts that have low or no malignant potential at time of evaluation in surgery clinic. a.   By analysis of blood 5.   Distinguish between cysts that have any malignant potential (high and low) and cysts that have no malignant potential (benign lesions) at time of evaluation in surgery clinic. a.   By analysis of blood 6.   Distinguish between a cyst that is mucinous (malignant potential) or non-mucinous (no malignant potential) at time of evaluation in surgery clinic. a.   By analysis of blood If successful in meeting these afore-mentioned objectives, these results can then be used in the development of future studies at identifying the need for additional evaluation (i.e., endoscopic ultrasound with fine needle aspiration) of an incidental cystic lesion identified on abdominal imaging.

  19. Living with Cystic Fibrosis

    MedlinePlus

    ... from the NHLBI on Twitter. Living With Cystic Fibrosis If you or your child has cystic fibrosis (CF), you should learn as much as you ... about CF Care Centers, go to the Cystic Fibrosis Foundation's Care Center Network Web page. It's standard ...

  20. Peripheral Calcifying Cystic Odontogenic Tumour - A Rare Case Report

    PubMed Central

    Shenoi, Ramakrishna; Gadve, Vandana; Rajderkar, Anand; Dive, Alka

    2015-01-01

    Odontogenic lesions are derived from remnants of the components of the developing tooth germ. The calcifying cystic odontogenic tumour (CCOT) is a benign cystic neoplasm of odontogenic origin that is characterized by ameloblastoma-like epithelial cells and ghost cells. Most peripheral CCOTs are located in the anterior gingiva of the mandible or maxilla. This is a rare case report of CCOT. The rare feature in our case was its peripheral nature of existence and its location in the left buccal vestibule and retromolar region. Based on the radiological, cytological and histopathological findings the lesion was surgically excised. PMID:26393218

  1. Mouse gastric mucin: cloning and chromosomal localization.

    PubMed Central

    Shekels, L L; Lyftogt, C; Kieliszewski, M; Filie, J D; Kozak, C A; Ho, S B

    1995-01-01

    Mucins protect gastric epithelium by maintaining a favourable pH gradient and preventing autodigestion. The purpose of this study was to clone a mouse gastric mucin which would provide a foundation for analysis of mucin gene regulation. Mucin was purified from the glandular portion of gastric specimens and deglycosylated by HF solvolysis. Antibodies against native and deglycosylated mouse gastric mucin (MGM) were raised in chickens. Screening of a mouse stomach cDNA library with the anti-(deglycosylated MGM) antibody yielded partial clones containing a 48 bp tandem repeat and 768 bp of non-repetitive sequence. The 16-amino-acid tandem repeat has a consensus sequence of QTSSPNTGKTSTISTT with 25% serine and 38% threonine. The MGM tandem repeat sequence bears no similarity to previously identified mucins. The MGM non-repetitive region shares sequence similarity with human MUC5AC and, to a lesser extent, human MUC2 and rat intestinal mucin. Northern blot analysis reveals a polydisperse message beginning at 13.5 kb in mouse stomach with no expression in oesophagus, trachea, small intestine, large intestine, caecum, lung or kidney. Immunoreactivity of antibodies against deglycosylated MGM and against a synthetic MGM tandem repeat peptide was restricted to superficial mucous cells, antral glands and Brunner's glands in the pyloric-duodenal region. DNA analysis shows that MGM recognizes mouse and rat DNA but not hamster, rabbit or human DNA. The MGM gene maps to a site on mouse chromosome 7 homologous to the location of a human secretory mucin gene cluster on human chromosome 11p15. Due to sequence similarity and predominant expression in the stomach, the MGM gene may be considered a MUC5AC homologue and named Muc5ac. Images Figure 1 Figure 6 Figure 7 Figure 8 Figure 9 Figure 10 PMID:7487932

  2. Abnormal fucosylation of-ileal mucus in cystic fibrosis: II. A histochemical study using monoclonal antibodies to fucosyl oligosaccharides.

    PubMed Central

    King, A; McLeish, M; Thiru, S

    1990-01-01

    Abnormal fucosylation of cystic fibrosis mucin was previously shown using peroxidase conjugated lectins on ileal tissue sections. These abnormally fucosylated glycoproteins were investigated further using monoclonal antibodies to fucosyl oligosaccharides based on type 1 and type 2 blood group precursor chains. The results of this study, using monoclonal antibodies to blood group glycoproteins in cystic fibrosis, were negative, yet abnormal fucosylation had been found using lectin histochemistry. Using monoclonal antibodies, lectins, and appropriate enzymes, such as glycosyl hydrolases, it should be possible to delineate further the abnormality found in glycoproteins in cystic fibrosis on appropriately fixed ileal sections, obtained from infants at term presenting with meconium ileus. Images PMID:2266174

  3. Apoptosis deregulation in myeloproliferative neoplasms

    PubMed Central

    Tognon, Raquel; Nunes, Natália de Souza; de Castro, Fabíola Attié

    2013-01-01

    ABSTRACT Philadelphia-chromosome negative chronic myeloproliferative neoplasms are clonal hematologic diseases characterized by hematopoietic progenitor independence from or hypersensitivity to cytokines. The cellular and molecular mechanisms involved in the pathophysiology of myeloproliferative neoplasms have not yet been fully clarified. Pathophysiologic findings relevant for myeloproliferative neoplasms are associated with genetic alterations, such as, somatic mutation in the gene that codifies JAK-2 (JAK V617F). Deregulation of the process of programmed cellular death, called apoptosis, seems to participate in the pathogenesis of these disorders. It is known that expression deregulation of pro- and anti-apoptotic genes promotes cell resistance to apoptosis, culminating with the accumulation of myeloid cells and establishing neoplasms. This review will focus on the alterations in apoptosis regulation in myeloproliferative neoplasms, and the importance of a better understanding of this mechanism for the development of new therapies for these diseases. PMID:24488400

  4. Low-grade mucinous neoplasia in a cecal diverticulum: A case report

    PubMed Central

    Nakatani, Kazuyoshi; Tokuhara, Katsuji; Sakaguchi, Tatsuma; Ryota, Hironori; Yoshioka, Kazuhiko; Kon, Masanori

    2015-01-01

    Introduction Low-grade mucinous neoplasia is an uncommon benign tumor that develops in the appendix. The development of mucocele disease has never been reported in a colonic diverticulum. We present a case developing low-grade mucinous neoplasia in a cecal diverticulum. Presentation of case A tumor in the ileocecal region was found during a medical examination of a 66-year-old woman. Three months later, the tumor was still present and the patient developed abdominal pain. Laparoscopic ileocecal resection with D2 lymph node dissection was performed. Histopathological examination revealed a low-grade mucinous neoplasm in a cecal diverticulum. Discussion Colonic mucoceles reportedly originate from the appendix. There are no previous reports of mucocele disease in a colonic diverticulum worldwide. This report reviews and discusses the management of the appendiceal mucoceles. Conclusion The incidence of colonic diverticula has recently begun to increase in Japan. The possibility of a mucocele within a colonic diverticulum should be considered in patients with submucosal colonic tumors. PMID:26318130

  5. Oxidation increases mucin polymer cross-links to stiffen airway mucus gels

    PubMed Central

    Yuan, Shaopeng; Hollinger, Martin; Lachowicz-Scroggins, Marrah E.; Kerr, Sheena C.; Dunican, Eleanor M.; Daniel, Brian M.; Ghosh, Sudakshina; Erzurum, Serpel C.; Willard, Belinda; Hazen, Stanley L.; Huang, Xiaozhu; Carrington, Stephen D.; Oscarson, Stefan; Fahy, John V.

    2015-01-01

    Airway mucus in cystic fibrosis (CF) is highly elastic, but the mechanism behind this pathology is unclear. We hypothesized that the biophysical properties of CF mucus are altered because of neutrophilic oxidative stress. Using confocal imaging, rheology, and biochemical measures of inflammation and oxidation, we found that CF airway mucus gels have a molecular architecture characterized by a core of mucin covered by a web of DNA and a rheological profile characterized by high elasticity that can be normalized by chemical reduction. We also found that high levels of reactive oxygen species in CF mucus correlated positively and significantly with high concentrations of the oxidized products of cysteine (disulfide cross-links). To directly determine whether oxidation can cross-link mucins to increase mucus elasticity, we exposed induced sputum from healthy subjects to oxidizing stimuli and found a marked and thiol-dependent increase in sputum elasticity. Targeting mucin disulfide cross-links using current thiol-amino structures such as N-acetylcysteine (NAC) requires high drug concentrations to have mucolytic effects. We therefore synthesized a thiol-carbohydrate structure (methyl 6-thio-6-deoxy-α-D-galactopyranoside) and found that it had stronger reducing activity than NAC and more potent and fast-acting mucolytic activity in CF sputum. Thus, oxidation arising from airway inflammation or environmental exposure contributes to pathologic mucus gel formation in the lung, which suggests that it can be targeted by thiol-modified carbohydrates. PMID:25717100

  6. Oxidation increases mucin polymer cross-links to stiffen airway mucus gels.

    PubMed

    Yuan, Shaopeng; Hollinger, Martin; Lachowicz-Scroggins, Marrah E; Kerr, Sheena C; Dunican, Eleanor M; Daniel, Brian M; Ghosh, Sudakshina; Erzurum, Serpel C; Willard, Belinda; Hazen, Stanley L; Huang, Xiaozhu; Carrington, Stephen D; Oscarson, Stefan; Fahy, John V

    2015-02-25

    Airway mucus in cystic fibrosis (CF) is highly elastic, but the mechanism behind this pathology is unclear. We hypothesized that the biophysical properties of CF mucus are altered because of neutrophilic oxidative stress. Using confocal imaging, rheology, and biochemical measures of inflammation and oxidation, we found that CF airway mucus gels have a molecular architecture characterized by a core of mucin covered by a web of DNA and a rheological profile characterized by high elasticity that can be normalized by chemical reduction. We also found that high levels of reactive oxygen species in CF mucus correlated positively and significantly with high concentrations of the oxidized products of cysteine (disulfide cross-links). To directly determine whether oxidation can cross-link mucins to increase mucus elasticity, we exposed induced sputum from healthy subjects to oxidizing stimuli and found a marked and thiol-dependent increase in sputum elasticity. Targeting mucin disulfide cross-links using current thiol-amino structures such as N-acetylcysteine (NAC) requires high drug concentrations to have mucolytic effects. We therefore synthesized a thiol-carbohydrate structure (methyl 6-thio-6-deoxy-α-D-galactopyranoside) and found that it had stronger reducing activity than NAC and more potent and fast-acting mucolytic activity in CF sputum. Thus, oxidation arising from airway inflammation or environmental exposure contributes to pathologic mucus gel formation in the lung, which suggests that it can be targeted by thiol-modified carbohydrates. PMID:25717100

  7. Sarcoma-Like Mural Nodule in a Borderline Mucinous Tumour of Ovary.

    PubMed

    Ahmed, Rabia; Din, Hafeez Ud; Hashmi, Shoaib Naiyar; Muhammad, Iqbal

    2016-05-01

    A 37-year female presented with complaint of lower abdominal pain and amenorrhoea to the Military Hospital, Rawalpindi. Ultrasound of pelvis showed a right adnexal cystic lesion. On investigation, CA-125 was raised. Her MRI scan of pelvis revealed a right adnexal mass of fluid intensity measuring 15.2 x 12.9 x 9.2 cm. Right ovarian cystectomy was performed and the specimen was sent for histopathological examination. Grossly, the mass was cystic in appearance and measured 13.5 x 10 x 10 cm. On sectioning, it was unilocular and filled with turbid material. The cyst wall showed multiple papillary structures along with a nodule measuring 1.1 x 1 cm. Microscopically, the sections revealed borderline mucinous tumour with a sarcoma-like mural nodule composed of spindle shaped cells and epulis-like giant cells. Sarcoma-like mural nodules are rare nodules which are associated with mucinous tumours of the ovary. However, they have excellent prognosis and should not be interpreted as malignant. PMID:27225154

  8. Ovarian Sertoli-Leydig Cell Tumor with Predominant Heterologous Mucinous Differentiation and Foci of Hepatocytic Differentiation: Case Report and Review of The Literature.

    PubMed

    Liang, Li; Menzin, Andrew; Lovecchio, John Louis; Navarro, Maria D

    2015-01-01

    Sertoli-Leydig cell tumor is a rare ovarian neoplasm and belongs to the group of sex cord stromal tumors. We present a case of a 15-year old girl diagnosed with Sertoli-Leydig cell tumor with heterologous elements consisting predominantly of mucinous epithelium and a sparse Sertoli-Leydig cell component, mimicking mucinous neoplasm. Furthermore, foci of hepatocytic differentiation were also identified. Immunohistochemical stains showed the component of Sertoli cell differentiation was positive for cytokeratin 18 and inhibin. The component of Leydig cell differentiation was strongly positive for inhibin. The component of hepatocytic differentiation was positive for low molecular weight keratin, HepPar1, alpha-fetoprotein and weakly positive for inhibin. Thus, this was a very rare case which created a challenge for pathologists, especially on frozen sections. PMID:26116602

  9. Contrast-enhanced ultrasound improves accurate identification of appendiceal mucinous adenocarcinoma in an old patient

    PubMed Central

    Shang, Jing; Ruan, Li-tao; Dang, Ying; Wang, Yun-yue; Song, Yan; Lian, Jie

    2016-01-01

    Abstract Background: Adenocarcinoma of appendiceal origin is far rarer than other colorectal carcinomas and its preoperative diagnosis is challenging. To our knowledge, utility of contrast-enhanced ultrasound (CEUS) to diagnose it is much less. Method: A 61-year-old man presented with abdominal pain in the right lower quadrant for 20 days. In order to fulfill an accurately preoperative diagnosis, he received laboratory and imaging tests such as carcinoembryonic antigen (CEA), computer tomography (CT), CEUS and endoscope. Diagnosis and Intervention: He was initially suspected of suffering appendicitis, while his white blood cell count was normal and carcinoembryonic antigen (CEA) in serum was remarkably increased. Both routine ultrasound and computer tomography (CT) examinations supported suppurative appendicitis. The overall data, however, failed to excluded neoplastic pathology thoroughly. Therefore, CEUS was carried out and showed an inhomogeneous enhancement intra the lesion located in the body of the appendix, which made our consideration of neoplasm. The result of the follow-up biopsy guided by endoscope was consistent with appendiceal tumor. The patient received laparoscopic right hemicolectomy. Histopathology confirmed as well differentiated mucinous adenocarcinoma of appendix origin. His postoperative course was uneventful, and he had a regular diet again without any complaint. Result: Serum CEA was remarkably increased (12.00 ng/mL). Both routine ultrasound and CT examinations supported suppurative appendicitis. However, CEUS examination showed an inhomogeneous enhancement intra the lesion located in the body of the appendix, which made our consideration of neoplasm. The follow-up biopsy guided by endoscope and surgical specimens confirmed as well differentiated mucinous adenocarcinoma of appendix origin. Conclusion: Most mucinous adenocarcinoma mimicking appendicitis results in difficult diagnosis preoperatively. Clinician and radiologist should be

  10. Genome-wide mutational landscape of mucinous carcinomatosis peritonei of appendiceal origin

    PubMed Central

    2014-01-01

    Background Mucinous neoplasms of the appendix (MNA) are rare tumors which may progress from benign to malignant disease with an aggressive biological behavior. MNA is often diagnosed after metastasis to the peritoneal surfaces resulting in mucinous carcinomatosis peritonei (MCP). Genetic alterations in MNA are poorly characterized due to its low incidence, the hypo-cellularity of MCPs, and a lack of relevant pre-clinical models. As such, application of targeted therapies to this disease is limited to those developed for colorectal cancer and not based on molecular rationale. Methods We sequenced the whole exomes of 10 MCPs of appendiceal origin to identify genome-wide somatic mutations and copy number aberrations and validated significant findings in 19 additional cases. Results Our study demonstrates that MNA has a different molecular makeup than colorectal cancer. Most tumors have co-existing oncogenic mutations in KRAS (26/29) and GNAS (20/29) and are characterized by downstream PKA activation. High-grade tumors are GNAS wild-type (5/6), suggesting they do not progress from low-grade tumors. MNAs do share some genetic alterations with colorectal cancer including gain of 1q (5/10), Wnt, and TGFβ pathway alterations. In contrast, mutations in TP53 (1/10) and APC (0/10), common in colorectal cancer, are rare in MNA. Concurrent activation of the KRAS and GNAS mediated signaling pathways appears to be shared with pancreatic intraductal papillary mucinous neoplasm. Conclusions MNA genome-wide mutational analysis reveals genetic alterations distinct from colorectal cancer, in support of its unique pathophysiology and suggests new targeted therapeutic opportunities. PMID:24944587

  11. Mucinous cystadenoma of the appendix associated with adenocarcinoma of the sigmoid colon and hepatocellular carcinoma of the liver: Report of a case

    PubMed Central

    Djuranovic, Srdjan P; Spuran, Milan M; Kovacevic, Nada V; Ugljesic, Milenko B; Kecmanovic, Dragutin M; Micev, Marjan T

    2006-01-01

    Mucinous cystadenoma of the appendix is a rare condition and represents one of the three entities with the common name mucocele of the appendix. It is characterized by a cystic dilatation of the lumen with stasis of mucus inside it. Histopathologically mucocele is divided into three groups: focal or diffuse mucosal hyperplasia, mucinous cystadenoma and mucinous cystadenocarcinoma. This condition is often associated with other neoplasia, especially adenocarcinoma of the colon and ovaries. We here describe a 57 year old male patient who presented with abdominal discomfort, constipation, fresh blood in stool and frequent urination. He had a big cystadenoma of the appendix associated with adenocarcinoma of the colon and hepatocellular carcinoma of the liver. The patient underwent right haemicolectomy, sigmoid colon resection and segmental resection of the liver. Now 3 years later he has no evidence of disease relapse. According to this, we stress the need of accurate preoperative diagnosis and intraoperative exploration of the whole abdomen in these patients. PMID:16610012

  12. Regulated Mucin Secretion from Airway Epithelial Cells

    PubMed Central

    Adler, Kenneth B.; Tuvim, Michael J.; Dickey, Burton F.

    2013-01-01

    Secretory epithelial cells of the proximal airways synthesize and secrete gel-forming polymeric mucins. The secreted mucins adsorb water to form mucus that is propelled by neighboring ciliated cells, providing a mobile barrier which removes inhaled particles and pathogens from the lungs. Several features of the intracellular trafficking of mucins make the airway secretory cell an interesting comparator for the cell biology of regulated exocytosis. Polymeric mucins are exceedingly large molecules (up to 3 × 106 Da per monomer) whose folding and initial polymerization in the ER requires the protein disulfide isomerase Agr2. In the Golgi, mucins further polymerize to form chains and possibly branched networks comprising more than 20 monomers. The large size of mucin polymers imposes constraints on their packaging into transport vesicles along the secretory pathway. Sugar side chains account for >70% of the mass of mucins, and their attachment to the protein core by O-glycosylation occurs in the Golgi. Mature polymeric mucins are stored in large secretory granules ∼1 μm in diameter. These are translocated to the apical membrane to be positioned for exocytosis by cooperative interactions among myristoylated alanine-rich C kinase substrate, cysteine string protein, heat shock protein 70, and the cytoskeleton. Mucin granules undergo exocytic fusion with the plasma membrane at a low basal rate and a high stimulated rate. Both rates are mediated by a regulated exocytic mechanism as indicated by phenotypes in both basal and stimulated secretion in mice lacking Munc13-2, a sensor of the second messengers calcium and diacylglycerol (DAG). Basal secretion is induced by low levels of activation of P2Y2 purinergic and A3 adenosine receptors by extracellular ATP released in paracrine fashion and its metabolite adenosine. Stimulated secretion is induced by high levels of the same ligands, and possibly by inflammatory mediators as well. Activated receptors are coupled to

  13. Development and Validation of a Mass Spectrometry-Based Assay for the Molecular Diagnosis of Mucin-1 Kidney Disease.

    PubMed

    Blumenstiel, Brendan; DeFelice, Matthew; Birsoy, Ozge; Bleyer, Anthony J; Kmoch, Stanislav; Carter, Todd A; Gnirke, Andreas; Kidd, Kendrah; Rehm, Heidi L; Ronco, Lucienne; Lander, Eric S; Gabriel, Stacey; Lennon, Niall J

    2016-07-01

    Mucin-1 kidney disease, previously described as medullary cystic kidney disease type 1 (MCKD1, OMIM 174000), is an autosomal dominant tubulointerstitial kidney disease recently shown to be caused by a single-base insertion within the variable number tandem repeat region of the MUC1 gene. Because of variable age of disease onset and often subtle signs and symptoms, clinical diagnosis of mucin-1 kidney disease and differentiation from other forms of hereditary kidney disease have been difficult. The causal insertion resides in a variable number tandem repeat region with high GC content, which has made detection by standard next-generation sequencing impossible to date. The inherently difficult nature of this mutation required an alternative method for routine detection and clinical diagnosis of the disease. We therefore developed and validated a mass spectrometry-based probe extension assay with a series of internal controls to detect the insertion event using 24 previously characterized positive samples from patients with mucin-1 kidney disease and 24 control samples known to be wild type for the variant. Validation results indicate an accurate and reliable test for clinically establishing the molecular diagnosis of mucin-1 kidney disease with 100% sensitivity and specificity across 275 tests called. PMID:27157321

  14. Myeloproliferative Neoplasms in Children

    PubMed Central

    Hofmann, Inga

    2015-01-01

    Myeloproliferative neoplasms (MPN) are a group of clonal hematopoietic stem cell disorders characterized by aberrant proliferation of one or more myeloid lineages often with increased immature cells in the peripheral blood. The three classical BCR-ABL-negative MPNs are: 1) polycythemia vera (PV), 2) essential thrombocythemia (ET), and 3) primary myelofibrosis (PMF), which are typically disorders of older adults and are exceedingly rare in children. The diagnostic criteria for MPNs remain largely defined by clinical, laboratory and histopathology assessments in adults, but they have been applied to the pediatric population. The discovery of the JAK2 V617F mutation, and more recently, MPL and CALR mutations, are major landmarks in the understanding of MPNs. Nevertheless, they rarely occur in children, posing a significant diagnostic challenge given the lack of an objective, clonal marker. Therefore, in pediatric patients, the diagnosis must rely heavily on clinical and laboratory factors, and exclusion of secondary disorders to make an accurate diagnosis of MPN. This review focuses on the clinical presentation, diagnostic work up, differential diagnosis, treatment and prognosis of the classical BCR-ABL-negative MPNs (PV, ET and PMF) in children and highlights key differences to the adult diseases. Particular attention will be given to pediatric PMF, as it is the only disorder of this group that is observed in infants and young children, and in many ways appears to be a unique entity compared to adult PMF. PMID:26609329

  15. Myeloproliferative neoplasms and thrombosis.

    PubMed

    Barbui, Tiziano; Finazzi, Guido; Falanga, Anna

    2013-09-26

    Major causes of morbidity and mortality in myeloproliferative neoplasms are represented by arterial and venous complications, progression to myelofibrosis, and transformation to acute leukemia. The pathogenesis of thrombosis results from a complex interplay of clinical and disease-related factors. Abnormalities of blood cells arising from the clonal proliferation of hematopoietic stem cells involve not only quantitative changes but also qualitative modifications that characterize the switch of these cells from a resting to a procoagulant phenotype. According to age and previous thrombosis, patients are classified in a "high risk" or "low risk". Novel disease-related determinants such as leukocytosis and JAK2V617F mutational status and/or mutational burden are now under active investigation. In low-risk polycythemia vera patients, only phlebotomy and primary antithrombotic prophylaxis with aspirin is recommended, while in high-risk patients cytotoxic therapy is considered. Whether novel drugs targeting the constitutively active JAK2/STAT pathway will improve the management of thrombosis is a challenge for future studies. PMID:23823316

  16. Myeloproliferative Neoplasms (MPNs) Patient Registry

    ClinicalTrials.gov

    2016-04-28

    Primary Myelofibrosis; Polycythemia Vera; Essential Thrombocythemia; Mastocytosis; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative; Leukemia, Myelomonocytic, Juvenile; Chronic Eosinophilic Leukemia-not Otherwise Specified; Myelodysplastic-Myeloproliferative Diseases; Neoplasms; Leukemia, Myelomonocytic, Chronic

  17. Molecular analysis of mucinous nonneoplastic cyst of the pancreas.

    PubMed

    Zhu, Bing; Finkelstein, Sydney D; Feng, Gong; Keswani, Rajesh N; Lin, Xiaoqi

    2016-09-01

    Although a mucinous nonneoplastic cyst (MNNC) of the pancreas is defined as a benign nonneoplastic lesion with no malignant potential, its histogenesis and etiology are still uncertain. To explore the origin and development of MNNC, we searched for neoplasia-associated mutational change in oncogene and tumor suppressor genes. Specifically, we analyzed KRAS oncogene mutations by polymerase chain reaction/dideoxy DNA (Sanger) sequencing and tumor suppression gene deletion by loss of heterozygosity (LOH) using polymerase chain reaction/capillary gel electrophoresis for a panel of 16 polymorphic microsatellite repeat markers targeting common tumor suppression gene loci at 1p, 3p, 5q, 9p, 10q, 17p, 17q, 18q, 21q, and 22q on DNA isolated from the cystic lining epithelium microdissected from 15 surgically diagnosed MNNCs by microdissection of unstained histologic sections of fixed resection specimens. DNA mutations were demonstrated in 4 of 15 cases: 1 with KRAS mutation at codon 12 glycine (G) substitution by aspartic acid (D) (G12D), 1 with KRAS mutation at 12 glycine (G) substitution by arginine (R) (G12R), 1 with LOH at 10q (PTEN), and 1 with LOH at 17q (RNF43). Therefore, although the genomic mutation rate detected in MNNC is relatively low, our results indicate that MNNCs may acquire genetic alteration similar to low-grade pancreatic intraepithelial neoplasia, furthering debate of the true nature of these lesions. PMID:27246174

  18. Pyocyanin-induced mucin production is associated with redox modification of FOXA2

    PubMed Central

    2013-01-01

    Background The redox-active pyocyanin (PCN) is a toxic, secondary metabolite secreted by the respiratory pathogen Pseudomonas aeruginosa (PA). Previously, we have shown that mouse lungs chronically exposed to PCN develop goblet cell hyperplasia and metaplasia (GCHM) and mucus hypersecretion, fibrosis and emphysema. These pathological features are commonly found in the airways of several chronic lung diseases, including cystic fibrosis (CF), as well as in mouse airways deficient in the forkhead box A2 (FOXA2), a transcriptional repressor of goblet GCHM and mucus biosynthesis. Furthermore, PCN inhibits FOXA2 by activating the pro-GCHM signaling pathways Stat6 and EGFR. However, it is not known whether PCN-generated reactive oxygen (ROS) and nitrogen (RNS) species posttranslationally modify and inactivate FOXA2. Methods We examined the posttranslational modifications of FOXA2 by PCN using specific antibodies against oxidation, nitrosylation, acetylation and ubiquitination. Electrophoretic mobility shift assay (EMSA) was used to examine the ability of modified FOXA2 to bind the promoter of MUC5B mucin gene. In addition, we used quantitative real time PCR, ELISA, immunofluorescence and mouse lung infection to assess whether the loss of FOXA2 function caused GCHM and mucin overexpression. Finally, we examined the restoration of FOXA2 function by the antioxidant glutathione (GSH). Results We found that PCN-generated ROS/RNS caused nitrosylation, acetylation, ubiquitination and degradation of FOXA2. Modified FOXA2 had reduced ability to bind the promoter of the MUC5B gene. The antioxidant GSH alleviated the modification of FOXA2 by PCN, and inhibited the overexpression of MUC5AC and MUC5B mucins. Conclusion These results suggest that PCN-mediated posttranslational modifications of FOXA2 are positively correlated with GCHM and overexpression of airway mucins. Furthermore, antioxidant treatment restores the function of FOXA2 to attenuate GCHM and mucus hypersecretion. PMID

  19. [Cystic renal pathology].

    PubMed

    Rosi, P; Cesaroni, M; Bracarda, S; Rociola, W; Virgili, G

    1993-08-01

    Ultrasonography has a great interest in diagnosis of cystic kidney disorders for typical eco-pattern of this pathology. In this work we show the eco-pattern of the most common cystic kidney disorders. Particularly we examine simple cysts (typical, atypical, complicated), multicystic kidney dysplasia, autosomal recessive polycystic kidney disease (infantile) autosomal dominant polycystic kidney disease (adult age). The so-called neoplastic cysts (multiloculated cysts, multiloculated cysts nephroma, cystic nephroblastoma), medullar cysts (medullary sponge kidney, medullary cystic disease), parapyelic cysts, acquired cystic kidney disease in renal failure patients, parasitic cysts, epidermoid cysts. About this disorders we present the more typical and expressive ultrasonographic appearance and we define the role and the opportunity of diagnostic setting by echography, moreover ultrasonography allows us to make a differential diagnosis between cystic kidney disorders and other kidney disease. PMID:8353538

  20. How Is Cystic Fibrosis Diagnosed?

    MedlinePlus

    ... from the NHLBI on Twitter. How Is Cystic Fibrosis Diagnosed? Doctors diagnose cystic fibrosis (CF) based on ... to see whether the baby has CF. Cystic Fibrosis Carrier Testing People who have one normal CFTR ...

  1. Mucinous borderline ovarian tumor with ascites.

    PubMed

    Batool, Tahira; Ullah, Nasreen Rehmat

    2014-11-01

    Borderline mucinous tumors are epithelial ovarian tumors with low rate of growth and low potential to invade or metastasize and associated with significantly better prognosis and excellent disease-free survival after surgical removal than other epithelial ovarian cancers. The accepted initial treatment is surgical removal of the tumor. Fertility-sparing surgery may suffice in young patients with tumors confined to the ovary. Radical surgery is recommended in patients with advanced disease and advanced age. Long-term surveillance is recommended to document and treat late recurrences. We report a case of a 59 years old postmenopausal patient with complex ovarian mucinous tumor and gross ascites; she had received three lines of chemotherapeutic agents pre-operatively, without any favorable outcome. Then, she went for staging laparotomy and histopathology showed borderline ovarian mucinous tumor required no further treatment and is fine till date. PMID:25518783

  2. In vitro utilization of mucin by Bacteroides fragilis.

    PubMed Central

    Roberton, A M; Stanley, R A

    1982-01-01

    A method for isolating pig colon mucin in a soluble high-molecular-weight form, suitable for addition to bacterial growth media, is described. This preparation was utilized as a sole carbohydrate energy source by two strains of Bacteroides fragilis. The extent of degradation was compared with that of commercial pig gastric mucin by the same strains. Gas-liquid chromatographic analysis of the mucin carbohydrates and gel chromatography of the preparations were carried out before and after in vitro degradation. The mucin carbohydrates were utilized only to a very limited extent, colon mucin being more resistant to degradation than gastric mucin. Both mucins chromatographed at or near the excluded volume on Sepharose 4B, and only in the case of ATCC 25285 grown on gastric mucin was a significant degradation peak detected. If mucins are degraded in vivo by the sequential action of several bacteria, a pure culture in vitro might be expected to degrade mucins to a limited extent only. Techniques previously used to examine mucin utilization by pure cultures may have overlooked limited mucin degradation demonstrated by the methods used in this work. PMID:6174077

  3. Actin - Lysozyme Interactions in Model Cystic Fibrosis Sputum

    NASA Astrophysics Data System (ADS)

    Sanders, Lori; Slimmer, Scott; Angelini, Thomas; Wong, Gerard C. L.

    2003-03-01

    Cystic fibrosis sputum is a complex fluid consisting of mucin (a glycoprotein), lysozyme (a cationic polypeptide), water, salt, as well as a high concentration of a number of anionic biological polyelectrolytes such as DNA and F-actin. The interactions governing these components are poorly understood, but may have important clinical consequences. For example, the formation of these biological polyelectrolytes into ordered gel phases may contribute significantly to the observed high viscosity of CF sputum. In this work, a number of model systems containing actin, lysozyme, and KCl were created to simulate CF sputum in vitro. These model systems were studied using small angle x-ray scattering and confocal fluorescence microscopy. Preliminary results will be presented. This work was supported by NSF DMR-0071761, the Beckman Young Investigator Program, and the Cystic Fibrosis Foundation.

  4. Polyelectrolyte Structure and Interactions in Model Cystic Fibrosis Sputum

    NASA Astrophysics Data System (ADS)

    Slimmer, Scott; Angelini, Thomas; Liang, Hongjun; Butler, John; Wong, Gerard C. L.

    2002-03-01

    Cystic fibrosis sputum is a complex fluid consisting of a number of components, including mucin (a glycoprotein), lysozyme (a cationic polypeptide), water, salt, as well as a high concentration of a number of anionic biological polyelectrolytes such as DNA and F-actin. The interactions governing these components are poorly understood, but may have important clinical consequences. For example, the formation of these biological polyelectrolytes into ordered gel phases may contribute significantly to the observed high viscosity of CF sputum. In this work, a number of model systems were created to simulate CF sputum in vitro, in order to elucidate the contributions of the different components. Preliminary results will be presented. This work was supported by NSF DMR-0071761, DOE DEFG02-91ER45439, the Beckman Young Investigator Program, and the Cystic Fibrosis Foundation.

  5. Surgical Management of Minor Salivary Gland Neoplasms of the Palate

    PubMed Central

    Moore, Brian A.; Burkey, Brian B.; Netterville, James L.; Butcher, R. Brent; Amedee, Ronald G.

    2008-01-01

    Objective: Minor salivary gland tumors are uncommon, accounting for up to 15% of salivary gland neoplasms. We describe our experience with both benign and malignant tumors of the palatal minor salivary glands, focusing on the extent of resection and options for defect reconstruction. Study Design: Retrospective review of medical records. Results: From 1994 to 2002, 37 patients with primary neoplasms originating in the palatal minor salivary glands were treated at a single institution. Patients ranged in age from the second to the seventh decades, with a female preponderance. Twenty-four percent of the lesions were benign. The most common malignant tumor encountered was low grade polymorphous adenocarcinoma, followed by mucoepidermoid carcinoma, and adenoid cystic carcinoma. The extent of surgical resection was dictated by tumor pathology and evidence of perineural spread, and defects were reconstructed with a variety of techniques. Postoperative complications included velopharyngeal insufficiency, flap fistulization or loss, and trismus. After 1 month to 8 years of follow-up, 1 patient has died with regional and systemic metastases. Conclusions: Neoplasms of the minor salivary glands in the palate may be excised, with limits dictated by tumor histopathology and perineural invasion. Improved functional results may be achieved by immediately reconstructing the defects with rotational flaps, reserving free flaps for more extensive defects of the maxilla and infratemporal fossa. PMID:21603498

  6. The role of disulphide bonds in human intestinal mucin

    PubMed Central

    Forstner, Janet F.; Jabbal, Inderjit; Qureshi, Rauf; Kells, David I. C.; Forstner, Gordon G.

    1979-01-01

    Goblet-cell mucin (mucin 1) was isolated and purified from human small-intestinal scrapings. After application of mucin 1 to DEAE-Bio-Gel (A) columns, most of the glycoprotein (76–94% of hexoses) was eluted in the first peak (designated mucin 2). Minor amounts of acidic glycoproteins were eluted with 0.2m- and 0.4m-NaCl in later peaks. Analyses of mucin 1 and mucin 2 revealed mucin 2 to be a monodisperse highly glycosylated glycoprotein containing 6.3% by wt. of protein, N-acetylgalactosamine, N-acetylglucosamine, galactose and fucose. Mucin 1 was similar in composition, but was polydisperse and contained more protein (12.3% by wt.) as well as N-acetylneuraminic acid. Analytical CsCl-gradient ultracentrifugation showed both mucin 1 and mucin 2 to have a major component with an average buoyant density of 1.47000g/ml. Mucin 1 also contained a slightly less-dense minor glycoprotein component. After exhaustive reduction and alkylation mucin 1 retained its major component, but partly dissociated into two lighter glycoprotein components. Mucin 2, in contrast, did not change its density distribution after reduction. Band ultracentrifugation in 2H2O-containing iso-osmotic buffers showed that mucin 1 contained a major fast-sedimenting component (so=37±2S), and a minor amount of a slower-sedimenting component. After reduction there was an increased quantity of the latter component, for which an so value of 14.5S was calculated. In contrast, mucin 2 was unaltered by reduction (so=33±2S). These findings indicate that the major component of goblet-cell mucin (mucin 2) does not dissociate after S–S-bond reduction, and thus does not apparently rely for its polymeric structure on the association of subunits through covalent disulphide bonds. However, the effects of reduction on mucin 1 suggest that in the native mucin intramolecular disulphide bonds in the minor glycoproteins may stabilize their structure, permitting secondary non-covalent interactions to develop with the

  7. A case of mixed adenoneuroendocrine carcinoma of the gallbladder arising from an intracystic papillary neoplasm associated with pancreaticobiliary maljunction.

    PubMed

    Meguro, Yoshiyuki; Fukushima, Noriyoshi; Koizumi, Masaru; Kasahara, Naoya; Hydo, Masanobu; Morishima, Kazue; Sata, Naohiro; Lefor, Alan T; Yasuda, Yoshikazu

    2014-09-01

    A 54-year-old Japanese woman was referred with a gallbladder tumor. Based on the results of the computed tomography scan, endoscopic retrograde cholangiopancreatography, and magnetic resonance cholangiopancreatography, a mucin-producing neoplasm of the gallbladder associated with pancreaticobiliary maljunction was diagnosed. Extended cholecystectomy, extrahepatic bile duct resection, and choledochojejunostomy were performed, and she remains free of recurrence 24 months after resection. Histopathological examination revealed that the papillary component of the lesion was an intracystic papillary neoplasm with diverse characteristics of pancreaticobiliary epithelium and intestinal epithelium including mucin. In this component, most of the papillary lesion was a high-grade intraepithelial neoplasm, but also showed slight invasion into the muscular layer. The nodular component consisted of both poorly differentiated biliary type adenocarcinoma and large cell neuroendocrine carcinoma. We report a rare case of a mixed adenoneuroendocrine carcinoma arising from an intracystic papillary neoplasm associated with pancreaticobiliary maljunction. As for the histogenesis of this tumor, based on the histopathologic appearance, transdifferentiation from poorly differentiated biliary type adenocarcinoma to large cell neuroendocrine carcinoma is considered the most possible histogenesis of this tumor. PMID:25146100

  8. Biomarker validation for intraductal papillary mucinous neoplasms of the pancreas | Division of Cancer Prevention

    Cancer.gov

    DESCRIPTION (provided by applicant): A critical component to successful cancer screening is the identification of a lesion for which intervention will result in prolonged survival or cure.The five-year survival of patients with resected stage IA pancreas cancer (the earliest identifiable lesion and |

  9. Time to reconsider Spitzoid neoplasms?

    PubMed Central

    Urso, Carmelo

    2016-01-01

    SUMMARY Background: Spitzoid neoplasms may pose significant diagnostic problems because in a fraction of them it is quite difficult or impossible to establish if they are benign or malignant lesions. An extraordinarily large number of studies have been made in attempts to solve this problem; regrettably, the histological criteria proposed and the various special sophisticated techniques employed have proven to be ineffective in making this distinction with confidence. Objectives: To explore the possible causes for this diagnostic failure and an attempt to identify the source of this problem. Method: A historical and technical analysis of the specialized literature is performed, critically evaluating the main points of this controversial topic. Results: The reasons for the diagnostic failure in Spitzoid neoplasms are not clear but could be the result of inappropriate conceptual representation. The analysis of available data and a rational review of old and new assumptions and concepts may suggest a different representation for Spitzoid neoplasms: Spitz nevus, atypical Spitz tumor and Spitzoid melanoma, rather than being three different tumors that are difficult or impossible to distinguish with assurance, could be viewed as one unique entity, Spitz tumor (ST). This tumor is a low-grade malignant neoplasm, in which the amount of intrinsic risk is variable, ranging from very low to high (ST1, ST2, ST3), and malignant potential could be estimated. Conclusions: The proposed alternative representation of Spitzoid neoplasms as a unique tumor may help in overcoming the difficulty in diagnosis of these tumors. PMID:27222771

  10. Mucins in lung cancer: diagnostic, prognostic, and therapeutic implications.

    PubMed

    Lakshmanan, Imayavaramban; Ponnusamy, Moorthy P; Macha, Muzafar A; Haridas, Dhanya; Majhi, Prabin Dhangada; Kaur, Sukhwinder; Jain, Maneesh; Batra, Surinder K; Ganti, Apar Kishor

    2015-01-01

    Aberrant expression of mucins is associated with cancer development and metastasis. An overexpression of few mucins contributes to oncogenesis by enhancing cancer cell growth and providing constitutive survival signals. This review focuses on the importance of mucins both in the normal bronchial epithelial cells and the malignant tumors of the lung and their contribution in the diagnosis and prognosis of lung cancer patients. During lung cancer progression, mucins either alone or through their interaction with many receptor tyrosine kinases mediate cell signals for growth and survival of cancer cells. Also, stage-specific expression of certain mucins, like MUC1, is associated with poor prognosis from lung cancer. Thus, mucins are emerging as attractive targets for developing novel therapeutic approaches for lung cancer. Several strategies targeting mucin expression and function are currently being investigated to control lung cancer progression. PMID:25319180

  11. Pleuropulmonary blastoma in a young adult presenting as a ruptured cystic teratoma in radiology.

    PubMed Central

    Lee, Chang Hun; Kim, Keun Il; Kim, Young Dae; Lee, Min Ki; Kim, Jee Yeon; Park, Do Youn; Sol, Mee Young; Suh, Kang Suek

    2003-01-01

    Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left chest pain of two weeks' duration. A computed tomography scan revealed a large, multicystic tumor occupying the left lower hemithorax, leading to the impression of a ruptured mediastinal cystic teratoma. A thoracotomy for resection of the tumor was performed. On histologic examination, the tumor consisted of cystic walls and associated solid lesions which showed undifferentiated blastemal tissues with focal fibrosarcomatous and rhabdoid features. Immunohistochemically the tumor cells only showed diffuse strong positivity for vimentin. The histologic findings corresponded to a type II PPB. The authors suggest that PPB, especially of type I or II, should be included in the radiologic differential diagnosis of mediastinal cystic neoplasms in a young adult. PMID:12923341

  12. Role of endoscopic ultrasound-guided fine needle aspiration and ultrasound-guided fine-needle aspiration in diagnosis of cystic pancreatic lesions

    PubMed Central

    Okasha, Hussein Hassan; Ashry, Mahmoud; Imam, Hala M. K.; Ezzat, Reem; Naguib, Mohamed; Farag, Ali H.; Gemeie, Emad H.; Khattab, Hani M.

    2015-01-01

    Background and Objective: The addition of fine-needle aspiration (FNA) to different imaging modalities has raised the accuracy for diagnosis of cystic pancreatic lesions. We aim to differentiate benign from neoplastic pancreatic cysts by evaluating cyst fluid carcinoembryonic antigen (CEA), carbohydrate antigen (CA19-9), and amylase levels and cytopathological examination, including mucin stain. Patients and Methods: This prospective study included 77 patients with pancreatic cystic lesions. Ultrasound-FNA (US-FNA) or endoscopic ultrasound-FNA (EUS-FNA) was done according to the accessibility of the lesion. The aspirated specimens were subjected to cytopathological examination (including mucin staining), tumor markers (CEA, CA19-9), and amylase level. Results: Cyst CEA value of 279 or more showed high statistical significance in differentiating mucinous from nonmucinous lesions with sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of 73%, 60%, 50%, 80%, and 65%, respectively. Cyst amylase could differentiate between neoplastic and nonneoplastic cysts at a level of 1043 with sensitivity of 58%, specificity of 75%, PPV of 73%, NPV of 60%, and accuracy of 66%. CA19-9 could not differentiate between neoplastic and nonneoplastic cysts. Mucin examination showed a sensitivity of 85%, specificity of 95%, PPV of 92%, NPV of 91%, and accuracy of 91% in differentiating mucinous from non-mucinous lesions. Cytopathological examination showed a sensitivity of 81%, specificity of 94%, PPV of 94%, NPV of 83%, and accuracy of 88%. Conclusion: US or EUS-FNA with analysis of cyst CEA level, CA19-9, amylase, mucin stain, and cytopathological examination increases the diagnostic accuracy of cystic pancreatic lesions. PMID:26020048

  13. Quantitative microscopic evaluation of mucin areas and its percentage in mucinous carcinoma of the breast using tissue histological images.

    PubMed

    Saha, Monjoy; Arun, Indu; Basak, Bijan; Agarwal, Sanjit; Ahmed, Rosina; Chatterjee, Sanjoy; Bhargava, Rohit; Chakraborty, Chandan

    2016-06-01

    Mucinous carcinoma (MC) of the breast is very rare (∼1-7% of all breast cancers), invasive ductal carcinoma. Presence of pools of extracellular mucin is one of the most important histological features for MC. This paper aims at developing a quantitative computer-aided methodology for automated identification of mucin areas and its percentage using tissue histological images. The proposed method includes pre-processing (i.e., colour space transformation and colour normalization), mucin regions segmentation, post-processing, and performance evaluation. The proposed algorithm achieved 97.74% segmentation accuracy in comparison to ground truths. In addition, the percentage of mucin present in the tissue regions is calculated by the mucin index (MI) for grading MC (pure, moderately, minimally mucinous). PMID:26971129

  14. Staging of neoplasms. Volume 7

    SciTech Connect

    Glazer, G.M.

    1986-01-01

    This book is divided into ten chapters. The first, an overview of the importance of staging, is followed by separate chapters on computed tomographic (CT) evaluation of lymph node metastases; metastatic disease to the thorax; staging of laryngeal, hypopharyngeal, esophageal, non-small cell lung, and renal carcinoma; and pediatric abdominal malignancies. CT staging of lymphomas is dealt with in a separate chapter. The final chapter summarizes initial experiences with staging of neoplasms by magnetic resonance (MR) imaging. Other neoplasms, such as pelvic, pancreatic, and gastrointestinal, are not discussed in depth. The book concludes with ten case studies, most of which deal with pelvic and gastrointestinal malignancies.

  15. Evaluation of p27 Expression in Salivary Gland Neoplasms; A Step Forward in Unveiling the Role of p27

    PubMed Central

    Malgaonkar, Nikhil I.; Abuderman, Abdulwahab; Kharma, MY; Al-Maweri, SA; Alaizari, NA; Altamimi, MA.; Darwish, S.

    2016-01-01

    Introduction Salivary gland neoplasms are not uncommon lesions that are seen in the head and neck region. The role of cell cycle regulators as well as that of oncogenes remains unexplored in the pathogenesis of these neoplasms. Aim Present study was conducted to evaluate the expression of p27 in the three common salivary gland neoplasms. Materials and Methods A total of 34 cases (19 pleomorphic adenoma, 8 mucoepidermoid carcinoma and 7 adenoid cystic carcinoma) were included. The sections were subjected to p27 staining and rated for the expression. Results Of the total 52.6% of pleomorphic adenoma cases, 25% of mucoepidermoid carcinoma cases and only 14.2% of adenoid cystic carcinoma cases showed strong expression suggesting variable p27 expression in both malignant neoplasms. Normal salivary gland tissue was stained as a positive control for the evaluation. Conclusion The results of the study suggest an important role for p27 in pathogenesis of mucoepidermoid carcinoma as well as adenoid cystic carcinoma while its role in pathogenesis of pleomorphic adenoma remains questionable keeping in view the strong expression of p27 in the same.

  16. Intraductal tubulopapillary neoplasm of the bile duct: A case report and review of the published work.

    PubMed

    Nakagawa, Takashi; Arisaka, Yoshifumi; Ajiki, Tetsuo; Fujikura, Kohei; Masuda, Atsuhiro; Takenaka, Mamoru; Shiomi, Hideyuki; Okabe, Yoshihiro; Fukumoto, Takumi; Ku, Yonson; Azuma, Takeshi; Zen, Yoh

    2016-06-01

    Recent studies have suggested that a peculiar tumor mimicking intraductal tubulopapillary neoplasms (ITPN) of the pancreas can develop in the bile duct. Here, we present a similar case and review the published work on the newly proposed biliary neoplasm. A 68-year-old woman was referred to us for further evaluation of a liver mass. On imaging, the tumor was 40 mm in size and intraductal in location, and was enhanced slightly on the contrast-enhanced computed tomography. No mucin overproduction was found. She underwent right hepatectomy for suspected intraductal papillary neoplasm of the bile duct. The tumor histologically consisted of the biliary-type epithelium arranged in a tubular architecture within the dilated bile duct. Tumor cells had features of high-grade dysplasia but no stroma invasion. No expressions of MUC2 and MUC5AC were noted. On molecular studies, KRAS and GNAS appeared to be wild-type genotypes. These features were in keeping with ITPN. In the published work review, "biliary ITPN" described in four manuscripts were characterized by predominantly intrahepatic/hilar in location, histological tubular architecture, negative expressions of MUC2 and MUC5AC, and uncommon alterations of KRAS, GNAS and BRAF. Although invasive malignancy was present in 71%, the outcome was favorable with the 5-year survival expected to be approximately 90%. Biliary ITPN are supposedly uncommon, but a greater awareness of this condition may give more chance to diagnose the underrecognized neoplasm. PMID:26459784

  17. Inherited renal cystic diseases.

    PubMed

    Kim, Bohyun; King, Bernard F; Vrtiska, Terri J; Irazabal, Maria V; Torres, Vicente E; Harris, Peter C

    2016-06-01

    A number of inherited renal diseases present with renal cysts and often lead to end-stage renal disease. With recent advances in genetics, increasing number of genes and mutations have been associated with cystic renal diseases. Although genetic testing can provide a definite diagnosis, it is often reserved for equivocal cases or for ongoing investigational research. Therefore, imaging findings are essential in the routine diagnosis, follow-up, and detection of complications in patients with inherited cystic renal diseases. In this article, the most recent classification, genetic analysis, clinical presentations, and imaging findings of inherited cystic renal diseases will be discussed. PMID:27167233

  18. Drugs Approved for Myeloproliferative Neoplasms

    Cancer.gov

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for myeloproliferative neoplasms. The list includes generic names, brand names, and common drug combinations, which are shown in capital letters. The drug names link to NCI's Cancer Drug Information summaries.

  19. Perforated mucinous cystadenoma of the vermiform appendix: an overview in reasoning clinical decisions

    PubMed Central

    Papadopoulos, Iordanis N; Christodoulou, Spyridon; Kokoropoulos, Panayiotis; Konstantudakis, George; Economopoulos, Nikolaos; Leontara, Vassilia

    2011-01-01

    Recent advances in the management of appendiceal mucinous neoplasms (AMN) such as peritonectomy combined with hyperthermic intraperitoneal chemotherapy have introduced new standards of care. However, many dilemmas are encountered in decision making as in the following patient. A 74-year-old woman was admitted with an appendiceal cystadenoma found in a preadmission CT scan. However, the tumour was not documented by the in hospital investigation due to its perforation and its reduction in size. Consequently, a series of management dilemmas were encountered that were solved by cautious evaluation of the pre and peroperative findings. She was submitted to a right hemicolectomy. A spontaneous perforation was suspected, but the accurate diagnosis was documented postoperatively by histopathology. This paradigm motivated this review which concluded that reasoning clinical decisions in the light of recent advances and appropriate care based on the disease-stage are essential for an optimal outcome in the management of AMN. PMID:22689271

  20. Primary and secondary neoplasms of the spleen

    PubMed Central

    Azar, S.; Al-Hawary, M.M.; Francis, I.R.

    2010-01-01

    Abstract With the exception of lymphoma involving the spleen, other primary and secondary neoplasms are rare and infrequently encountered. Primary malignant neoplasms involving the spleen are lymphoma and angiosarcoma. Primary benign neoplasms involving the spleen include hemangioma, lymphangioma, littoral cell angioma and splenic cyst and solid lesions such as hamartoma and inflammatory pseudotumor. PMID:20713317

  1. 9 CFR 311.11 - Neoplasms.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ... 9 Animals and Animal Products 2 2014-01-01 2014-01-01 false Neoplasms. 311.11 Section 311.11... CERTIFICATION DISPOSAL OF DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.11 Neoplasms. (a) An individual organ or other part of a carcass affected with a neoplasm shall be condemned. If there is...

  2. 9 CFR 311.11 - Neoplasms.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ... 9 Animals and Animal Products 2 2011-01-01 2011-01-01 false Neoplasms. 311.11 Section 311.11... CERTIFICATION DISPOSAL OF DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.11 Neoplasms. (a) An individual organ or other part of a carcass affected with a neoplasm shall be condemned. If there is...

  3. 9 CFR 311.11 - Neoplasms.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ... 9 Animals and Animal Products 2 2013-01-01 2013-01-01 false Neoplasms. 311.11 Section 311.11... CERTIFICATION DISPOSAL OF DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.11 Neoplasms. (a) An individual organ or other part of a carcass affected with a neoplasm shall be condemned. If there is...

  4. 9 CFR 311.11 - Neoplasms.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ... 9 Animals and Animal Products 2 2012-01-01 2012-01-01 false Neoplasms. 311.11 Section 311.11... CERTIFICATION DISPOSAL OF DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.11 Neoplasms. (a) An individual organ or other part of a carcass affected with a neoplasm shall be condemned. If there is...

  5. 9 CFR 311.11 - Neoplasms.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Neoplasms. 311.11 Section 311.11... CERTIFICATION DISPOSAL OF DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.11 Neoplasms. (a) An individual organ or other part of a carcass affected with a neoplasm shall be condemned. If there is...

  6. Mucinous ovarian cancer: A therapeutic review.

    PubMed

    Xu, Wen; Rush, Jack; Rickett, Kirsty; Coward, Jermaine I G

    2016-06-01

    Mucinous ovarian cancer represents approximately 3% of epithelial ovarian cancers (EOC). Despite this seemingly low prevalence, it remains a diagnostic and therapeutic conundrum that has resulted in numerous attempts to adopt novel strategies in managing this disease. Anecdotally, there has been a prevailing notion that established gold standard systemic regimens should be substituted for those utilised in cancers such as gastrointestinal (GI) malignancies; tumours that share more biological similarities than other EOC subtypes. This review summarises the plethora of small studies which have adopted this philosophy and influenced the design of the multinational GOG142 study, which was ultimately terminated due to poor accrual. To date, there is a paucity of evidence to support delivering 'GI style' chemotherapy for mucinous ovarian cancer over and above carboplatin-paclitaxel doublet therapy. Hence there is an urge to develop studies focused on targeted therapeutic agents driven by refined mutational analysis and conducted within the context of harmonised international collaborations. PMID:27083591

  7. Cystic Adenomatoid Odontogenic Tumor

    PubMed Central

    Grover, Sonal; Rahim, Ahmed Mujib Bangalore; Parakkat, Nithin Kavassery; Kapoor, Shekhar; Mittal, Kumud; Sharma, Bhushan; Shivappa, Anil Bangalore

    2015-01-01

    Adenomatoid Odontogenic Tumor (AOT) is a well-established benign epithelial lesion of odontogenic origin. Rightfully called “the master of disguise,” this lesion has been known for its varied clinical and histoarchitectural patterns. Not only does AOT predominantly present radiologically as a unilocular cystic lesion enclosing the unerupted tooth (which is commonly mistaken as a dentigerous cyst) but the lesion also presents rarely with a cystic component histopathologically. We present one such unusual case of cystic AOT associated with an impacted canine, mimicking a dentigerous cyst. The present case aims to highlight the difference between cystic AOT and dentigerous cyst radiographically. The exact histogenesis of AOT and its variants still remains obscure. An attempt has been made to hypothesize the new school of thought regarding the origin of AOT. PMID:26579317

  8. Acquired Cystic Kidney Disease

    MedlinePlus

    ... a kidney transplant or blood-filtering treatments called dialysis. The cysts are more likely to develop in people who are on kidney dialysis. The chance of developing acquired cystic kidney disease ...

  9. Giant mucinous cystadenocarcinoma of ovary: A case report and review of literature

    PubMed Central

    Katke, Rajshree Dayanand

    2016-01-01

    Giant cystadenocarcinomas of the ovary are rarely described. Huge ovarian masses are mostly benign, but malignancy should be ruled out by investigations and clinical assessment. Giant cysts require resection because of compressive symptoms or risk of malignancy and their management invariably requires laparotomy to prevent perforation and spillage of the cyst fluid into peritoneal cavity. Here, we present a case of a 42-year-old female with severe and rapidly growing abdominal distension operated for exploratory laparotomy for cystic mass excision. On histology, mass was found to be metastatic mucinous cystadenocarcinoma with omental metastasis. The diagnostic and management challenges posed by this unexpected and unusual presentation of an ovarian cystadenocarcinoma are discussed. The main aim of this report is to draw attention to huge ovarian epithelial cysts with unsuspected presentation contributing to a decrease in any underdiagnosis, misdiagnosis, and mismanagement that might occur. PMID:27134482

  10. Synchronous Nesidioblastosis, Endocrine Microadenoma, and Intraductal Papillary Mucinous Neoplasia in a Man Presenting With Hyperinsulinemic Hypoglycemia.

    PubMed

    De Sousa, Sunita M C; Haghighi, Koroush S; Qiu, Min Ru; Greenfield, Jerry R; Chen, Daniel L T

    2016-01-01

    Herein, we report the first case of concomitant nesidioblastosis, pancreatic neuroendocrine tumor, and intraductal papillary mucinous neoplasia. The combination is significant as each of these pathological entities is independently very rare. The patient was a 33-year-old man who presented with symptomatic hyperinsulinemic hypoglycemia and no risk factors for pancreatic disease. Abdominal imaging showed an isolated 12 mm pancreatic lesion, whilst selective arterial calcium stimulation testing demonstrated multiple territories of insulin excess. He proceeded to subtotal pancreatectomy. Histopathology revealed an endocrine microadenoma, α and β cell nesidioblastosis, and multifocal intraductal papillary mucinous neoplasia. The endocrine microadenoma and nesidioblastosis stained for insulin, suggesting both likely contributed to hypoglycemia. Glucagon immunohistochemistry was also positive, though there were no clinical features of glucagon excess. Hypoglycemia resolved postoperatively. This case and other evidence from the literature suggest that hyperplasia and neoplasia may occur sequentially in the pancreas, and that endocrine and exocrine tumorigenesis may be linked in some individuals. Further study is required to identify a unifying mechanism, and to elucidate potential ramifications in the management of patients with pancreatic neoplasms. PMID:26658039

  11. Folding and Aggregation of Mucin Domains.

    NASA Astrophysics Data System (ADS)

    Urbanc, Brigita; Bansil, Rama; Turner, Bradley

    2007-03-01

    Mucin glycoproteins consist of tandem repeating glycosylated regions flanked by non-repetitive protein domains with little glycosylation. These non-repetitive domains are involved in polymerization of mucin via disulfide bonds and play an important role in the pH dependent gelation of gastric mucin, which is essential to protecting the stomach from autodigestion. We have examined the folding and aggregation of the non-repetitive sequence of von Willebrand factor vWF-C1 domain (67 amino acids) and PGM 2X (242 amino acids) using Discrete Molecular Dynamics (four-bead protein model with hydrogen bonding and amino acid-specific hydrophobic/hydrophilic and electrostatic interactions of side chains). Simulations of vWF C1 show 4-6 β-strands separated by turns/loops with more loops at lower pH. A simulation of several vWF C1 proteins at low pH shows aggregates still with a high content of β-strands and enhanced turn/loop regions. For the PGM 2X simulation the contact map shows several salt bridges enclosing hairpin turns. The implications of these simulations for describing the aggregation/gelation of PGM will be discussed.

  12. Primary vaginal mucinous adenocarcinoma of gastric type arising in adenosis: a report of 2 cases, 1 associated with uterus didelphys.

    PubMed

    Talia, Karen L; Scurry, James; Manolitsas, Thomas; McCluggage, W Glenn

    2012-03-01

    We report 2 cases of primary vaginal mucinous adenocarcinoma arising in adenosis in nondiethylstilbestrol-exposed women, 1 with uterus didelphys. Both tumors exhibited morphologic and immunohistochemical features (MUC6 and/or HIK 1083 positivity) identical to the recently described cervical gastric-type adenocarcinoma, a subtype of mucinous adenocarcinoma that is non-human papillomavirus related and possibly related to adenoma malignum. Both neoplasms were intensely p53 positive, suggesting that TP53 mutation may be implicated in their development. We believe that the vaginal tumors arose from adenosis through atypical adenosis, as benign and atypical glands were present at the periphery of the neoplasms. In reporting these cases, we discuss atypical adenosis and other types of non-diethylstilbestrol-associated vaginal adenocarcinomas. At least 9 other examples of primary vaginal, or more uncommonly cervical, adenocarcinomas arising in non-diethylstilbestrol-exposed women with congenital genitourinary malformations have been reported, suggesting a probable causal association between congenital malformation, vaginal adenosis, and vaginal adenocarcinoma. PMID:22317878

  13. Mucin-producing tumors of the ovary: MR imaging appearance.

    PubMed

    Laurent, P-E; Thomassin-Piana, J; Jalaguier-Coudray, A

    2015-11-01

    Mucin-producing tumors of the ovary can be either primary epithelial mucin-producing tumors or ovarian metastases from a remote adenocarcinoma usually originating from the gastrointestinal tract. The purpose of this pictorial review is to describe the main types of primary or secondary mucin-producing tumors of the ovary and to provide MR imaging diagnostic criteria in order to guide further therapy. PMID:25753545

  14. Expression of REG4 in ovarian mucinous tumors.

    PubMed

    Huang, Qiong; Chen, Xiaoduan; Lu, Weiguo; Lai, Maode; Lu, Bingjian

    2014-04-01

    Regenerating islet-deprived gene family, number 4 (REG4), is a novel marker for intestinal differentiation. We performed immunohistochemical studies on REG4, cytokeratin (CK)7, CK20, and caudal type homeobox 2 (CDX2) in 291 ovarian mucinous tumors. There were 226 primary tumors and 65 metastatic tumors. The primary tumors comprised 69/226 mucinous cystadenomas, 79/226 mucinous borderline tumors (64/79 intestinal-type and 15/79 endocervical-like tumors), and 78/226 mucinous carcinomas. We found that REG4 expression was significantly higher in mucinous borderline tumors (30/79, 38.0%) and primary mucinous carcinomas (26/78, 33.3%) than in mucinous cystadenomas (4/69, 5.8%; P<0.05). However, REG4 expression was more commonly associated with intestinal-type, borderline, mucinous tumors rather than the endocervical-like type (30/64 vs. 0/15, P<0.001). There was a significant correlation between the REG4 and CDX2 expression profiles in primary ovarian mucinous tumors (r=0.772, P<0.001). REG4, CDX2, and diffuse CK20 had higher expression frequencies in metastatic lower gastrointestinal adenocarcinoma than in primary mucinous tumors (P<0.01). The CK7/REG4 coordinate expression profile was comparable in diagnostic value to CK7/CK20 or CK7/CDX2 profile. We conclude that REG4 expression is common in mucinous borderline tumors of the intestinal type as it is absent in the endocervical-like form in this series. Expression of CK7/REG4 may contribute to the differential diagnosis between primary and metastatic ovarian mucinous tumors. PMID:23958547

  15. Automatic differential diagnosis of pancreatic serous and mucinous cystadenomas based on morphological features.

    PubMed

    Song, Jae-Won; Lee, Ju-Hong; Choi, Joon-Hyuk; Chun, Seok-Ju

    2013-01-01

    Generally, pathological diagnosis using an electron microscope is time-consuming and likely to result in a subjective judgment, because pathologists perform manual screening of tissue slides at high magnifications. Recently, the advent of digital pathology technology has provided the basis for convenient screening and quantitative analysis by digitizing tissue slides through a computer system. However, a screening process with high magnification still takes quite a long time. To solve these problems, recently the use of computer-aided design techniques for performing pathologic diagnosis has been increasing in digital pathology. For pathological diagnosis, we need different diagnostic methods for different regions with different characteristics. Therefore, in order to effectively diagnose different lesions and types of diseases, a quantitative method for extracting specific features is required in computerized pathologic diagnosis. This study is about an automated differential diagnosis system to differentiate between benign serous cystadenoma and possibly-malignant mucinous cystadenoma. In order to diagnose cystic tumors, the first step is identifying a cystic region and inspecting its epithelial cells. First, we identify the lumen boundary of a cyst using the Direction Cumulative Map considering 8-ways. Then, the Epithelial Nuclei Identification algorithm is used to discern epithelial nuclei. After that, three morphological features for the differential diagnosis of mucinous and serous cystadenomas are extracted. To demonstrate the superiority of the proposed features, the experiments compared performance of the classifiers learned by using the proposed morphological features and the classical morphological features based on nuclei. The classifiers in the simulations are as follows; Bayesian Classifier, k-Nearest Neighbors, Support Vector Machine, and Artificial Neural Network. The results show that all classifiers using the proposed features have the best

  16. Primary uterine müllerian mucinous borderline tumor (MMBT) associated with adenomyosis: a case report.

    PubMed

    Kawamura, Keiko; Kaneki, Eisuke; Ogawa, Shinji; Imamura, Hiroko; Ohishi, Yoshihiro; Kobayashi, Hiroaki; Kato, Kiyoko

    2014-03-01

    Müllerian mucinous borderline tumors (MMBTs) usually arise from the ovary. The present report is the first case of primary uterine MMBTs associated with adenomyosis. A 51-year-old woman was referred to our hospital for a complex cystic and solid 4×3 cm right adnexal mass. She had a history of a left ovarian endometriotic cyst and had undergone a left oophorectomy 2 yr prior. A laparotomy was performed, and the tumor was found to be originating in the posterior wall of the uterus. She underwent a total abdominal hysterectomy, right salpingo-oophorectomy, and left salpingectomy. Microscopically, the solid portion of the tumor contained papillary proliferations of glands, which were covered by a mucinous epithelium with mild to moderate nuclear atypia, accompanied by stromal infiltration of inflammatory cells. Islands of adenomyosis were also observed around the cyst. These pathologic findings were similar to the features of ovarian MMBT. We diagnosed this tumor as a uterine MMBT, probably arising from adenomyosis. PMID:24487469

  17. Cystic Fibrosis (CF) Respiratory Screen: Sputum

    MedlinePlus

    ... Cystic Fibrosis (CF) Chloride Sweat Test Lungs and Respiratory System Cystic Fibrosis: Diet and Nutrition Cystic Fibrosis Cystic Fibrosis: Diet and Nutrition Lungs and Respiratory System Contact Us Print Resources Send to a friend ...

  18. Interleukin-17 Pathophysiology and Therapeutic Intervention in Cystic Fibrosis Lung Infection and Inflammation.

    PubMed

    Hsu, Daniel; Taylor, Patricia; Fletcher, Dave; van Heeckeren, Rolf; Eastman, Jean; van Heeckeren, Anna; Davis, Pamela; Chmiel, James F; Pearlman, Eric; Bonfield, Tracey L

    2016-09-01

    Cystic fibrosis (CF) is characterized by an excessive neutrophilic inflammatory response within the airway as a result of defective cystic fibrosis transmembrane receptor (CFTR) expression and function. Interleukin-17A induces airway neutrophilia and mucin production associated with Pseudomonas aeruginosa colonization, which is associated with the pathophysiology of cystic fibrosis. The objectives of this study were to use the preclinical murine model of cystic fibrosis lung infection and inflammation to investigate the role of IL-17 in CF lung pathophysiology and explore therapeutic intervention with a focus on IL-17. Cftr-deficient mice (CF mice) and wild-type mice (WT mice) infected with P. aeruginosa had robust IL-17 production early in the infection associated with a persistent elevated inflammatory response. Intratracheal administration of IL-17 provoked a neutrophilic response in the airways of WT and CF animals which was similar to that observed with P. aeruginosa infection. The neutralization of IL-17 prior to infection significantly improved the outcomes in the CF mice, suggesting that IL-17 may be a therapeutic target. We demonstrate in this report that the pathophysiological contribution of IL-17 may be due to the induction of chemokines from the epithelium which is augmented by a deficiency of Cftr and ongoing inflammation. These studies demonstrate the in vivo contribution of IL-17 in cystic fibrosis lung disease and the therapeutic validity of attenuating IL-17 activity in cystic fibrosis. PMID:27271746

  19. Interaction of cellulose-based cationic polyelectrolytes with mucin.

    PubMed

    Mazoniene, Edita; Joceviciute, Simona; Kazlauske, Jurgita; Niemeyer, Bernd; Liesiene, Jolanta

    2011-03-01

    Mucoadhesivity of water-soluble polymers is an important factor, when testing their suitability for controlled drug delivery systems. For this purpose, the interaction of new cationic cellulose polyelectrolytes with lyophilized mucin was investigated by means of turbidimetric titration, microscopy and measurement of zeta potential and particle size changes in the system. Results show that the cellulose derivatives interact with mucin. This interaction became stronger if cellulose macromolecules contained positively charged groups and an electrostatic interaction with the negatively charged mucin particles occurred. Under certain conditions flocculation of mucin particles by the cellulose polyelectrolyte was observed. PMID:21134731

  20. Magnetic resonance imaging findings and prognosis of gastric-type mucinous adenocarcinoma (minimal deviation adenocarcinoma or adenoma malignum) of the uterine corpus: Two case reports

    PubMed Central

    HINO, MAYO; YAMAGUCHI, KEN; ABIKO, KAORU; YOSHIOKA, YUMIKO; HAMANISHI, JUNZO; KONDOH, EIJI; KOSHIYAMA, MASAFUMI; BABA, TSUKASA; MATSUMURA, NORIOMI; MINAMIGUCHI, SACHIKO; KIDO, AKI; KONISHI, IKUO

    2016-01-01

    Our group previously documented the first, very rare case of primary gastric-type mucinous adenocarcinoma of the uterine corpus. Although this type of endometrial cancer appears to be similar to the gastric-type adenocarcinoma of the uterine cervix, its main symptoms, appearance on magnetic resonance imaging (MRI) and prognosis have not been fully elucidated due to its rarity. We herein describe an additional case of gastric-type mucinous adenocarcinoma of the endometrium and review the relevant literature. The two cases at our institution (Kyoto University Hospital, Kyoto, Japan) involved postmenopausal women with a primary complaint of abnormal genital bleeding. Microscopic examination of the hysterectomy specimens indicated a highly differentiated mucinous adenocarcinoma with a desmoplastic stromal reaction. Immunohistochemistry for HIK1083 and/or MUC6 was positive in both cases, suggesting a gastric phenotype. Both patients were diagnosed at an advanced stage, they relapsed or recurred immediately after adjuvant chemotherapy, and eventually succumbed to the disease. The main symptom of gastric-type mucinous adenocarcinoma of the uterine cervix is watery discharge, whereas abnormal genital bleeding in addition to watery discharge is mainly observed in the mucinous type of endometrial adenocarcinoma. Cystic cavities in the tumor are present on MRI in cases of endometrial origin, and prognosis is very poor due to resistance to chemotherapy. Thus, gastric-type mucinous adenocarcinoma of the uterine endometrium exhibits a clinical behavior that is similar to tumors originating from the uterine cervix, but is associated with distinguishing clinical symptoms. The incidence of gastric-type endometrial adenocarcinoma may be higher than expected. PMID:27123265

  1. Spontaneous endomyometrial neoplasms in aging Chinese hamsters

    SciTech Connect

    Brownstein, D.G.; Brooks, A.L.

    1980-05-01

    Twenty-one endomyometrial neoplasms among 93 nulliparous noninbred Chinese hamsters were evaluated. The median survival time of the 93 females was 1040 days. The median age of hamsters with endomyometrial neoplasms was 1200 days. Neoplasms were classified as carcinomas or malignant mixed muellerian tumors of the endometrium and benign or malignant myometrial neoplasms. There were 13 endometrial adenocarcinomas. Three tumors were mixed adenosquamous carcinomas, which occurred in significantly older Chinese hamsters than did adenocarcinomas. Three malignant mixed muellerian tumors consisted of 2 carcinosarcomas and 1 mixed mesodermal tumor. The 2 myometrial neoplasms were a lelomyoma and a lelomyosarcoma. The classification and relative frequency of these neoplasms were similar to endomyometrial neoplasms of women, which makes Chinese hamsters useful subjects for studies of spontaneous endomyometrial cancers.

  2. Radiographic and ultrasonographic findings of uterine neoplasms in nine dogs.

    PubMed

    Patsikas, Michail; Papazoglou, Lysimachos G; Jakovljevic, Samuel; Papaioannou, Nikolaos G; Papadopoulou, Paraskevi L; Soultani, Christina B; Chryssogonidis, Ioannis A; Kouskouras, Konstantinos A; Tziris, Nikolaos E; Charitanti, Afroditi A

    2014-01-01

    The records of nine female intact dogs with histologically confirmed uterine tumors were reviewed retrospectively, and the related radiographic and ultrasonographic signs of the lesions detected were recorded. Radiography revealed a soft-tissue opacity between the urinary bladder and colon in six of seven dogs with uterine body and/or cervical tumors, and a soft-tissue opacity in the midventral abdomen in two dogs with uterine horn tumors. Ultrasonography revealed masses in all dogs with uterine body/cervical tumors and could delineate the origin of the mass in one of two dogs with uterine horn tumors. The mass was characterized ultrasonographically as solid in three dogs (all leiomyomas), solid with cystic component in four dogs (two adenocarcinomas, one leiomyoma, and one fibroleiomyoma), and cystic in two (both leiomyomas). Hyperechoic foci in the mass were observed in three dogs. Ultrasonography was a useful method for demonstrating uterine body and/or cervical tumors. However, it was not possible to ascertain sonographically that a mass originated in a uterine horn unless there was associated evidence of uterine horn to which the mass could be traced. The ultrasonographic appearance of uterine tumors was variable, and the type of neoplasm could only be determined by taking biopsies of the mass. PMID:25028432

  3. Mammary neoplasms of the bitch.

    PubMed

    Cotchin, E

    1958-01-01

    In this paper, the interrelationships of the neoplasms of the canine mammary gland are investigated. These neoplasms are a group of tumors of a great variety of histological structure and sometimes of uncertain histogenesis. Particular attention is given to the histogenesis of the mucoid, cartilaginous, and bony elements. From 1950-56, a macroscopic and histological examination of mammary neoplasms from 424 bitches (2-17 years of age) was made. The tumors from 381 bitches were removed surgically while the others came from 43 bitches who were examined postmortem. Of the 160 tumors whose location was recorded, 105 occurred in the 2 hinder glands, 19 in the middle glands, and 46 in one or another of the 2 anterior glands. 186 of the 424 bitches bore malignant mammary tumors (87 carcinomas, 73 sarcomas, 27 complex malignant tumors) and 249 had benign tumors (19 simple and 230 complex). 40 of the benign complex tumors contained bone, an additional 63 contained cartilage but no bone, and 67 showed mucoid tissue but no cartilage or bone. It is suggested that there is a predominant proliferation of myoepithelial cells which tend to become embedded in a mucoid or chondroid matrix. The bone in the tumors appears to be formed by endochondral ossification of preformed cartilage, or by intramembranous ossification in the connective tissue of the tumor. Metastases were present in 41 of the 424 bitches. PMID:12311486

  4. Multiple cystic lung disease.

    PubMed

    Ferreira Francisco, Flavia Angélica; Soares Souza, Arthur; Zanetti, Gláucia; Marchiori, Edson

    2015-12-01

    Multiple cystic lung disease represents a diverse group of uncommon disorders that can present a diagnostic challenge due to the increasing number of diseases associated with this presentation. High-resolution computed tomography of the chest helps to define the morphological aspects and distribution of lung cysts, as well as associated findings. The combination of appearance upon imaging and clinical features, together with extrapulmonary manifestations, when present, permits confident and accurate diagnosis of the majority of these diseases without recourse to open-lung biopsy. The main diseases in this group that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis and folliculin gene-associated syndrome (Birt-Hogg-Dubé); other rare causes of cystic lung disease, including cystic metastasis of sarcoma, are also discussed. Disease progression is unpredictable, and understanding of the complications of cystic lung disease and their appearance during evolution of the disease are essential for management. Correlation of disease evolution and clinical context with chest imaging findings provides important clues for defining the underlying nature of cystic lung disease, and guides diagnostic evaluation and management. PMID:26621970

  5. Heterogeneity and persistence length in human ocular mucins.

    PubMed Central

    Round, A N; Berry, M; McMaster, T J; Stoll, S; Gowers, D; Corfield, A P; Miles, M J

    2002-01-01

    Atomic force microscopy (AFM) has been used to investigate the heterogeneity and flexibility of human ocular mucins and their subunits. We have paid particular attention, in terms of theory and experiment, to the problem of inducing the polymers to assume equilibrium conformations at a surface. Mucins deposited from a buffer containing Ni(2+) ions adopt extended conformations on mica akin to those observed for DNA under similar conditions. The heterogeneity of the intracellular native mucins is evident from a histogram of contour lengths, reflecting, in part, the diversity of mucin gene products expressed. Reduction of the native mucin with dithiothreitol, thereby breaking the S==S bonds between cysteine residues, causes a marked reduction in polymer length. These results reflect the modes of transport and assembly of newly synthesized mucins in vivo. By modifying the worm-like chain model for applicability to two dimensions, we have confirmed that under the conditions employed mucin adsorbs to mica in an equilibrated conformation. The determined persistence length of the native mucin, 36 nm, is consistent with that of an extended, flexible polymer; such characteristics will influence the properties of the gels formed in vivo. PMID:12202389

  6. Annexin II mediates the neutrophil elastase-stimulated exocytosis of mucin 5ac.

    PubMed

    Xu, Rui; Li, Qi; Zhou, Xiangdong; Perelman, Juliy M; Kolosov, Victor P

    2014-01-01

    The overexpression and hypersecretion of mucus is a hallmark of several chronic pulmonary inflammatory diseases, including chronic obstructive pulmonary disease (COPD), asthma and cystic fibrosis. Mucin 5ac (MUC5AC) is a major component of airway mucus. Annexin II (ANXII) has been reported to be expressed in various cells and is associated with the fusion of secretory vesicles. Neutrophil elastase (NE) is present at high concentrations in the airway surface fluid in patients with cystic fibrosis and various other severe diseases. However, the role of ANXII in NE-induced secretion of MUC5AC granules remains unclear. It was determined that NE upregulates the transcription and protein synthesis of ANXII in 16HBE human bronchial epithelial cells. Following stimulation with NE, ANXII is recruited to the cell membrane, as visualised by cell immunochemistry and laser confocal microscopy, and the redistribution of ANXII is inhibited by the protein kinase-C (PKC) inhibitor bisindolylmaleimide I. Conversely, depleting endogenous ANXII decreases MUC5AC secretion into the cell culture supernatant and increases the levels of intracellular MUC5AC protein. The data indicated that ANXII is associated with the secretion of MUC5AC granules. PMID:24247640

  7. Structural insights into bacterial recognition of intestinal mucins.

    PubMed

    Etzold, Sabrina; Juge, Nathalie

    2014-10-01

    The mucosal layer covering our gut epithelium represents the first line of host defenses against the luminal content, while enabling contacts between the resident microbiota and the host. Mucus is mainly composed of mucins, large glycoproteins containing a protein core and a high number of O-linked oligosaccharides. Mucin glycans act as binding sites or carbon sources for the intestinal microbes, thereby functioning as a host-specific determinant affecting the microbiota composition and human health. Reflecting the structural diversity of mucin glycans and their prime location, commensal and pathogenic microbes have evolved a range of adhesins allowing their interaction with the host. However, despite the recognised importance of mucin glycans in modulating intestinal homeostasis, information on carbohydrate-binding proteins from gut bacteria is disparate. This review is focussed on recent structural insights into host-microbe interactions mediated by mucins. PMID:25106027

  8. Altered Mucins (MUC) Trafficking in Benign and Malignant Conditions

    PubMed Central

    Joshi, Suhasini; Kumar, Sushil; Choudhury, Amit; Ponnusamy, Moorthy P.; Batra, Surinder K.

    2014-01-01

    Mucins are high molecular weight O-glycoproteins that are predominantly expressed at the apical surface of epithelial cells and have wide range of functions. The functional diversity is attributed to their structure that comprises of a peptide chain with unique domains and multiple carbohydrate moieties added during posttranslational modifications. Tumor cells aberrantly overexpress mucins, and thereby promote proliferation, differentiation, motility, invasion and metastasis. Along with their aberrant expression, accumulating evidence suggest the critical role of altered subcellular localization of mucins under pathological conditions due to altered endocytic processes. The mislocalization of mucins and their interactions result in change in the density and activity of important cell membrane proteins (like, receptor tyrosine kinases) to facilitate various signaling, which help cancer cells to proliferate, survive and progress to more aggressive phenotype. In this review article, we summarize studies on mucins trafficking and provide a perspective on its importance to pathological conditions and to answer critical questions including its use for therapeutic interventions. PMID:25261375

  9. CT of soft-tissue neoplasms

    SciTech Connect

    Weekes, R.G.; McLeod, R.A.; Reiman, H.M.; Pritchard, D.J.

    1985-02-01

    The computed tomographic scans (CT) of 84 patients with untreated soft-tissue neoplasms were studied, 75 with primary and nine with secondary lesions. Each scan was evaluated using several criteria: homogeneity and density, presence and type of calcification, presence of bony destruction, involvement of multiple muscle groups, definition of adjacent fat, border definition, and vessel or nerve involvement. CT demonstrated the lesion in all 84 patients and showed excellent anatomic detail in 64 of the 75 patients with primary neoplasms. The CT findings were characteristic enough to suggest the histology of the neoplasm in only 13 lesions (nine lipomas, three hemangiomas, one neurofibroma). No malignant neoplasm had CT characteristics specific enough to differentiate it from any other malignant tumor. However, malignant neoplasms could be differentiated from benign neoplasms in 88% of the cases.

  10. Tear Film Mucins: Front Line Defenders of the Ocular Surface; Comparison with Airway and Gastrointestinal Tract Mucins

    PubMed Central

    Hodges, Robin R.; Dartt, Darlene A.

    2014-01-01

    The ocular surface including the cornea and conjunctiva and its overlying tear film are the first tissues of the eye to interact with the external environment. The tear film is complex containing multiple layers secreted by different glands and tissues. Each layer contains specific molecules and proteins that not only maintain the health of the cells on the ocular surface by providing nourishment and removal of waste products but also protect these cells from environment. A major protective mechanism that the corneal and conjunctival cells have developed is secretion of the innermost layer of the tear film, the mucous layer. Both the cornea and conjunctiva express membrane spanning mucins, whereas the conjunctiva also produces soluble mucins. The mucins present in the tear film serve to maintain the hydration of the ocular surface and to provide lubrication and anti-adhesive properties between the cells of the ocular surface and conjunctiva during the blink. A third function is to contribute to the epithelial barrier to prevent pathogens from binding to the ocular surface. This review will focus on the different types of mucins produced by the corneal and conjunctival epithelia. Also included in this review will be a presentation of the structure of mucins, regulation of mucin production, role of mucins in ocular surface diseases, and the differences in mucin production by the ocular surface, airways and gastrointestinal tract. PMID:23954166

  11. Cystic and nodular lung disease.

    PubMed

    Richards, J Caleb; Lynch, David A; Chung, Jonathan H

    2015-06-01

    Diffuse cystic and nodular lung diseases have characteristic imaging findings. The most common causes of cystic lung disease are lymphangioleiomyomatosis and Langerhans cell histiocytosis. Other less common cystic lung diseases include Birt-Hogg-Dube syndrome, lymphocytic interstitial pneumonitis, and light chain deposition disease. Computed tomography is used to differentiate cystic lung disease from emphysema, honeycombing, cavities, and bronchiectasis, which mimic cystic lung disease. Diffuse nodular lung disease are categorized as centrilobular, perilymphatic, and random types. In diffuse nodular lung disease, a specific diagnosis is achieved through a combination of history, physical examination, and imaging findings. PMID:26024606

  12. Testicular neoplasm diagnosed by ultrasound.

    PubMed

    Senay, B A; Stein, B S

    1986-06-01

    The diagnosis of testicular cancer is usually made by the findings of a testicular mass on physical examination. In rare cases a young man will present with retroperitoneal nodes and a normal testicular examination. In such cases a testicular ultrasound may localize the testis which harbors a subclinical neoplasm. In addition serum markers of B-HCG and AFP are essential. As a screening procedure a urine pregnancy test is helpful, since it can be obtained quickly while quantitative B-HCG and APF results are delayed. PMID:3523046

  13. How We Treat Myeloproliferative Neoplasms.

    PubMed

    Harrison, Claire N; McLornan, Donal P; Francis, Yvonne A; Woodley, Claire; Provis, Lizzie; Radia, Deepti H

    2015-06-01

    The present report focuses on management strategies for the myeloproliferative neoplasm according to the structure and processes we use within our center, a large tertiary unit in central London. The standard procedures for achieving an accurate diagnosis and risk stratification and therapeutic strategies for these diseases with a detailed focus on contentious areas are discussed. In the 9 years after the description of the Janus kinase 2 mutation, this field has altered quite radically in several aspects. For example, a new therapeutic paradigm exists, especially for myelofibrosis. We share how our unit has adapted to these changes. PMID:26297273

  14. Microbial-induced meprin β cleavage in MUC2 mucin and a functional CFTR channel are required to release anchored small intestinal mucus

    PubMed Central

    Schütte, André; Ermund, Anna; Becker-Pauly, Christoph; Johansson, Malin E. V.; Rodriguez-Pineiro, Ana M.; Bäckhed, Fredrik; Müller, Stefan; Lottaz, Daniel; Bond, Judith S.; Hansson, Gunnar C.

    2014-01-01

    The mucus that covers and protects the epithelium of the intestine is built around its major structural component, the gel-forming MUC2 mucin. The gel-forming mucins have traditionally been assumed to be secreted as nonattached. The colon has a two-layered mucus system where the inner mucus is attached to the epithelium, whereas the small intestine normally has a nonattached mucus. However, the mucus of the small intestine of meprin β-deficient mice was now found to be attached. Meprin β is an endogenous zinc-dependent metalloprotease now shown to cleave the N-terminal region of the MUC2 mucin at two specific sites. When recombinant meprin β was added to the attached mucus of meprin β-deficient mice, the mucus was detached from the epithelium. Similar to meprin β-deficient mice, germ-free mice have attached mucus as they did not shed the membrane-anchored meprin β into the luminal mucus. The ileal mucus of cystic fibrosis (CF) mice with a nonfunctional cystic fibrosis transmembrane conductance regulator (CFTR) channel was recently shown to be attached to the epithelium. Addition of recombinant meprin β to CF mucus did not release the mucus, but further addition of bicarbonate rendered the CF mucus normal, suggesting that MUC2 unfolding exposed the meprin β cleavage sites. Mucus is thus secreted attached to the goblet cells and requires an enzyme, meprin β in the small intestine, to be detached and released into the intestinal lumen. This process regulates mucus properties, can be triggered by bacterial contact, and is nonfunctional in CF due to poor mucin unfolding. PMID:25114233

  15. Application of X-Ray Fluorescence Analysis to Determine the Elemental Composition of Tissues from Different Ovarian Neoplasms

    NASA Astrophysics Data System (ADS)

    Motevich, I. G.; Strekal, N. D.; Papko, N. M.; Glebovich, M. I.; Shulha, A. V.; Maskevich, S. A.

    2015-03-01

    We present the results of x-ray fluorescence analysis of tissues from healthy ovaries and from ovaries with different pathologies: benign and borderline tumors, mucinous and endometrioid cancers, serous carcinomas. We determine the average copper, zinc, calcium, selenium, cadmium, lead, and mercury levels. We observed that in the benign ovarian tumors, we see a significant decrease in the cadmium, mercury, and lead levels compared with healthy tissues. In the borderline neoplasms, the copper level is reduced relative to zinc (Cu/Zn), cadmium, mercury, and lead, and also the zinc concentration is increased. In the ovarian carcinomas, we observed changes in the ratio of the chemical elements in the tumor tissues, depending on the histologic type. The results obtained can be used for differentiation, diagnosis, and adjustment of treatment for different ovarian neoplasms.

  16. Penile mucinous carcinoma: A case report

    PubMed Central

    ÖZTÜRK, HAKAN

    2015-01-01

    Penile cancer is an extremely rare form of urological cancer that usually originates in the epithelium of the inner preputium or glans. Major etiological factors include phimosis, poor penile hygiene and smoking. Nearly 95% of penile cancers are squamous cell carcinomas and usually occur in the sixth decade of life. The disease exhibits two variants, namely metastatic and atypical disease. Squamous differentiation may also present with mucinous metaplasia. An extremely limited number of case reports on penile cancer are available in the literature. The present study reports the case of a 39-year-old patient with penile mucinous adenocarcinoma who was admitted with the complaint of perineal discharge, which is, to the best of our knowledge, the first case in the literature. The patient underwent total penectomy and inguinal lymph node dissection. The tumor was staged as T4N1M0, according to the European Association of Urology’s tumor-node-metastasis classification system. The patient was treated with pelvic radiotherapy and six cycles of systemic neoadjuvant chemotherapy with cisplatin and paclitaxel simultaneously, over a period of four months. After nine months of follow-up the patient succumbed to the disease due to widespread metastases. PMID:25663900

  17. Mucins Suppress Virulence Traits of Candida albicans

    PubMed Central

    Kavanaugh, Nicole L.; Zhang, Angela Q.; Nobile, Clarissa J.; Johnson, Alexander D.

    2014-01-01

    ABSTRACT Candida albicans is the most prevalent fungal pathogen of humans, causing a variety of diseases ranging from superficial mucosal infections to deep-seated systemic invasions. Mucus, the gel that coats all wet epithelial surfaces, accommodates C. albicans as part of the normal microbiota, where C. albicans resides asymptomatically in healthy humans. Through a series of in vitro experiments combined with gene expression analysis, we show that mucin biopolymers, the main gel-forming constituents of mucus, induce a new oval-shaped morphology in C. albicans in which a range of genes related to adhesion, filamentation, and biofilm formation are downregulated. We also show that corresponding traits are suppressed, rendering C. albicans impaired in forming biofilms on a range of different synthetic surfaces and human epithelial cells. Our data suggest that mucins can manipulate C. albicans physiology, and we hypothesize that they are key environmental signals for retaining C. albicans in the host-compatible, commensal state. PMID:25389175

  18. Cone Beam Computed Tomography Findings in Calcifying Cystic Odontogenic Tumor Associated with Odontome: A Case Report

    PubMed Central

    Phulambrikar, Tushar; Vilas Kant, Sanchita; Kode, Manasi; Magar, Shaliputra

    2015-01-01

    The calcifying cystic odontogenic tumor (CCOT) is a rare cystic odontogenic neoplasm frequently found in association with odontome. This report documents a case of CCOT associated with an odontome arising in the anterior maxilla in a 28-year-old man. Conventional radiographs showed internal calcification within the lesion but were unable to visualize its relation with the adjacent structures and its accurate extent. In this case cone beam computed tomography (CBCT) could accurately reveal the extent and the internal structure of the lesion which aided the presumptive diagnosis of the lesion as CCOT. This advanced imaging technique proved to be extremely useful in the radiographic assessment and management of this neoplasm of the maxilla. PMID:26636128

  19. The Spindle Cell Neoplasms of the Oral Cavity

    PubMed Central

    Shamim, Thorakkal

    2015-01-01

    Spindle cell neoplasms are defined as neoplasms that consist of spindle-shaped cells in the histopathology. Spindle cell neoplasms can affect the oral cavity. In the oral cavity, the origin of the spindle cell neoplasms may be traced to epithelial, mesenchymal and odontogenic components. This article aims to review the spindle cell neoplasms of the oral cavity with emphasis on histopathology. PMID:26351482

  20. [News in cystic fibrosis].

    PubMed

    Delaisi, B

    2013-08-01

    The improvement over the last two decades in the treatment of cystic fibrosis led to an increase in life expectancy approaching 40 years at birth. Logically, the population of adult patients has been increasing and is currently 50% of patients followed in France. These therapeutic advances have justified the establishment in 2003 of a generalized neonatal screening for cystic fibrosis. The latest data of this screening show an incidence of CF of 1/5359 live births, far below the incidence of 1/2500 which was widely accepted twenty years ago. The performance of this screening is currently based on the dosage of trypsin immuno reactive, followed in case of exceeding the threshold of a search of the 30 most common mutations, can detect around 96% of 150 to 200 CF cases every year. Therefore, the possibility of a false negative of the screening cannot be excluded and evocative symptoms of cystic fibrosis, even for children born after 2003, will lead to prescribe a sweat test. While treatments available so far goal consequences of cystic fibrosis, a new therapeutic class to correct the functional defect of the mutated protein, called CFTR modulators, is emerging. Ivacaftor, leader of this new class, belonging to the category of "CFTR potentiator" got its access on the market in September 2012 for patients carrying the G551D mutation. New other molecules, named "CFTR correctors" which can have synergistic effect with ivacaftor and concern patients carrying the most common mutation--DF 508--are under development. PMID:23856023

  1. Uterine adenosarcomas are mesenchymal neoplasms.

    PubMed

    Piscuoglio, Salvatore; Burke, Kathleen A; Ng, Charlotte K Y; Papanastasiou, Anastasios D; Geyer, Felipe C; Macedo, Gabriel S; Martelotto, Luciano G; de Bruijn, Ino; De Filippo, Maria R; Schultheis, Anne M; Ioris, Rafael A; Levine, Douglas A; Soslow, Robert A; Rubin, Brian P; Reis-Filho, Jorge S; Weigelt, Britta

    2016-02-01

    Uterine adenosarcomas (UAs) are biphasic lesions composed of a malignant mesenchymal (ie stromal) component and an epithelial component. UAs are generally low-grade and have a favourable prognosis, but may display sarcomatous overgrowth (SO), which is associated with a worse outcome. We hypothesized that, akin to breast fibroepithelial lesions, UAs are mesenchymal neoplasms in which clonal somatic genetic alterations are restricted to the mesenchymal component. To characterize the somatic genetic alterations in UAs and to test this hypothesis, we subjected 20 UAs to a combination of whole-exome (n = 6), targeted capture (n = 13) massively parallel sequencing (MPS) and/or RNA sequencing (n = 6). Only three genes, FGFR2, KMT2C and DICER1, were recurrently mutated, all in 2/19 cases; however, 26% (5/19) and 21% (4/19) of UAs harboured MDM2/CDK4/HMGA2 and TERT gene amplification, respectively, and two cases harboured fusion genes involving NCOA family members. Using a combination of laser-capture microdissection and in situ techniques, we demonstrated that the somatic genetic alterations detected by MPS were restricted to the mesenchymal component. Furthermore, mitochondrial DNA sequencing of microdissected samples revealed that epithelial and mesenchymal components of UAs were clonally unrelated. In conclusion, here we provide evidence that UAs are genetically heterogeneous lesions and mesenchymal neoplasms. PMID:26592504

  2. Neurobehavioral presentations of brain neoplasms.

    PubMed

    Filley, C M; Kleinschmidt-DeMasters, B K

    1995-07-01

    We studied 8 patients with frontal or temporolimbic neoplasms who had psychiatric presentations to clarify diagnostic criteria for distinguishing psychiatric disease from structural brain lesions and to examine brain-behavior relationships associated with cerebral neoplasms using modern neuroimaging techniques. Medical records were retrospectively reviewed for evidence of neurobehavioral and neurologic manifestations, tumor histologic features, and the results of treatment. Clinical presentations were correlated with tumor location as determined by computed tomography and magnetic resonance imaging. Patients with frontal lobe tumors presented with abulia, personality change, or depression, whereas those with temporolimbic tumors had auditory and visual hallucinations, mania, panic attacks, or amnesia. After treatment, neurobehavioral syndromes abated or resolved in 7 of 8 patients. We recommend that any patient 40 years of age or older with a change in mental state, cognitive or emotional, should have neuroimaging of the brain. Any patient with a psychiatric presentation who has specific neurobehavioral or neurologic findings or an unexpectedly poor response to psychopharmacologic treatment should also have brain imaging. These case reports extend and update observations on the importance of frontal and temporolimbic systems in the pathogenesis of neurobehavioral disorders. PMID:7667978

  3. Human papillomavirus-associated neoplasms of the sinonasal tract and nasopharynx.

    PubMed

    Thavaraj, Selvam

    2016-03-01

    It is now well established that human papillomavirus (HPV) is an important causative factor in a subgroup of head and neck cancer. In the head and neck, while HPV is strongly associated with squamous cell carcinoma arising in the oropharynx, there is a growing interest in HPV-associated neoplasms of non-oropharyngeal origin including those which arise within sinonasal and nasopharyngeal mucosa. This article reviews current literature on the association of HPV with Scheiderian papillomas, sinonasal squamous cell carcinoma, sinonasal undifferentiated carcinoma, carcinoma with adenoid cystic-like features, and nasopharyngeal carcinoma. Several clinical implications of HPV detection in sinonasal and nasopharyngeal carcinomas are briefly discussed. PMID:26482046

  4. The physicochemical characterisation of pepsin degraded pig gastric mucin.

    PubMed

    Abodinar, Atiga; Tømmeraas, Kristoffer; Ronander, Elena; Smith, Alan M; Morris, Gordon A

    2016-06-01

    Mucins are the main macromolecular components of the mucus secretions that cover the oral cavity, gastrointestinal and urogenital tracts of animals. The properties of the mucus secretions are therefore directly correlated with the physicochemical properties of mucin glycoproteins. In this study, mucins were obtained from pig gastric mucous after digestion with pepsin at 37°C for 4h, these mucins were characterised in terms of compositional and hydrodynamic properties. Compositional analysis showed that this mucin contains protein (15%), carbohydrates (55%) of which the constituents are: fucose (4%), galactose (9%), glucosamine (55%), glucosamine (33%) and sialic acid (2%). The latter component gives the mucin polymer a pH-dependant negative charge, with a ζ-potential of -3mV at pH 1.2 up to -11mV at pH 7.4. The weight average molar mass was ∼1×10(6)g/mol and intrinsic viscosity was ∼0.42dL/g although there was a small pH dependency due to the polyelectrolyte behavior of the polymer. The measurements of viscosity versus shear rate showed shear thinning behavior and the critical overlap concentration was determined to be 10-11% w/v indicating a compact structure. Knowledge of these properties is fundamental to the understanding interactions of mucins, with for example, novel drug delivery systems. PMID:26944659

  5. Mucin glycan foraging in the human gut microbiome

    PubMed Central

    Tailford, Louise E.; Crost, Emmanuelle H.; Kavanaugh, Devon; Juge, Nathalie

    2015-01-01

    The availability of host and dietary carbohydrates in the gastrointestinal (GI) tract plays a key role in shaping the structure-function of the microbiota. In particular, some gut bacteria have the ability to forage on glycans provided by the mucus layer covering the GI tract. The O-glycan structures present in mucin are diverse and complex, consisting predominantly of core 1-4 mucin-type O-glycans containing α- and β- linked N-acetyl-galactosamine, galactose and N-acetyl-glucosamine. These core structures are further elongated and frequently modified by fucose and sialic acid sugar residues via α1,2/3/4 and α2,3/6 linkages, respectively. The ability to metabolize these mucin O-linked oligosaccharides is likely to be a key factor in determining which bacterial species colonize the mucosal surface. Due to their proximity to the immune system, mucin-degrading bacteria are in a prime location to influence the host response. However, despite the growing number of bacterial genome sequences available from mucin degraders, our knowledge on the structural requirements for mucin degradation by gut bacteria remains fragmented. This is largely due to the limited number of functionally characterized enzymes and the lack of studies correlating the specificity of these enzymes with the ability of the strain to degrade and utilize mucin and mucin glycans. This review focuses on recent findings unraveling the molecular strategies used by mucin-degrading bacteria to utilize host glycans, adapt to the mucosal environment, and influence human health. PMID:25852737

  6. Mucin glycan foraging in the human gut microbiome.

    PubMed

    Tailford, Louise E; Crost, Emmanuelle H; Kavanaugh, Devon; Juge, Nathalie

    2015-01-01

    The availability of host and dietary carbohydrates in the gastrointestinal (GI) tract plays a key role in shaping the structure-function of the microbiota. In particular, some gut bacteria have the ability to forage on glycans provided by the mucus layer covering the GI tract. The O-glycan structures present in mucin are diverse and complex, consisting predominantly of core 1-4 mucin-type O-glycans containing α- and β- linked N-acetyl-galactosamine, galactose and N-acetyl-glucosamine. These core structures are further elongated and frequently modified by fucose and sialic acid sugar residues via α1,2/3/4 and α2,3/6 linkages, respectively. The ability to metabolize these mucin O-linked oligosaccharides is likely to be a key factor in determining which bacterial species colonize the mucosal surface. Due to their proximity to the immune system, mucin-degrading bacteria are in a prime location to influence the host response. However, despite the growing number of bacterial genome sequences available from mucin degraders, our knowledge on the structural requirements for mucin degradation by gut bacteria remains fragmented. This is largely due to the limited number of functionally characterized enzymes and the lack of studies correlating the specificity of these enzymes with the ability of the strain to degrade and utilize mucin and mucin glycans. This review focuses on recent findings unraveling the molecular strategies used by mucin-degrading bacteria to utilize host glycans, adapt to the mucosal environment, and influence human health. PMID:25852737

  7. Expression profile of mucins in ovarian mucinous tumors: distinguishing primary ovarian from metastatic tumors.

    PubMed

    Wang, Jayson; El-Bahrawy, Mona A

    2014-03-01

    Ovarian mucinous tumors (OMTs) of the intestinal type share morphologic features with primary tumors of other sites, and it can often be difficult to distinguish primary ovarian from metastatic mucinous tumors. MUC1, MUC2, MUC5AC, and MUC6 expressions were studied by immunohistochemistry in 36 OMTs of intestinal type (17 malignant, 19 borderline), 18 pancreatic, 12 biliary, 15 esophageal, 9 gastric, and 7 colorectal/appendiceal adenocarcinomas. All samples were from primary sites, except for colorectal tumors which were from ovarian metastases. Borderline and malignant OMTs show similar mucin immunoprofile, being strongly and uniformly positive for MUC5AC (97.2% of cases), whereas only focally positive for MUC1 (19.4%), MUC2 (38.9%), and MUC6 (22.2%). The positive frequencies of pancreatic adenocarcinomas for MUC1, MUC2, MUC5AC, and MUC6, respectively, were 100%, 16.7%, 94.4%, and 61.1%; for biliary (cholangiocarcinomas) were 91.7%, 0%, 16.7%, and 8.3%; for esophageal carcinomas were 73.3%, 33.3%, 53.3%, and 26.7%; for gastric carcinomas were 44.4%, 44.4%, 44.4%, and 0% and for lower gastrointestinal tract cancers were 28.6%, 85.7%, 42.9%, and 0%. Our study shows that OMTs are usually MUC5AC+/MUC1-, which is different from pancreatic, biliary, esophageal, gastric, and colorectal/appendiceal carcinomas. We recommend that these mucin stains be added to the panel of immunostains to differentiate metastatic tumors to the ovary from primary OMTs. PMID:24487472

  8. Computed tomography examination of periampullary neoplasms.

    PubMed

    Darweesh, R M; Thorsen, M K; Dodds, W J; Kishk, S M; Lawson, T L; Stewart, E T

    1988-01-01

    The hospital records of 24 patients with periampullary neoplasms were reviewed. The clinical triad of jaundice, pain, and weight loss and the radiographic imaging triad of dilated biliary ducts, dilated pancreatic duct, and periampullary mass should suggest the diagnosis of periampullary neoplasm. PMID:3349797

  9. Macromolecular properties and polymeric structure of canine tracheal mucins.

    PubMed Central

    Shankar, V; Virmani, A K; Naziruddin, B; Sachdev, G P

    1991-01-01

    Two high-Mr mucus glycoproteins (mucins), CTM-A and CTM-B, were highly purified from canine tracheal pouch secretions, and their macromolecular properties as well as polymeric structure were investigated. On SDS/composite-gel electrophoresis, a diffuse band was observed for each mucin. Polyacrylamide-gel electrophoresis using 6% gels also showed the absence of low-Mr contaminants in the mucins. Comparison of chemical and amino acid compositions revealed significant differences between the two mucins. Using a static-laser-light-scattering technique, CTM-A and CTM-B were found to have weight-average Mr values of about 11.0 x 10(6) and 1.4 x 10(6) respectively. Both mucins showed concentration-dependent aggregation in buffer containing 6 M-guanidine hydrochloride. Under similar experimental conditions, reduced-alkylated CTM-A had an Mr of 5.48 x 10(6) and showed no concentration-dependent aggregation. Hydrophobic properties of the mucins, investigated by the fluorescent probe technique using mansylphenylalanine as the probe, showed the presence of a large number of low-affinity (KD approx. 10(5) M) binding sites. These sites appeared to be located on the non-glycosylated regions of the protein core, since Pronase digestion of the mucins almost completely eliminated probe binding. Reduction of disulphide bonds of CTM-A and CTM-B did not significantly alter the probe-binding properties. Also, addition of increasing NaCl concentrations (0.03-1.0 M) to the buffer caused only a small change in the hydrophobic properties of native and reduced-alkylated mucins. CTM-A was deglycosylated, without notable in the hydrophobic properties of native and reduced-alkylated mucins. CTM-A was deglycosylated, without notable degradation, using a combination of chemical and enzymic methods. On SDS/PAGE the protein core was estimated to have an Mr of approx. 60,000. On the basis of the protein and carbohydrate contents of the major mucin CTM-A, the mucin monomer was calculated to have an

  10. [Physiological role of mucins in the colonic barrier integrity].

    PubMed

    Gaudier, Estelle; Hoebler, Christine

    2006-01-01

    Colonic mucus is a key element of colonic barrier as it is located at the frontier between luminal microflora and colonic mucosa itself. Colonic mucus is mainly composed of high molecular weight glycoproteins called mucins that can be either secreted or membrane-linked. The expression of various colonic mucins is altered in colorectal cancers or inflammations. The aim of this review is to highlight the crucial role played by colonic mucins in the maintenance of colonic barrier integrity, both because they are part of the protective mucus layer, and because they individually exert specific functions involved in epithelial barrier, like cell growth and differentiation, immunomodulation, signal transduction or cell adhesion. PMID:17075443

  11. KRAS mutation in adenocarcinoma of the gastrointestinal type arising from a mature cystic teratoma of the ovary.

    PubMed

    Li, Yan; Zhang, Ruiguang; Pan, Danzhen; Huang, Bangxing; Weng, Mixia; Nie, Xiu

    2014-01-01

    Mature cystic teratomas (MCT) in the ovary rarely undergo malignant transformation. Moreover, adenocarcinoma of the gastrointestinal type is much rarer. We present two cases of perimenopausal female pateints with mature cystic teratoma of single ovary, while local adenocarcinoma arising in the MCT. The malignancies showed immunohistochemical features of intestinal differentiation, such as strong positivity for CDX-2, villin and CK-20, and negativity for CK-7. Furthermore, the mutation analysis of molecular alteration revealed a KRAS gene mutation in the intestinal adenocarcinoma part, extending into benign intestinal-type epithelium linings. Yet the mutation was not present in the epidermal component of the teratoma. We present these as two unique cases of mucinous adenocarcinoma of the intestinal type arising from mature cystic teratoma. Moreover, we also submit that this KRAS mutation might contribute to identify malignant transformation of a MCT and suggest possible effect on targeted treatment decisions for anti-epidermal growth factor receptor (EGFR) therapy in metastasized patients. PMID:25297496

  12. [Lysophosphatidic acid and malignant neoplasms].

    PubMed

    Jesionowska, Anna; Cecerska-Heryć, Elżbieta; Marczuk, Natalia; Safranow, Krzysztof; Dołęgowska, Barbara

    2015-01-01

    Lysophosphatidic acid (LPA) is a lipid compound which plays an important role in the human body, enabling its proper development and functioning. The extracellular LPA is mainly formed of lysophospholipids by the action of autotaxin. LPA activates specific G protein coupled receptors on the cell surface, which results in activation of intracellular signaling pathways, resulting in an increased production of proteins such as VEGF, MMP and uPA. The effect is increased cell proliferation, migration, survival and morphological changes. Aberrant expression of LPA receptors or autotaxin is present in various neoplasms. LPA may be used as a potential diagnostic marker, because its concentrations in the plasma of ovarian cancer patients are significantly higher than in the control group. Scientific research is focused on the searching for the compounds that inhibit the effects of LPA. The promising results of preclinical trials suggest potential usefulness of these compounds in the fight against cancer. PMID:27048092

  13. [Benign neoplasms of female urethra].

    PubMed

    Usunova, I; Vladimirov, V

    2009-01-01

    In clinical practice neoplasms of female urethra are found usually in adult women. They can also be found in adolescent girls and as rare congenital abnormality. Those conditions are most frequently detected during gynecological or urological examination. Symptoms are few. Lesions are situated at the outer orifice of urethra at the broad basis. Authors have diagnosed and treated 331 patients between 26 and 87 years. Electro coagulation has been performed in 185 patients. Surgical excision has been performed in 41 patients. Excision with following electrocoagulation has been performed in 18 patients. Conservative treatment has been performed in 87 patients. Histological sample analysis has provided diagnosis of urethral polyp, caruncle and mucosal prolaps. Second electrocoagulation after surgical excision has been performed in 5 patients. Collaboration between urologists and gynecologists is essential for early diagnosis, prophylaxis and successful treatment of above mentioned diseases. PMID:19496462

  14. The Cystic Fibrosis Intestine

    PubMed Central

    De Lisle, Robert C.; Borowitz, Drucy

    2013-01-01

    The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis transmembrane regulator protein (CFTR). The majority of people with CF have a limited life span as a consequence of CFTR dysfunction in the respiratory tract. However, CFTR dysfunction in the gastrointestinal (GI) tract occurs earlier in ontogeny and is present in all patients, regardless of genotype. The same pathophysiologic triad of obstruction, infection, and inflammation that causes disease in the airways also causes disease in the intestines. This article describes the effects of CFTR dysfunction on the intestinal tissues and the intraluminal environment. Mouse models of CF have greatly advanced our understanding of the GI manifestations of CF, which can be directly applied to understanding CF disease in humans. PMID:23788646

  15. Cystic Lymphangioma of Pancreas.

    PubMed

    Bihari, Chhagan; Rastogi, Archana; Rajesh, S; Arora, Ankur; Arora, Asit; Kumar, Nikhil

    2016-03-01

    Lymphangiomas are benign hamartomatous malformations which can arise either from congenitally sequestered lymphatic channels or due to acquired obstruction caused by fibrosis of lymph channels. They are common in the pediatric age group in the soft tissue of neck and the axilla. Abdominal lymphangiomas are rare; even rarer is the primary involvement of pancreas. It occurs more frequently in females and is often located in the distal pancreas. The authors report the case of cystic lymphangioma of pancreas in a 26-year old female presenting with recurrent episodes of upper abdominal pain that was treated with laparoscopic cyst excision. Although exceptionally rare, lymphangioma of the pancreas should be considered in the differential diagnosis of pancreatic cystic lesions, especially in young women. PMID:27065694

  16. Cervicothoracic cystic dysraphism.

    PubMed

    Valeur, Natalie S; Iyer, Ramesh S; Ishak, Gisele E

    2016-09-01

    Cystic dysraphism of the cervical and upper thoracic spine is very rare. It differs from the much more common lumbosacral dysraphism in appearance and structure, and usually portends a better prognosis due to lack of functional neurological tissue in the dysraphic sac and absent or less severe intracranial anomalies. There is ambiguity in the literature regarding terminology because of the paucity of cases. We present cases of the most common type of cervicothoracic cystic dysraphism and emphasize differences from lumbosacral myelomeningocele. Patient outcome depends on the presence of associated anomalies and whether complete surgical resection is performed. Imaging plays a critical role in surgical planning, screening the central nervous system for additional anomalies, and in the postoperative setting for evaluation of retethering. PMID:27147079

  17. Stratified mucin-producing intraepithelial lesion with invasive carcinoma: 12 cases with immunohistochemical and ultrastructural findings.

    PubMed

    Onishi, Junji; Sato, Yuichiro; Sawaguchi, Akira; Yamashita, Atsushi; Maekawa, Kazunari; Sameshima, Hiroshi; Asada, Yujiro

    2016-09-01

    Stratified mucin-producing intraepithelial lesion (SMILE) is considered to be a variant of adenocarcinoma in situ (defined as intraepithelial malignant glandular epithelium without invasion) or adenosquamous carcinoma in situ of the uterine cervix. However, recent study suggested that SMILE is more similar to high-grade squamous epithelial lesion by their immunohistochemical findings. An invasive form of SMILE "invasive stratified mucin-producing carcinoma (ISMC)" has been also proposed, but immunohistochemical features are not well documented. Therefore, this study aimed to clarify the immunohistochemical characteristics of SMILE and ISMC. Twelve cases of SMILE were found among 445 patients (2.7%) with high-grade intraepithelial lesions or invasive carcinomas, 3 of whom had solely intraepithelial disease with SMILE component (mean age, 37 years; range, 30-48 years) and 9 with invasive carcinomas (mean age, 47 years; range, 37-66 years; including ISMC). Immunohistochemically, SMILE and ISMC were diffusely positive for p16 and CAM5.2, focally for IMP3, and almost negative or only focally positive for p63. Nuclear signals in SMILE and invasive carcinomas were detected by human papillomavirus (HPV) in situ hybridization; 5 cases showed HPV16 and/or HPV18 polymerase chain reaction products. The ultrastructural study of 1 case showed surface microvilli and small vacuolar structure in SMILE; ISMC had mucous-like vacuoles, many mitochondria and intracytoplasmic lumen but lacked tonofilament. These findings were more similar to adenocarcinoma in situ or adenocarcinoma than squamous intraepithelial lesion or squamous cell carcinoma. We suggest that SMILE is an intraepithelial neoplasm and ISMC is an invasive form of SMILE. PMID:27237368

  18. Cystic pulmonary hydatidosis

    PubMed Central

    Sarkar, Malay; Pathania, Rajnish; Jhobta, Anupam; Thakur, Babu Ram; Chopra, Rajesh

    2016-01-01

    Cystic echinococcosis (CE) is a zoonotic parasitic disease caused by the larval stages of the cestode Echinococcus granulosus. Worldwide, pulmonary hydatid cyst is a significant problem medically, socially, and economically. Surgery is the definitive therapy of pulmonary hydatidosis. Benzimidazoles may be considered in patients with a surgical contraindication. This review will focus on pathogenesis, lifecycle, clinical features, and management of pulmonary hydatid disease. PMID:27051107

  19. Epidemiology of Cystic Fibrosis.

    PubMed

    Spoonhower, Kimberly A; Davis, Pamela B

    2016-03-01

    Improved quality of care and rapidly emerging therapeutic strategies to restore chloride transport profoundly impact the epidemiology and pathobiology of cystic fibrosis (CF) in the twenty-first century. CF now serves as a model for chronic illness management, continuous quality improvement via registry data, and a seamless link between basic science research, translational studies, clinical trials, and outcomes research to enable rapid expansion of treatment options. PMID:26857763

  20. Calcifying cystic odontogenic tumor associated with an odontome – a diverse lesion encountered

    PubMed Central

    Radheshyam, Chourasia; Alokenath, Bandyopadhyay; Kumar, Harish; Abikshyeet, Panda

    2015-01-01

    The human jaw is an exclusive habitat for odontogenic lesions. Ghost cells associated odontogenic lesions are a diverse group with a variety of presentations in the jaws. Calcifying cystic odontogenic tumor is a benign cystic neoplasm of odontogenic origin which demonstrates ghost cells in the epithelial component. This tumor sometimes mimics the features of a cyst clinically and radiographically, but histopathologically as well as behavior-wise shows the features of a tumor. Many classification systems have been proposed and revised from time to time. Presently a dualistic concept is highlighted to classify this group of lesions. The present case highlights a case of calcifying cystic odontogenic tumor associated with a complex composite odontome, which appeared like a cyst clinically and radiographically. PMID:26345145

  1. Mucin-Type O-Glycosylation in Gastric Carcinogenesis.

    PubMed

    Duarte, Henrique O; Freitas, Daniela; Gomes, Catarina; Gomes, Joana; Magalhães, Ana; Reis, Celso A

    2016-01-01

    Mucin-type O-glycosylation plays a crucial role in several physiological and pathological processes of the gastric tissue. Modifications in enzymes responsible for key glycosylation steps and the consequent abnormal biosynthesis and expression of their glycan products constitute well-established molecular hallmarks of disease state. This review addresses the major role played by mucins and associated O-glycan structures in Helicobacter pylori adhesion to the gastric mucosa and the subsequent establishment of a chronic infection, with concomitant drastic alterations of the gastric epithelium glycophenotype. Furthermore, alterations of mucin expression pattern and glycan signatures occurring in preneoplastic lesions and in gastric carcinoma are also described, as well as their impact throughout the gastric carcinogenesis cascade and in cancer progression. Altogether, mucin-type O-glycosylation alterations may represent promising biomarkers with potential screening and prognostic applications, as well as predictors of cancer patients' response to therapy. PMID:27409642

  2. Respiratory mucins: identification of core proteins and glycoforms.

    PubMed

    Thornton, D J; Carlstedt, I; Howard, M; Devine, P L; Price, M R; Sheehan, J K

    1996-06-15

    At least eight mucin apoproteins are expressed by the tracheobronchial epithelium, but it is not known which, if any, of these are major constituents of the respiratory secretions responsible for the formation of the mucus gel. To address this we have isolated mucins from normal, asthmatic and chronic bronchitic secretions. The asthmatic mucin reduced subunits were fractionated into four populations (I-IV) by anion-exchange HPLC. Amino acid and monosaccharide compositional analysis, as well as M(r) and size measurements, indicate that two of these populations (I and II) are glycoforms of the same or related apoprotein(s) and that the other populations contain two different apoproteins. A panel of antibodies and antisera recognizing the variable number tandem repeat (VNTR) of specific mucin apoproteins did not, as predicted, react with the glycosylated molecules, but after deglycosylation the majority of these probes (with the exception of those to MUC2, which were negative) reacted at a low level with each of the subunit populations. In contrast, an antiserum against a non-VNTR sequence of MUC5AC identified one of the populations (III) as the MUC5AC mucin. The MUC5AC reduced subunit had an M(r) of 2.2 x 10(6) and an RG (radius of gyration) of 57 nm. The genetic identities of the major mucin (populations I and II) and a minor component (population IV) were not established. The MUC5AC mucin was also identified as a major component in the pooled normal secretions from 20 individuals, whereas in a chronic bronchitic sample it was only a minor constituent. Furthermore, in all these different respiratory secretions the MUC5AC mucin appears as a similar biochemical entity, as assessed by Mono Q chromatography and agarose electrophoresis, suggesting that it may have a well-defined pattern of glycosylation in the respiratory tract. PMID:8670177

  3. Solid Variant of Adenoid Cystic Carcinoma: Difficulties in Diagnostic Recognition.

    PubMed

    Ben Salha, Imen; Bhide, Shree; Mourtzoukou, Despoina; Fisher, Cyril; Thway, Khin

    2016-08-01

    Adenoid cystic carcinoma (ACC) is a malignant neoplasm that mainly affects the salivary glands but has been described in many other anatomical sites. It is composed of basaloid cells with myoepithelial/basal cell differentiation and ductal epithelial cells that proliferate in a fibrous stroma, with variable amounts of myxohyaline material. Three patterns (cribriform, tubular, and solid) occur, and the solid variant is characterized by a predominant compact sheet-like and nested pattern of rounded basaloid cells lacking obvious cribriform or tubular architecture. The solid variant has significant morphological and immunohistochemical overlap with a large range of neoplasms of different lineages, including other carcinomas and sarcomas. We describe a case of solid variant ACC of the paranasal sinuses, which showed an almost entirely solid pattern of growth (in >95% of cells) and which on initial biopsy showed no features of classical ACC. This highlights the potential for diagnostic misinterpretation with a variety of other neoplasms, which is particularly important because of the significant difference in treatment for ACC and tumors in its differential diagnosis. PMID:27069025

  4. Pulmonary-type adenocarcinoma and signet ring mucinous adenocarcinoma arising in an ovarian dermoid cyst: report of a unique case.

    PubMed

    Boyd, Clinton; Patel, Kaushik; O'Sullivan, Brendan; Taniere, Philippe; McCluggage, W Glenn

    2012-11-01

    Dermoid cysts are common benign ovarian germ cell neoplasms. Occasionally, one of the mature elements undergoes malignant transformation resulting in the formation of a somatic malignancy; most commonly this is squamous carcinoma. We report a unique case where 2 separate malignancies arose within a dermoid cyst, one a signet ring mucinous adenocarcinoma and the other a pulmonary-type adenocarcinoma. There have been only occasional earlier case reports of a possible pulmonary-type adenocarcinoma arising in a dermoid cyst. In the case we report, the pulmonary-type adenocarcinoma was closely associated with a bronchial structure and exhibited diffuse positive immunohistochemical staining with TTF1, PE10, and napsin A. Molecular studies revealed no evidence of epidermal growth factor receptor mutation, a molecular alteration which may be found in primary pulmonary adenocarcinoma. PMID:23026196

  5. Chemically tunable mucin chimeras assembled on living cells

    PubMed Central

    Kramer, Jessica R.; Onoa, Bibiana; Bustamante, Carlos; Bertozzi, Carolyn R.

    2015-01-01

    Mucins are a family of secreted and transmembrane glycoproteins characterized by a massive domain of dense O-glycosylation on serine and threonine residues. Mucins are intimately involved in immunity and cancer, yet elucidation of the biological roles of their glycodomains has been complicated by their massive size, domain polymorphisms, and variable glycosylation patterns. Here we developed a synthetic route to a library of compositionally defined, high-molecular weight, dual end-functionalized mucin glycodomain constructs via N-carboxyanhydride polymerization. These glycopolypeptides are the first synthetic analogs to our knowledge to feature the native α-GalNAc linkage to serine with molecular weights similar to native mucins, solving a nearly 50-year synthetic challenge. Physical characterization of the mimics revealed insights into the structure and properties of mucins. The synthetic glycodomains were end-functionalized with an optical probe and a tetrazine moiety, which allowed site-specific bioorthogonal conjugation to an engineered membrane protein on live mammalian cells. This strategy in protein engineering will open avenues to explore the biological roles of cell surface mucins. PMID:26420872

  6. Regulation of mucin secretion from in vitro cellular models.

    PubMed

    Davis, C William

    2002-01-01

    Conceptually, in vitro models for airway mucin secretion may provide useful information pertinent to many aspects of goblet cell biology/physiology. Such models may be especially useful in identifying potential secretagogues, probing the distribution of receptors between goblet cell apical and basolateral membrane domains, and revealing intracellular messenger pathways underlying receptor activation. We have focused most recently on human bronchial epithelial cell cultures grown as tracheal xenografts and SPOC1 cell cultures. These two models are remarkably similar with respect to the regulation of mucin secretion: luminal challenges with the P2Y2 purinoceptor agonists ATP or UTP elicit mucin secretion with EC50s of about 3 microM and archetypal agonists to other purinoceptors test negative. P2Y2 purinoceptors typically couple via Gq to phospholipase C, suggesting that intracellular Ca2+ and protein kinase C (PKC) are important in activating intracellular pathways leading to goblet cell mucin release. Consistent with this notion, phorbol myristate acetate and ionomycin elicit mucin secretion from SPOC1 cells and HBE xenografts, whereas cyclic nucleotides do not. Delineation of the molecules comprising these receptor/messenger interactions and their supporting pathways remains an important challenge for the development of drugs effective in therapeutic interventions in mucin hypersecretory airway diseases; with these models we have initiated the process. PMID:12568491

  7. Mucin function in inflammatory bowel disease: an update.

    PubMed

    Boltin, Doron; Perets, Tsachi T; Vilkin, Alex; Niv, Yaron

    2013-02-01

    MUC2 is the primary component of the mucin barrier that separates the intestinal microbiota and the intestinal epithelium. This mucous barrier is affected by both luminal/microbial factors and host/immune factors, both of which have genetic and environmental determinants. The complex interactions between these players in health and disease states are not fully understood. Inflammatory bowel disease (IBD) has both genetic and environmental etiologies that lead to the breakdown of the epithelial barrier. In this review, we explore the up-to-date evidence that implicates mucin in the pathogenesis of IBD. In IBD, quantitative changes in mucin secretion occur, as well as structural changes in mucin's glycoprotein core and the sulfation and sialylation of mucin's oligosaccharide residues. These changes are associated with a diminished functionality of the mucous barrier. We identify the various genetic mutations associated with these changes and outline the animal models that have enhanced the current understanding of the genetic basis for IBD. Further study is needed to better characterize the immune and genetic influences on mucin expression and secretion and role of endoplasmic reticulum stress and a defective unfolded protein response in mediating these changes. PMID:23164684

  8. Antiviral activity of purified human breast milk mucin.

    PubMed

    Habte, Habtom H; Kotwal, Girish J; Lotz, Zoë E; Tyler, Marilyn G; Abrahams, Melissa; Rodriques, Jerry; Kahn, Delawir; Mall, Anwar S

    2007-01-01

    Human breast milk is known to contain numerous biologically active components which protect breast fed infants against microbes, viruses, and toxins. The purpose of this study was to purify and characterize the breast milk mucin and determine its anti-poxvirus activity. In this study human milk mucin, free of contaminant protein and of sufficient quantity for further analysis, was isolated and purified by Sepharose CL-4B gel filtration and cesiumchloride density-gradient centrifugation. Based on the criteria of size and appearance of the bands and their electrophoretic mobility on sodium dodecyl sulfate polyacrylamide-gel electrophoresis, Western blotting together with the amino acid analysis, it is very likely that the human breast milk mucin is MUC1. It was shown that this breast milk mucin inhibits poxvirus activity by 100% using an inhibition assay with a viral concentration of 2.4 million plaque-forming units/ml. As the milk mucin seems to aggregate poxviruses prior to their entry into host cells, it is possible that this mucin may also inhibit other enveloped viruses such as HIV from entry into host cells. PMID:17361093

  9. Male reproductive system neoplasms. Special listing

    SciTech Connect

    Not Available

    1981-07-01

    This Special Listing of Current Cancer Research Projects is a publication of the International Cancer Research Data Bank (ICRDB) Program of the National Cancer Institute. Each Listing contains descriptions of ongoing projects in one selected cancer research area. The research areas include: Experimental prostate carcinogenesis and related biology; Epidemiology of prostatic neoplasms; Preclinical studies of prostatic cancers; Diagnosis and prognosis of prostatic cancer; Therapy of prostatic cancer; Experimental testicular carcinogenesis and related biology; Epidemiology of testicular cancer; Diagnosis, prognosis and treatment of testicular neoplasms; Penile and other reproductive system neoplasms.

  10. Microfocus of Anaplastic Carcinoma Arising in Mural Nodule of Ovarian Mucinous Borderline Tumor With Very Rapid and Fatal Outcome.

    PubMed

    Mhawech-Fauceglia, Paulette; Ramzan, Amin; Walia, Saloni; Pham, Huyen Q; Yessaian, Annie

    2016-07-01

    A 36-yr-old woman presented with abdominal discomfort. A computed tomography scan revealed a large left cystic and solid pelvic mass without evidence of metastatic disease. Total hysterectomy with bilateral salpingo-oophorectomy and tumor staging was performed. Grossly, the ovarian mass measured 20×18 cm and the cut surface was multiloculated with 1 single mural nodule measuring 2×1.5 cm. The histologic diagnosis of ovarian mucinous borderline tumor with a microfocus of anaplastic carcinoma arising in sarcoma-like mural nodule, FIGO Stage IA was rendered. After 3 mo, the patient returned with symptomatic anemia. A computed tomography scan showed enlarged retroperitoneal and pelvic lymph nodes. Image-guided biopsy of the pelvic lymph node showed a metastatic anaplastic carcinoma from her primary ovarian carcinoma. Chemotherapy was initiated, but the patient developed fulminant disseminated intravascular coagulation within <1 wk of her presentation which was fatal. PMID:26598983

  11. Recurrence of cystic adventitial disease in an interposed vein graft.

    PubMed

    Ohta, T; Kato, R; Sugimoto, I; Kondo, M; Tsuchioka, H

    1994-09-01

    A case of cystic adventitial disease (CAD) of the popliteal artery with intermittent claudication in the left calf is reported. This patient was first treated by total excision of the cyst and the involved artery followed by graft interposition with an autogenous saphenous vein. Recurrence of CAD in the interposed vein graft was noted after 6 months, and excision of the cyst with the involved graft and graft interposition with an autogenous saphenous vein was again required. We consider that the definitive evidence from early recurrence in the interposed vein graft shown in this case will close the discussion of the cause of CAD. The cystic lesion is thought by some to originate from the adventitia; traumatic, embryologic, or systemic abnormality theories were proposed. We assume that the mucin-secreting synovial cells originating from the neighboring joint capsule, tendon sheath, or, in some cases, from the ganglion itself, directly invade the adventitia through any rough or injured crack caused by trauma to the adventitia or simply attach to and encircle the adventitia. Total resection of the lesion with grafting is the recommended treatment for CAD because of the excellent results. However, as shown in our case, complete removal of the synovial cells even by resection technique may be difficult; therefore intensive follow-up is fundamentally necessary in this disease. PMID:8079189

  12. Myeloproliferative Neoplasms: A Contemporary Review.

    PubMed

    Tefferi, Ayalew; Pardanani, Animesh

    2015-04-01

    Polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) constitute the BCR-ABL1-negative myeloproliferative neoplasms and are characterized by mutually exclusive Janus kinase 2 (JAK2), calreticulin (CALR), and myeloproliferative leukemia virus oncogene (MPL) mutations; respective frequencies of these mutations are approximately 95%, 0%, and 0% in PV, 60%, 20%, and 3% in ET, and 60%, 25%, and 7% in PMF. These mutations might be accompanied by other mutations that are less specific to myeloproliferative neoplasms but are prognostically relevant, such as additional sex combs-like 1 (ASXL1). Characteristic bone marrow morphology is required for World Health Organization-compliant diagnosis, especially in distinguishing ET from prefibrotic PMF and masked PV. Survival is the longest in ET, although still inferior to that of the age- and sex-matched control population; median survivals for patients younger than 60 years are approximately 33 years for ET, 24 for PV, and 15 for PMF. Major disease complications include thrombosis and leukemic or fibrotic transformation. In PV and ET, risk factors for survival include older age, leukocytosis, and thrombosis, whereas JAK2 mutation in ET is associated with increased risk of thrombosis. In PMF, type 1 or type 1-like CALR mutations are associated with superior and ASXL1 with inferior survival. Prevention of thrombosis in PV is secured by phlebotomy (hematocrit target <45%) and in both PV and ET by low-dose aspirin therapy; high-risk patients derive additional antithrombotic benefit from cytoreductive therapy with hydroxyurea as first-line and interferon-alfa and busulfan as second-line drugs of choice. Although the JAK inhibitor ruxolitinib was recently approved for use in hydroxyurea-resistant PV, its role in routine clinical practice remains debatable. In myelofibrosis, stem cell transplant is the current treatment of choice for genetically or clinically high-risk disease; for all other patients

  13. Usefulness of KL-6 in the subtyping of intraductal papillary mucinous neoplasia of the pancreas, including carcinoma, dysplasia, and hyperplasia.

    PubMed

    Ohtsuki, Yuji; Watanabe, Ryohei; Kimura, Masashi; Nomura, Katsuyoshi; Maeda, Tomoharu; Kito, Katsumi; Takeji, Miyuki; Lee, Gang-Hong; Furihata, Mutsuo

    2015-06-01

    KL-6 is known as a useful serum biomarker of the disease activity in interstitial pneumonias. We investigated its usefulness as a biomarker for subtyping intraductal papillary mucinous neoplasms (IPMNs) of the pancreas. IPMNs are generally divided into 4 subtypes, namely pancreatobiliary (PB), intestinal (INT), gastric (GS), and oncocytic (ONC). Aside from the KL-6 antibody, the MUC1, MUC2, MUC5AC, MUC6, and MIB-1 antibodies were also examined. Eighteen IPMN cases were examined, including 12 cases of intraductal papillary mucinous carcinomas (IPMCs) simultaneously associated with dysplasia (IPMDs) and hyperplasia (IPMHs) and 6 IPMD cases with IPMH. KL-6 antibody was positive in the 8 IPMC cases, corresponding to a MUC2-negative PB subtype, but negative in 4 IPMC cases, corresponding to the INT subtype, which is positive for MUC2. IPMD of moderate-to-severe degree positively stained for the KL-6 antibody in the IPMC cases of the PB subtype but not in those of the INT subtype. The IPMH cases were mostly negative for KL-6, similar to the mild IPMD cases. In the 6 cases of mild IPMD and/or IPMH, KL-6 and MUC2 expressions were mostly negative. In conclusion, the KL-6 antibody is immunohistochemically a good biomarker of the PB subtype of IPMC, but not the INT subtype. Identifying IPMN subtypes based on KL-6 stainability would be useful. Clinicopathological studies with more IPMC cases might be needed for further progress in this field of study. PMID:24908068

  14. Pseudomonas aeruginosa pyocyanin modulates mucin glycosylation with sialyl-Lewis(x) to increase binding to airway epithelial cells.

    PubMed

    Jeffries, J L; Jia, J; Choi, W; Choe, S; Miao, J; Xu, Y; Powell, R; Lin, J; Kuang, Z; Gaskins, H R; Lau, G W

    2016-07-01

    Cystic fibrosis (CF) patients battle life-long pulmonary infections with the respiratory pathogen Pseudomonas aeruginosa (PA). An overabundance of mucus in CF airways provides a favorable niche for PA growth. When compared with that of non-CF individuals, mucus of CF airways is enriched in sialyl-Lewis(x), a preferred binding receptor for PA. Notably, the levels of sialyl-Lewis(x) directly correlate with infection severity in CF patients. However, the mechanism by which PA causes increased sialylation remains uncharacterized. In this study, we examined the ability of PA virulence factors to modulate sialyl-Lewis(x) modification in airway mucins. We found pyocyanin (PCN) to be a potent inducer of sialyl-Lewis(x) in both mouse airways and in primary and immortalized CF and non-CF human airway epithelial cells. PCN increased the expression of C2/4GnT and ST3Gal-IV, two of the glycosyltransferases responsible for the stepwise biosynthesis of sialyl-Lewis(x), through a tumor necrosis factor (TNF)-α-mediated phosphoinositol-specific phospholipase C (PI-PLC)-dependent pathway. Furthermore, PA bound more efficiently to airway epithelial cells pre-exposed to PCN in a flagellar cap-dependent manner. Importantly, antibodies against sialyl-Lewis(x) and anti-TNF-α attenuated PA binding. These results indicate that PA secretes PCN to induce a favorable environment for chronic colonization of CF lungs by increasing the glycosylation of airway mucins with sialyl-Lewis(x). PMID:26555707

  15. Pseudomonas aeruginosa pyocyanin modulates mucin glycosylation with sialyl-Lewisx to increase binding to airway epithelial cells

    PubMed Central

    Choi, Woosuk; Choe, Shawn; Miao, Jinfeng; Xu, Ying; Powell, Rebecca; Lin, Jingjun; Kuang, Zhizhou; Gaskins, H Rex; Lau, Gee W.

    2015-01-01

    Cystic fibrosis (CF) patients battle life-long pulmonary infections with the respiratory pathogen Pseudomonas aeruginosa (PA). An overabundance of mucus in CF airways provides a favorable niche for PA growth. When compared to that of non-CF individuals, mucus of CF airways is enriched in sialyl-Lewisx, a preferred binding receptor for PA. Notably, the levels of sialyl-Lewisx directly correlate with infection severity in CF patients. However, the mechanism by which PA causes increased sialylation remains uncharacterized. In this study, we examined the ability of PA virulence factors to modulate sialyl-Lewisx modification in airway mucins. We found pyocyanin (PCN) to be a potent inducer of sialyl-Lewisx in both mouse airways and in primary and immortalized CF and non-CF human airway epithelial cells. PCN increased the expression of C2/4GnT and ST3Gal-IV, two of the glycosyltransferases responsible for the stepwise biosynthesis of sialyl-Lewisx, through a TNF-α-mediated phosphoinositol-specific phospholipase C (PI-PLC) dependent pathway. Furthermore, PA bound more efficiently to airway epithelial cells pre-exposed to PCN through a flagellar cap-dependent manner. Importantly, antibodies against sialyl-Lewisx and anti-TNF-α attenuated PA binding. These results indicate that PCN secretes PCN to induce a favorable environment for chronic colonization of CF lungs by increasing the glycosylation of airway mucins with sialyl-Lewisx. PMID:26555707

  16. Neoplasms of the Small Bowel

    PubMed Central

    Silberman, Howard; Crichlow, Robert W.; Caplan, Howard S.

    1974-01-01

    Small bowel tumors are unusual lesions exhibiting nonspecific clinical features often diagnosed at an advanced stage. In the cases studied at the Hospital of the University of Pennsylvania nearly all the 32 patients with malignancies were symptomatic whereas in the 34 patients with benign lesions the condition was discovered as an incidental finding in about half of the patients. Weight loss, palpable mass or anemia usually indicated malignancy. Small bowel radiography was the most useful diagnostic aid in the present series. While the etiology of these lesions is unknown, villous adenomas probably bear a relationship to carcinoma. The association between chronic regional enteritis and small bowel tumors is unestablished but suggestive. An analysis of reported series reveals a disproportionate incidence of additional primary tumors in patients with small bowel neoplasms. Surgical extirpation is indicated for curative treatment. In the present series, resection in hope of cure was carried out in 25 of 32 malignant tumors resulting in eight five-year survivals. One of these latter lived nine years with disseminated malignant carcinoid reflecting the occasional indolent course of this tumor. PMID:4842978

  17. Histopathology of gastrointestinal neuroendocrine neoplasms

    PubMed Central

    Hirabayashi, Kenichi; Zamboni, Giuseppe; Nishi, Takayuki; Tanaka, Akira; Kajiwara, Hiroshi; Nakamura, Naoya

    2013-01-01

    Gastrointestinal neuroendocrine neoplasms (GI-NENs) arise from neuroendocrine cells distributed mainly in the mucosa and submucosa of the gastrointestinal tract. In 2010, the World Health Organization (WHO) classification of NENs of the digestive system was changed, categorizing these tumors as grade 1 neuroendocrine tumor (NET), grade-2NET, neuroendocrine carcinoma (large- or small-cell type), or mixed adenoneuroendocrine carcinoma (MANEC). Such a classification is based on the Ki-67 index and mitotic count in histological material. For the accurate pathological diagnosis and grading of NENs, it is important to clearly recognize the characteristic histological features of GI-NENs and to understand the correct method of counting Ki-67 and mitoses. In this review, we focus on the histopathological features of GI-NENs, particularly regarding biopsy and cytological diagnoses, neuroendocrine markers, genetic and molecular features, and the evaluation of the Ki-67 index and mitotic count. In addition, we will address the histological features of GI-NEN in specific organs. PMID:23346552

  18. Histopathology of gastrointestinal neuroendocrine neoplasms.

    PubMed

    Hirabayashi, Kenichi; Zamboni, Giuseppe; Nishi, Takayuki; Tanaka, Akira; Kajiwara, Hiroshi; Nakamura, Naoya

    2013-01-01

    Gastrointestinal neuroendocrine neoplasms (GI-NENs) arise from neuroendocrine cells distributed mainly in the mucosa and submucosa of the gastrointestinal tract. In 2010, the World Health Organization (WHO) classification of NENs of the digestive system was changed, categorizing these tumors as grade 1 neuroendocrine tumor (NET), grade-2NET, neuroendocrine carcinoma (large- or small-cell type), or mixed adenoneuroendocrine carcinoma (MANEC). Such a classification is based on the Ki-67 index and mitotic count in histological material. For the accurate pathological diagnosis and grading of NENs, it is important to clearly recognize the characteristic histological features of GI-NENs and to understand the correct method of counting Ki-67 and mitoses. In this review, we focus on the histopathological features of GI-NENs, particularly regarding biopsy and cytological diagnoses, neuroendocrine markers, genetic and molecular features, and the evaluation of the Ki-67 index and mitotic count. In addition, we will address the histological features of GI-NEN in specific organs. PMID:23346552

  19. Cerebral thrombosis and myeloproliferative neoplasms.

    PubMed

    Artoni, Andrea; Bucciarelli, Paolo; Martinelli, Ida

    2014-11-01

    Myeloproliferative neoplasms (MPN) are acquired clonal disorders characterized by the proliferation of bone marrow myeloid cells. Different somatic mutations have been recently associated with MPN, the most common being JAK-2 V617F. Among MPN, polycythemia vera and essential thrombocythemia are particularly associated with an increased risk to develop thrombotic complications, either arterial or venous. Cerebrovascular events (stroke and transient ischemic attacks) are prevalent, accounting for approximately two-thirds of all events. Also cerebral vein thrombosis can complicate MPN and can be the first manifestation of the disease. Risk factors for thrombosis in patients with MPN are related or unrelated to the disease. Among the former there are cellular risk factors, such as increased white blood cell counts, vascular cell activation, endothelial dysfunction, and plasmatic risk factors, such as increased plasma viscosity, reduced levels of protein S, increased thrombin generation. The latter include increased age and previous thrombotic events. In addition, common cardiovascular risk factors (smoking, hypertension, diabetes, dyslipidemia, obesity) contribute to the pathogenesis of arterial events, whereas circumstantial risk factors (particularly oral contraceptive use and pregnancy/puerperium) to that of venous events. Primary prevention of arterial thrombosis with antiplatelet therapy is warranted in the majority of patients with MPN, whereas primary prevention of venous thrombosis is limited to anticoagulant prophylaxis during high-risk situations. Secondary prevention includes long-term antiplatelet therapy for arterial and short- or long-term anticoagulant therapy for venous thrombosis, depending on the risk factors present at the first event. PMID:25217248

  20. Molecular diagnostics of myeloproliferative neoplasms.

    PubMed

    Langabeer, Stephen E; Andrikovics, Hajnalka; Asp, Julia; Bellosillo, Beatriz; Carillo, Serge; Haslam, Karl; Kjaer, Lasse; Lippert, Eric; Mansier, Olivier; Oppliger Leibundgut, Elisabeth; Percy, Melanie J; Porret, Naomi; Palmqvist, Lars; Schwarz, Jiri; McMullin, Mary F; Schnittger, Susanne; Pallisgaard, Niels; Hermouet, Sylvie

    2015-10-01

    Since the discovery of the JAK2 V617F mutation in the majority of the myeloproliferative neoplasms (MPN) of polycythemia vera, essential thrombocythemia and primary myelofibrosis ten years ago, further MPN-specific mutational events, notably in JAK2 exon 12, MPL exon 10 and CALR exon 9 have been identified. These discoveries have been rapidly incorporated into evolving molecular diagnostic algorithms. Whilst many of these mutations appear to have prognostic implications, establishing MPN diagnosis is of immediate clinical importance with selection, implementation and the continual evaluation of the appropriate laboratory methodology to achieve this diagnosis similarly vital. The advantages and limitations of these approaches in identifying and quantitating the common MPN-associated mutations are considered herein with particular regard to their clinical utility. The evolution of molecular diagnostic applications and platforms has occurred in parallel with the discovery of MPN-associated mutations, and it therefore appears likely that emerging technologies such as next-generation sequencing and digital PCR will in the future play an increasing role in the molecular diagnosis of MPN. PMID:25951317

  1. Cystic lesions of the pancreas

    PubMed Central

    Hruban, Ralph H.; Fukushima, Noriyoshi

    2008-01-01

    Summary In contrast to the relatively uniform pathology and the unyielding dismal outcome associated with infiltrating ductal adenocarcinoma of the pancreas, cystic lesions have a broad spectrum of gross and microscopic pathologies, and a range of clinical outcomes. The common cystic lesions of the pancreas are reviewed with emphasis on practical tips for distinguishing between the main entities. PMID:20953247

  2. Mature Cystic Teratoma of Liver

    PubMed Central

    Gupta, Richa; Bansal, Kalpana; Manchanda, Vivek

    2013-01-01

    A four-year-old boy presented with constipation and mild abdominal distention for one year. Radiologic investigations showed a multiloculated cystic lesion in the caudate lobe of liver with focal calcification in the wall. The child underwent laparotomy with marsupialization of the cystic lesion. Histopathologic examination showed mature teratoma of liver. PMID:24040591

  3. Bone morbidity in chronic myeloproliferative neoplasms.

    PubMed

    Farmer, Sarah; Ocias, Lukas Frans; Vestergaard, Hanne; Broesby-Olsen, Sigurd; Hermann, Anne Pernille; Frederiksen, Henrik

    2015-08-01

    Patients with the classical Philadelphia chromosome-negative chronic myeloproliferative neoplasms including essential thrombocythemia, polycythemia vera and primary myelofibrosis often suffer from comorbidities, in particular, cardiovascular diseases and thrombotic events. Apparently, there is also an increased risk of osteoporotic fractures among these patients. However, the true prevalence, mechanisms involved and therapeutic implications are not well described. In this review, we summarize what is currently known about possible associations between bone disease and chronic myeloproliferative neoplasms. Chronic inflammation has been suggested to explain the initiation of clonal development and progression in chronic myeloproliferative neoplasms. Decreased bone mineral density and enhanced fracture risk are well-known manifestations of many chronic systemic inflammatory diseases. As opposed to systemic mastocytosis (SM) where pathogenic mechanisms for bone manifestations probably involve effects of mast cell mediators on bone metabolism, the mechanisms responsible for increased fracture risk in other chronic myeloproliferative neoplasms are not known. PMID:26036168

  4. Management of Inoperable Malignant Neoplasms.

    PubMed

    Kiess, Ana P; Quon, Harry

    2016-01-01

    For patients with inoperable salivary gland malignancy, radiation therapy has significant limitations but has been the mainstay of treatment. With standard photon radiation (X-rays), the 10-year loco-regional control (LRC) and overall survival rates are only ∼25%. Neutron radiation has potential biological advantages over photon radiation because it causes increased DNA damage, and studies of patients with inoperable salivary gland malignancy have shown improved 6-year LRC and overall survival of ∼60%. However, neutron radiation may also increase the risk of late toxicities, especially central nervous system toxicities after treatment of tumors involving the base of the skull. Proton radiation has potential physical advantages due to minimal exit dose through normal tissues, and a recent study has demonstrated 90% 5-year LRC after combined proton/photon radiation for adenoid cystic carcinoma involving the base of the skull. Stereotactic radiosurgery has also been used in combination with neutrons or standard photons as a technique to boost the skull base. The use of concurrent chemotherapy as a radiosensitizer has been considered based on extrapolation of data on squamous cell carcinomas, but further data are needed on inoperable salivary gland malignancies. Newer targeted therapies are also under investigation, and clinical trial enrollment is encouraged. PMID:27093559

  5. Cystic Fibrosis Therapeutics

    PubMed Central

    Ramsey, Bonnie W.

    2013-01-01

    A great deal of excitement and hope has followed the successful trials and US Food and Drug Administration approval of the drug ivacaftor (Kalydeco), the first therapy available that targets the underlying defect that causes cystic fibrosis (CF). Although this drug has currently demonstrated a clinical benefit for a small minority of the CF population, the developmental pathway established by ivacaftor paves the way for other CF transmembrane conductance regulator (CFTR) modulators that may benefit many more patients. In addition to investigating CFTR modulators, researchers are actively developing numerous other innovative CF therapies. In this review, we use the catalog of treatments currently under evaluation with the support of the Cystic Fibrosis Foundation, known as the Cystic Fibrosis Foundation Therapeutics Pipeline, as a platform to discuss the variety of candidate treatments for CF lung disease that promise to improve CF care. Many of these approaches target the individual components of the relentless cycle of airway obstruction, inflammation, and infection characteristic of lung disease in CF, whereas others are aimed directly at the gene defect, or the resulting dysfunctional protein, that instigates this cycle. We discuss how new findings from the laboratory have informed not only the development of novel therapeutics, but also the rationales for their use and the outcomes used to measure their effects. By reviewing the breadth of candidate treatments currently in development, as well as the recent progress in CF therapies reflected by the evolution of the therapeutics pipeline over the past few years, we hope to build upon the optimism and anticipation generated by the recent success of Kalydeco. PMID:23276843

  6. Computerized tomography in evaluation of hepatic neoplasms

    SciTech Connect

    Luna, R.F.; Resende, C.; Tishler, J.M.A.; Aldrete, J.S.; Shin, M.S.; Rubin, E.; Rahn, N.H.

    1984-08-01

    The authors reviewed their experience with computerized tomography (CT) of the abdomen in 212 patients with histologically documented liver neoplasms seen during a 30-month period. The CT findings in cavernous hemangioma and focal nodular hyperplasia were specific, and permitted accurate diagnosis of this lesion before biopsy. The CT appearance of all other lesions was variable. CT is useful in providing an accurate evaluation of the intrahepatic and extrahepatic extent of the neoplasm.

  7. Radiation-induced neoplasms of the brain

    SciTech Connect

    Kumar, P.P.; Good, R.R.; Skultety, F.M.; Leibrock, L.G.; Severson, G.S.

    1987-04-01

    The histopathology of two patients with radiation-induced neoplasms of the brain following therapeutic irradiation for intracranial malignancies is described. The second neoplasms were an atypical meningioma and a polymorphous cell sarcoma, respectively. They occurred 12 and 23 years after irradiation (4000 rad), within the original field of irradiation. In both cases, the radiation-induced tumors were histologically distinct from the initial medulloblastomas. Both patients were retreated with local irradiation using permanent implantation of radioactive iodine-125 seeds.

  8. Automated screening of pigmentary skin neoplasms

    NASA Astrophysics Data System (ADS)

    Kudrin, Konstantin G.; Matorin, Oleg V.; Reshetov, Igor V.

    2015-01-01

    We have analysed the clinical symptoms and the malignization signs of pigmented skin neoplasms. We have estimated the complex of clinical parameters which could be measured for the purpose of skin screening diagnostic via digital image processing. Allowable errors of clinical parameter characterization have been calculated, and the origin of these errors has been discussed. Proposed technique for automated screening of pigmentary skin neoplasms should become an effective tool for early skin diagnostics.

  9. Mucin Promotes Rapid Surface Motility in Pseudomonas aeruginosa

    PubMed Central

    Yeung, Amy T. Y.; Parayno, Alicia; Hancock, Robert E. W.

    2012-01-01

    ABSTRACT An important environmental factor that determines the mode of motility adopted by Pseudomonas aeruginosa is the viscosity of the medium, often provided by adjusting agar concentrations in vitro. However, the viscous gel-like property of the mucus layer that overlays epithelial surfaces is largely due to the glycoprotein mucin. P. aeruginosa is known to swim within 0.3% (wt/vol) agar and swarm on the surface at 0.5% (wt/vol) agar with amino acids as a weak nitrogen source. When physiological concentrations or as little as 0.05% (wt/vol) mucin was added to the swimming agar, in addition to swimming, P. aeruginosa was observed to undergo highly accelerated motility on the surface of the agar. The surface motility colonies in the presence of mucin appeared to be circular, with a bright green center surrounded by a thicker white edge. While intact flagella were required for the surface motility in the presence of mucin, type IV pili and rhamnolipid production were not. Replacement of mucin with other wetting agents indicated that the lubricant properties of mucin might contribute to the surface motility. Based on studies with mutants, the quorum-sensing systems (las and rhl) and the orphan autoinducer receptor QscR played important roles in this form of surface motility. Transcriptional analysis of cells taken from the motility zone revealed the upregulation of genes involved in virulence and resistance. Based on these results, we suggest that mucin may be promoting a new or highly modified form of surface motility, which we propose should be termed “surfing.” PMID:22550036

  10. Analysis of Candida albicans adhesion to salivary mucin.

    PubMed Central

    Hoffman, M P; Haidaris, C G

    1993-01-01

    Clearance of Candida albicans from the oral cavity is thought to be mediated via specific receptor-ligand interactions between salivary constituents and the fungus. Since surfaces in the oral cavity are normally coated with a saliva-derived pellicle, specific interactions between salivary constituents and C. albicans may also contribute to adhesion of C. albicans to the oral mucosa and dental prostheses. Therefore, the purpose of this study was to identify salivary constituents to which C. albicans is capable of binding. A solid-phase overlay assay was used in which electrophoretically separated rat and human salivary constituents bound to membrane filters were incubated with radiolabelled C. albicans cells. C. albicans adhered to a single salivary component from each host. Correlation of cell-binding activity with specific monoclonal antibody (MAb)-binding activity indicated that the constituent bound by C. albicans in human saliva was low-molecular-weight mucin (MG2) and that in rat saliva was rat submandibular gland (RSMG) mucin. Further studies showed an identical cell hybridization signal and MAb colocalization by using RSMG ductal saliva and an aqueous RSMG extract in the solid-phase overlay assay. Analysis of cell binding to the aqueous extract of RSMG fractionated by anion-exchange chromatography demonstrated that C. albicans binding was restricted to an acidic subfraction of the RSMG extract, which also bound the RSMG mucin-specific MAb. The Candida-binding fraction contained predominantly RSMG mucin glycoprotein and also a noncovalently associated, chloroform-extractable material. Furthermore, we identified two strains of C. albicans which differed severalfold in the ability to bind RSMG mucin in the overlay assay. These results suggest that C. albicans binds to only a specific subfraction of RSMG mucin and that the two C. albicans strains tested differ in the ability to bind RSMG mucin subfractions. Images PMID:8478083

  11. Composite pheochromocytoma masquerading as solid-pseudopapillary neoplasm of pancreas.

    PubMed

    Gupta, Geetanjali; Saran, Ravindra Kumar; Godhi, Satyajit; Srivastava, Siddharth; Saluja, Sundeep Singh; Mishra, Pramod Kumar

    2015-05-16

    Pheochromocytoma and ganglioneuroma form rare composite tumours of the adrenal medulla comprising less than 3% of all sympathoadrenal tumours. We present a case of intraoperatively detected adrenal medullary tumour of composite pheochromocytoma and ganglioneuroma diagnosed on histopathology, in a normotensive patient. A 50-year-old male with a past history of chronic obstructive pulmonary disease presented with abdominal pain and significant weight loss since one month. Ultrasound and contrast-enhanced computed tomography abdomen revealed a large lobulated lesion in the distal body and tail of pancreas suggestive of solid and papillary neoplasm of body and tail of pancreas. Intra-operatively, a 15 cm × 10 cm solid lesion with cystic areas was seen arising from the left lower pole of the adrenal gland pushing the pancreas which appeared unremarkable. In our case, exploratory laparotomy with tumour excision was done. Extensive sectioning and microscopic examination of this adrenal tumour confirmed a diagnosis of composite Pheochromocytoma with Ganglioneuroma on histopathology. Immunophenotyping with S-100 further supported the diagnosis. The goal of this report is to increase the awareness of this rare disease and to further identify its variable presentation. PMID:25984524

  12. Complete sequence of the human mucin MUC4: a putative cell membrane-associated mucin.

    PubMed Central

    Moniaux, N; Nollet, S; Porchet, N; Degand, P; Laine, A; Aubert, J P

    1999-01-01

    The MUC4 gene, which encodes a human epithelial mucin, is expressed in various epithelial tissues, just as well in adult as in poorly differentiated cells in the embryo and fetus. Its N-terminus and central sequences have previously been reported as comprising a 27-residue peptide signal, followed by a large domain varying in length from 3285 to 7285 amino acid residues. The present study establishes the whole coding sequence of MUC4 in which the C-terminus is 1156 amino acid residues long and shares a high degree of similarity with the rat sialomucin complex (SMC). SMC is a heterodimeric glycoprotein complex composed of mucin (ascites sialoglycoprotein 1, ASGP-1) and transmembrane (ASGP-2) subunits. The same organization is found in MUC4, where the presence of a GlyAspProHis proteolytic site may cleave the large precursor into two subunits, MUC4alpha and MUC4beta. Like ASGP-2, which binds the receptor tyrosine kinase p185(neu), MUC4beta possesses two epidermal growth factor-like domains, a transmembrane sequence and a potential phosphorylated site. MUC4, the human homologue of rat SMC, may be a heterodimeric bifunctional cell-surface glycoprotein of 2.12 micrometers. These results confer a new biological role for MUC4 as a ligand for ErbB2 in cell signalling. PMID:10024507

  13. Thomsen-Friedenreich glycotope is expressed in developing and normal kidney but not in renal neoplasms.

    PubMed

    Toma, V; Zuber, C; Sata, T; Komminoth, P; Hailemariam, S; Eble, J N; Heitz, P U; Roth, J

    2000-06-01

    The Thomsen-Friedenreich glycotope (TF) is considered a general carcinoma autoantigen and is therefore of importance in cancer diagnosis and immunotherapy. We report the distribution of the TF glycotope in developing and adult human kidney and renal neoplasms. A monoclonal antibody and the lectin amaranthin were used to study the TF and its sialylated, masked form by immunohistochemistry and immunoblotting. In developing kidney, the TF was restricted to the loop of Henle, distal tubules, and peripheral collecting ducts, whereas its sialylated form was detectable in all epithelial differentiations derived from the 2 embryonic anlagen, the metanephrogenic blastema being unreactive. This pattern was essentially preserved in adult kidney, with TF labeling beginning in the thick ascending limb and extending into the collecting ducts of outer medulla. The sialylated TF glycotope was additionally observed in ascending thin limbs. The TF was exclusively expressed in the luminal cell surface and hence was inaccessible to immune reactions. Analysis of a spectrum of renal neoplasms failed to detect the TF, with the exception of occasional staining of tubules in nephroblastoma. Moreover, the sialylated TF was only detectable in oncocytoma, chromophobe renal cell carcinoma, cystic nephroma, nephroblastoma, and nephroblastomatosis complex and occasionally in type 1 papillary renal cell carcinoma. Thus, the TF and its sialylated form are expressed in normal developing and adult kidney. However, the TF does not seem to represent a tumor-associated glycotope in human kidney, nor does it appear to be of value in diagnosis and immunotherapy of renal neoplasms. PMID:10872656

  14. Hyper-osmolarity and calcium chelation: Effects on cystic fibrosis mucus.

    PubMed

    Ermund, Anna; Meiss, Lauren N; Gustafsson, Jenny K; Hansson, Gunnar C

    2015-10-01

    A non-functional Cystic Fibrosis Transmembrane conductance Regulator (CFTR) leads to the disease cystic fibrosis (CF). Although the CFTR is expressed in multiple organs, pulmonary disease is the major cause of illness and death in patients with CF. Stagnant mucus, causing airway obstruction, bacterial overgrowth, persistent inflammation and tissue destruction characterizes the disease, but how the defect in CFTR function is coupled to the mucus phenotype is still controversial. We have recently shown that bicarbonate ions passing through CFTR are necessary for proper unfolding of the MUC2 mucin, thus highlighting the importance of bicarbonate ion transport via the CFTR and the ability of these ions to raise the pH and chelate calcium bound to the mucin as the important steps in forming normal mucus. In order to find potential CF treatments and expand our knowledge about the usefulness of bicarbonate as an active ingredient in formulations to alleviate mucus plugging, we used an Ussing-type chamber and explants from the F508del-CFTR mutant mouse ileum to test the effect of calcium chelators on mucus attachment, either in isolation or in combination with osmolytes such as mannitol or hypertonic saline. We found that increasing the concentration of bicarbonate, both alone or in combination with increased osmolarity of the solution, detached the otherwise attached CF mucus. PMID:26134505

  15. Calreticulin Mutations in Myeloproliferative Neoplasms

    PubMed Central

    Lavi, Noa

    2014-01-01

    With the discovery of the JAK2V617F mutation in patients with Philadelphia chromosome-negative (Ph−) myeloproliferative neoplasms (MPNs) in 2005, major advances have been made in the diagnosis of MPNs, in understanding of their pathogenesis involving the JAK/STAT pathway, and finally in the development of novel therapies targeting this pathway. Nevertheless, it remains unknown which mutations exist in approximately one-third of patients with non-mutated JAK2 or MPL essential thrombocythemia (ET) and primary myelofibrosis (PMF). At the end of 2013, two studies identified recurrent mutations in the gene encoding calreticulin (CALR) using whole-exome sequencing. These mutations were revealed in the majority of ET and PMF patients with non-mutated JAK2 or MPL but not in polycythemia vera patients. Somatic 52-bp deletions (type 1 mutations) and recurrent 5-bp insertions (type 2 mutations) in exon 9 of the CALR gene (the last exon encoding the C-terminal amino acids of the protein calreticulin) were detected and found always to generate frameshift mutations. All detected mutant calreticulin proteins shared a novel amino acid sequence at the C-terminal. Mutations in CALR are acquired early in the clonal history of the disease, and they cause activation of JAK/STAT signaling. The CALR mutations are the second most frequent mutations in Ph− MPN patients after the JAK2V617F mutation, and their detection has significantly improved the diagnostic approach for ET and PMF. The characteristics of the CALR mutations as well as their diagnostic, clinical, and pathogenesis implications are discussed in this review. PMID:25386351

  16. Calreticulin mutations in myeloproliferative neoplasms.

    PubMed

    Lavi, Noa

    2014-10-01

    With the discovery of the JAK2V617F mutation in patients with Philadelphia chromosome-negative (Ph(-)) myeloproliferative neoplasms (MPNs) in 2005, major advances have been made in the diagnosis of MPNs, in understanding of their pathogenesis involving the JAK/STAT pathway, and finally in the development of novel therapies targeting this pathway. Nevertheless, it remains unknown which mutations exist in approximately one-third of patients with non-mutated JAK2 or MPL essential thrombocythemia (ET) and primary myelofibrosis (PMF). At the end of 2013, two studies identified recurrent mutations in the gene encoding calreticulin (CALR) using whole-exome sequencing. These mutations were revealed in the majority of ET and PMF patients with non-mutated JAK2 or MPL but not in polycythemia vera patients. Somatic 52-bp deletions (type 1 mutations) and recurrent 5-bp insertions (type 2 mutations) in exon 9 of the CALR gene (the last exon encoding the C-terminal amino acids of the protein calreticulin) were detected and found always to generate frameshift mutations. All detected mutant calreticulin proteins shared a novel amino acid sequence at the C-terminal. Mutations in CALR are acquired early in the clonal history of the disease, and they cause activation of JAK/STAT signaling. The CALR mutations are the second most frequent mutations in Ph(-) MPN patients after the JAK2V617F mutation, and their detection has significantly improved the diagnostic approach for ET and PMF. The characteristics of the CALR mutations as well as their diagnostic, clinical, and pathogenesis implications are discussed in this review. PMID:25386351

  17. Global coagulation in myeloproliferative neoplasms.

    PubMed

    Tripodi, Armando; Chantarangkul, Veena; Gianniello, Francesca; Clerici, Marigrazia; Lemma, Laura; Padovan, Lidia; Gatti, Loredana; Mannucci, Pier Mannuccio; Peyvandi, Flora

    2013-12-01

    In spite of their recognized risk of thrombosis, patients with myeloproliferative neoplasms (MPN) show little or no abnormalities of traditional coagulation tests, perhaps because these are unable to represent the balance between pro- and anticoagulants nor the effect of platelets and blood cells. We investigated whether global tests such as thrombin generation in platelet-rich plasma (PRP) or thromboelastometry in whole blood were able to detect signs of procoagulant imbalance in MPN. The endogenous thrombin potential (ETP) of 111 patients and 89 controls was measured in PRP with platelet count adjusted to the original patient- or control-count. Testing was performed with and without thrombomodulin (the physiological protein C activator) and results were expressed as ETP ratios (with/without thrombomodulin). High ETP ratios reflect resistance to thrombomodulin and were taken as indexes of procoagulant imbalance. Patients were also investigated by thromboelastometry that provides such parameters as the clot formation time (CFT) and maximal clot firmness (MCF). Short CFT or high MCF were taken as indexes of procoagulant imbalance. ETP ratios were higher in patients than in controls and were directly correlated with platelet counts and inversely with the plasma levels of free protein S, protein C and antithrombin. Patients on hydroxyurea had lower ETP ratios than those on other treatments. CFT was shorter and MCF was greater in patients than controls; CFT and MCF were correlated with platelet counts. In conclusion, patients with MPN display a procoagulant imbalance detectable by thrombin generation and thromboelastometry. These tests might be useful in the frame of clinical trials to assess their association with the occurrence of thrombosis and with the effect of therapeutic strategies in MPN. PMID:23820940

  18. Biomimetic mucin modified PLGA nanoparticles for enhanced blood compatibility.

    PubMed

    Thasneem, Y M; Rekha, M R; Sajeesh, S; Sharma, Chandra P

    2013-11-01

    Efforts to develop long circulating polymeric nanoparticles have propelled many strategies in nanoparticle surface modification to bypass immune surveillance and systemic clearance. In this context, our present study reports on the preparation and evaluation of mucin functionalized poly lactic-co-glycolic acid (PLGA) nanoparticles as hemocompatible, cell penetrating nanoparticulate drug delivery system. Amino groups of mucin were conjugated to the terminal carboxylic acid groups on PLGA to be followed by nanoparticle synthesis via standard solvent evaporation technique. Detailed in vitro experiments were performed to illustrate the significance of alternating copolymer structured mucin modified PLGA nanoparticles in terms of enhanced hemocompatibility and cellular uptake. Mucylation proved promising in controlling PLGA nanoparticle- interaction with plasma proteins (opsonins) and blood components via hemolysis, thrombogenecity and complement activation. Besides hemocompatibility, the modified and unmodified nanoparticles were also found to be cytocompatible with L929 and C6 cell lines. The fluorescent and confocal image analysis evaluated the extent of cellular uptake of nanoparticles into C6 cells. Specifically the combination of stealth properties and cellular internalization capacity of mucin modified PLGA nanoparticle (PLGA-Mucin) lead us to propose it as a safe, efficient and multifunctional nanoplatform for disease specific intravenous drug delivery applications as far as in vitro experiments are concerned. PMID:23978287

  19. Covalently-crosslinked mucin biopolymer hydrogels for sustained drug delivery.

    PubMed

    Duffy, Connor V; David, Laurent; Crouzier, Thomas

    2015-07-01

    The sustained delivery of both hydrophobic and hydrophilic drugs from hydrogels has remained a challenge requiring the design and scalable production of complex multifunctional synthetic polymers. Here, we demonstrate that mucin glycoproteins, the gel-forming constituents of native mucus, are suitable for assembly into robust hydrogels capable of facilitating the sustained release of hydrophobic and hydrophilic drugs. Covalently-crosslinked mucin hydrogels were generated via exposure of methacrylated mucin to ultraviolet light in the presence of a free radical photoinitiator. The hydrogels exhibited an elastic modulus similar to that of soft mammalian tissue and were sensitive to proteolytic degradation by pronase. Paclitaxel, a hydrophobic anti-cancer drug, and polymyxin B, a positively-charged hydrophilic antibacterial drug, were retained in the hydrogels and released linearly with time over seven days. After four weeks of drug release, the hydrogels continued to release sufficient amounts of active paclitaxel to reduce HeLa cell viability and sufficient amounts of active polymyxin B to prevent bacterial proliferation. Along with previously-established anti-inflammatory, anti-viral, and hydrocarbon-solubilizing properties of mucin, the results of this study establish mucin as a readily-available, chemically-versatile, naturally-biocompatible alternative to complex multifunctional synthetic polymers as building blocks in the design of biomaterials for sustained drug delivery. PMID:25818947

  20. Human milk mucin inhibits rotavirus replication and prevents experimental gastroenteritis.

    PubMed Central

    Yolken, R H; Peterson, J A; Vonderfecht, S L; Fouts, E T; Midthun, K; Newburg, D S

    1992-01-01

    Acute gastrointestinal infections due to rotaviruses and other enteric pathogens are major causes of morbidity and mortality in infants and young children throughout the world. Breast-feeding can reduce the rate of serious gastroenteritis in infants; however, the degrees of protection offered against rotavirus infection vary in different populations. The mechanisms associated with milk-mediated protection against viral gastroenteritis have not been fully elucidated. We have isolated a macromolecular component of human milk that inhibits the replication of rotaviruses in tissue culture and prevents the development of gastroenteritis in an animal model system. Purification of the component indicates that the antiviral activity is associated with an acidic fraction (pI = 4.0-4.6), which is free of detectable immunoglobulins. Furthermore, high levels of antiviral activity are associated with an affinity-purified complex of human milk mucin. Deglycosylation of the mucin complex results in the loss of antiviral activity. Further purification indicated that rotavirus specifically binds to the milk mucin complex as well as to the 46-kD glycoprotein component of the complex. Binding to the 46-kD component was substantially reduced after chemical hydrolysis of sialic acid. We have documented that human milk mucin can bind to rotavirus and inhibit viral replication in vitro and in vivo. Variations in milk mucin glycoproteins may be associated with different levels of protection against infection with gastrointestinal pathogens. Images PMID:1331178

  1. Mucin 1 is a potential therapeutic target in cutaneous T-cell lymphoma.

    PubMed

    Jain, Salvia; Stroopinsky, Dina; Yin, Li; Rosenblatt, Jacalyn; Alam, Maroof; Bhargava, Parul; Clark, Rachael A; Kupper, Thomas S; Palmer, Kristen; Coll, Maxwell D; Rajabi, Hasan; Pyzer, Athalia; Bar-Natan, Michal; Luptakova, Katarina; Arnason, Jon; Joyce, Robin; Kufe, Donald; Avigan, David

    2015-07-16

    Cutaneous T-cell lymphoma (CTCL) is an aggressive neoplasm with limited treatments for patients with advanced disease. The mucin 1 C-terminal subunit (MUC1-C) oncoprotein plays a critical role in regulating cell proliferation, apoptosis, and protection from cytotoxic injury mediated by reactive oxygen species (ROS). Although CTCL cells exhibit resistance to ROS-induced apoptosis, the expression and functional significance of MUC1 in CTCL have not been previously investigated. Present studies demonstrate that MUC1-C is overexpressed in CTCL cell lines and primary CTCL cells but is absent in resting T cells from healthy donors and B-cell lymphoma cells. We have developed a cell-penetrating peptide that disrupts homodimerization of the MUC1-C subunit necessary for its nuclear translocation and downstream signaling. We show that treatment of CTCL cells with the MUC1-C inhibitor is associated with downregulation of the p53-inducible regulator of glycolysis and apoptosis and decreases in reduced NAD phosphate and glutathione levels. In concert with these results, targeting MUC1-C in CTCL cells increased ROS and, in turn, induced ROS-mediated late apoptosis/necrosis. Targeting MUC1-C in CTCL tumor xenograft models demonstrated significant decreases in disease burden. These findings indicate that MUC1-C maintains redox balance in CTCL cells and is thereby a novel target for the treatment of patients with CTCL. PMID:26048911

  2. Mucin 1 is a potential therapeutic target in cutaneous T-cell lymphoma

    PubMed Central

    Stroopinsky, Dina; Yin, Li; Rosenblatt, Jacalyn; Alam, Maroof; Bhargava, Parul; Clark, Rachael A.; Kupper, Thomas S.; Palmer, Kristen; Coll, Maxwell D.; Rajabi, Hasan; Pyzer, Athalia; Bar-Natan, Michal; Luptakova, Katarina; Arnason, Jon; Joyce, Robin; Kufe, Donald; Avigan, David

    2015-01-01

    Cutaneous T-cell lymphoma (CTCL) is an aggressive neoplasm with limited treatments for patients with advanced disease. The mucin 1 C-terminal subunit (MUC1-C) oncoprotein plays a critical role in regulating cell proliferation, apoptosis, and protection from cytotoxic injury mediated by reactive oxygen species (ROS). Although CTCL cells exhibit resistance to ROS-induced apoptosis, the expression and functional significance of MUC1 in CTCL have not been previously investigated. Present studies demonstrate that MUC1-C is overexpressed in CTCL cell lines and primary CTCL cells but is absent in resting T cells from healthy donors and B-cell lymphoma cells. We have developed a cell-penetrating peptide that disrupts homodimerization of the MUC1-C subunit necessary for its nuclear translocation and downstream signaling. We show that treatment of CTCL cells with the MUC1-C inhibitor is associated with downregulation of the p53-inducible regulator of glycolysis and apoptosis and decreases in reduced NAD phosphate and glutathione levels. In concert with these results, targeting MUC1-C in CTCL cells increased ROS and, in turn, induced ROS-mediated late apoptosis/necrosis. Targeting MUC1-C in CTCL tumor xenograft models demonstrated significant decreases in disease burden. These findings indicate that MUC1-C maintains redox balance in CTCL cells and is thereby a novel target for the treatment of patients with CTCL. PMID:26048911

  3. Gli2 protein expression level is a feasible marker of ligand-dependent hedgehog activation in pancreatic neoplasms.

    PubMed

    Sugiyama, Y; Sasajima, J; Mizukami, Y; Koizumi, K; Kawamoto, T; Ono, Y; Karasaki, H; Tanabe, H; Fujiya, M; Kohgo, Y

    2016-06-01

    The hedgehog pathway is known to promote proliferation of pancreatic ductal adenocarcinoma (PDA) and has been shown to restrain tumor progression. To understand how hedgehog causes these effects, we sought to carefully examine protein expression of hedgehog signaling components during different tumor stages. Genetically engineered mice, Pdx1-Cre;LSL-KrasG12D and Pdx1-Cre;LSL-KrasG12D;p53lox/+, were utilized to model distinct phases of tumorigenesis, pancreatic intraepithelial neoplasm (PanIN) and PDA. Human pancreatic specimens of intraductal papillary mucinous neoplasm (IPMN) and PDA were also employed. PanIN and IPMN lesions highly express Sonic Hedgehog, at a level that is slightly higher than that observed in PDA. GLI2 protein is also expressed in both PanIN/IPMN and PDA. Although there was no difference in the nuclear staining, the cytoplasmic GLI2 level in PDA was modest in comparison to that in PanIN/IPMN. Hedgehog interacting protein was strongly expressed in the precursors, whereas the level in PDA was significantly attenuated. There were no differences in expression of Patched1 at early and late stages. Finally, a strong correlation between Sonic Hedgehog and GLI2 staining was found in both human and murine pancreatic tumors. The results indicate that the GLI2 protein level could serve as a feasible marker of ligand-dependent hedgehog activation in pancreatic neoplasms. PMID:27543868

  4. Gastrointestinal Manifestations of Cystic Fibrosis

    PubMed Central

    2016-01-01

    Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis. PMID:27330503

  5. Chloride impermeability in cystic fibrosis

    NASA Astrophysics Data System (ADS)

    Quinton, Paul M.

    1983-02-01

    Cystic fibrosis is the most common fatal genetic disease affecting Caucasians and is perhaps best characterized as an exocrinopathy involving a disturbance in fluid and electrolyte transport1. A high NaCl concentration in the sweat is characteristic of patients with this disease; the basic physiological reason for this abnormality is unknown. We have microperfused isolated sweat ducts from control subjects and cystic fibrosis patients, and report here results which suggest that abnormally low Cl- permeability in cystic fibrosis leads to poor reabsorption of NaCl in the sweat duct, and hence to a high concentration of NaCl in the sweat.

  6. Aggregation of mucin by chromium(III) complexes as revealed by electrokinetic and rheological studies: influence on the tryptic and O-glycanase digestion of mucin.

    PubMed

    Shrivastava, H Yamini; Sreeram, K J; Nair, Balachandran Unni

    2004-04-01

    In the present study, the impact of chromium(III) complexes ([Cr(salen)(H2O)2](+) (1), [Cr(en)3]3+ (2) and [Cr(EDTA)(H2O)]- (3)) on the biophysical properties of mucin like specific viscosity, zeta potential and particle size has been investigated. It is evident from the present investigation that the nature of the coordinated ligand has a major role to play in bringing about the changes in the physical characteristics of the glycoprotein. It was observed that (1) and (3) because of their coordinate mode of binding lead to decrease in the specific viscosity of mucin, whereas (2) on the other hand was found to bring about drastic increase in the mucin viscosity due to sol-gel transition in the mucin conformation. Complex (2) was found to gradually lower the zeta potential value of mucin (particle size=51.5 nm) from -24.8 +/- 1.31 mV to -0.58 +/- 0.30 mV, which reveals aggregation (particle size=216 nm) and subsequent sedimentation of mucin with an increase in the average diameter of mucin particles. The binding of (2) to mucin was found to impart resistance to mucin against both tryptic and O-glycanase digestion, suggesting that, the aggregation of mucin causes conformational as well as configurational changes in the glycoprotein; thus perturbing the location of carbohydrate domains. PMID:14769060

  7. Primary testicular mucinous cystadenoma: Case report and literature review.

    PubMed

    de Lima, Mário Maciel; de Lima, Mário Maciel; Granja, Fabiana

    2015-01-01

    Testicular mucinous cystadenomas are rare in urological practice, and their histogenesis, course and management are debated. We report a primary testicular mucinous cystadenoma in a 54-year old male who presented with left testicular swelling and pain. He denied having a history of cryptorchidism, testicular trauma, infections, urinary complaints, or febrile illnesses. He did not have diabetes, but was on treatment for hypertension. The patient underwent a left inguinal radical orchiectomy, and histological examination of the resected tumour confirmed a primary testicular mucinous cystadenoma. The patient had an uneventful recovery, and is being followed up. Conclusively, urologists need to maintain a high index of suspicion of these tumours and their differentiation from metastatic tumours to ensure optimal therapeutic outcomes. PMID:26600891

  8. Gelation of mucin: Protecting the stomach from autodigestion

    NASA Astrophysics Data System (ADS)

    Bansil, Rama

    2011-03-01

    In this talk I will describe the molecular mechanisms involved in the remarkable ability of the mucus lining of the stomach for protecting the stomach from being digested by the acidic gastric juices that it secretes. These physical properties can be attributed to the presence of a high molecular weight glycoprotein found in mucus, called mucin. Rheology and other measurements show that gastric mucin forms a gel under acidic pH. A model of gelation based on the interplay of hydrophobic and electrostatic interactions will be discussed. Molecular Dynamics simulation studies of folding and aggregation of mucin domains provide further support for this model. The relevance of gelation to the motion of the ulcer causing bacterium H. pylori will be discussed.

  9. Age-related changes in mucins from human whole saliva.

    PubMed

    Denny, P C; Denny, P A; Klauser, D K; Hong, S H; Navazesh, M; Tabak, L A

    1991-10-01

    The predominant mucins in human whole saliva, MG1 and MG2, serve to protect and to lubricate the oral cavity. In this study, both unstimulated and stimulated whole salivas were collected from two groups of subjects: young (18-35 years of age) and aged (65-83 years of age). The subjects were in apparent good health. Saliva samples from each subject were analyzed by SDS-PAGE. The gels were stained with Stains-all, and both MG1 and MG2 were quantitated by video-image densitometry. The protocol gave reproducible values for each mucin. The stimulated and unstimulated salivas from aged subjects showed significant reductions in concentrations of both MG1 and MG2, as quantitated in mucin dye-binding units. Possible associations of these reductions with the aging process are discussed. PMID:1719051

  10. Mucin biopolymers as broad-spectrum antiviral agents

    PubMed Central

    Lieleg, Oliver; Lieleg, Corinna; Bloom, Jesse; Buck, Christopher B.; Ribbeck, Katharina

    2012-01-01

    Mucus is a porous biopolymer matrix that coats all wet epithelia in the human body and serves as the first line of defense against many pathogenic bacteria and viruses. However, under certain conditions viruses are able to penetrate this infection barrier, which compromises the protective function of native mucus. Here, we find that isolated porcine gastric mucin polymers, key structural components of native mucus, can protect an underlying cell layer from infection by small viruses such as human papillomavirus (HPV), Merkel cell polyomavirus (MCV), or a strain of influenza A virus. Single particle analysis of virus mobility inside the mucin barrier reveals that this shielding effect is in part based on a retardation of virus diffusion inside the biopolymer matrix. Our findings suggest that purified mucins may be used as a broad-range antiviral supplement to personal hygiene products, baby formula or lubricants to support our immune system. PMID:22475261

  11. Divergent Mechanisms of Interaction of Helicobacter pylori and Campylobacter jejuni with Mucus and Mucins

    PubMed Central

    Naughton, Julie Ann; Mariño, Karina; Dolan, Brendan; Reid, Colm; Gough, Ronan; Gallagher, Mary E.; Kilcoyne, Michelle; Gerlach, Jared Q.; Joshi, Lokesh; Rudd, Pauline; Carrington, Stephen; Bourke, Billy

    2013-01-01

    Helicobacter pylori and Campylobacter jejuni colonize the stomach and intestinal mucus, respectively. Using a combination of mucus-secreting cells, purified mucins, and a novel mucin microarray platform, we examined the interactions of these two organisms with mucus and mucins. H. pylori and C. jejuni bound to distinctly different mucins. C. jejuni displayed a striking tropism for chicken gastrointestinal mucins compared to mucins from other animals and preferentially bound mucins from specific avian intestinal sites (in order of descending preference: the large intestine, proximal small intestine, and cecum). H. pylori bound to a number of animal mucins, including porcine stomach mucin, but with less avidity than that of C. jejuni for chicken mucin. The strengths of interaction of various wild-type strains of H. pylori with different animal mucins were comparable, even though they did not all express the same adhesins. The production of mucus by HT29-MTX-E12 cells promoted higher levels of infection by C. jejuni and H. pylori than those for the non-mucus-producing parental cell lines. Both C. jejuni and H. pylori bound to HT29-MTX-E12 mucus, and while both organisms bound to glycosylated epitopes in the glycolipid fraction of the mucus, only C. jejuni bound to purified mucin. This study highlights the role of mucus in promoting bacterial infection and emphasizes the potential for even closely related bacteria to interact with mucus in different ways to establish successful infections. PMID:23716616

  12. Genome wide analysis of the bovine mucin genes and their gastrointestinal transcription profile

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Mucins are large glycoproteins implicated in protection of all mucosal surfaces. In humans and rodents, the mucin gene family has been well described and previous studies have investigated the distribution and function of mucins in the respiratory, urogenital and gastrointestinal (GI) tracts. In con...

  13. GABA selectively increases mucin-1 expression in isolated pig jejunum.

    PubMed

    Braun, Hannah-Sophie; Sponder, Gerhard; Pieper, Robert; Aschenbach, Jörg R; Deiner, Carolin

    2015-11-01

    The inhibitory neurotransmitter GABA (γ-aminobutyric acid) is synthesized by glutamic acid decarboxylase, which is expressed in the central nervous system and in various other tissues including the intestine. Moreover, GABA can be ingested in vegetarian diets or produced by bacterial commensals in the gastrointestinal tract. As previous studies in lung have suggested a link between locally increased GABA availability and mucin 5AC production, the present study sought to test whether the presence or lack of GABA (and its precursor glutamine) has an effect on intestinal mucin expression. Porcine jejunum epithelial preparations were incubated with two different amounts of GABA or glutamine on the mucosal side for 4 h, and changes in the relative gene expression of seven different mucins, enzymes involved in mucin shedding, GABA B receptor, enzymes involved in glutamine/GABA metabolism, glutathione peroxidase 2, and interleukin 10 were examined by quantitative PCR (TaqMan(®) assays). Protein expression of mucin-1 (MUC1) was analyzed by Western blot. On the RNA level, only MUC1 was significantly up-regulated by both GABA concentrations compared with the control. Glutamine-treated groups showed the same trend. On the protein level, all treatment groups showed a significantly higher MUC1 expression than the control group. We conclude that GABA selectively increases the expression of MUC1, a cell surface mucin that prevents the adhesion of microorganisms, because of its size and negative charge, and therefore propose that the well-described positive effects of glutamine on enterocytes and intestinal integrity are partly attributable to effects of its metabolite GABA. PMID:26471792

  14. Neutrophils in cystic fibrosis.

    PubMed

    Laval, Julie; Ralhan, Anjali; Hartl, Dominik

    2016-06-01

    Cystic fibrosis (CF) lung disease is characterized by chronic infection and inflammation. Among inflammatory cells, neutrophils represent the major cell population accumulating in the airways of CF patients. While neutrophils provide the first defensive cellular shield against bacterial and fungal pathogens, in chronic disease conditions such as CF these short-lived immune cells release their toxic granule contents that cause tissue remodeling and irreversible structural damage to the host. A variety of human and murine studies have analyzed neutrophils and their products in the context of CF, yet their precise functional role and therapeutic potential remain controversial and incompletely understood. Here, we summarize the current evidence in this field to shed light on the complex and multi-faceted role of neutrophils in CF lung disease. PMID:26854289

  15. [Cystic kidney diseases].

    PubMed

    Zerres, K; Ortiz Brüchle, N

    2012-04-01

    Cystic kidney diseases are clinically and genetically heterogeneous. The most important entities are autosomal-dominant and autosomal-recessive polycystic kidney diseases. The proteins encoded by the involved genes are referred to as cystoproteins, which are located predominantly in the primary cilia. Primary cilia play an important role in cyst formation. Inherited polycystic kidney diseases belong to the increasing number of reported ciliopathies, including several syndromic entities. An exact diagnosis is the basis for medical care and genetic counselling; thus, the diagnostic algorithm should include clinical, ultrasonographic and morphological features of the underlying kidney disease, knowledge about further features and family history. Molecular genetic testing may contribute important information towards a definite diagnosis. PMID:22410941

  16. [Rhinosinusitis in cystic fibrosis].

    PubMed

    Mainz, J G; Gerber, A; Arnold, C; Baumann, J; Baumann, I; Koitschev, A

    2015-11-01

    In cystic fibrosis (CF) mucociliary clearance of the entire respiratory system is impaired. This allows pathogens, such as Pseudomonas aeruginosa to persist and proliferate, which by progressive pulmonary destruction causes 90 % of premature deaths due to this inherited disease. The dramatic improvement in life expectation of patients due to intensive therapy has resulted in the inevitable but variably expressed sinonasal involvement coming into the clinical and scientific focus. Thereby, almost all CF patients reveal sinonasal pathology and many suffer from chronic rhinosinusitis. Recently, the sinonasal niche has been recognized as a site of initial and persistent colonization by pathogens. This article presents the pathophysiological background of this multiorgan disease as well as general diagnostic and therapeutic standards. The focus of this article is on sinonasal involvement and conservative and surgical options for treatment. Prevention of pathogen acquisition is an essential issue in the otorhinolaryngological treatment of CF patients. PMID:26495450

  17. Mucinous Borderline Ovarian Tumor in Very Old Aged Postmenopausal Woman

    PubMed Central

    Lee, Seung-Hee; Lee, Hae-Hyeog; Lee, Arum; Kim, Yeon-Suk; Jeon, Dong-Su; Kwak, Jeong Ja; Yang, Yo-Sep

    2015-01-01

    Mucinous borderline ovarian tumors (BOTs) occur most often in women between the ages of 20 and 30. Early-stage detection of the condition has a more favorable prognosis. In this case report, the authors present an elderly 93-year old woman who visited our hospital due to severe abdominal pain after being diagnosed with a pelvic mass 2 years ago and not undergoing any treatment since the diagnosis was made. She underwent emergency left salpingo-oophorectomy and was diagnosed with mucinous BOT according to biopsy results. PMID:26793682

  18. Cystic fibrosis in pregnancy.

    PubMed Central

    Kent, N E; Farquharson, D F

    1993-01-01

    OBJECTIVE: To review the outcomes of pregnancies in women with cystic fibrosis (CF) and to address issues pertinent to the obstetric care of such women. DATA SOURCES: English-language case reports and case series published from 1960 to 1991 identified through a search of MEDLINE and Index Medicus. The terms of reference were "cystic fibrosis" and "pregnancy". Not all the reports reviewed addressed all the outcomes under consideration. STUDY SELECTION: A total of 20 reports citing cases of pregnancy in women with CF. DATA EXTRACTION: Outcomes included the number of spontaneous abortions, pregnancies continued beyond 20 weeks, preterm deliveries, maternal deaths at 6 months and 2 years after delivery and perinatal deaths. Breast-feeding was addressed. Measures to assess the severity of maternal disease included the mean age at diagnosis of CF, weight gain during pregnancy, pulmonary function studies if available and the need for pancreatic enzyme replacement therapy. DATA SYNTHESIS: Of 217 pregnancies in 162 women spontaneous abortion occurred in 10 (4.6%). Pregnancy progressed beyond 20 weeks in 81.6% of cases; 24.3% of the deliveries were preterm. The maternal death rate did not exceed that among age-related women with CF who were not pregnant. The rate of perinatal death was 7.9%. Breast milk was not hypernatremic. Poor outcomes were associated with a weight gain of less than 4.5 kg and a forced vital capacity of less than 50% of the predicted value. CONCLUSIONS: Premature labour and delivery remain a significant risk for pregnant women with CF, contributing to a high rate of perinatal death. Maternal illness and death result from deteriorating pulmonary function. Breast-feeding is not contraindicated. Attention to energy intake and pulmonary function is important. PMID:8374843

  19. Genetics Home Reference: cystic fibrosis

    MedlinePlus

    ... protects the linings of the airways, digestive system, reproductive system, and other organs and tissues. In people with ... experience health problems affecting the respiratory, digestive, and reproductive systems. Most men with cystic fibrosis have congenital bilateral ...

  20. Steady Advances Against Cystic Fibrosis

    MedlinePlus

    ... age 2, he grew up playing everything from football and lacrosse to ice hockey and golf. And ... Who's at Risk? Cystic fibrosis affects males and females from all racial and ethnic groups. It is ...

  1. Multiple cystic lymphangiomas of the spleen: radiologic and histopathologic findings.

    PubMed

    Kim, Se-Young; Kwon, Heon-Ju; Park, Hae Won; Lee, So-Yeon; Son, Byung Ho; Kim, Mi Sung

    2015-07-01

    Splenic cyst(s) may be noted as an incidental finding on conventional imaging techniques, or as a result of evaluation of a patient with left upper quadrant pain, left shoulder pain, abdominal enlargement, or splenomegaly. Lymphangioma of the spleen is an extremely rare and benign neoplasm in adults, which is characterized by cystic dilatation of the lymphatic vessels in splenic parenchyma. This report describes a case of multiple splenic lymphangiomas in a 41-year-old female. She underwent abdominal ultrasonography for a health check-up. She had no symptoms, and physical examination did not reveal any abnormalities apart from splenomegaly. Ultrasonography and computed tomography showed multiple variable-sized cysts replacing the normal parenchyma of the spleen. There were wall calcifications in several cysts. The patient underwent laparoscopic splenectomy, and the final diagnosis was multiple lymphangiomas of the spleen. PMID:26576795

  2. Diagnosis and management of endocrine gland neoplasms

    SciTech Connect

    Weller, R.E.

    1989-05-01

    Functional and nonfunctional neoplasms of the endocrine glands constitute some of the more challenging diagnostic and therapeutic problems in veterinary cancer medicine. The clinical signs are usually the result of an overproduction of hormones that are normally biosynthesized by the neoplastic endocrine gland (orthoendocrine syndromes), as opposed to those that are the result of hormones that are not normally biosynthesized and secreted by those cells that have undergone neoplastic transformation (paraendocrine syndromes, also known as endocrine paraneoplastic syndromes or ectopic hormone syndromes). The biological effects produced by a neoplasm may be out of proportion to the actual size of the tumor. This report focuses on the clinical signs and syndromes associated with neoplasms of the thyroid, adrenal glands and pancreas. Discussion will focus on the mechanisms producing the clinical signs, diagnosis, staging, therapy and prognosis. 2 tabs.

  3. Radiofrequency ablation technique eradicating palpebral margin neoplasm

    PubMed Central

    Jiang, Tian-Yu; Wang, Xing-Lin; Suo, Wei; He, Qing-Hua; Xiao, Hong-Yu

    2011-01-01

    AIM To report the study on radiofrequency ablation technique for eradication of palpebral margin neoplasm and its clinical effects. METHODS One hundred and six cases with the palpebral margin neoplasm were performed surgical removal with radiofrequency ablation technique. The 1-2 months postoperative follow-up was investigated and the lost cases were excluded from statistics. The continuing follow-up lasted about 6-16months. RESULTS One hundred cases underwent one treatment and 6 cases underwent two treatments. Six cases were missed. All the cases followed up healed well without pigmentation or scar left, nor eyelash loss or palpebral margin deformation. No case was recurrent. CONCLUSION Radiofrequency ablation has significant efficiency in eradicating the palpebral margin neoplasm. PMID:22553639

  4. Skin neoplasms of dogs in Sydney.

    PubMed

    Rothwell, T L; Howlett, C R; Middleton, D J; Griffiths, D A; Duff, B C

    1987-06-01

    In a survey of dogs in Sydney, mastocytomas (16.1%) and histiocytomas (14.0%) were the most common in a total of 1,000 skin neoplasms. The basal cell and appendage group provided 25.5% of the neoplasms. The prevalence of the various neoplasms, the age of affected dogs, the proportion in the sexes, the common sites of occurrence and prevalence in the different breeds were broadly similar to findings in surveys in other countries, except that in the Syndeny dogs there was a greater prevalence of histiocytomas and haemangiopericytomas, a more common occurrence of histiocytomas in mature dogs, an occurrence of histiocytomas in similar numbers on the head, trunk and limbs, and a remarkably common development of squamous cell carcinomas in Dalmatians. PMID:3115242

  5. Conventional radiological strategy of common gastrointestinal neoplasms

    PubMed Central

    Li, Yi-Zhuo; Wu, Pei-Hong

    2015-01-01

    This article summarizes the clinical characteristics and imaging features of common gastrointestinal (GI) neoplasms in terms of conventional radiological imaging methods. Barium studies are readily available for displaying primary malignancies and are minimally or not at all invasive. A neoplasm may be manifested as various imaging findings, including mucosal disruption, soft mass, ulcer, submucosal invasion and lumen stenosis on barium studies. Benign tumors typically appear as smoothly marginated intramural masses. Malignant neoplasms most often appear as irregular infiltrative lesions on barium examination. Tumor extension to adjacent GI segments may be indistinct on barium images. Cross-sectional images such as computed tomography and magnetic resonance imaging may provide more accurate details of the adjacent organ invasion, omental or peritoneal spread. PMID:25628800

  6. Frequent KRAS mutation in complex mucinous epithelial lesions of the endometrium.

    PubMed

    Alomari, Ahmed; Abi-Raad, Rita; Buza, Natalia; Hui, Pei

    2014-05-01

    KRAS mutation correlates with mucinous differentiation in various human cancers, and recently, was found in a high proportion of a small cohort of papillary mucinous lesions of the endometrium. In this study, a large number of endometrial mucinous lesions were analyzed for the presence of KRAS mutation along with clinical progression. A total of 45 endometrial biopsy/curettage cases were included in the study and classified into the following categories: simple mucinous change (5 cases), complex mucinous change (33 cases) and mucinous adenocarcinoma (7 cases). Follow-up hysterectomy specimens were available in 14 of 33 patients (42%) with complex mucinous lesions, of which 9 cases (64%) showed atypical complex hyperplasia with an average interval of 21 weeks. None of the 5 cases of simple mucinous change showed KRAS mutation. KRAS mutation was observed in 18 of 33 patients with complex mucinous lesions (55%) and in 6 of 7 cases of mucinous adenocarcinoma (86%). Overall, KRAS mutation has a positive predictive value (PPV) of 88% (7/8 cases) for complex atypical hyperplasia or adenocarcinoma in the follow-up hysterectomy. In conclusion, the current data further emphasizes the architectural complexity as an important prognostic indicator for patients with mucinous endometrial lesions. The presence of KRAS mutation in both mucinous adenocarcinoma and complex mucinous changes indicates that KRAS mutational activation is implicated in the pathogenesis of a significant subset of endometrial mucinous carcinoma. With a high PPV, KRAS mutation analysis may offer an additional discriminatory power to refine risk stratification algorithm for patients with endometrial mucinous lesions. PMID:24186144

  7. Radiological and surgical management of thyroid neoplasms.

    PubMed

    Takami, H; Ikeda, Y; Miyabe, R; Okinaga, H; Kameyama, K; Fukunari, N

    2004-01-01

    Recent advances in the radiological diagnosis in thyroid neoplasms have been achieved by high-resolution ultrasonography and color-Doppler, and the ultrasound-guided fine-needle aspiration biopsy and ultrasound-guided percutaneous ethanol injection therapy have been developed on the basis of these modalities. Ultrasonography and ultrasound-guided fine-needle aspiration biopsy have made minimally invasive thyroid surgery possible. The surgical procedures are classified into three main categories according to the approach, and each approach has its own advantages and disadvantages. Surgeons have to select the most suitable approach from one of these categories of approaches for each patient with a thyroid neoplasm. PMID:15271417

  8. Colonic mucin synthesis is increased by sodium butyrate.

    PubMed

    Finnie, I A; Dwarakanath, A D; Taylor, B A; Rhodes, J M

    1995-01-01

    The effects of sodium butyrate and sodium bromo-octanoate (an inhibitor of beta oxidation) on colonic mucus glycoprotein (mucin) synthesis have been assessed using tissue from colonic resection samples. Epithelial biopsy specimens were incubated for 16 hours in RPMI 1640 with glutamine, supplemented with 10% fetal calf serum and N-acetyl-[3H]-glucosamine ([3H]-Glc NAc), and differing concentrations of sodium butyrate. Incorporation of [3H] Glc NAc into mucin by normal epithelium at least 10 cm distant from colonic cancer was increased in the presence of sodium butyrate in a dose dependent manner, with maximum effect (476%) at a concentration of 0.1 mM (number of specimens = 24 from six patients, p < 0.001). The increase in response to butyrate was not seen when specimens were incubated in the presence of the beta oxidation inhibitor sodium bromo-octanoate 0.05 M. The striking increase in mucin synthesis that results when butyrate is added to standard nutrient medium suggests that this may be an important mechanism affecting the rate of mucin synthesis in vivo and may also explain the therapeutic effect of butyrate in colitis. PMID:7890244

  9. Mupirocin-mucin agar for selective enumeration of Bifidobacterium bifidum.

    PubMed

    Pechar, Radko; Rada, Vojtech; Parafati, Lucia; Musilova, Sarka; Bunesova, Vera; Vlkova, Eva; Killer, Jiri; Mrazek, Jakub; Kmet, Vladimir; Svejstil, Roman

    2014-11-17

    Bifidobacterium bifidum is a bacterial species exclusively found in the human intestinal tract. This species is becoming increasingly popular as a probiotic organism added to lyophilized products. In this study, porcine mucin was used as the sole carbon source for the selective enumeration of B. bifidum in probiotic food additives. Thirty-six bifidobacterial strains were cultivated in broth with mucin. Only 13 strains of B. bifidum utilized the mucin to produce acids. B. bifidum was selectively enumerated in eight probiotic food supplements using agar (MM agar) containing mupirocin (100 mg/L) and mucin (20 g/L) as the sole carbon source. MM agar was fully selective if the B. bifidum species was presented together with Bifidobacterium animalis subsp. lactis, Bifidobacterium breve, and Bifidobacterium longum subsp. longum species and with lactic acid bacteria (lactobacilli, streptococci). Isolated strains of B. bifidum were identified using biochemical, PCR, MALDI-TOF procedures and 16S rRNA gene sequencing. The novel selective medium was also suitable for the isolation of B. bifidum strains from human fecal samples. PMID:25217723

  10. Mucins and Cytokeratins as Serum Tumor Markers in Breast Cancer.

    PubMed

    Nicolini, Andrea; Ferrari, Paola; Rossi, Giuseppe

    2015-01-01

    Structural and functional characteristics of mucins and cytokeratins are shortly described. Thereafter, those commonly used in breast cancer as serum tumor markers are considered. First CA15.3, MCA, CA549, CA27.29 mucins and CYFRA21.1, TPA, TPS cytokeratins alone or in association have been examined in different stages and conditions. Then their usefulness in monitoring disease-free breast cancer patients is evaluated. The central role of the established cut-off and critical change, the "early" treatment of recurrent disease and the potential benefit in survival are other issues that have been highlighted and discussed. The successive sections and subsections deal with the monitoring of advanced disease. In them, the current recommendations and the principal findings on using the above mentioned mucins and cytokeratins have been reported. A computer program for interpreting consecutive measurements of serum tumor markers also has been illustrated. The final part of the chapter is devoted to mucins and cytokeratins as markers of circulating and disseminated tumor cells and their usefulness for prognosis. PMID:26530368

  11. Physical Properties of Human Whole Salivary Mucin:A Dynamic Light Scattering Study

    NASA Astrophysics Data System (ADS)

    Mahajan, Manish; Kumar, Vijay; Saraswat, Mayank; Yadav, Savita; Shukla, N. K.; Singh, T. P.

    2008-04-01

    Human salivary mucin, a primary mucous membrane coating glycoprotein forms the first line of defense against adverse environments, attributed to the complex formation between mucin subunits and non mucin species. Aim of the study was to emphasize the effect of pH, denaturants (guanidinum hydrochloride, urea) and detergents (CHAPS, TRITON X -100, SDS on human whole salivary mucin. Hydrodynamic size distribution was measured using DLS. It was observed that aggregation was due to increase in hydrophobic interactions, believed to be accomplished by unfolding of the protein core. Whereas, the detergents which solubilize the proteins by decreasing hydrophobicity lead to disaggregation of mucin into smaller fragments. Mucin subjected to tobacco extract and upon subsequent addition of nicotine was found to have a disaggregating effect on it, suggesting nicotine may be one of the factors responsible for the disaggregating effect of tobacco on mucin, an important carcinogenetic mechanism.

  12. ALK-rearranged adenocarcinoma with extensive mucin production can mimic mucinous adenocarcinoma: clinicopathological analysis and comprehensive histological comparison with KRAS-mutated mucinous adenocarcinoma.

    PubMed

    Cha, Yoon Jin; Han, Joungho; Hwang, Soo Hyun; Lee, Tae Bum; Kim, Hojoong; Zo, Jea Ill

    2016-06-01

    We aimed to investigate clinicopathological features and histology of ALK-rearranged adenocarcinomas with extensive mucin production (AEM) that mimic mucinous adenocarcinoma (MA). Retrospectively, 12 cases of AEM and 25 cases of MA harbouring KRAS mutation were retrieved. The clinicopathological profile and detailed histological features were analysed and compared based on the ALK and KRAS status. AEMs occurred in younger patients (p = 0.044) and were characterised by floating tubulopapillary pattern (p < 0.001), prominent nucleolus (p < 0.001), and apical cytoplasmic snouts (p < 0.001). In contrast, KRAS-mutated MAs lacked ALK-specific histological patterns (p < 0.05). Instead, tumour-infiltrating leukocytes (p = 0.018) and smooth cytoplasmic borders (p < 0.001) with vesicular nuclei (p = 0.004) were prominent in KRAS-mutated MAs. AEMs demonstrated characteristic tubulopapillary pattern and apical snouts, which were distinguishing features from MAs with KRAS mutation. Apical snouts can be a useful histological surrogate for ALK rearrangement in the pulmonary adenocarcinomas showing extensive mucin that mimic MA. PMID:27114375

  13. [Modern imaging of liver and pancreatic neoplasms].

    PubMed

    Mátéka, Ilona; Bikhazi, Ziad; Bartha, Éva; Palkó, András

    2015-03-01

    Modern imaging modalities play an outstanding role in the detection, characterization, staging, therapy planning, treatment outcome evaluation and follow-up of patients with liver and pancreatic neoplasms. Diagnostic performance and accuracy of the available modalities are continuously improving therefore, it is necessary to overview from time to time the diagnostic protocols and algorithms. PMID:25763911

  14. Cholescintigraphy: gallbladder nonvisualization secondary to neoplasm

    SciTech Connect

    Lecklitner, M.L.; Rosen, P.R.; Nusynowitz, M.L.

    1981-08-01

    Whereas the diagnosis of acute cholecystitis is characterized by nonvisualization of the gallbladder with Tc-99m iminodiacetic acid derivatives, nonvisualization is not specific for acute cholecystitis. The first reported case of nonvisualization of the gallbladder due to neoplasm is added to an expanding list of causes of nonvisualization other than the more frequent causes: acute and chronic cholecystitis.

  15. SNP Array in Hematopoietic Neoplasms: A Review

    PubMed Central

    Song, Jinming; Shao, Haipeng

    2015-01-01

    Cytogenetic analysis is essential for the diagnosis and prognosis of hematopoietic neoplasms in current clinical practice. Many hematopoietic malignancies are characterized by structural chromosomal abnormalities such as specific translocations, inversions, deletions and/or numerical abnormalities that can be identified by karyotype analysis or fluorescence in situ hybridization (FISH) studies. Single nucleotide polymorphism (SNP) arrays offer high-resolution identification of copy number variants (CNVs) and acquired copy-neutral loss of heterozygosity (LOH)/uniparental disomy (UPD) that are usually not identifiable by conventional cytogenetic analysis and FISH studies. As a result, SNP arrays have been increasingly applied to hematopoietic neoplasms to search for clinically-significant genetic abnormalities. A large numbers of CNVs and UPDs have been identified in a variety of hematopoietic neoplasms. CNVs detected by SNP array in some hematopoietic neoplasms are of prognostic significance. A few specific genes in the affected regions have been implicated in the pathogenesis and may be the targets for specific therapeutic agents in the future. In this review, we summarize the current findings of application of SNP arrays in a variety of hematopoietic malignancies with an emphasis on the clinically significant genetic variants. PMID:27600067

  16. Neoplasms identified in free-flying birds

    USGS Publications Warehouse

    Siegfried, L.M.

    1983-01-01

    Nine neoplasms were identified in carcasses of free-flying wild birds received at the National Wildlife Health Laboratory; gross and microscopic descriptions are reported herein. The prevalence of neoplasia in captive and free-flying birds is discussed, and lesions in the present cases are compared with those previously described in mammals and birds.

  17. Effect of dexamethasone and ACC on bacteria-induced mucin expression in human airway mucosa.

    PubMed

    Hauber, Hans-Peter; Goldmann, Torsten; Vollmer, Ekkehard; Wollenberg, Barbara; Zabel, Peter

    2007-11-01

    Gram-negative bacteria can stimulate mucin production, but excessive mucus supports bacterial infection and consequently leads to airway obstruction. Therefore, the effect of dexamethasone (DEX) and the antioxidant acetyl-cysteine (ACC) on bacteria-induced mucus expression was investigated. Explanted human airway mucosa and mucoepidermoid cells (Calu-3) were stimulated with lipopolysaccharide (LPS) or PAM3 (a synthetic lipoprotein). DEX or ACC were added to either LPS- or PAM3-stimulated airway mucosa or Calu-3 cells. Mucin mRNA expression (MUC5AC) and total mucus glycoconjugates (mucin protein) were quantified using real-time PCR and periodic acid Schiff staining. LPS and PAM3 significantly increased mucin expression in airway mucosa and Calu-3 cells (P < 0.05). DEX alone had no significant effect on mucin expression in airway mucosa or Calu-3 cells (P > 0.05). In contrast, DEX significantly reduced LPS- and PAM3-induced mucin expression in explanted mucosal tissue and mucin expression in Calu-3 cells (P < 0.05). In explanted human airway mucosa ACC alone significantly increased mucin expression (P < 0.05). In contrast, ACC significantly decreased LPS- and PAM3-induced mucin expression (P < 0.05). In Calu-3 cells ACC alone had no significant effect on mucin expression (P > 0.05). ACC decreased LPS- and PAM3-induced mucin expression, but this effect was not significant (P > 0.05). These data suggest that DEX can effectively reduce bacteria-induced mucin expression in the airways. ACC alone may increase mucin expression in noninfected mucosa, but it decreased bacteria-induced mucin expression. Further studies are warranted to evaluate whether the effect of DEX or ACC is clinically relevant. PMID:17600317

  18. Pediatric cystic nephromas: distinctive features and frequent DICER1 mutations.

    PubMed

    Cajaiba, Mariana M; Khanna, Geetika; Smith, Ethan A; Gellert, Lan; Chi, Yueh-Yun; Mullen, Elizabeth A; Hill, Dana A; Geller, James I; Dome, Jeffrey S; Perlman, Elizabeth J

    2016-02-01

    Cystic nephromas (CNs) are uncommon benign renal neoplasms that present with a bimodal age distribution, affecting either infants/young children or adult females. Although differences between these age groups have been suggested, large studies of pediatric CN have not been conducted. As a result, the nomenclature and diagnostic criteria for these lesions remain controversial. In addition, the morphological overlap seen between CN and cystic partially differentiated nephroblastoma (CPDN) can result in diagnostic dilemmas. This study reviews the morphologic and radiographic features of 44 pediatric CN prospectively enrolled on a Children's Oncology Group protocol from 2007 to 2013. Although the typical multicystic architecture with thin septa described in adult CN was present in all of our pediatric cases, differences were also identified. We report distinctive features that add to the morphological spectrum of CN in children. Of the 44 cases, 16 had been previously analyzed and reported for DICER1 mutation, and either loss of function or missense mutations or both were identified in 15 of 16. In contrast, we analyzed 10 cases of adult CN, and all were negative for DICER1 mutations; similarly, 6 CPDNs previously analyzed and reported were negative for DICER1 mutations. Therefore, the clinical, morphological, and genetic differences between pediatric and adult CN, as well as between CN and CPDN, suggest that these 3 lesions represent distinct entities. PMID:26772403

  19. Molecular Pathology: Prognostic and Diagnostic Genomic Markers for Myeloid Neoplasms.

    PubMed

    Kuo, Frank C

    2016-09-01

    Application of next-generation sequencing (NGS) on myeloid neoplasms has expanded our knowledge of genomic alterations in this group of diseases. Genomic alterations in myeloid neoplasms are complex, heterogeneous, and not specific to a disease entity. NGS-based panel testing of myeloid neoplasms can complement existing diagnostic modalities and is gaining acceptance in the clinics and diagnostic laboratories. Prospective, randomized trials to evaluate the prognostic significance of genomic markers in myeloid neoplasms are under way in academic medical centers. PMID:27523973

  20. PULMONARY CYSTIC ECHINOCOCCOSIS

    PubMed Central

    Santivanez, Saul; Garcia, Hector H.

    2012-01-01

    Pulmonary cystic echinococosis, a zoonosis caused by the larvae of the dog tapeworm Echinococcus granulosus, is considered as a major public health problem in those countries where dogs are used to care for large herds because of the incapacitating effects produced in affected population. The ratio lung:liver involvement is higher in children than in adults. A higher proportion of lung cases are discovered incidentally on a routine x-ray evaluation; the majority of infected people remain asymptomatic until the cyst enlarges sufficiently to cause symptoms. The majority of symptoms are caused by mass effect from the cyst volume; the presence of complications caused by cysts broke changes the clinical presentation; the principal complication is cyst rupture, producing cough, chest pain, hemoptysis, or vomica. Diagnosis is obtained by imaging evaluation (Chest X-ray or CT scan), supported by serology in the majority of cases. Surgery is the main therapeutic approach, having as principal objective, the removal of the parasite, preventing intraoperative dissemination; the use of pre surgical chemotherapy reduces the chances of seeding and recurrence; treatment using benzimidazoles is the preferred treatment when surgery is not available, or complete removal is not feasible PMID:20216420

  1. Cystic Fibrosis Sinusitis.

    PubMed

    Le, Christopher; McCrary, Hilary C; Chang, Eugene

    2016-01-01

    Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the CF transmembrane conductance regulator gene(CFTR) resulting in impaired ion transport. Nearly all people with CF will develop chronic rhino-sinusitis (CRS) and present with the characteristic viscous mucus, impaired mucociliary clearance and chronic inflammation/infection of the sinonasal cavity. While some individuals with CF can appear relatively asymptomatic in terms of their sinus disease, commonly reported symptoms include anosmia, headache, facial pain, nasal obstruction, chronic congestion and nasal discharge. Nasal endoscopy typically reveals mucosal edema, purulent discharge and nasal polyposis. Computed tomography (CT) imaging classically demonstrates the distinguishing findings of sinus hypoplasia or aplasia with generalized opacification, medial bulging of the lateral sinonasal sidewall and a demineralized uncinate process. Current treatment for CF sinusitis includes the use of hypertonic saline, topical and systemic steroids, antibiotics and endoscopic surgery. Research investigating novel therapies designed at targeting the primary defect of CF is showing promise for reversal of CF sinus disease, in addition to potential for disease prevention. PMID:27466844

  2. The O-Linked Glycome and Blood Group Antigens ABO on Mucin-Type Glycoproteins in Mucinous and Serous Epithelial Ovarian Tumors

    PubMed Central

    Vitiazeva, Varvara; Kattla, Jayesh J.; Flowers, Sarah A.; Lindén, Sara K.; Premaratne, Pushpa; Weijdegård, Birgitta; Sundfeldt, Karin; Karlsson, Niclas G.

    2015-01-01

    Background Mucins are heavily O-glycosylated proteins where the glycosylation has been shown to play an important role in cancer. Normal epithelial ovarian cells do not express secreted mucins, but their abnormal expression has previously been described in epithelial ovarian cancer and may relate to tumor formation and progression. The cyst fluids were shown to be a rich source for acidic glycoproteins. The study of these proteins can potentially lead to the identification of more effective biomarkers for ovarian cancer. Methods In this study, we analyzed the expression of the MUC5AC and the O-glycosylation of acidic glycoproteins secreted into ovarian cyst fluids. The samples were obtained from patients with serous and mucinous ovarian tumors of different stages (benign, borderline, malignant) and grades. The O-linked oligosaccharides were released and analyzed by negative-ion graphitized carbon Liquid Chromatography (LC) coupled to Electrospray Ionization tandem Mass Spectrometry (ESI-MSn). The LC-ESI-MSn of the oligosaccharides from ovarian cyst fluids displayed differences in expression of fucose containing structures such as blood group ABO antigens and Lewis-type epitopes. Results The obtained data showed that serous and mucinous benign adenomas, mucinous low malignant potential carcinomas (LMPs, borderline) and mucinous low-grade carcinomas have a high level of blood groups and Lewis type epitopes. In contrast, this type of fucosylated structures were low abundant in the high-grade mucinous carcinomas or in serous carcinomas. In addition, the ovarian tumors that showed a high level of expression of blood group antigens also revealed a strong reactivity towards the MUC5AC antibody. To visualize the differences between serous and mucinous ovarian tumors based on the O-glycosylation, a hierarchical cluster analysis was performed using mass spectrometry average compositions (MSAC). Conclusion Mucinous benign and LMPs along with mucinous low-grade carcinomas

  3. Sclerosing stromal tumour of the ovary presenting as precocious puberty: a rare neoplasm.

    PubMed

    Chaurasia, Jai Kumar; Afroz, Nishat; Maheshwari, Veena; Naim, Mohammed

    2014-01-01

    A 7-year-old Indian girl presented with symptoms of excessive development of breasts, early menarche, growth of pubic hairs, accelerated growth and abdominal distension. On clinical examination, a large right abdominopelvic mass was palpable. MRI revealed a large, heterogeneous, solid and cystic tumour in the right adnexal region, suggestive of an ovarian neoplasm. The hormonal profile showed markedly elevated oestradiol and low follicle-stimulating hormone levels. Clinical diagnosis of precocious puberty with right ovarian mass was concluded. Right-sided salpingo-oophorectomy was performed. Histopathology showed features consistent with sclerosing stromal tumour of the ovary. Postoperatively, signs and symptoms of precocity gradually regressed and her serum oestradiol level came down to normal. This is the first reported case from India. PMID:24686794

  4. Unplugging Mucus in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.

    PubMed

    Mall, Marcus A

    2016-04-01

    Airway mucus obstruction is a key feature of cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD). The thin layer of mucus that covers healthy airway surfaces has important protective functions in lung defense. However, excess mucus produces airflow obstruction and provides a nidus for bacterial infection and inflammation. Despite its importance in pathogenesis, understanding of the mechanisms underlying airway mucus obstruction, as well as therapeutic options, remain limited. Studies in the rare genetic disease CF identified airway surface dehydration due to cystic fibrosis transmembrane conductance regulator (CFTR) gene dysfunction as an important disease mechanism that may explain mucus stasis and plugging in a spectrum of muco-obstructive lung diseases, including COPD. This concept is supported by the phenotype of the β-epithelial Na(+) channel-transgenic mouse that exhibits airway surface dehydration and develops a spontaneous lung disease that shares key features with CF and COPD, such as airway mucus plugging, chronic neutrophilic inflammation, and structural lung damage. Furthermore, preclinical testing demonstrated that hydration strategies, including osmotically active hypertonic saline and preventive inhibition of the amiloride-sensitive epithelial Na(+) channel are effective in unplugging airways in this mouse model of chronic obstructive lung disease. On the other hand, genetic deletion of neutrophil elastase, a potent stimulus for mucus hypersecretion, reduced goblet cell metaplasia and mucin expression but had no effect on mucus obstruction in vivo. Collectively, these studies demonstrate that airway surface dehydration is sufficient to produce mucus obstruction even in the absence of mucus hypersecretion and support further clinical testing of hydrating agents as a promising therapeutic strategy to unplug mucus in CF and COPD. PMID:27115954

  5. Using Cystic Fibrosis Therapies for Non-Cystic Fibrosis Bronchiectasis.

    PubMed

    ElMaraachli, Wael; Conrad, Douglas J; Wang, Angela C C

    2016-03-01

    Non-cystic fibrosis bronchiectasis (NCFB) is an increasingly prevalent disease that places a significant burden on patients and health systems globally. Although many of the therapies used to treat NCFB were originally developed as cystic fibrosis (CF) therapies, not all of them have been demonstrated to be efficacious in NCFB and some may even be harmful. This article explores the evidence for which therapeutic strategies used to treat CF have been translated into the care of NCFB. The conclusion is that therapies for adult NCFB cannot be simply extrapolated from CF clinical trials, and in some instances, doing so may actually result in harm. PMID:26857775

  6. [Cystic cancer of the kidney].

    PubMed

    el Moussaoui, A; Dakir, M; Sarf, I; Aboutaeib, R; el Mrini, M; Benjelloun, S

    1997-01-01

    Cystic renal cancer is uncommon and raises real preoperative diagnostic problems, requiring the use of medical imaging, and sometimes even surgery. The authors report 3 cases of cystic renal cancer in 2 men and 1 woman, aged 87, 67 and 20 years, respectively. Three patients presented with the urological triad (haematuria, pain and lumbar mass). Ultrasonography suggested the diagnosis of cystic cancer in all 3 cases. Computed tomography was performed in 2 patients and more precisely confirmed the ultrasound findings. Selective arteriography, performed in one patient, confirmed the hypothesis of malignancy. Surgical exploration resulted in radical total nephrectomy. Histology confirmed the diagnosis of adenocarcinoma. The course was favourable in 2 cases after a follow-up of 4 years. One patient presented a local recurrence with pulmonary metastases 6 months after the operation. A review of the literature illustrates the diagnostic difficulties of this form of renal cancer. PMID:9509236

  7. Intracholecystic papillary-tubular neoplasms (ICPN) of the gallbladder (neoplastic polyps, adenomas, and papillary neoplasms that are ≥1.0 cm): clinicopathologic and immunohistochemical analysis of 123 cases.

    PubMed

    Adsay, Volkan; Jang, Kee-Taek; Roa, Juan Carlos; Dursun, Nevra; Ohike, Nobuyuki; Bagci, Pelin; Basturk, Olca; Bandyopadhyay, Sudeshna; Cheng, Jeanette D; Sarmiento, Juan M; Escalona, Oscar Tapia; Goodman, Michael; Kong, So Yeon; Terry, Paul

    2012-09-01

    displayed some foci of low-grade dysplasia, and 15/47 (32%) had no identifiable invasion. (4) Overall, 55% of the cases had an associated invasive carcinoma (pancreatobiliary type, 58; others, 10). Factors associated significantly with invasion were the extent of high-grade dysplasia, cell type (biliary or foveolar), and papilla formation. Among systematically analyzed invasive carcinomas, tumoral intraepithelial neoplasia was detected in 6.4% (39/606). (5) The 3-year actuarial survival was 90% for cases without invasion and 60% for those associated with invasion. In contrast, those associated with invasion had a far better clinical outcome compared with pancreatobiliary-type GB carcinomas (3-yr survival, 27%), and this survival advantage persisted even with stage-matched comparison. Death occurred in long-term follow-up even in a few noninvasive cases (4/55; median 73.5 mo) emphasizing the importance of long-term follow-up. In conclusion, tumoral preinvasive neoplasms (≥1 cm) in the GB are analogous to their pancreatic and biliary counterparts (biliary intraductal papillary neoplasms, pancreatic intraductal papillary mucinous neoplasms, and intraductal tubulopapillary neoplasms). They show variable cellular lineages, a spectrum of dysplasia, and a mixture of papillary or tubular growth patterns, often with significant overlap, warranting their classification under 1 unified parallel category, intracholecystic papillary-tubular neoplasm. Intracholecystic papillary-tubular neoplasms are relatively indolent neoplasia with significantly better prognosis compared with pancreatobiliary-type GB carcinomas. In contrast, even seemingly innocuous examples such as those referred to as "pyloric gland adenomas" can progress to carcinoma and be associated with invasion and fatal outcome. PMID:22895264

  8. Structural investigation of porcine stomach mucin by X-ray fiber diffraction and homology modeling

    SciTech Connect

    Veluraja, K.; Vennila, K.N.; Umamakeshvari, K.; Jasmine, A.; Velmurugan, D.

    2011-03-25

    Research highlights: {yields} Techniques to get oriented mucin fibre. {yields} X-ray fibre diffraction pattern for mucin. {yields} Molecular modeling of mucin based on X-ray fibre diffraction pattern. -- Abstract: The basic understanding of the three dimensional structure of mucin is essential to understand its physiological function. Technology has been developed to achieve orientated porcine stomach mucin molecules. X-ray fiber diffraction of partially orientated porcine stomach mucin molecules show d-spacing signals at 2.99, 4.06, 4.22, 4.7, 5.37 and 6.5 A. The high intense d-spacing signal at 4.22 A is attributed to the antiparallel {beta}-sheet structure identified in the fraction of the homology modeled mucin molecule (amino acid residues 800-980) using Nidogen-Laminin complex structure as a template. The X-ray fiber diffraction signal at 6.5 A reveals partial organization of oligosaccharides in porcine stomach mucin. This partial structure of mucin will be helpful in establishing a three dimensional structure for the whole mucin molecule.

  9. Rat and human colonic mucins bind to and inhibit adherence lectin of Entamoeba histolytica.

    PubMed Central

    Chadee, K; Petri, W A; Innes, D J; Ravdin, J I

    1987-01-01

    Establishment of adherence by Entamoeba histolytica is mediated by a 170-kD Gal/GalNAc inhibitable lectin and is required for cytolysis and phagocytosis of mammalian target cells. We studied the biochemical mechanisms of the in vitro interaction between rat and human colonic mucins and axenic E. histolytica trophozoites. Crude mucus prevented amebic adherence to Chinese hamster ovary (CHO) cells by up to 70%. Purification of the colonic mucins by Sepharose 4B chromatography, nuclease digestion, and cesium chloride gradient centrifugation resulted in a 1,000-fold enrichment of the inhibitory mucins. Purified rat mucin inhibited amebic adherence to and cytolysis of homologous rat colonic epithelial cells. Oxidation and enzymatic cleavage of rat mucin Gal and GalNAc residues completely abrogated mucin inhibition of amebic adherence. The binding of rat 125I-mucin to amebae was galactose specific, saturable, reversible, and pH dependent. A monoclonal antibody specific for the 170-kD amebic Gal/GalNAc lectin completely inhibited the binding of rat 125I-mucin. Rat mucin bound to Affigel affinity purified the amebic lectin from conditioned medium. Colonic mucin glycoproteins act as an important host defense by binding to the parasite's adherence lectin, thus preventing amebic attachment to and cytolysis of host epithelial cells. Images PMID:2890655

  10. [Historical compilation of cystic fibrosis].

    PubMed

    Navarro, Salvador

    2016-01-01

    Cystic fibrosis is the most common life-shortening recessively inherited disorder in the Caucasian population. The genetic mutation that most frequently provokes cystic fibrosis (ΔF508) appeared at least 53,000years ago. For many centuries, the disease was thought to be related to witchcraft and the "evil eye" and it was only in 1938 that Dorothy H. Andersen characterized this disorder and suspected its genetic origin. The present article reviews the pathological discoveries and diagnostic and therapeutic advances made in the last 75 years. The review ends with some considerations for the future. PMID:26070393

  11. Diagnostic Testing in Cystic Fibrosis.

    PubMed

    Brewington, John; Clancy, J P

    2016-03-01

    Cystic Fibrosis (CF) is a rare, multisystem disease leading to significant morbidity and mortality. CF is caused by defects in the cystic fibrosis transmembrane conductance regulator protein (CFTR), a chloride and bicarbonate transporter. Early diagnosis and access to therapies provides benefits in nutrition, pulmonary health, and cognitive ability. Several screening and diagnostic tests are available to support a diagnosis. We discuss the characteristics of screening and diagnostic tests for CF and guideline-based algorithms using these tools to establish a diagnosis. We discuss classification and management of common "diagnostic dilemmas," including the CFTR-related metabolic syndrome and other CFTR-associated diseases. PMID:26857766

  12. Parasite molecules and host responses in cystic echinococcosis.

    PubMed

    Díaz, A; Casaravilla, C; Barrios, A A; Ferreira, A M

    2016-03-01

    Cystic echinococcosis is the infection by the larvae of cestode parasites belonging to the Echinococcus granulosus sensu lato species complex. Local host responses are strikingly subdued in relation to the size and persistence of these larvae, which develop within mammalian organs as 'hydatid cysts' measuring up to tens of cm in diameter. In a context in which helminth-derived immune-suppressive, as well as Th2-inducing, molecules garner much interest, knowledge on the interactions between E. granulosus molecules and the immune system lags behind. Here, we discuss what is known and what are the open questions on E. granulosus molecules and structures interacting with the innate and adaptive immune systems, potentially or in demonstrated form. We attempt a global biological approach on molecules that have been given consideration primarily as protective (Eg95) or diagnostic antigens (antigen B, antigen 5). We integrate glycobiological information, which traverses the discussions on antigen 5, the mucin-based protective laminated layer and immunologically active preparations from protoscoleces. We also highlight some less well-known molecules that appear as promising candidates to possess immune-regulatory activities. Finally, we point out gaps in the molecular-level knowledge of this infectious agent that hinder our understanding of its immunology. PMID:26425838

  13. Ovarian seromucinous carcinoma: report of a series of a newly categorized and uncommon neoplasm.

    PubMed

    Taylor, Jennifer; McCluggage, W Glenn

    2015-07-01

    Seromucinous neoplasms are a new category of ovarian epithelial tumor in the revised World Health Organization Classification of Tumours of the Female Reproductive Organs. Borderline variants are well described, but there have been few reports of seromucinous carcinomas. We report the clinicopathologic features in 19 cases of ovarian seromucinous carcinoma in patients aged 16 to 79 years (mean 47). In 16 cases, the neoplasm was unilateral and in 3 cases bilateral. The tumors ranged in size from 1.8 to 18 cm (mean 10.5 cm). The tumors were stage I (n=15), stage II (n=1), and stage III (n=3). The histologic features were highly variable both within and between individual tumors. The majority of neoplasms (12 cases) exhibited a predominant papillary architecture with lesser components of glandular, microglandular, and solid growth. A predominant glandular architecture was present in 6 cases, whereas 1 had a predominantly solid growth. A characteristic feature was an admixture of cell types. Most of the tumors (15 cases) were mainly composed of endocervical-like mucinous cells, whereas in 4 cases there was predominant endometrioid differentiation. Other cell types, present in varying proportions, included hobnail cells, eosinophilic cells, squamous cells, clear cells, and signet-ring cells. An infiltrate of neutrophil polymorphs was a prominent feature in most cases. Most cases also exhibited areas of microglandular architecture with cytoplasmic clearing and intraluminal polymorphs, the features closely resembling cervical microglandular hyperplasia. Areas of stromal hyalinization, adenofibromatous growth, and psammoma bodies were present in a minority of cases. Endometriosis was identified in the same ovary in 10 cases, and in 10 there was a component of seromucinous borderline tumor. Thirteen, 5, and 1 tumor were of grades 1, 2, and 3, respectively (using the FIGO grading system for endometrioid adenocarcinomas of the uterine corpus). A synchronous uterine

  14. Endoscopic submucosal dissection for gastrointestinal neoplasms

    PubMed Central

    Kakushima, Naomi; Fujishiro, Mitsuhiro

    2008-01-01

    Endoscopic submucosal dissection (ESD) is an advanced technique of therapeutic endoscopy for superficial gastrointestinal neoplasms. Three steps characterize it: injecting fluid into the submucosa to elevate the lesion, cutting the surrounding mucosa of the lesion, and dissecting the submucosa beneath the lesion. The ESD technique has rapidly permeated in Japan for treatment of early gastric cancer, due to its excellent results of en-bloc resection compared to endoscopic mucosal resection (EMR). Although there is still room for improvement to lessen its technical difficulty, ESD has recently been applied to esophageal and colorectal neoplasms. Favorable short-term results have been reported, but the application of ESD should be well considered by three aspects: (1) the possibility of nodal metastases of the lesion, (2) technical difficulty such as location, ulceration and operator’s skill, and (3) organ characteristics. PMID:18494043

  15. Intrathoracic neoplasms in the dog and cat

    SciTech Connect

    Weller, R.E.

    1994-03-01

    Very little is known regarding the epidemiology, etiology, and mechanisms of spontaneous intrathoracic neoplasia in companion animals. Much of what we know or suspect about thoracic neoplasia in animals has been extrapolated from experimentally-induced neoplasms. Most studies of thoracic neoplasia have focused on the pathology of primary and metastatic neoplasms of the lung with little attention given to diagnostic and therapeutic considerations. Although the cited incidence rate for primary respiratory tract neoplasia is low, 8.5 cases per 100,000 dogs and 5.5 cases per 100,000 cats, intrathoracic masses often attract attention out of proportion to their actual importance since they are often readily visualized on routine thoracic radiographs.

  16. Endoscopic submucosal dissection for colorectal neoplasms

    PubMed Central

    Takamaru, Hiroyuki; Mori, Genki; Yamada, Masayoshi; Kinjo, Yuzuru; So, Eriko; Abe, Seiichiro; Otake, Yosuke; Nakajima, Takeshi; Matsuda, Takahisa; Saito, Yutaka

    2014-01-01

    Endoscopic submucosal dissection (ESD) is an established therapeutic technique for the treatment of gastrointestinal neoplasms. Because it is typically completed as en bloc resection, this technique provides a complete specimen for precise pathological evaluation. On the other hand, ESD is not as widely applied in treating colorectal neoplasms as with gastric cancers, due to its technical difficulty, longer procedure time, and increased risk of perforation. However, some devices that facilitate ESD and improve the safety of the procedure have been recently reported, and the use of the technique has gradually spread worldwide. Endoscopists who begin to perform ESD need to recognize the indications of ESD, the technical issue involved in this procedure, and its associated complications. This review outlines the methods and certain types of devices used for colorectal ESD. PMID:25333002

  17. [Grading of head and neck neoplasms].

    PubMed

    Agaimy, A; Weichert, W

    2016-07-01

    Tumors of the head and neck form a heterogeneous group of benign and malignant neoplasms with significant differences in biological behavior and therapeutic strategies. Squamous cell carcinomas (SCC) of the larynx, pharynx and oral cavity represent the most frequent and, thus, clinically most important malignant neoplasms in this anatomical region. Similar to other neoplasms, grading of head and neck malignancies is based on evaluation of the tumor histology usually including both architectural and cytological features; however, the current consensus grading for head and neck SCC is of limited prognostic and therapeutic value and the reproducibility is low. Therefore, novel grading criteria have been proposed that are based on additional parameters, such as the type of tumor growth pattern at the invasive front (so-called tumor budding). These novel algorithms, however, have not yet been officially endorsed into guidelines. Salivary gland (SG) neoplasms, although less frequent, constitute a second important pathologically and clinically complex group of tumors at this location. In contrast to SCC, grading of these tumors is of high clinical importance. Based on the large variety of carcinoma entities of the SG, both entity-specific (e. g. mucoepidermoid carcinoma) algorithms but also algorithms, which are solely based on the recognition of a specific carcinoma variant with subsequent automatic assignment of the tumor grade (e. g. acinic cell carcinoma and salivary duct carcinoma) are in use. In the sinonasal tract, grading is important for non-intestinal type adenocarcinoma and esthesioneuroblastoma. In this article the most important grading schemes and criteria for head and neck malignancies are presented and their prognostic and therapeutic implications are discussed. PMID:27342593

  18. Granular Cell Tumor: An Uncommon Benign Neoplasm

    PubMed Central

    Gayen, Tirthankar; Das, Anupam; Shome, Kaushik; Bandyopadhyay, Debabrata; Das, Dipti; Saha, Abanti

    2015-01-01

    Granular cell tumor is a distinctly rare neoplasm of neural sheath origin. It mainly presents as a solitary asymptomatic swelling in the oral cavity, skin, and rarely internal organs in the middle age. Histopathology is characteristic, showing polyhedral cells containing numerous fine eosinophilic granules with indistinct cell margins. We present a case of granular cell tumor on the back of a 48-year-old woman which was painful, mimicking an adnexal tumor. PMID:26120181

  19. Benign Diseases and Neoplasms of the Penis.

    PubMed

    Wasco, Matthew J; Shah, Rajal B

    2009-03-01

    This article provides comprehensive review of benign diseases and neoplastic conditions of the penis. It describes and provides representative images of clinical, key pathologic features and ancillary techniques to aid in differential diagnoses. It examines these diseases from the epidemiologic standpoint, looks at environmental and genetic factors, and outlines the new histologic entities for penile neoplasms with distinct outcomes and clinical behavior that have been proposed in recent years. PMID:26838101

  20. Enzymatically active biomimetic micropropellers for the penetration of mucin gels

    PubMed Central

    Walker, Debora; Käsdorf, Benjamin T.; Jeong, Hyeon-Ho; Lieleg, Oliver; Fischer, Peer

    2015-01-01

    In the body, mucus provides an important defense mechanism by limiting the penetration of pathogens. It is therefore also a major obstacle for the efficient delivery of particle-based drug carriers. The acidic stomach lining in particular is difficult to overcome because mucin glycoproteins form viscoelastic gels under acidic conditions. The bacterium Helicobacter pylori has developed a strategy to overcome the mucus barrier by producing the enzyme urease, which locally raises the pH and consequently liquefies the mucus. This allows the bacteria to swim through mucus and to reach the epithelial surface. We present an artificial system of reactive magnetic micropropellers that mimic this strategy to move through gastric mucin gels by making use of surface-immobilized urease. The results demonstrate the validity of this biomimetic approach to penetrate biological gels, and show that externally propelled microstructures can actively and reversibly manipulate the physical state of their surroundings, suggesting that such particles could potentially penetrate native mucus. PMID:26824056

  1. Synthetic Oral Mucin Mimic from Polymer Micelle Networks

    PubMed Central

    2015-01-01

    Mucin networks are formed in the oral cavity by complexation of glycoproteins with other salivary proteins, yielding a hydrated lubricating barrier. The function of these networks is linked to their structural, chemical, and mechanical properties. Yet, as these properties are interdependent, it is difficult to tease out their relative importance. Here, we demonstrate the ability to recreate the fibrous like network through a series of complementary rinses of polymeric worm-like micelles, resulting in a 3-dimensional (3D) porous network that can be deposited layer-by-layer onto any surface. In this work, stability, structure, and microbial capture capabilities were evaluated as a function of network properties. It was found that network structure alone was sufficient for bacterial capture, even with networks composed of the adhesion-resistant polymer, poly(ethylene glycol). The synthetic networks provide an excellent, yet simple, means of independently characterizing mucin network properties (e.g., surface chemistry, stiffness, and pore size). PMID:24992241

  2. [Microbiocenosis of parietal mucin in the gastrointestinal tract of rats].

    PubMed

    Vorob'ev, A A; Nesvizhskiĭ, Iu V; Bogdanova, E A; Korneev, L M

    2005-01-01

    The qualitative and quantitative composition of the microbial community in parietal mucin at different areas of the gastrointestinal tract (GIT) of rats was revealed. The pronounced variability in the quantitative and qualitative characteristics of microbiocenosis in parietal mucin of rats at different sections was revealed. The differences were most pronounced in the passage from upper to lower GIT sections, the large intestine found to be the richest biocenosis. The microbial composition of rat feces was faintly associated with the GIT parietal microbiocenosis. The individual areas of GIT mucosa were unique of their microbial characteristics and organization. This makes it possible to regard them as relatively independent biotopes and indicates that it is impossible to evaluate the microbial community by one of the colonic mucosal sifes. PMID:16438365

  3. [Molecular pathology of plasma cell neoplasms].

    PubMed

    Fend, F

    2010-10-01

    Plasma cell myeloma (PCM) and related immunosecretory disorders are a group of B-cell proliferations with a wide clinical and prognostic spectrum, characterized by the production of monoclonal immunoglobulin by immortalized plasma cells. Recent years have seen an explosion in knowledge on the genetic basis and biology of these diseases, followed by improved clinical risk stratification and the introduction of novel therapeutic concepts, such as treatment with proteasome inhibitors or immunomodulatory substances. PCM is a common malignancy, accounting for approximately 10% of all hematological neoplasms. There is good evidence to support a multistep transformation process in plasma cell neoplasms, which corresponds to clinically discernible disease stages. Monoclonal gammopathy of unknown significance is a common asymptomatic precursor lesion for PCM which carries an approximately 1% annual risk for progression. Terminal disease stages are characterized by increasing genetic complexity and independence from bone marrow stromal cells and show a rapidly increasing tumour load with severe clinical symptoms. Modern diagnostics of plasma cell neoplasms require inclusion of clinical, morphological, immunophenotypical and cytogenetic features to allow for individual risk assessment and therapy planning. PMID:20852863

  4. Salivary Gland Neoplasms in Children and Adolescents.

    PubMed

    Bradley, Patrick J; Eisele, David W

    2016-01-01

    Salivary gland neoplasms (SGNs) in children are uncommon. Epithelial SGNs (ESGNs) comprise the majority (95%), with the remaining being mesenchymal SGNs (MeSGNs). Pleomorphic adenoma is the most frequently encountered benign neoplasm, mucoepidermoid carcinoma is the most frequent malignant ESGN, and rhabdomyosarcoma is the most frequent malignant MeSGN. ESGN presents in the second decade, whereas MeSGN presents in the first and second decades. Swelling without pain or neurological signs is the main presentation of both benign and malignant neoplasms. Making an accurate preoperative histological diagnosis is important, so a needle biopsy or a perioperative frozen section is useful when there is doubt about the disease status of the patient; the excised tumour margin is also important. Surgical excision should aim to achieve clear margin excision in benign and malignant ESGNs, minimising the need for adjuvant radiotherapy and maximising the long-term likelihood of patient cure. Benign ESGNs are uncommon, and excision is curative, whereas malignant ESGN and MeSGN should be managed by a multidisciplinary paediatric oncology team. PMID:27093697

  5. Endocrine neoplasms in familial syndromes of hyperparathyroidism.

    PubMed

    Li, Yulong; Simonds, William F

    2015-06-01

    Familial syndromes of hyperparathyroidism, including multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2A (MEN2A), and the hyperparathyroidism-jaw tumor (HPT-JT), comprise 2-5% of primary hyperparathyroidism cases. Familial syndromes of hyperparathyroidism are also associated with a range of endocrine and nonendocrine tumors, including potential malignancies. Complications of the associated neoplasms are the major causes of morbidities and mortalities in these familial syndromes, e.g., parathyroid carcinoma in HPT-JT syndrome; thymic, bronchial, and enteropancreatic neuroendocrine tumors in MEN1; and medullary thyroid cancer and pheochromocytoma in MEN2A. Because of the different underlying mechanisms of neoplasia, these familial tumors may have different characteristics compared with their sporadic counterparts. Large-scale clinical trials are frequently lacking due to the rarity of these diseases. With technological advances and the development of new medications, the natural history, diagnosis, and management of these syndromes are also evolving. In this article, we summarize the recent knowledge on endocrine neoplasms in three familial hyperparathyroidism syndromes, with an emphasis on disease characteristics, molecular pathogenesis, recent developments in biochemical and radiological evaluation, and expert opinions on surgical and medical therapies. Because these familial hyperparathyroidism syndromes are associated with a wide variety of tumors in different organs, this review is focused on those endocrine neoplasms with malignant potential. PMID:27207564

  6. Follicular pancreatitis: a distinct form of chronic pancreatitis-an additional mimic of pancreatic neoplasms.

    PubMed

    Gupta, Rajib K; Xie, Bill H; Patton, Kurt T; Lisovsky, Mikhail; Burks, Eric; Behrman, Stephen W; Klimstra, David; Deshpande, Vikram

    2016-02-01

    Follicular pancreatitis is a recently described variant of chronic pancreatitis characterized clinically by the formation of a discrete pancreatic mass and histologically by the presence of florid lymphoid aggregates with reactive germinal centers. Our aim was to study the clinical and histologic features of follicular pancreatitis, as well as to critically examine potential overlap with autoimmune pancreatitis. Immunohistochemistry for Bcl-2, CD21, κ and λ light chains as well as IgG4 and IgG were performed. We found a total of 6 patients (male-female ratio, 2:1; mean age, 57 years) who fulfilled the diagnosis of follicular pancreatitis in our institutions. Four had an incidental diagnosis, while two presented with abdominal pain, fatigue, and elevated liver enzymes. On imaging, 3 patients had a discrete solid mass, whereas 2 cases showed a dilated main pancreatic duct, mimicking an intraductal pancreatic mucinous neoplasm on imaging. One patient had a lesion in the intra-pancreatic portion of the common bile duct. On histopathology, all cases showed numerous lymphoid follicles with Bcl-2-negative germinal centers either in a periductal or in a more diffuse (periductal and intra-parenchymal) fashion, but without attendant storiform fibrosis, obliterative phlebitis, or granulocytic epithelial lesions. IgG4-to-IgG ratio was <40% in 5 cases. A comparison cohort revealed germinal centers in 25% of type 1 autoimmune pancreatitis and 2% of type 2 autoimmune pancreatitis cases, but none were periductal in location. In conclusion, follicular pancreatitis, an under-recognized mimic of pancreatic neoplasms is characterized by intrapancreatic lymphoid follicles with reactive germinal centers. PMID:26563969

  7. Chemical modification of carbohydrates in tissue sections may unmask mucin antigens.

    PubMed

    Kirkeby, S

    2013-01-01

    Expression of mucins in cells and tissues is of great diagnostic and prognostic importance, and immunohistochemistry frequently is used to detect them. Reports concerning mucin localization in sections sometimes are conflicting, however, partly because immunogenic regions of the mucin molecule may be masked and thus not available for binding to an antibody. We modified carbohydrates in tissue sections chemically to enhance the binding of monoclonal mucin antibodies and of the lectin, Vicia villosa B4, to human tissue. The immunohistochemical localization of MUC1 and the simple mucin-type antigens, Tn and sialyl-Tn, was influenced by oxidation with periodic acid and by β-elimination before incubation. In some epithelial cells the staining was prevented by these procedures while in other cells it was evident. It appears that chemical modification can either destroy some antigen binding sites or unmask cryptic antigen binding sites in the mucin molecule and thereby make them accessible for immunohistochemical detection. PMID:22998734

  8. Diffusion through Pig Gastric Mucin: Effect of Relative Humidity

    PubMed Central

    Runnsjö, Anna; Dabkowska, Aleksandra P.; Sparr, Emma; Kocherbitov, Vitaly; Arnebrant, Thomas; Engblom, Johan

    2016-01-01

    Mucus covers the epithelium found in all intestinal tracts, where it serves as an important protecting barrier, and pharmaceutical drugs administrated by the oral, rectal, vaginal, ocular, or nasal route need to penetrate the mucus in order to reach their targets. Furthermore, the diffusion in mucus as well as the viscosity of mucus in the eyes, nose and throat can change depending on the relative humidity of the surrounding air. In this study we have investigated how diffusion through gels of mucin, the main protein in mucus, is affected by changes in ambient relative humidity (i.e. water activity). Already a small decrease in water activity was found to give rise to a significant decrease in penetration rate through the mucin gel of the antibacterial drug metronidazole. We also show that a decrease in water activity leads to decreased diffusion rate in the mucin gel for the fluorophore fluorescein. This study shows that it is possible to alter transport rates of molecules through mucus by changing the water activity in the gel. It furthermore illustrates the importance of considering effects of the water activity in the mucosa during development of potential pharmaceuticals. PMID:27336158

  9. Polymorphisms in mucin genes in the development of gastric cancer

    PubMed Central

    Wen, Rong; Gao, Fang; Zhou, Cheng-Jiang; Jia, Yan-Bin

    2015-01-01

    Gastric cancer (GC) is the third leading cause of cancer-related death worldwide. In areas of high prevalence, such as Japan, South Korea and China, most cases of GC are related to Helicobacter pylori (H. pylori), which involves well-characterized sequential stages, including infection, atrophic gastritis, intestinal metaplasia, dysplasia, and GC. Mucins are the most abundant high-molecular-weight glycoproteins in mucus, which is the first line of defense and plays a major role in blocking pathogenic factors. Normal gastric mucosa shows expression of MUC1, MUC5AC and MUC6 that is specific to cell type. However, the specific pattern of MUC1, MUC5AC and MUC6 expression is changed in gastric carcinogenesis, accompanied by de novo expression of secreted MUC2. Recent studies have provided evidence that variations in these mucin genes affect many steps of GC development, such as H. pylori infection, and gastric precancerous lesions. In this review, we focus on studies of the association between polymorphisms in mucin genes and development of GC. This information should be helpful for the early detection, surveillance, and treatment of GC. PMID:26600932

  10. Mucins help to avoid alloreactivity at the maternal fetal interface.

    PubMed

    Redzovic, Arnela; Laskarin, Gordana; Dominovic, Marin; Haller, Herman; Rukavina, Daniel

    2013-01-01

    During gestation, many different mechanisms act to render the maternal immune system tolerant to semi-allogeneic trophoblast cells of foetal origin, including those mediated via mucins that are expressed during the peri-implantation period in the uterus. Tumour- associated glycoprotein-72 (TAG-72) enhances the already established tolerogenic features of decidual dendritic cells with the inability to progress towards Th1 immune orientation due to lowered interferon (IFN)- γ and interleukin (IL)-15 expression. Mucine 1 (Muc 1) supports alternative activation of decidual macrophages, restricts the proliferation of decidual regulatory CD56(+) bright natural killer (NK) cells, and downregulates their cytotoxic potential, including cytotoxic mediator protein expression. Removing TAG-72 and Muc 1 from the eutopic implantation site likely contributes to better control of trophoblast invasion by T cells and NK cells and appears to have important immunologic advantages for successful implantation, in addition to mechanical advantages. However, these processes may lead to uncontrolled trophoblast growth after implantation, inefficient defence against infection or tumours, and elimination of unwanted immunocompetent cells at the maternal-foetal interface. The use of mucins by tumour cells to affect the local microenvironment in order to avoid the host immune response and to promote local tumour growth, invasion, and metastasis confirms this postulation. PMID:23864879

  11. Tea derived galloylated polyphenols cross-link purified gastrointestinal mucins.

    PubMed

    Georgiades, Pantelis; Pudney, Paul D A; Rogers, Sarah; Thornton, David J; Waigh, Thomas A

    2014-01-01

    Polyphenols derived from tea are thought to be important for human health. We show using a combination of particle tracking microrheology and small-angle neutron scattering that polyphenols acts as cross-linkers for purified gastrointestinal mucin, derived from the stomach and the duodenum. Both naturally derived purified polyphenols, and green and black tea extracts are shown to act as cross-linkers. The main active cross-linking component is found to be the galloylated forms of catechins. The viscosity, elasticity and relaxation time of the mucin solutions experience an order of magnitude change in value upon addition of the polyphenol cross-linkers. Similarly small-angle neutron scattering experiments demonstrate a sol-gel transition with the addition of polyphenols, with a large increase in the scattering at low angles, which is attributed to the formation of large scale (>10 nm) heterogeneities during gelation. Cross-linking of mucins by polyphenols is thus expected to have an impact on the physicochemical environment of both the stomach and duodenum; polyphenols are expected to modulate the barrier properties of mucus, nutrient absorption through mucus and the viscoelastic microenvironments of intestinal bacteria. PMID:25162539

  12. Salivary Mucins Protect Surfaces from Colonization by Cariogenic Bacteria

    PubMed Central

    Frenkel, Erica Shapiro

    2014-01-01

    Understanding how the body's natural defenses function to protect the oral cavity from the myriad of bacteria that colonize its surfaces is an ongoing topic of research that can lead to breakthroughs in treatment and prevention. One key defense mechanism on all moist epithelial linings, such as the mouth, gastrointestinal tract, and lungs, is a layer of thick, well-hydrated mucus. The main gel-forming components of mucus are mucins, large glycoproteins that play a key role in host defense. This study focuses on elucidating the connection between MUC5B salivary mucins and dental caries, one of the most common oral diseases. Dental caries is predominantly caused by Streptococcus mutans attachment and biofilm formation on the tooth surface. Once S. mutans attaches to the tooth, it produces organic acids as metabolic by-products that dissolve tooth enamel, leading to cavity formation. We utilize CFU counts and fluorescence microscopy to quantitatively show that S. mutans attachment and biofilm formation are most robust in the presence of sucrose and that aqueous solutions of purified human MUC5B protect surfaces by acting as an antibiofouling agent in the presence of sucrose. In addition, we find that MUC5B does not alter S. mutans growth and decreases surface attachment and biofilm formation by maintaining S. mutans in the planktonic form. These insights point to the importance of salivary mucins in oral health and lead to a better understanding of how MUC5B could play a role in cavity prevention or diagnosis. PMID:25344244

  13. A distinct molecular profile associated with mucinous epithelial ovarian cancer

    PubMed Central

    Heinzelmann-Schwarz, V A; Gardiner-Garden, M; Henshall, S M; Scurry, J P; Scolyer, R A; Smith, A N; Bali, A; Bergh, P Vanden; Baron-Hay, S; Scott, C; Fink, D; Hacker, N F; Sutherland, R L; O'Brien, P M

    2006-01-01

    Mucinous epithelial ovarian cancers (MOC) are clinically and morphologically distinct from the other histological subtypes of ovarian cancer. To determine the genetic basis of MOC and to identify potential tumour markers, gene expression profiling of 49 primary ovarian cancers of different histological subtypes was performed using a customised oligonucleotide microarray containing >59 000 probesets. The results show that MOC express a genetic profile that both differs and overlaps with other subtypes of epithelial ovarian cancer. Concordant with its histological phenotype, MOC express genes characteristic of mucinous carcinomas of varying epithelial origin, including intestinal carcinomas. Differences in gene expression between MOC and other histological subtypes of ovarian cancer were confirmed by RT–PCR and/or immunohistochemistry. In particular, galectin 4 (LGALS4) was highly and specifically expressed in MOC, but expressed at lower levels in benign mucinous cysts and borderline (atypical proliferative) tumours, supporting a malignant progression model of MOC. Hence LGALS4 may have application as an early and differential diagnostic marker of MOC. PMID:16508639

  14. Cystic pineocytoma--case report.

    PubMed

    Momozaki, N; Ikezaki, K; Abe, M; Fukui, M; Fujii, K; Kishikawa, T

    1992-03-01

    Pineocytoma and pineoblastoma, originating from pineal parenchyma, are rare and usually solid. An unusual case of totally cystic pineocytoma in a 37-year-old female is reported. The tumor showed neuronal differentiation and had a good outcome. Prominent calcification associated with pineocytoma and pineoblastoma is an useful finding to differentiate these from benign pineal cysts. PMID:1377802

  15. Cutaneous manifestations of cystic fibrosis.

    PubMed

    Bernstein, Megan L; McCusker, Meagen M; Grant-Kels, Jane M

    2008-01-01

    Cystic fibrosis is an autosomal recessive disease reported in 1 in 2500 live births in Northern American and Northern European Caucasian populations. Classic disease findings include chronic bacterial infection of airways and sinuses, malabsorption of fat, infertility in men, and elevated concentrations of chloride in sweat. Less well-recognized findings associated with cystic fibrosis include cutaneous findings, which can be primary or secondary manifestations of the disease process. Patients demonstrate more atopic and drug hypersensitivity reactions than the general population, but have similar rates of urticaria compared with the general population. In atypical presentations of cystic fibrosis, the nutrient deficiency dermatitis of the disease may aid with diagnosis, and notably can be the presenting sign. Other dermatologic manifestations of cystic fibrosis include early aquagenic skin wrinkling and cutaneous vasculitis, which can be associated with arthralgias. Familiarity with the nutrient deficiency dermatitis of this entity may play a role in the timely diagnosis of the disease, and the other cutaneous findings add to our understanding of the protean nature of its manifestations. PMID:18429769

  16. Mucin Agarose Gel Electrophoresis: Western Blotting for High-molecular-weight Glycoproteins.

    PubMed

    Ramsey, Kathryn A; Rushton, Zachary L; Ehre, Camille

    2016-01-01

    Mucins, the heavily-glycosylated proteins lining mucosal surfaces, have evolved as a key component of innate defense by protecting the epithelium against invading pathogens. The main role of these macromolecules is to facilitate particle trapping and clearance while promoting lubrication of the mucosa. During protein synthesis, mucins undergo intense O-glycosylation and multimerization, which dramatically increase the mass and size of these molecules. These post-translational modifications are critical for the viscoelastic properties of mucus. As a result of the complex biochemical and biophysical nature of these molecules, working with mucins provides many challenges that cannot be overcome by conventional protein analysis methods. For instance, their high-molecular-weight prevents electrophoretic migration via regular polyacrylamide gels and their sticky nature causes adhesion to experimental tubing. However, investigating the role of mucins in health (e.g., maintaining mucosal integrity) and disease (e.g., hyperconcentration, mucostasis, cancer) has recently gained interest and mucins are being investigated as a therapeutic target. A better understanding of the production and function of mucin macromolecules may lead to novel pharmaceutical approaches, e.g., inhibitors of mucin granule exocytosis and/or mucolytic agents. Therefore, consistent and reliable protocols to investigate mucin biology are critical for scientific advancement. Here, we describe conventional methods to separate mucin macromolecules by electrophoresis using an agarose gel, transfer protein into nitrocellulose membrane, and detect signal with mucin-specific antibodies as well as infrared fluorescent gel reader. These techniques are widely applicable to determine mucin quantitation, multimerization and to test the effects of pharmacological compounds on mucins. PMID:27341489

  17. Palliative Surgical Approach in Advanced Nonresponsive Mucinous Ovarian Cancer: A Rare Case Report

    PubMed Central

    Agarwal, Manika; Kumar, Ritesh; Topno, Noor; Mishra, Shweta; Dhirasaria, Ashish; Singh, A Santa

    2016-01-01

    Advanced mucinous ovarian cancer is a separate entity and has different biological behaviour. There is a wide range of therapeutic challenges and dilemmas in the management of these patients. The authors present a case of advanced ovarian mucinous cystadenocarcinoma with pseudomyxoma peritonei who had poor response to standard neoadjuvant chemotherapy. This case is highlighted to emphasize the challenges in the decision making for the management of advanced mucinous ovarian cancer. PMID:27162429

  18. [Therapeutic update in cystic fibrosis].

    PubMed

    Durupt, S; Nove Josserand, R; Durieu, I

    2014-06-01

    We present the recent therapeutic advances in the cystic fibrosis care. It concerns improvements in symptomatic treatment with the development of dry powder inhaled antibiotics that improved quality of life, and innovative treatments namely the modulators of the cystic fibrosis transmembrane protein conductance regulator (CFTR), molecules which act specifically at the level of the defective mechanisms implied in the disease. The life expectancy of cystic fibrosis patients born after 2000, is estimated now to be about 50 years. This improvement of survival was obtained with the organization of the care within the specialized centers for cystic fibrosis (Centre de ressource et de compétences de la mucoviscidose) and remains still based on heavy symptomatic treatments. Dry powder inhaled antibiotics constitute a significant time saving for patients to whom all the care can achieve two hours daily. Since 2012, the modulators of CFTR, molecules allowing a pharmacological approach targeted according to the type of the mutations, allows a more specific approach of the disease. Ivacaftor (Kalydeco(®)) which potentialises the function of the CFTR protein expressed on the cellular surface is now available for patients with the G551D mutation. Lumacaftor is going to be tested in association with ivacaftor in patients with the F508del mutation, that is present in at least 75% of the patients. The ataluren which allows the production of a functional protein CFTR in patients with a no sense mutation is the third representing of this new therapeutic class. We presently have numerous symptomatic treatments for the cystic fibrosis care. The development of CFTR modulators, today available to a restricted number of patients treated with ivacaftor represents a very promising therapeutic avenue. It will represent probably the first step to a personalized treatment according to CFTR genotype. PMID:24309546

  19. Genomic Loss of DUSP4 Contributes to the Progression of Intraepithelial Neoplasm of Pancreas to Invasive Carcinoma.

    PubMed

    Hijiya, Naoki; Tsukamoto, Yoshiyuki; Nakada, Chisato; Tung Nguyen, Lam; Kai, Tomoki; Matsuura, Keiko; Shibata, Kohei; Inomata, Masafumi; Uchida, Tomohisa; Tokunaga, Akinori; Amada, Kohei; Shirao, Kuniaki; Yamada, Yasunari; Mori, Hiromu; Takeuchi, Ichiro; Seto, Masao; Aoki, Masahiro; Takekawa, Mutsuhiro; Moriyama, Masatsugu

    2016-05-01

    The progression from precursor lesions of pancreatic cancer, including pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasm (IPMN), to invasive disease is characterized by stepwise accumulation of genetic alterations. However, it remains unclear whether additional alterations are required for the progression of high-grade neoplasms to invasive pancreatic carcinoma. We compared the genomic profiles of paired noninvasive and invasive carcinoma tissues collected from patients with IPMN. We demonstrate that the frequency of genomic copy-number aberrations significantly increased during the course of invasion, and the loss of 8p11.22-ter was more often associated with invasive tissues. Expression profiling in pancreatic cancer cell lines with and without 8p11.22-ter revealed that DUSP4, an MAPK phosphatase, was significantly downregulated in cells lacking 8p11.22-ter as well as in invasive carcinomas due to genomic loss. Restoration of DUSP4 expression in pancreatic cancer cells significantly suppressed invasiveness and anoikis resistance via ERK inactivation. Accordingly, we found that blockade of ERK signaling by MEK inhibition was effective in an orthotopic xenograft model and significantly extended survival. Collectively, our findings demonstrate a genetic mechanism by which pancreatic precursor lesions progress to invasive carcinomas and highlight DUSP4 as a novel invasion suppressor that can be therapeutically exploited through manipulation of ERK signaling. Cancer Res; 76(9); 2612-25. ©2016 AACR. PMID:26941286

  20. Emerging Potential of Natural Products for Targeting Mucins for Therapy Against Inflammation and Cancer

    PubMed Central

    Macha, Muzafar A.; Krishn, Shiv Ram; Jahan, Rahat; Banerjee, Kasturi; Batra, Surinder K.; Jain, Maneesh

    2015-01-01

    Deregulated mucin expression is a hallmark of several inflammatory and malignant pathologies. Emerging evidence suggests that, apart from biomarkers, these deregulated mucins are functional contributors to pathogenesis in inflammation and cancer. Both overexpression and downregulation of mucins in various organ systems is associated with pathobiology of inflammation and cancer. Restoration of mucin homeostasis has become an important goal for therapy and management of such disorders and has fueled the quest for selective mucomodulators. With improved understanding of mucin regulation and mechanistic insights into their pathobiological roles, there is optimism to find selective non-toxic agents capable of modulating mucin expression and function. Recently, natural compounds derived from dietary sources have drawn attention due to their anti-inflammatory and anti-oxidant properties and low toxicity. Considerable efforts have been directed towards evaluating dietary natural products as chemopreventive and therapeutic agents; identification, characterization and synthesis of their active compounds; and improving their delivery and bioavailability. We describe the current understanding of mucin regulation, rationale for targeting mucins with natural products and discuss some natural products that modulate mucin expression and functions. We further discuss the approaches and parameters that should guide future research to identify and evaluate selective natural mucomodulators for therapy. PMID:25624117

  1. The effect of the space flight environment on mucin production in the mouse uterine tube

    NASA Astrophysics Data System (ADS)

    Svalina, Gorica; Forsman, Allan D.

    2013-06-01

    Numerous studies have indicated that the microgravity environment of space has harmful effects on several tissues throughout the body. Although this phenomenon is well documented, research in this area is still in its relative infancy. This study investigates the effects of space flight on mucin production of the uterine tubes of mice. This study examined the epithelium of the uterine tubes from female mice that were flown on the space shuttle Endeavour for 13 days in August, 2007 and their concomitant controls. The tissue was qualitatively analyzed for the type of mucin produced, i.e., acidic, neutral, acidic/neutral mixture. Further, the tissue was quantitatively analyzed for the amounts of mucins produced by measuring the thickness of the mucin layer for each region of the uterine tube: isthmus, ampulla, and infundibulum. One way ANOVA tests were used to compare mucin thickness between all three sets of animals. Results indicate similar but not identical results between the three regions of the uterine tube. The Baseline tissue had the thickest mucin layer regardless of treatment group. In the ampulla the mucin layer was the thinnest in the Flight tissue, followed by the Ground Control, with the Baseline being the thickest. Analysis of the mucin layer of the infundibulum of the three treatment groups indicated no difference in its thickness between the three regions of the uterine tube. These results indicate a trend toward thinning of the mucin layer of the uterine tube in space flight, but also indicate an influence by the housing environment.

  2. Molecular analysis of ovarian mucinous carcinoma reveals different cell of origins.

    PubMed

    Wang, Yihong; Shwartz, Lauren Ende; Anderson, Derek; Lin, Ming-Tseh; Haley, Lisa; Wu, Ren-Chin; Vang, Russell; Shih, Ie-Ming; Kurman, Robert J

    2015-09-01

    It is believed that a subset of primary ovarian mucinous tumors is derived from mature teratomas [1-5]. To confirm this, we performed microsatellite genotyping using a variety of short tandem repeat makers and analyzed allelotypes of 8 mucinous tumors (4 mucinous carcinomas, 3 atypical proliferative mucinous tumors and 1 mucinous cystadenoma) associated with a teratoma to determine whether they were clonally related. 7 of the 8 mucinous tumors showed complete or a high degree of homozygosity. Among the 6 pairs of tumors with teratoma tissue available for comparison, 5 of 6 showed a high or complete degree of allelotypes matching, which differed from the somatic allelotypes of the normal control tissue. A discrepancy was detected between carcinoma and teratoma in one pair at several loci, with different X-chromosome inactivation patterns revealed by the HUMARA clonality assay. We also investigated the allelotypes of 16 ovarian mucinous carcinomas without a teratoma in young patients (range 13-30) and in 6 older patients (range 40-67) using the same method. None of these tumors showed pure homozygosity. The number of homozygous loci in this cohort was significantly lower than that in the first. Our results suggest first, that most mucinous tumors associated with a teratoma are derived from the teratoma but occasionally they could be collision tumors and second that the majority of pure mucinous tumors in young women in whom a teratoma is not present are not derived from a teratoma. PMID:26355245

  3. Ocular surface mucins and local inflammation-studies in genetically modified mouse lines.

    PubMed

    Shirai, Kumi; Saika, Shizuya

    2015-01-01

    Mucins locate to the apical surfaces of all wet-surfaced epithelia including ocular surface. The functions of the mucins include anti-adhesive, lubrication, water retention, allergens and pathogen barrier function. Ocular surface pathologies, i.e. dry eye syndrome or allergic conjunctivitis, are reportedly associated with alteration of expression pattern of mucin components. Recent investigations indicated anti-bacterial adhesion or anti-inflammatory effects of members of mucins in non-ocular tissues, i.e., gastrointestinal tracts or airway tissues, by using genetically modified mouse lines that lacks an expression of a mucin member. However, examination of ocular phenotypes of each of mucin gene-ablated mouse lines has not yet fully performed. Muc16-dficient mouse is associated with spontaneous subclinical inflammation in conjunctiva. The article reviews the roles of mucin members in modulation of local inflammation in mucous membrane tissues and phenotype of mouse lines with the loss of a mucin gene. Analysis of ocular surface of mucin-gene related mutant mouse lines are to be further performed. PMID:26818460

  4. Cysteamine and prostaglandin F2 beta stimulate rat gastric mucin release

    SciTech Connect

    Lamont, J.T.; Ventola, A.S.; Maull, E.A.; Szabo, S.

    1983-02-01

    Gastric mucin glycoproteins form an adherent gel over the surface epithelium that is thought to protect the stomach against chemical and physical damage. The purpose of this study was to measure the release of mucin glycoproteins from rat stomach after treatment with cysteamine and prostaglandin F2 beta, two structurally unrelated drugs that have been shown to protect the stomach against the noxious effects of alcohol and other damaging agents. Gastric mucin was separated into soluble (washout) and insoluble (adherent) phases before colorimetric quantitation of total mucin, protein-bound hexose, and sialic acid. Cysteamine produced a dose-dependent increase in release of soluble and gel mucin. Prostaglandin F2 beta caused a dose-dependent release of hexose-containing mucin but had no effect on sialic acid-containing glycoproteins. Sepharose 4B chromatography of both the soluble and adherent mucus revealed that greater than 90% was a high molecular weight glycoprotein fraction. N-Ethylmaleimide, a known inhibitor of cytoprotection by cysteamine, had no effect on mucin secretion. Similarly, indomethacin inhibited mucin secretion by cysteamine but did not significantly influence cytoprotection. Thus the secretion of mucin by cytoprotective agents is unlikely by itself to explain the ability of the stomach to resist chemical or physical damage.

  5. Emerging potential of natural products for targeting mucins for therapy against inflammation and cancer.

    PubMed

    Macha, Muzafar A; Krishn, Shiv Ram; Jahan, Rahat; Banerjee, Kasturi; Batra, Surinder K; Jain, Maneesh

    2015-03-01

    Deregulated mucin expression is a hallmark of several inflammatory and malignant pathologies. Emerging evidence suggests that, apart from biomarkers, these deregulated mucins are functional contributors to the pathogenesis in inflammation and cancer. Both overexpression and downregulation of mucins in various organ systems is associated with pathobiology of inflammation and cancer. Restoration of mucin homeostasis has become an important goal for therapy and management of such disorders has fueled the quest for selective mucomodulators. With improved understanding of mucin regulation and mechanistic insights into their pathobiological roles, there is optimism to find selective non-toxic agents capable of modulating mucin expression and function. Recently, natural compounds derived from dietary sources have drawn attention due to their anti-inflammatory and anti-oxidant properties and low toxicity. Considerable efforts have been directed towards evaluating dietary natural products as chemopreventive and therapeutic agents; identification, characterization and synthesis of their active compounds; and improving their delivery and bioavailability. We describe the current understanding of mucin regulation, rationale for targeting mucins with natural products and discuss some natural products that modulate mucin expression and functions. We further discuss the approaches and parameters that should guide future research to identify and evaluate selective natural mucomodulators for therapy. PMID:25624117

  6. Benign mucinous metaplasia of the penis. A lesion resembling extramammary Paget's disease.

    PubMed

    Val-Bernal, J F; Hernández-Nieto, E

    2000-02-01

    Benign mucinous metaplasia in the surface epithelium of the genital area is rare and has only been reported once in the vulva. A unique case of benign mucinous metaplasia of the prepuce in a 65-year-old man is reported here. The lesion measured 0.6 cm, was located in the mucous surface of the foreskin, and showed acid mucin containing cells. We regard benign mucinous metaplasia as a reactive rather than a neoplastic process. The main lesions to be considered in the differential diagnosis are mucinous syringometaplasia, extramammary Paget's disease, cutaneous squamous cell carcinoma in situ with mucinous metaplasia, superficial spreading malignant melanoma, and epidermotropic metastasis. The confinement of mucin-containing cells to the epidermis, the absence of nuclear atypia, the basal orientation of the nuclei, the predominant location of the cells in the upper layers of the epithelium, and the fact that the mucinous cells are replacing the squamous epithelium rather that infiltrating it, all assist in recognizing mucinous metaplasia of the penis as a specific and benign entity. PMID:10678702

  7. Clinicopathological analysis of primary epithelial appendiceal neoplasms.

    PubMed

    Gupta, Sachin; Parsa, Venkata; Adsay, Volkan; Heilbrun, Lance K; Smith, Daryn; Shields, Anthony F; Weaver, Donald; Philip, Philip A; El-Rayes, Bassel F

    2010-12-01

    Appendiceal carcinomas are classified into three distinct histopathological disease entities: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), or peritoneal mucinous carcinomatosis with intermediate or discorant features (PMCA I/D). The treatment of appendiceal tumors should be based on accurate histopathological classification, per previously reported case series. The objective of this study was to evaluate the clinicopathologic presentation and outcome of patients with appendiceal tumors treated at our institution over a 15-year period. We identified patients with appendiceal tumors diagnosed or treated at our institution from January 1989 through May 2004. Chart review for age, gender, signs and symptoms at diagnosis, and treatment was performed. Review of the pathologic specimens was performed and tumors were classified as DPAM, PMCA I/D, or PMCA. Forty patients were identified (median age 52.5 years; males 38%). The number of patients with DPAM, PMCA I/D, and PMCA was 15 (38%), 6 (15%), and 18 (46%), respectively. Peritoneal involvement was seen in 11 (73%) of patients with DPAM, 5 (83%) of PMCA I/D, and 11 (61%) of PMCA. The median survival for patients with DPAM, PMCA I/D, and PMCA was 7.7 years (90% CI: 2.9--upper limit not estimable), 1.2 years (90% CI: 0.9-1.6), and 0.7 years (90% CI: 0.4-1.5), respectively. The difference in survival across the three groups was statistically significant. Three distinct histopathological disease entities exist in appendiceal tumors. The prognosis and management of these tumors should be based on the extent of disease and pathologic diagnosis. PMID:19851895

  8. Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis.

    PubMed Central

    Cheng, P W; Boat, T F; Cranfill, K; Yankaskas, J R; Boucher, R C

    1989-01-01

    Cystic fibrosis (CF) respiratory epithelia exhibit abnormal anion transport that may be linked to abnormal lung defense. In these studies, we investigated whether primary cultures of CF respiratory epithelial cells regulate abnormally the sulfate content of high molecular weight glycoconjugates (HMG) participating in airways' mucosal defense. HMG, including glycosaminoglycans and mucin-type glycoproteins released spontaneously into medium and HMG released from cell surfaces by trypsin, were metabolically labeled with 35SO4- and [6-3H]-glucosamine (GlcN) or 35SO4- and [3H]serine. All three classes of HMG from CF cells exhibited 35S/3H labeling ratios 1.5-4-fold greater than HMG from normal or disease control cells. Differences for labeling ratios of HMG from CF cells were shown to be the consequence of increased 35SO4- incorporation rather than decreased peptide synthesis and release or HMG glycosylation. The buoyant density of CF mucin-type HMG also was increased, consistent with increased sulfation. These observations suggest that oversulfation of a spectrum of HMG is a genetically determined characteristic of CF epithelial cells and may play an important pathophysiological role by altering the properties of mucous secretions and/or the interactions between selected bacteria and HMG at the airways' surface. Images PMID:2738159

  9. Efficacy of a novel mucolytic agent on pseudomyxoma peritonei mucin, with potential for treatment through peritoneal catheters.

    PubMed

    Akhter, Javed; Pillai, Krishna; Chua, Terence C; Alzarin, Naeef; Morris, David Lawson

    2014-01-01

    Compared to current treatment for pseudomyxoma peritonei (PMP), the extraction of solubilised mucin through peritoneal catheter can be minimally invasive. However, mucin has variable appearance that may influence mucolysis. Hence, we investigated the mucolysis of 36 mucin samples with a novel agent. Using visual inspection and hardness index, PMP mucin was classified into three grades. The mucin pathological category was identified from patient record. Subsequently, the dissolution of the samples was tested. For in vitro, 1 g of mucin was treated to the mucolytic agent in 10 ml TRIS buffer at 37 deg. Celsius for 3 hours, with weighing of residual mucin. Control treatment was similar but received TRIS buffer. For in vivo, 2 g of implanted intra-peritoneal mucin in nude rats was treated to mucolytic (2 X 500 ul/24 hr, over 48 hours, plus another treatment before sacrifice at 56 hours, with weighing of residual mucin. Controls were treated but only with TRIS buffer. Six animals were used for each mucin grade (3 mucolytic treated & and 3 controls). Grades of mucin were soft mucin (62%), semi hard (20%) and hard mucin (18%). Diffuse peritoneal adenomucinosis had 50% of soft mucin and peritoneal mucinous carcinoma had 11% (P = 0.0382). In vitro and in vivo absolute disintegration was 100% for soft, 57.38% and 48.67% for semi hard, 50% and 28.67% for hard mucin. Majority of mucin were soft with complete disintegration, the rest showed variable disintegration, suggesting that the mucolytic has potential for treating PMP. PMID:25232491

  10. Efficacy of a novel mucolytic agent on pseudomyxoma peritonei mucin, with potential for treatment through peritoneal catheters

    PubMed Central

    Akhter, Javed; Pillai, Krishna; Chua, Terence C; Alzarin, Naeef; Morris, David Lawson

    2014-01-01

    Compared to current treatment for pseudomyxoma peritonei (PMP), the extraction of solubilised mucin through peritoneal catheter can be minimally invasive. However, mucin has variable appearance that may influence mucolysis. Hence, we investigated the mucolysis of 36 mucin samples with a novel agent. Using visual inspection and hardness index, PMP mucin was classified into three grades. The mucin pathological category was identified from patient record. Subsequently, the dissolution of the samples was tested. For in vitro, 1 g of mucin was treated to the mucolytic agent in 10 ml TRIS buffer at 37 deg. Celsius for 3 hours, with weighing of residual mucin. Control treatment was similar but received TRIS buffer. For in vivo, 2 g of implanted intra-peritoneal mucin in nude rats was treated to mucolytic (2 X 500 ul/24 hr, over 48 hours, plus another treatment before sacrifice at 56 hours, with weighing of residual mucin. Controls were treated but only with TRIS buffer. Six animals were used for each mucin grade (3 mucolytic treated & and 3 controls). Grades of mucin were soft mucin (62%), semi hard (20%) and hard mucin (18%). Diffuse peritoneal adenomucinosis had 50% of soft mucin and peritoneal mucinous carcinoma had 11% (P = 0.0382). In vitro and in vivo absolute disintegration was 100% for soft, 57.38% and 48.67% for semi hard, 50% and 28.67% for hard mucin. Majority of mucin were soft with complete disintegration, the rest showed variable disintegration, suggesting that the mucolytic has potential for treating PMP. PMID:25232491

  11. Depletion of mucin in mucin-producing human gastrointestinal carcinoma: Results from in vitro and in vivo studies with bromelain and N-acetylcysteine

    PubMed Central

    Amini, Afshin; Masoumi-Moghaddam, Samar; Ehteda, Anahid; Liauw, Winston; Morris, David L.

    2015-01-01

    Aberrant expression of membrane-associated and secreted mucins, as evident in epithelial tumors, is known to facilitate tumor growth, progression and metastasis, and to provide protection against adverse growth conditions, chemotherapy and immune surveillance. Emerging evidence provides support for the oncogenic role of MUC1 in gastrointestinal carcinomas and relates its expression to an invasive phenotype. Similarly, mucinous differentiation of gastrointestinal tumors, in particular increased or de novo expression of MUC2 and/or MUC5AC, is widely believed to imply an adverse clinicopathological feature. Through formation of viscous gels, too, MUC2 and MUC5AC significantly contribute to the biology and pathogenesis of mucin-secreting gastrointestinal tumors. Here, we investigated the mucin-depleting effects of bromelain (BR) and N-acetylcysteine (NAC), in nine different regimens as single or combination therapy, in in vitro (MKN45, KATOIII and LS174T cell lines) and in vivo (female nude mice bearing intraperitoneal MKN45 and LS174T) settings. The inhibitory effects of the treatment on cancer cell growth and proliferation were also evaluated in vivo. Our results suggest that a combination of BR and NAC with dual effects on growth and mucin products of mucin-expressing tumor cells is a promising candidate towards the development of novel approaches to gastrointestinal malignancies with the involvement of mucin pathology. This capability supports the use of this combination formulation in locoregional approaches for reducing the adverse effects of the aberrantly secreted gel-forming mucins, as in pseudomyxoma peritonei and similar pathologies with ectopic production of mucin. PMID:26436698

  12. Depletion of mucin in mucin-producing human gastrointestinal carcinoma: Results from in vitro and in vivo studies with bromelain and N-acetylcysteine.

    PubMed

    Amini, Afshin; Masoumi-Moghaddam, Samar; Ehteda, Anahid; Liauw, Winston; Morris, David L

    2015-10-20

    Aberrant expression of membrane-associated and secreted mucins, as evident in epithelial tumors, is known to facilitate tumor growth, progression and metastasis, and to provide protection against adverse growth conditions, chemotherapy and immune surveillance. Emerging evidence provides support for the oncogenic role of MUC1 in gastrointestinal carcinomas and relates its expression to an invasive phenotype. Similarly, mucinous differentiation of gastrointestinal tumors, in particular increased or de novo expression of MUC2 and/or MUC5AC, is widely believed to imply an adverse clinicopathological feature. Through formation of viscous gels, too, MUC2 and MUC5AC significantly contribute to the biology and pathogenesis of mucin-secreting gastrointestinal tumors. Here, we investigated the mucin-depleting effects of bromelain (BR) and N-acetylcysteine (NAC), in nine different regimens as single or combination therapy, in in vitro (MKN45, KATOIII and LS174T cell lines) and in vivo (female nude mice bearing intraperitoneal MKN45 and LS174T) settings. The inhibitory effects of the treatment on cancer cell growth and proliferation were also evaluated in vivo. Our results suggest that a combination of BR and NAC with dual effects on growth and mucin products of mucin-expressing tumor cells is a promising candidate towards the development of novel approaches to gastrointestinal malignancies with the involvement of mucin pathology. This capability supports the use of this combination formulation in locoregional approaches for reducing the adverse effects of the aberrantly secreted gel-forming mucins, as in pseudomyxoma peritonei and similar pathologies with ectopic production of mucin. PMID:26436698

  13. Baseline Goblet Cell Mucin Secretion in the Airways Exceeds Stimulated Secretion over Extended Time Periods, and Is Sensitive to Shear Stress and Intracellular Mucin Stores

    PubMed Central

    Doyle, Sean P.; Nguyen, Kristine; Ribeiro, Carla M. P.; Vasquez, Paula A.; Forest, M. Gregory; Lethem, Michael I.; Dickey, Burton F.; Davis, C. William

    2015-01-01

    Airway mucin secretion studies have focused on goblet cell responses to exogenous agonists almost to the exclusion of baseline mucin secretion (BLMS). In human bronchial epithelial cell cultures (HBECCs), maximal agonist-stimulated secretion exceeds baseline by ~3-fold as measured over hour-long periods, but mucin stores are discharged completely and require 24 h for full restoration. Hence, over 24 h, total baseline exceeds agonist-induced secretion by several-fold. Studies with HBECCs and mouse tracheas showed that BLMS is highly sensitive to mechanical stresses. Harvesting three consecutive 1 h baseline luminal incubations with HBECCs yielded equal rates of BLMS; however, lengthening the middle period to 72 h decreased the respective rate significantly, suggesting a stimulation of BLMS by the gentle washes of HBECC luminal surfaces. BLMS declined exponentially after washing HBECCs (t1/2 = 2.75 h), to rates approaching zero. HBECCs exposed to low perfusion rates exhibited spike-like increases in BLMS when flow was jumped 5-fold: BLMS increased >4 fold, then decreased within 5 min to a stable plateau at 1.5–2-fold over control. Higher flow jumps induced proportionally higher BLMS increases. Inducing mucous hyperplasia in HBECCs increased mucin production, BLMS and agonist-induced secretion. Mouse tracheal BLMS was ~6-fold higher during perfusion, than when flow was stopped. Munc13-2 null mouse tracheas, with their defect of accumulated cellular mucins, exhibited similar BLMS as WT, contrary to predictions of lower values. Graded mucous metaplasia induced in WT and Munc13-2 null tracheas with IL-13, caused proportional increases in BLMS, suggesting that naïve Munc13-2 mouse BLMS is elevated by increased mucin stores. We conclude that BLMS is, [i] a major component of mucin secretion in the lung, [ii] sustained by the mechanical activity of a dynamic lung, [iii] proportional to levels of mucin stores, and [iv] regulated differentially from agonist-induced mucin

  14. Baseline Goblet Cell Mucin Secretion in the Airways Exceeds Stimulated Secretion over Extended Time Periods, and Is Sensitive to Shear Stress and Intracellular Mucin Stores.

    PubMed

    Zhu, Yunxiang; Abdullah, Lubna H; Doyle, Sean P; Nguyen, Kristine; Ribeiro, Carla M P; Vasquez, Paula A; Forest, M Gregory; Lethem, Michael I; Dickey, Burton F; Davis, C William

    2015-01-01

    Airway mucin secretion studies have focused on goblet cell responses to exogenous agonists almost to the exclusion of baseline mucin secretion (BLMS). In human bronchial epithelial cell cultures (HBECCs), maximal agonist-stimulated secretion exceeds baseline by ~3-fold as measured over hour-long periods, but mucin stores are discharged completely and require 24 h for full restoration. Hence, over 24 h, total baseline exceeds agonist-induced secretion by several-fold. Studies with HBECCs and mouse tracheas showed that BLMS is highly sensitive to mechanical stresses. Harvesting three consecutive 1 h baseline luminal incubations with HBECCs yielded equal rates of BLMS; however, lengthening the middle period to 72 h decreased the respective rate significantly, suggesting a stimulation of BLMS by the gentle washes of HBECC luminal surfaces. BLMS declined exponentially after washing HBECCs (t1/2 = 2.75 h), to rates approaching zero. HBECCs exposed to low perfusion rates exhibited spike-like increases in BLMS when flow was jumped 5-fold: BLMS increased >4 fold, then decreased within 5 min to a stable plateau at 1.5-2-fold over control. Higher flow jumps induced proportionally higher BLMS increases. Inducing mucous hyperplasia in HBECCs increased mucin production, BLMS and agonist-induced secretion. Mouse tracheal BLMS was ~6-fold higher during perfusion, than when flow was stopped. Munc13-2 null mouse tracheas, with their defect of accumulated cellular mucins, exhibited similar BLMS as WT, contrary to predictions of lower values. Graded mucous metaplasia induced in WT and Munc13-2 null tracheas with IL-13, caused proportional increases in BLMS, suggesting that naïve Munc13-2 mouse BLMS is elevated by increased mucin stores. We conclude that BLMS is, [i] a major component of mucin secretion in the lung, [ii] sustained by the mechanical activity of a dynamic lung, [iii] proportional to levels of mucin stores, and [iv] regulated differentially from agonist-induced mucin

  15. PAX2, PAX8 and CDX2 Expression in Metastatic Mucinous, Primary Ovarian Mucinous and Seromucinous Tumors and Review of the Literature.

    PubMed

    Ates Ozdemir, D; Usubutun, A

    2016-07-01

    Ovarian cancer is the most common cause of gynecologic cancer death. Both morphologically and immunohistochemically, metastatic mucinous tumors are the best mimickers of mucinous ovarian tumors; its pathogenesis still remains a mystery. PAX2 and PAX8 immunohisyochemistries are useful for differentiating numerous primary tumour types from metastatic ones. There are few studies in literature about PAX expressions in mucinous and seromucinous tumors. None of these are takes into account the histologic type (whether it is seromucinous or mucinous) or the metastatic origin. With this purpose hematoxylin and eosine slides of ovarian mucinous and seromucinous tumors were re-evaluated and one block was chosen for each case. The study included 76 ovarian mucinous and seromucinous tumors of the ovary reported in Hacettepe University department of pathology between 2000 and 2013. Tissue microarray (TMA) was designed from the chosen blocks, PAX2, PAX8, CDX2 immunostains was preformed to the TMA slides. As a result, most of the metastatic cases were negative for PAX2 (91.2 %) and PAX8 (86.3 %), many were diffusely and strongly positive for CDX2 (68.2 %). Seromucinous tumors were devoid of CDX2 expression; but all cases (except one) displayed strong and diffuse positivity with PAX8. In other words differing from mucinous tumors, seromucinous tumors show strong PAX8 positivity-similar to serous tumors. This study shows that PAX8 and CDX2 could be useful in differentiating primary mucinous from metastatic tumor. Furthermore unlike the homogeneity in seromucinous tumors for PAX8 and CDX2 mucinous tumors shows heterogeneity with different expression patterns. PMID:26797858

  16. [Epithelial tumor-like changes, precancerous conditions and skin neoplasms (standardization study)].

    PubMed

    Bednár, B; Stanová, M

    1976-05-01

    A retrospective study of bioptic material was used to design the following outline of a histological classification of epithelial skin tumours tentatively compared with handbooks published by the WHO (1) and AFIP (2): I. Tumour-like changes: 1. senile verruca (mixed, acanthotic, melanoacanthotic, hyperkeratonic, reticular, inverted). 2. Virus verrucosities (v. vulgaris, v. plana, c. accuminatum, molluscom contagiosum). 3. Hamartogenic verrucosities (naevus verrucosus, n. comedonicus, fibroepithelial papilloma. 4. Genetically undefined verrucosities (acanthosis nigricans, light cell acanthoma, verrucous dyskeratosis). 5. Cysts (atheroma, epidermoid cyst, dermoid cyst, others). 6. Unclassified. II. Precanceroses: 1. Pseudoepitheliomatous hyperplasis, 2. keratosis senilis, 3. Radiation dermatosis, 4. Unclassified. III. Epithelial tumours A. From surface epithelium 1. Spinocellular carcinoma (basic type, anaplastic, adenoid, sarcomatoid, clear cell carcinoma, intraepidermal). 2. Basocellular carcinoma: a) varieties derived from surface epithelium (intraepithelial, superficial, solid, cystic, invasive), b) varieties with adenoid features (cylindromatous, fibroepithelia), c) varieties with trichoepithelial features (keratinizing, pigment-type, clear cell type), d) naevus varieties (basocellular naevi). 3. Spinobasocellular carcinoma. 4. Unclassifiable. B. Sweat gland tumours: 1. syringocystadenoma papilliferum, 2. hidradenoma papillare, 3. nodular hidradenoma (eccrine spiradenoma, eccrine acrospiroma, myxochondroepithelioma, myoepithelioma, mucinous epithelioma), 4. syringoma, 5. eccrine cylindroma, 6. hidrocystoma, 7. eccrine poroma, 8. carcinomas (so called extramammary Paget carcinoma), 9. unclassifiable. C. Sebaceous gland tumours: 1. adenoma sebaceum, 2. carcinoma sebaceum, 3. quasi tumours (naevus sebaceus, Pringle's hamartoma, steatocystoma multiplex, hyperplasia), 4. unclassifiable. D. Trichoepithelial tumours: 1. trichofolliculoma, 2. follicular poroma, 3

  17. Mucins as Diagnostic and Prognostic Biomarkers in a Fish-Parasite Model: Transcriptional and Functional Analysis

    PubMed Central

    Pérez-Sánchez, Jaume; Estensoro, Itziar; Redondo, María José; Calduch-Giner, Josep Alvar; Kaushik, Sadasivam; Sitjà-Bobadilla, Ariadna

    2013-01-01

    Mucins are O-glycosylated glycoproteins present on the apex of all wet-surfaced epithelia with a well-defined expression pattern, which is disrupted in response to a wide range of injuries or challenges. The aim of this study was to identify mucin gene sequences of gilthead sea bream (GSB), to determine its pattern of distribution in fish tissues and to analyse their transcriptional regulation by dietary and pathogenic factors. Exhaustive search of fish mucins was done in GSB after de novo assembly of next-generation sequencing data hosted in the IATS transcriptome database (www.nutrigroup-iats.org/seabreamdb). Six sequences, three categorized as putative membrane-bound mucins and three putative secreted-gel forming mucins, were identified. The transcriptional tissue screening revealed that Muc18 was the predominant mucin in skin, gills and stomach of GSB. In contrast, Muc19 was mostly found in the oesophagus and Muc13 was along the entire intestinal tract, although the posterior intestine exhibited a differential pattern with a high expression of an isoform that does not share a clear orthologous in mammals. This mucin was annotated as intestinal mucin (I-Muc). Its RNA expression was highly regulated by the nutritional background, whereas the other mucins, including Muc2 and Muc2-like, were expressed more constitutively and did not respond to high replacement of fish oil (FO) by vegetable oils (VO) in plant protein-based diets. After challenge with the intestinal parasite Enteromyxum leei, the expression of a number of mucins was decreased mainly in the posterior intestine of infected fish. But, interestingly, the highest down-regulation was observed for the I-Muc. Overall, the magnitude of the changes reflected the intensity and progression of the infection, making mucins and I-Muc, in particular, reliable markers of prognostic and diagnostic value of fish intestinal health. PMID:23776483

  18. Myeloproliferative neoplasms: Morphology and clinical practice.

    PubMed

    Barbui, Tiziano; Thiele, Jürgen; Vannucchi, Alessandro M; Tefferi, Ayalew

    2016-06-01

    In myeloproliferative neoplasms (MPNs), controversy persists regarding the usefulness and reproducibility of bone marrow (BM) features. Disagreements concerning the WHO classification are mainly focused on the discrimination between essential thrombocythemia (ET) and prefibrotic/early primary myelofibrosis (prePMF) and prodromal polycythemia vera (PV). Criticism mostly refers to lack of standardization of distinctive BM features precluding correct morphological pattern recognition. The distinction between WHO-defined ET and prePMF is not trivial because outcome is significantly worse in prePMF. Morphology was generally considered to be non-specific for the diagnosis of PV. Recent studies have revealed under-diagnosis of morphologically and biologically consistent PV. PMID:26718907

  19. Computed tomography of cardiac pseudotumors and neoplasms.

    PubMed

    Anavekar, Nandan S; Bonnichsen, Crystal R; Foley, Thomas A; Morris, Michael F; Martinez, Matthew W; Williamson, Eric E; Glockner, James F; Miller, Dylan V; Breen, Jerome F; Araoz, Philip A

    2010-07-01

    Important features of cardiac masses can be clearly delineated on cardiac computed tomography (CT) imaging. This modality is useful in identifying the presence of a mass, its relationship with cardiac and extracardiac structures, and the features that distinguish one type of mass from another. A multimodality approach to the evaluation of cardiac tumors is advocated, with the use of echocardiography, CT imaging and magnetic resonance imaging as appropriately indicated. In this article, various cardiac masses are described, including pseudotumors and true cardiac neoplasms, and the CT imaging findings that may be useful in distinguishing these rare entities are presented. PMID:20705174

  20. Pulmonary disease in gestational trophoblastic neoplasms.

    PubMed Central

    McNair, O. M.; Polk, O. D.

    1992-01-01

    Gestational trophoblastic neoplasms can present as pulmonary nodules without significant disease of the reproductive organs. This article describes a case of metastatic gestational trophoblastic disease to the lungs. This entity must be considered in the differential diagnosis in any female of reproductive age who presents with multiple pulmonary nodules. Thoracotomy has a limited role in the initial evaluation of patients with this disease. However, it may be needed in patients who have evidence of persistent pulmonary disease, despite appropriate therapy. Images Figure 1 Figure 2 Figure 3 PMID:1324326

  1. Uterine neoplasms, version 1.2014.

    PubMed

    Koh, Wui-Jin; Greer, Benjamin E; Abu-Rustum, Nadeem R; Apte, Sachin M; Campos, Susana M; Chan, John; Cho, Kathleen R; Cohn, David; Crispens, Marta Ann; Dupont, Nefertiti; Eifel, Patricia J; Fader, Amanda Nickles; Fisher, Christine M; Gaffney, David K; George, Suzanne; Han, Ernest; Huh, Warner K; Lurain, John R; Martin, Lainie; Mutch, David; Remmenga, Steven W; Reynolds, R Kevin; Small, William; Teng, Nelson; Tillmanns, Todd; Valea, Fidel A; McMillian, Nicole; Hughes, Miranda

    2014-02-01

    Adenocarcinoma of the endometrium (also known as endometrial cancer or more broadly as uterine cancer or carcinoma of the uterine corpus) is the most common malignancy of the female genital tract in the United States. An estimated 49,560 new uterine cancer cases will occur in 2013, with 8190 deaths resulting from the disease. Uterine sarcomas (stromal/mesenchymal tumors) are uncommon malignancies, accounting for approximately 3% of all uterine cancers. The NCCN Guidelines for Uterine Neoplasms describe malignant epithelial carcinomas and uterine sarcomas; each of these major categories contains specific histologic groups that require different management. This excerpt of these guidelines focuses on early-stage disease. PMID:24586086

  2. Mesenteric cystic lymphangioma mimicking malignancy.

    PubMed

    Hureibi, Khalid; Sunidar, Osama A

    2014-01-01

    Mesenteric cystic lymphangiomas are benign tumours arising from the mesentery, and have no known aetiology. Patients might be discovered incidentally to have asymptomatic mesenteric cysts, or they can present with symptoms such as pain, nausea and vomiting. A 27-year-old man presented with vague abdominal pain, loss of appetite, postprandial fullness and significant weight loss. There was no lymphadenopathy, and abdominal examination was unremarkable. CT showed a mesenteric mass and a diagnosis of abdominal lymphoma was suggested. There was no evidence of pulmonary tuberculosis on chest X-ray and the purified protein derivative test was negative. On laparotomy, a 5×9×7 cm sessile cyst containing thick white fluid and arising from the ileal mesentery was found and completely removed. Histopathology proved a diagnosis of mesenteric cystic lymphangioma. The patient made uneventful recovery, and was asymptomatic on clinical follow-up after 6 weeks. PMID:25178885

  3. Mesenteric cystic lymphangioma mimicking malignancy

    PubMed Central

    Hureibi, Khalid; Sunidar, Osama A

    2014-01-01

    Mesenteric cystic lymphangiomas are benign tumours arising from the mesentery, and have no known aetiology. Patients might be discovered incidentally to have asymptomatic mesenteric cysts, or they can present with symptoms such as pain, nausea and vomiting. A 27-year-old man presented with vague abdominal pain, loss of appetite, postprandial fullness and significant weight loss. There was no lymphadenopathy, and abdominal examination was unremarkable. CT showed a mesenteric mass and a diagnosis of abdominal lymphoma was suggested. There was no evidence of pulmonary tuberculosis on chest X-ray and the purified protein derivative test was negative. On laparotomy, a 5×9×7 cm sessile cyst containing thick white fluid and arising from the ileal mesentery was found and completely removed. Histopathology proved a diagnosis of mesenteric cystic lymphangioma. The patient made uneventful recovery, and was asymptomatic on clinical follow-up after 6 weeks. PMID:25178885

  4. Pharmacogenetics of cystic fibrosis treatment.

    PubMed

    Carter, Suzanne C; McKone, Edward F

    2016-08-01

    Cystic fibrosis (CF) is genetic autosomal recessive disease caused by reduced or absent function of CFTR protein. Treatments for patients with CF have primarily focused on the downstream end-organ consequences of defective CFTR. Since the discovery of the CFTR gene that causes CF in 1989 there have been tremendous advances in our understanding of the genetics and pathophysiology of CF. This has recently led to the development of new CFTR mutation-specific targeted therapies for select patients with CF. This review will discuss the characteristics of the CFTR gene, the CFTR mutations that cause CF and the new mutation specific pharmacological treatments including gene therapy that are contributing to the dawning of a new era in cystic fibrosis care. PMID:27490265

  5. [Cystic fibrosis and associated complications].

    PubMed

    Schwarz, C; Staab, D

    2015-03-01

    Cystic fibrosis (CF) is an autosomal recessive inherited metabolic disease. The mutation is located on the long arm of chromosome 7. Due to a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, chloride ion transport is reduced across the cell membrane. As a result, the disease can be described as an exocrinopathy. In all organs with exocrine glands, disorders occur in association with the defective chloride transport. The main impact of this defect is manifested in the lungs. Therefore, the most common cause of death is pulmonary disease with respiratory insufficiency due to recurrent infections. Unfortunately, a cure for the disease is still not available. However, new therapies that may affect the CFTR mutation more specifically give new hope for better therapeutic options in the future. The long-term goal of therapy is to develop a causal therapy for all six different mutation classes and thus for about 2000 mutations. PMID:25693903

  6. Etanercept-induced cystic acne.

    PubMed

    Kashat, Maria; Caretti, Katherine; Kado, Jessica

    2014-07-01

    Tumor necrosis factor α antagonists are potent biologics used to treat a variety of autoimmune disorders such as rheumatoid arthritis, ankylosing spondylitis, Crohn disease, psoriasis, and psoriatic arthritis. These medications are known to have many side effects (eg, infusion reactions, cytopenia, risk for infection, heart failure); however, only a few cases of acne vulgaris have been associated with the use of these biologics, particularly infliximab and adalimumab. We report a rare case of etanercept-induced cystic acne. PMID:25101341

  7. [Imaging spinal cord cystic lesions in adults].

    PubMed

    Kremer, S; Bierry, G; Abu Eid, M; Bogorin, A; Koob, M; Zöllner, G; Dietemann, J L

    2007-05-01

    Intrarachidian cystic lesions are frequent, with highly varied causes. They can be classified according to their location into intramedullary cystic lesions and extramedullary cystic lesions. In these two categories, they can then be regrouped according to the tissue from which they develop. MRI is the first-choice examination for the study of the intracanal contents and the differential diagnosis between the various lesions. PMID:17541357

  8. Congenital Cystic Lesions of the Lung: Congenital Cystic Adenomatoid Malformation and Bronchopulmonary Sequestration

    PubMed Central

    Sfakianaki, Anna K; Copel, Joshua A

    2012-01-01

    Congenital cystic lesions of the lung in fetuses are rare. The most common malformations of the lower respiratory tract are congenital cystic adenomatoid malformation and bronchopulmonary sequestration. With the increased use of obstetric ultrasound, cystic lung lesions are detected more often antenatally, which allows for proper planning of peripartum and neonatal management. This article discusses a range of diagnostic and management options. PMID:22866187

  9. Novel bifidobacterial glycosidases acting on sugar chains of mucin glycoproteins.

    PubMed

    Katayama, Takane; Fujita, Kiyotaka; Yamamoto, Kenji

    2005-05-01

    Bifidobacterium bifidum was found to produce a specific 1,2-alpha-L-fucosidase. Its gene (afc A) has been cloned and the DNA sequence was determined. The Afc A protein consisting of 1959 amino acid residues with a predicted molecular mass of 205 kDa can be divided into three domains; the N-terminal function-unknown domain (576 aa), the catalytic domain (898 aa), and the C-terminal bacterial Ig-like domain (485 aa). The recombinant catalytic domain specifically hydrolyzed the terminal alpha-(1-->2)-fucosidic linkages of various oligosaccharides and sugar chains of glycoproteins. The primary structure of the catalytic domain exhibited no similarity to those of any glycoside hydrolases but showed similarity to those of several hypothetical proteins in a database, which resulted in establishment of a novel glycoside hydrolase family (GH family 95). Several bifidobacteria were found to produce a specific endo-alpha-N-acetylgalactosaminidase, which is the endoglycosidase liberating the O-glycosidically linked galactosyl beta1-->3 N-acetylgalactosamine disaccharide from mucin glycoprotein. The molecular cloning of endo-alpha-N-acetylgalactosaminidase was carried out on Bifidobacterium longum based on the information in the database. The gene was found to comprise 1966 amino acid residues with a predicted molecular mass of 210 kDa. The recombinant protein released galactosyl beta1-->3 N-acetylgalactosamine disaccharide from natural glycoproteins. This enzyme of B. longum is believed to be involved in the catabolism of oligosaccharide of intestinal mucin glycoproteins. Both 1,2-alpha-L-fucosidase and endo-alpha-N-acetylgalactosaminidase are novel and specific enzymes acting on oligosaccharides that exist mainly in mucin glycoproteins. Thus, it is reasonable to conclude that bifidobacteria produce these enzymes to preferentially utilize the oligosaccharides present in the intestinal ecosystem. PMID:16233817

  10. Prognostic comparison between mucinous and nonmucinous adenocarcinoma in colorectal cancer.

    PubMed

    Park, Jong Seob; Huh, Jung Wook; Park, Yoon Ah; Cho, Yong Beom; Yun, Seong Hyeon; Kim, Hee Cheol; Lee, Woo Yong; Chun, Ho-Kyung

    2015-04-01

    Mucinous adenocarcinoma (MAC) is a histological subtype of colorectal cancer. The oncologic behavior of MAC differs from nonmucinous adenocarcinoma (non-MAC). Our aim in this study was to characterize patients with colorectal MAC through evaluation of a large, institutional-based cohort with long-term follow-up. A total of 6475 patients with stages I to III colorectal cancer who underwent radical surgery were enrolled from January 2000 to December 2010. Prognostic comparison between MAC (n = 274, 4.2%) and non-MAC was performed. The median follow-up period was 48.0 months. Patients with MAC were younger than those without MAC (P = 0.012) and had larger tumor size (P < 0.001), higher preoperative carcinoembryonic antigen (P < 0.001), higher pathologic T stage (P < 0.001), more right-sided colon cancer (49.3%, P < 0.001), and more frequent high-frequency microsatellite instability (10.2%, P < 0.001). Five-year disease-free survival (DFS) was 76.5% in the MAC group and 83.2% in the non-MAC group (P = 0.008), and 5-year overall survival was 81.4% versus 87.4%, respectively (P = 0.005). Mucinous histology (MAC vs non-MAC) in the entire cohort was not an independent prognostic factor of DFS but had a statistical tendency (P = 0.071). In subgroup analysis of colon cancer without rectal cancer, mucinous histology was an independent prognostic factor (P = 0.026). MAC was found at more advanced stage, located mainly at the right side and was an independent factor of survival in colon cancer. Because of the unique biological behavior of MAC, patients with MAC require special consideration during follow-up. PMID:25881840

  11. Different staining patterns of ovarian Brenner tumor and the associated mucinous tumor.

    PubMed

    Roma, Andres A; Masand, Ramya P

    2015-02-01

    The association of ovarian Brenner tumors and adjacent mucinous tumors is well known but not completely understood. In this study, we analyzed immunohistochemical markers on Brenner tumors and their associated mucinous tumor to explore Mullerian as well as Wolffian and germ cell derivation and determine if the mucinous component is independent or related to the Brenner tumor. Of 32 consecutive cases of Brenner tumors, 8 were identified with significant mucinous component, and 7 additional cases included foci of mucinous epithelium within the Brenner transitional nests. All Brenner tumors were diffusely positive for GATA3 and negative for Paired box gene 8, PAX2, and Sal-like protein 4. Interestingly, the areas of mucinous epithelium as well as mucinous tumors, intermixed and adjacent to the Brenner tumor, were negative for all 4 markers; however, occasional basal-like cells retained expression of GATA3. The immunoprofile of mucinous tumors associated with Brenner tumors shares the lack of Mullerian markers PAX2 and Paired box gene 8 with the Brenner tumor but differs in the expression of GATA3 only in the Brenner tumor component. PMID:25596159

  12. Mechanophysical Stimulations of Mucin Secretion in Cultures of Nasal Epithelial Cells

    PubMed Central

    Even-Tzur Davidovich, Nurit; Kloog, Yoel; Wolf, Michael; Elad, David

    2011-01-01

    Nasal epithelial cells secret mucins and are exposed in vivo to airflow-induced mechanophysical stresses, including wall shear stress (WSS), temperature, and humidity. In this work, human nasal epithelial cells cultured under air-liquid interface conditions were subjected to fields of airflow-induced oscillatory WSS at different temperature and humidity conditions. Changes in mucin secretion due to WSS were measured and the role of the cytoskeleton in mucin secretion was explored. Mucin secretion significantly increased in response to WSS in a magnitude-dependent manner with respect to static cultures and independently of the airflow temperature and humidity. In static cultures, mucin secretion decreased at high humidity with or without elevation of the temperature with respect to cultures at a comfortable climate. In cultures exposed to WSS, mucin secretion increased at high temperature with respect to cultures at comfortable climate conditions. The polymerization of actin microfilaments was shown to increase mucin secretion under WSS, whereas the dynamics of microtubule polymerization did not affect secretion. In conclusion, the data in this study show that mucin secretion is sensitive to oscillatory WSS as well as high temperature and humidity conditions. PMID:21689518

  13. Mucin aggregation from a rod-like meso-scale model

    NASA Astrophysics Data System (ADS)

    Moreno, Nicolas; Perilla, Jairo E.; Colina, Coray M.; Lísal, Martin

    2015-05-01

    Dissipative particle dynamics, a meso-scale particle-based model, was used to study the aggregation of mucins in aqueous solutions. Concentration, strength of the mucin-water interactions, as well as the effects of size, shape, and composition of the model molecules were studied. Model proteins were represented as rod-like objects formed by coarse-grained beads. In the first model, only one type of beads formed the mucin. It was found that all the surfaces were available to form aggregates and the conformation of the aggregates was a function of the strength of the mucin-water interaction. With this model, the number of aggregates was unaffected by the initial position of the mucins in the simulation box, except for the lowest mucin concentration. In a more refined mucin model, two kinds of beads were used in the molecule in order to represent the existence of cysteine-like terminal groups in the actual molecule. With this new scheme, aggregation took place by the interaction of the terminal groups between model molecules. The kinetic analysis of the evolution of the number of aggregates with time was also studied for both mucin models.

  14. Antigenic and structural features of goblet-cell mucin of human small intestine.

    PubMed Central

    Mantle, M; Forstner, G G; Forstner, J F

    1984-01-01

    With the use of a newly developed solid-phase radioimmunoassay method, the major antigenic determinants of human small-intestinal goblet-cell mucin were investigated and related to the overall tertiary structure of the mucin. Preliminary hapten inhibition studies with various oligosaccharides of known sequence and structure suggested that the determinants did not reside in carbohydrate. Exhaustive thiol reduction, however, almost abolished antigenicity, caused breakdown of the mucin into small heterogeneous glycopeptides, and liberated a 'link' peptide of Mr 118000. Western 'blots' of reduced mucin from polyacrylamide gels on to nitrocellulose sheets showed that a small amount of residual antigenicity remained in large-Mr glycopeptides (Mr greater than 200000). The 'link' peptide was not antigenic. Timed Pronase digestion of native mucin resulted in a progressive loss of antigenic determinants. Gel electrophoresis revealed that after 8h of digestion the 118000-Mr peptide had disappeared, whereas antigenicity, which was confined to large-Mr glycopeptides, was destroyed much more slowly with time (70% by 24h, 100% by 72h). Despite the loss of antigenicity, 72h-Pronase-digested glycopeptides retained all of the carbohydrate of the native mucin. Therefore the antibody to human small-intestinal mucin appears to recognize a 'naked' (non-glycosylated and Pronase-susceptible) peptide region(s) of mucin glycopeptides. For full antigenicity, however, disulphide bonds are required to stabilize a specific three-dimensional configuration of the 'naked' region. Images Fig. 4. Fig. 6. PMID:6199017

  15. Digestive neuroendocrine neoplasms: A 2016 overview.

    PubMed

    Merola, Elettra; Rinzivillo, Maria; Cicchese, Noemi; Capurso, Gabriele; Panzuto, Francesco; Delle Fave, Gianfranco

    2016-08-01

    Digestive neuroendocrine neoplasms (DNENs) have an incidence of 2.39 per 100,000 inhabitants per year, and a prevalence of 35 cases per 100,000; the gap between these rates is to be referred to the relatively long survival that characterizes the majority of these tumors, which can be thus considered as chronic oncological diseases. Up to 80% of patients are stage IV since the first diagnosis, presenting a 5-yr overall survival rate of 35%-55% and a twice higher mortality than limited disease. DNENs express somatostatin receptors in more than 80% of cases, detected through immunohistochemistry or functional imaging tests (FITs). This feature identifies patients who may benefit from "cold" somatostatin analogs (SSAs) or peptide receptors radionuclide therapy, although SSAs are sometimes used also with a negative uptake at FITs. The therapeutic options have been recently increased after the identification of molecular pathways involved in DNENs pathogenesis, and the subsequent use of targeted therapies (i.e., Everolimus and Sunitinib) for these neoplasms. This review offers an overview about pancreatic and small bowel NENs, critically underlining the issues that still need to be clarified and the future perspectives to be investigated. PMID:27212431

  16. Neoplasms in young dogs after perinatal irradiation

    SciTech Connect

    Benjamin, S.A.; Lee, A.C.; Angleton, G.M.; Saunders, W.J.; Miller, G.K.; Williams, J.S.; Brewster, R.D.; Long, R.I.

    1986-08-01

    For a study of the life-time effects of irradiation during development, 1,680 beagles were given single, whole-body exposures to /sup 60/Co gamma-radiation at one of three prenatal (preimplantation, embryonic, and fetal) or at one of three postnatal (neonatal, juvenile, and young adult) ages. Mean doses were 0, 0.16, or 0.83 Gy. For comparison with data on childhood cancer after prenatal irradiation, examination was made of tumors occurring in young dogs in this life-span experiment. Up to 4 years of age, 18 dogs had neoplasms diagnosed, 2 of these being in controls. Four dogs that were irradiated in the perinatal (late fetal or neonatal) period died of cancers prior to 2 years of age. This risk was of significant increase compared to the risks for other experimental groups and for the canine population in general. Overall, 71% (5 of 7) of all cancers and 56% (10 of 18) of all benign and malignant neoplasms seen in the first 4 years of life occurred in 29% (480 of 1680) of the dogs irradiated in the perinatal period. These data suggest an increased risk for neoplasia after perinatal irradiation in dogs.

  17. Management of early gastrointestinal neuroendocrine neoplasms

    PubMed Central

    Scherübl, Hans; Jensen, Robert T; Cadiot, Guillaume; Stölzel, Ulrich; Klöppel, Günter

    2011-01-01

    Neuroendocrine neoplasms (NENs) of the stomach, duodenum, appendix or rectum that are small (≤ 1 cm) and well differentiated can be considered “early” tumors, since they generally have a (very) good prognosis. In the new WHO classification of 2010, these neoplasms are called neuroendocrine tumors/ carcinoids (NETs), grade (G) 1 or 2, and distinguished from poorly differentiated neuroendocrine carcinomas (NECs), G3. NETs are increasing, with a rise in the age-adjusted incidence in the U.S.A. by about 700 % in the last 35 years. Improved early detection seems to be the main reason for these epidemiological changes. Both the better general availability of endoscopy, and imaging techniques, have led to a shift in the discovery of smaller-sized (≤ 10-20 mm) intestinal NETs/carcinoids and earlier tumor stages at diagnosis. Endoscopic screening is therefore effective in the early diagnosis, not only of colorectal adenocarcinomas, but also of NETs/carcinoids. Endoscopic removal, followed up with endoscopic surveillance is the treatment of choice in NETs/carcinoids of the stomach, duodenum and rectum that are ≤ 10 mm in size, have a low proliferative activity (G1), do not infiltrate the muscular layer and show no angioinvasion. In all the other intestinal NENs, optimal treatment generally needs surgery and/or medical therapy depending on type, biology and stage of the tumor, as well as the individual situation of the patient. PMID:21860682

  18. Advances in the Diagnosis of Neuroendocrine Neoplasms.

    PubMed

    Kulkarni, Harshad R; Singh, Aviral; Baum, Richard P

    2016-09-01

    Somatostatin receptor PET/CT using (68)Ga-labeled somatostatin analogs, is a mainstay for the evaluation of the somatostatin receptor status in neuroendocrine neoplasms. In addition, the assessment of glucose metabolism by (18)F-FDG PET/CT at diagnosis can overcome probable shortcomings of histopathologic grading. This offers a systematic theranostic approach for the management of neuroendocrine neoplasms, that is, patient selection for the appropriate treatment-surgery, somatostatin analogs, peptide receptor radionuclide therapy, targeted therapies like everolimus and sunitinib, or chemotherapy-and also for therapy response monitoring. Novel targets, for example, the chemokine receptor CXCR4 in higher-grade tumors and glucagon like peptide-1 receptor in insulinomas, appear promising for imaging. Scandium-44 and Copper-64, especially on account of their longer half-life (for pretherapeutic dosimetry) and cyclotron production (which favors mass production), might be the potential alternatives to (68)Ga for PET/CT imaging. The future of molecular imaging lies in Radiomics, that is, qualitative and quantitative characterization of tumor phenotypes in correlation with tumor genomics and proteomics, for a personalized cancer management. PMID:27553465

  19. Mucins and Their Sugars. Critical Mediators of Hyperreactivity and Inflammation.

    PubMed

    Raclawska, Dorota S; Ttofali, Fani; Fletcher, Ashley A; Harper, Daniel N; Bochner, Bruce S; Janssen, William J; Evans, Christopher M

    2016-03-01

    Excessive mucus causes severe airflow obstruction in fatal asthma. It is also present in mild to moderate disease, but is poorly understood and treated. Mucus overproduction is associated with dysregulated expression of the mucins MUC5AC and MUC5B. Whereas increased MUC5AC is a consistent finding, MUC5B varies-remaining stably produced in some patients but strongly repressed in others (>90%). Patients with lower MUC5B display worsened asthma phenotypes including airway hyperreactivity (AHR) to methacholine (MCh) and eosinophilic inflammation. To better understand the roles of mucins in asthma, we generated Muc5ac and Muc5b knockout ((-/-)) mice. AHR to MCh was abolished in antigen-challenged Muc5ac(-/-) mice, due to prevention of heterogeneous mucous plugging that occurred in allergic wild-type mice during MCh-induced bronchoconstriction. Thus, in addition to the established role of smooth muscle-mediated airway narrowing, Muc5ac is an essential noncontractile AHR component. We also found that, unlike Muc5ac(-/-) mice, Muc5b-deficient mice were not protected from asthma phenotypes. Furthermore, whereas inflammation was unaffected by Muc5ac deficiency, it was exaggerated in the absence of Muc5b. On the basis of these differential effects, we are now determining how asthma phenotypes are regulated by mucin isoform specificity. Glycosylation is dramatically different: Muc5ac is heavily fucosylated whereas Muc5b is mainly sialylated. Fucosylation increases mucus viscoelasticity, and FUT2, the enzyme that catalyzes mucin α1,2-fucosylation, is associated with severe asthma exacerbation risk. Sialylation is required for binding to siglec (sialic acid-binding immunoglobulin-like lectin) receptors on leukocytes. Eosinophils express Siglec-F (mouse) or Siglec-8 (human). Engagement by sialoside ligands induces eosinophil apoptosis, and Muc5b via sialylated termini that require the α2,3-sialyltransferase ST3Gal3 for synthesis binds Siglec-F and induces apoptosis in mouse

  20. Colonic mucin composition in primates. Selective alterations associated with spontaneous colitis in the cotton-top tamarin.

    PubMed

    Podolsky, D K; Madara, J L; King, N; Sehgal, P; Moore, R; Winter, H S

    1985-01-01

    Heterogeneity of colonic mucin glycoprotein was examined in rectal mucosal biopsy specimens from a variety of primate species (Saguinus oedipus, n = 18; Macaca mulatta, n = 2; Macaca fascicularis, n = 2; Aotus trivirgatus, n = 2; Saimiri sciureus, n = 2; and Callithrix jacchus, n = 2). After initial separation of radiolabeled mucin and nonmucin glycoproteins solubilized from mucosal biopsy specimens, at least five labeled mucin components were found in monkey rectal mucosa in contrast to the six mucin fractions observed in the human colon. Although primates consistently lacked the earliest eluting component present in human colonic mucin, other mucin components cochromatographed with comparable fractions previously identified in human colonic biopsy specimens. The relative proportions of each fraction were consistent throughout all species except the cotton-top tamarin (S. oedipus), an animal that develops a chronic colitis. The cotton-top tamarin was found to have a markedly reduced amount of one mucin component (IV) in a manner analogous to the reduction in a human mucin fraction previously noted in patients with ulcerative colitis. Sequential evaluation of mucin profiles in cotton-top tamarins (n = 12) treated with sulfasalazine (50 mg/kg X day) or placebo in a 10-wk double-blind crossover study demonstrated the persistence of the selective reduction in tamarin species IV unrelated to disease activity. In contrast, the relative amount of tamarin mucin III was greater in association with increased disease activity than that observed in association with reduced disease activity (46% +/- 11% total mucin vs. 19% +/- 7% total mucin posttreatment). PMID:3917260