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Sample records for giant cystic malignant

  1. Giant cystic pheochromocytoma located in the renal hilus.

    PubMed

    Melegh, Zsombor; Rényi-Vámos, Ferenc; Tanyay, Zoltán; Köves, István; Orosz, Zsolt

    2002-01-01

    A malignant tumor in the past medical history of a patient often makes the differential diagnosis of a second tumor more difficult, especially if one of the tumors does not show its characteristic features. The authors report a case of a 55-year-old male who presented with a malignant melanoma on his left shoulder. A retroperitoneal giant cystic mass, 200 mm in diameter, was found incidentally. Adrenal origin was ruled out by imaging techniques. The absence of typical clinical symptoms made a correct preoperative diagnosis unlikely, and severe cardiovascular complications set in during surgery. Considering the characteristics of the cutaneous malignant melanoma, the metastatic origin of the giant retroperitoneal tumor was not likely either. During surgery the left kidney, with a cystic tumor located in the hilus, was removed. The postoperative pathologic diagnosis was pheochromocytoma located in the hilus of the left kidney.

  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. Giant cystic pheochromocytoma: A silent entity.

    PubMed

    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.

  4. 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

  5. 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

  6. Parotid Cystic Lesion in Amelanotic Malignant Melanoma.

    PubMed

    Santos, Vitorino Modesto Dos; Gondim Neto, Manoel da Costa; de Melo, Tereza Rodrigues de Carvalho Vieira; Motta, Iara Machado

    2016-09-01

    A 60-year Brazilian woman, presented with an enlarged lymph node in the neck for one year, and a superficial nonulcerated lesion was observed in the scalp. Fine needle aspiration and biopsy of the lymph node revealed amelanocytic metastasis, and immunohistochemistry study showed Melan-A/ Mart-1 antigen (clone A103 and S-100 protein). The entire suspected area of the scalp was further resected and an amelanotic melanoma without angiolymphatic invasion was diagnosed. Ultrasonography and PET-computed tomography showed hypermetabolic cystic area in the right parotid. Furthermore, aspiration biopsy and surgical samples from parotid cyst confirmed the malignant amelanotic melanoma. Cystic metastases are scarcely reported in parotid gland, and can pose diagnostic challenges. PMID:27671185

  7. A Case of Giant Uterine Lipoleiomyoma Simulating Malignancy

    PubMed Central

    Karaman, Erbil; Çim, Numan; Bulut, Gülay; Elçi, Gülhan; Andıç, Esra; Tekin, Mustafa; Kolusarı, Ali

    2015-01-01

    Introduction. Uterine leiomyoma is the most common benign pathology in women and lipoleiomyoma is an extremely rare and specific type of leiomyoma. Here, we report an unusual case of giant pedunculated subserous lipoleiomyoma misdiagnosed preoperatively as leiomyosarcoma. Case. A 45-year-old woman admitted to our gynecology outpatient clinic for complaints of abdominal distention, tiredness, and pelvic pain for the last 6 months. Sonography and abdominal magnetic resonance imaging (MRI) showed a giant semisolid mass that filled whole abdominal cavity from pelvis to subdiaphragmatic area. A primary diagnosis of uterine sarcoma or ovarian malignancy was made. On operation, total abdominal hysterectomy with a pedunculated mass of size 30 × 23 × 12 cm and weighing 5.4 kg and bilateral salpingo-oophorectomy were performed. The histopathology revealed a lipoleiomyoma with extensive cystic and fatty degeneration without any malignancy. Discussion. The diagnosis of leiomyoma is done usually with pelvic ultrasound but sometimes it is difficult to reach a correct diagnosis especially in cases of giant and pedunculated lipoleiomyoma that included fatty tissue which may mimick malignancy. Conclusion. Subserous pedunculated giant lipoleiomyoma should be kept in mind in the differential diagnosis of leiomyosarcoma or ovarian malignancy. PMID:26266066

  8. [Giant retroperitoneal cystic mass: appendiceal mucocele].

    PubMed

    Rodríguez Alonso, A; Suárez Pascual, G; Bonelli Martín, C; González Blanco, A; Lorenzo Franco, J; Cuerpo Pérez, M A; Nogueira Carballedo, C; Alvarez Fernández, J C; Nieto García, J

    2004-04-01

    Appendiceal mucocele is a term used to describe the dilatation of the vermiform appendix produced by an intraluminal accumulation of mucus. Four pathological processes have been described that may lead to an appendiceal mucocele: obturation of cecoappendiceal communication, mucosal hyperplasia, mucinous cystadenoma and mucinous cystoadenocarcinoma. The most frequent is mucinous cystadenoma, seen in 50% of appendiceal mucoceles. 6% of patients with appendiceal mucocele develop peritoneal pseudomixoma, possibly through dissemination of the epithelial cells into the abdominal cavity. Preoperative diagnosis of the lesion is particularly important in order to deal with it carefully during surgery. CT scan is the most precise radiological exploration, although there are no pathognomonic signs of mucocele. Typical CT finding of a mucocele is a well-defined cystic mass that compresses the cecum without any peripheral inflammatory reaction, and with low levels of attenuation that vary between water and soft tissue density. We present a case of an appendiceal mucocele caused by a mucinous cystadenoma clinically presented as a giant retroperitoneal mass. Diagnosis was postoperatively made, after pathological study of the surgical sample.

  9. Unusual Severe Complication Following Transarterial Chemoembolization for Metastatic Malignant Melanoma: Giant Intrahepatic Cyst and Fatal Hepatic Failure

    SciTech Connect

    Ataergin, Selmin; Tasar, Mustafa; Solchaga, Luis; Ozet, Ahmet; Arpaci, Fikret

    2009-03-15

    We describe a 45-year-old male patient with malignant melanoma who underwent hepatic arterial chemoembolization due to liver metastases. Four months after the procedure, the patient developed a giant cystic cavity in the liver. Cytologic examination of the cystic fluid retention revealed necrotic tumor material. The fluid was drained by percutaneous catheter, but the patient developed hepatic failure. This case represents another rare complication of transarterial chemoembolization and shows that transarterial chemoembolization may have rare fatal complications.

  10. Giant metastasizing malignant hidradenoma in a child

    PubMed Central

    Bajaj, Sunil K; Misra, Ritu; Gupta, Rohini; Bansal, Anju

    2016-01-01

    An 8-year-old girl presented with a scalp swelling. The swelling was recurrent, reappearing everytime after local excision. She underwent surgery and the histopathologic diagnosis was malignant hidradenoma. This very rare and aggressive tumor is known to occur in elderly population and is histopathologically distinct from its commonly occuring benign counterpart. Malignant hidradenoma is resistant to chemotherapy and radiotherapy. We empahsize that being cognizant of the possibility of this rare tumor would assist in timely action in the form of wide resection, with possible reduction in morbidity and mortality. PMID:27730041

  11. Giant malignant phyllodes tumour of breast.

    PubMed

    Krishnamoorthy, Ramakrishnan; Savasere, Thejas; Prabhuswamy, Vinod Kumar; Babu, Rajashekhara; Shivaswamy, Sadashivaiah

    2014-01-01

    The term phyllodes tumour includes lesions ranging from completely benign tumours to malignant sarcomas. Clinically phyllodes tumours are smooth, rounded, and usually painless multinodular lesions indistinguishable from fibroadenomas. Percentage of phyllodes tumour classified as malignant ranges from 23% to 50%. We report a case of second largest phyllodes tumour in a 35-year-old lady who presented with swelling of right breast since 6 months, initially small in size, that progressed gradually to present size. Examination revealed mass in the right breast measuring 36×32 cms with lobulated firm surface and weighing 10 kgs. Fine needle aspiration cytology was reported as borderline phyllodes; however core biopsy examination showed biphasic neoplasm with malignant stromal component. Simple mastectomy was done and specimen was sent for histopathological examination which confirmed the core biopsy report. Postoperatively the patient received chemotherapy and radiotherapy. The patient is on follow-up for a year and has not shown any evidence of metastasis or recurrence. PMID:25548696

  12. Giant cystic leiomyoma of the uterus occupying the retroperitoneal space.

    PubMed

    Funaki, Kaoru; Fukunishi, Hidenobu; Tsuji, Yoshihiko; Maeda, Tetsuo; Takahashi, Takuya

    2013-12-01

    A 31-year-old nulliparous woman visited our hospital complaining of abdominal distension. Abdominal ultrasonography and computed tomography revealed a 40 × 40 × 30-cm, multilocular cystic mass extending from the upper abdomen to the pelvis. Magnetic resonance imaging (MRI) revealed a cystic tumor that was hypointense on T1-weighted images and was heterogeneously hyperintense on T2-weighted images. The final diagnosis was an 8 kg leiomyoma with cystic degeneration. Uterine leiomyomas are common benign tumors in females of reproductive age. However, subserosal leiomyomas with complete cystic degeneration of the retroperitoneal space are rare, and they are difficult to accurately diagnosis without pathological examination.

  13. Recurrent giant cell fibroblastoma: Malignancy predisposition in Kabuki syndrome revisited.

    PubMed

    Karagianni, Paraskevi; Lambropoulos, Vassilios; Stergidou, Dorothea; Fryssira, Helena; Chatziioannidis, Ilias; Spyridakis, Ioannis

    2016-05-01

    Kabuki syndrome is a genetic condition characterized by distinctive facial phenotype, mental retardation, and internal organ malformations. Mutations of the epigenetic genes KMT2D and KDM6A cause dysregulation of certain developmental genes and account for the multiple congenital anomalies of the syndrome. Eight cases of malignancies have been reported in young patients with Kabuki syndrome although a causative association to the syndrome has not been established. We report a case of a 12-year-old girl with Kabuki syndrome who developed a tumor on the right side of her neck. A relapsing tumor 19 months after initial excision, proved to be giant cell fibroblastoma. Τhis is the first report of giant cell fibroblastoma -a rare tumor of childhood- in a patient with Kabuki syndrome. PMID:26898171

  14. Malignant giant solitary fibrous tumor of the mediastinum.

    PubMed

    De Raet, Jan; Sacré, Robert; Hoorens, Anne; Fletcher, Christopher; Lamote, Jan

    2008-09-01

    Malignant giant solitary fibrous tumor (SFT) of the mediastinum is a rare neoplasm derived from mesenchymal tissue. Owing to its large size, a complete resection of the tumor can present many challenges, particularly given its proximity to vital neighboring structures. We report a successful en-bloc resection of a massive mediastinal SFT, which was compressing the inferior trachea and heart, by means of a median sternotomy and an anterior left thoracotomy. We emphasize the rarity of this uncommon mediastinal mass. Key points of mediastinal SFT are discussed.

  15. Giant cystic primary mucoepidermoid carcinoma of mandible: a rare case and literature review.

    PubMed

    Verma, Roshan Kumar; Sunku, Satheesh Kumar; Bal, Amanjeet; Panda, Naresh K

    2014-01-01

    Primary intra-osseous mucoepidermoid carcinoma arising from jaw is an extremely rare condition accounting to less than 2% of all mucoepidermoid carcinomas. In the jaw, it occurs more commonly in mandible than maxilla. They are low-grade cancers and affect jaw as uni- or multi-locular radiographic lesions. Here we discuss a rare case of giant cystic primary intra-cystic mucoepidermoid carcinoma of the mandible which was excised in toto. Here we discuss the clinical features, radiological and histological characteristics of this rare lesion, and review the literature.

  16. [EUS results of malignant rectal giant condyloma acuminatum (Buschke-Loewenstein tumor)].

    PubMed

    Strock, Paul; Barrioz, Thierry; Lauroy, Johny; Babin, Philippe; Mordi, Abdel; Fort, Eric; Laurin, Christine; Sevestre, Christian; Silvain, Christine

    2004-01-01

    Giant condyloma acuminatum is a large, exophytic, cauliflower-like lesion that usually affects the anogenital region. Localisation in the rectum is uncommon and has a high rate of malignant transformation but does not lead to develop distant metastases. For the time, we report the endosonographic appearance of a malignant intrarectal giant condyloma acuminatum.

  17. Histological and clinical characteristics of malignant giant cell tumor of bone.

    PubMed

    Gong, Lihua; Liu, Weifeng; Sun, Xiaoqi; Sajdik, Constantin; Tian, Xinxia; Niu, Xiaohui; Huang, Xiaoyuan

    2012-03-01

    Malignant giant cell tumors of bone (MGCTB) are rare, and the diagnosis can be difficult due to the occurrence of a variety of malignant tumors containing giant cells. To better understand its clinicopathological features, we have reviewed our experience with 17 cases of MGCTB. Five cases were primary malignant giant cell tumor of bone (PMGCTB), and 12 cases were giant cell tumors of bone initially diagnosed as benign but malignant in a recurrent lesion (secondary MGCTB, SMGCTB). The patients included six women and 11 men (age ranged from 17 to 52 years; mean, 30.5 years). The tumor arose in the femur (six cases), the tibia (seven cases), the humerus (three cases), and the fibula (one case). Microscopically, PMGCTB showed both conventional giant cell tumor and malignant sarcoma features. SMGCTB were initially diagnosed as conventional giant cell tumor of bone, the recurrent lesion showing malignant features. Histologically, the malignant components included osteosarcoma (11 cases), undifferentiated high-grade pleomorphic sarcoma (two cases), and fibrosarcoma (four cases). SMGCTB cases showed strong expression of p53. Follow-up information revealed that four patients died of lung metastasis, two patients are alive with lung metastases, and 11 patients are alive without tumor. MGCTB should be considered as a high-grade sarcoma. It must be distinguished from GCTB and other malignant tumors containing giant cells. p53 might play a role in the malignant transformation of GCTB. PMID:22350004

  18. Giant cystic lymphangioma originating from the cardia of the stomach: A case report

    PubMed Central

    CHEN, GENG; LIU, MINGQING; MALIK, TAYYAB HAMID; LI, SHOUYING; TANG, YING; XU, HONG

    2016-01-01

    Cystic lymphangiomas are rare benign tumors involving the neck, head, and axilla, and most frequently occur in children before the age of 5 years. In the present study, the case of a giant cystic lymphangioma originating in the cardia of the stomach was reported in an 18-year-old female complaining of abdominal distention. Contrast-enhanced computed tomography and endoscopic ultrasonography revealed a large, multilobulated cystic mass located between the cardia and esophagogastric junction with a diameter of 4.0 cm. The lesion was successfully removed by endoscopic submucosal dissection. Subsequent immunohistochemical analysis of the lymphatic endothelium-specific O-linked sialoglycoprotein D2-40 confirmed the diagnosis of cystic lymphangioma. No complications associated with the tumor dissection occurred, and the patient did not report any further complaints or any signs of recurrence at 6- and 18-month follow-up. The present case demonstrates that a diagnosis of cystic lymphangioma should be considered in non-pediatric patients suffering aspecific abdominal complaints. PMID:27168832

  19. Acute Cholecystitis Caused by Malignant Cystic Duct Obstruction: Treatment with Metallic Stent Placement

    SciTech Connect

    Miyayama, Shiro Yamashiro, Masashi; Takeda, Taro; Aburano, Hiroyuki; Komatsu, Tetsuya; Sanada, Taku; Kosaka, Shotaro; Toya, Daisyu; Matsui, Osamu

    2008-07-15

    We report the successful management of acute cholecystitis using cystic duct stent placement in 3 patients with inoperable malignant cystic duct obstruction (2 cholangiocarcinoma and 1 pancreatic carcinoma). All patients underwent stent placement in the bile duct, using an uncovered stent in 2 and a covered stent in 1, to relieve jaundice occurring 8-184 days (mean 120 days) before the development of acute cholecystitis. The occluded cystic duct was traversed by a microcatheter and a stent was implanted 4-17 days (mean 12 days) after cholecystostomy. Acute cholecystitis was improved after the procedure in all patients. Two patients died 3 and 10 months later, while 1 has survived without cholecystitis for 22 months after the procedure to date.

  20. Giant mucinous cystic adenoma with pancreatic atrophy mimicking dorsal agenesis of the pancreas

    PubMed Central

    Gagnière, Johan; Dupré, Aurélien; Ines, David Da; Tixier, Lucie; Pezet, Denis; Buc, Emmanuel

    2014-01-01

    Mucinous cystic adenoma (MCA) of the pancreas is a rare benign cystic tumor with ovarian-like stroma and lack of communication with the pancreatic ductal system. The ovarian tissue is incorporated from the left gonad within the dorsal pancreas during embryogenesis. Consequently, congenital dorsal agenesis of the pancreas (DAP) cannot be associated with MCA. We report the case of a giant MCA associated with atrophy of the dorsal pancreas mimicking complete DAP. Pancreato-magnetic resonance imaging failed to identify the dorsal pancreas but the absence of diabetes mellitus and compression of the splenic vein with major tributaries rectified the diagnosis of secondary atrophy of the distal pancreas. Unusual proximal location of the cyst in the pancreas may have induced chronic obstruction of both the dorsal pancreatic duct and the splenic vein, with secondary atrophy of the distal pancreas. PMID:24672649

  1. The Malignant Transformation of Retrorectal Cystic Hamartomas With Blood Irregular Antibodies Positive: A Case Report.

    PubMed

    Zhao, Xiang-Rong; Gao, Chao; Zhang, Yong; Yu, Yong-Hua

    2015-12-01

    Retrorectal cystic hamartomas are rare congenital presacral lesions and malignancy is extremely rare. Although surgical excision is the essential for treatment, a unique feature of our case compared with previously reported tailgut cysts is that this patient's blood irregular antibodies are positive with higher operational risks.A 44-year-old woman presented to our department complaining of pelvic and perineal pain for 6 months. Computed tomography (CT) scan of the abdomen and pelvis demonstrated a well-demarcated hypodense, multilocular cystic lesion, 10 cm in size, in the presacral region of the right of the midline. We found her blood irregular antibodies were positive in the preoperative examination. So she quitted surgery. Exploratory laparotomy and incision and drainage of pelvic tumor were operated. Postoperative routine pathology showed: (retroperitoneal tumors) moderately differentiated adenocarcinoma. Combined with clinical symptom and imaging, malignant transformation of retrorectal cystic hamartomas (tailgut cysts) was diagnosed. Taking into account that cyst is not sensitive to radiotherapy, so tumor necrosis factor (TNF) and raltitrexed were infused into the cysts and 3 cycles oxaliplatin (130 mg/m) were completed. Now although the lesion is shrink, but yellow, viscous mucus still secrete constantly, 100 ml/w.Given surgical excision is the essential for treatment, complete surgical excision should be implemented as far as possible. But if surgery cannot be carried out like the presented case, systemic chemotherapy and local radiotherapy are also available, which can alleviate the symptoms of oppression and improve the quality of life partly.

  2. Malignant fibrous histiocytoma (giant cell type) associated with a malignant mixed tumor in the salivary gland of a dog.

    PubMed

    Pérez-Martínez, C; García Fernández, R A; Reyes Avila, L E; Pérez-Pérez, V; González, N; García-Iglesias, M J

    2000-07-01

    A 12-year-old male Boxer dog presented with a 5 x 5 x 7-cm partially encapsulated mass in the right mandibular salivary gland. Histologically, the mass was composed of neoplastic epithelial and mesenchymal cells. The mesenchymal component consisted of two cell populations arranged in different patterns: coalescing nodules of neoplastic mononuclear cells with rare osteoid and numerous osteoclastlike giant cells; and sheets of neoplastic spindle cells intermingled with neoplastic epithelial cells and containing osteoid and well-formed bone trabeculae lined by osteoblasts and few osteoclastlike giant cells. On the basis of these histological features, two malignant salivary tumors were diagnosed: a malignant fibrous histiocytoma (giant cell type) and a malignant mixed tumor. Immunohistochemical studies demonstrated keratin 5 and 8 expression by the neoplastic epithelial cells, indicating a probable salivary ductal origin, and vimentin expression by all mesenchymal elements, suggesting a fibroblastic line of differentiation.

  3. Secondary malignant giant-cell tumor of bone. Clinicopathological assessment of nineteen patients

    SciTech Connect

    Rock, M.G.; Sim, F.H.; Unni, K.K.; Witrak, G.A.; Frassica, F.J.; Schray, M.F.; Beabout, J.W.; Dahlin, D.C.

    1986-09-01

    Twenty-six patients who had a malignant giant-cell tumor of bone--a sarcoma either juxtaposed to a zone of typical benign giant-cell tumor or occurring at the site of a previously documented benign giant-cell tumor--have been seen at the Mayo Clinic. Of the twenty-six tumors, nineteen were secondary to a previous attempt at local control of a benign giant-cell tumor. All but one of these nineteen patients with a secondary tumor had received therapeutic irradiation four to thirty-nine years earlier. The nature and duration of the symptoms and the sites of predilection of the malignant giant-cell tumors were the same as for benign giant-cell tumor. Fibrosarcoma occurred three times as frequently as osteosarcoma. The best results of treatment of the secondary sarcoma were obtained with early ablation.

  4. Giant cell rich osteosarcoma of the mandible with abundant spindle cells and osteoclast-like giant cells mimicking malignancy in giant cell tumor

    PubMed Central

    Sun, Li-Mei; Zhang, Qing-Fu; Tang, Na; Mi, Xiao-Yi; Qiu, Xue-Shan

    2015-01-01

    Giant cell rich osteosarcoma is a relatively unusual histological form of osteosarcoma, common lesion usually presenting in the long bones of the appendicular skeleton. The occurrence in the mandible is exceptional rare. Histologically, this tumor tends to be a highly anaplastic, pleomorphic tumor in which the tumor cells may be: plasmacytoid, fusiform, ovoid, small round cells, clear cells, mono-or multinucleated giant cells, or, spindle cells. Herein, we present a case with the sternum and first thoracic vertebra metastasis from primary giant cell rich osteosarcoma of the mandible in a 28 year-old Chinese female. The tumor was predominantly composed of abundant spindle cells with marked atypia and numerous osteoclast-like giant cells reminiscent of malignancy in giant cell tumor. The unusual histological appearance can pose a great diagnostic challenge. It may be easily misdiagnosed, especially if the specimen is limited or from fine-needle aspiration. PMID:26464744

  5. Ovarian mucinous cystic tumor of borderline malignancy with a mural nodule of anaplastic spindle cell carcinoma: a case report.

    PubMed

    Yamazaki, Hitoshi; Matsuzawa, Akiyo; Shoda, Takashi; Iguchi, Hiroyoshi; Kyushima, Noriyuki

    2013-12-05

    Ovarian cystic tumors with a mural nodule are a rare entity. We report a case of a mural nodule of anaplastic spindle cell carcinoma in an ovarian mucinous cystic tumor of borderline malignancy. The patient was a 45-years-old Japanese woman who presented with an ovarian cyst. She suffered from mature cystic teratoma of both ovaries 9 years before the present history. Image analysis and laboratory data showing a high serum CA19-9 level suggested ovarian malignancy. She underwent bilateral salpingo-oophorectomy with hysterectomy and omentectomy. There was a mural nodule in the ovarian mucinous cystic lesion. Microscopically, the nodule was composed of spindle-shaped cells with severe nuclear atypia. Immunohistochemical analysis allowed the cells to be categorized as anaplastic spindle cell carcinoma. Fifteen months after the operation the patient is alive without any clinical findings of tumor recurrence. To the best of our knowledge in the English literature, this is the first report of a mural nodule of an anaplastic spindle cell carcinoma within an ovarian mucinous cystic borderline tumor harboring previously confirmed cystic teratoma.

  6. Malignant giant pheochromocytoma: a case report and review of the literature

    PubMed Central

    Arcos, Cristina Torres; Luque, Virgilio Ruiz; Luque, José Aguilar; García, Pablo Martínez; Jiménez, Antonia Brox; Muñoz, Macarena Márquez

    2009-01-01

    Malignant pheochromocytoma is a rare disease and surgical resection is the only curative treatment. There are no definitive histological or cytological criteria of malignancy, as it is impossible to determine this condition in the absence of advanced locoregional disease or metastases. We report a case of a patient with a giant retroperitoneal tumour, the second largest to be published, which was diagnosed as a malignant pheochromocytoma; it was treated with surgery. The literature is reviewed to evaluate tumour features and criteria to distinguish between benign and malignant pheochromocytomas. PMID:20019963

  7. Giant cystic meconium peritonitis presenting in a neonate with classic radiographic eggshell calcifications and treated with an elective surgical approach: a case report

    PubMed Central

    2012-01-01

    Introduction Giant cystic meconium peritonitis is relatively rare. Patients often present with nonspecific physical findings such as distension and emesis. Plain abdominal films remain invaluable for identifying the characteristic calcifications seen with a meconium pseudocyst, and large eggshell calcifications are pathognomonic for the giant cystic subtype. Case presentation We present classic plain X-ray findings and an intraoperative image of a premature low birth weight two-day-old Hispanic male baby treated for giant cystic meconium peritonitis with a staged procedure involving peritoneal drainage, ostomy creation and closure. Conclusion Pediatric surgeons have a range of potential therapeutic approaches for giant cystic meconium peritonitis. A delay of definitive surgical management in the setting of massive abdominal soiling is a safe and acceptable strategy if adequate temporizing drainage is performed in the early perinatal period. PMID:22857611

  8. Radical resection of a giant, invasive and symptomatic malignant Solitary Fibrous Tumour (SFT) of the pleura.

    PubMed

    Filosso, Pier Luigi; Asioli, Sofia; Ruffini, Enrico; Rovea, Paolo; Macri', Luigia; Sapino, Anna; Bretti, Sergio; Lyberis, Paraskevas; Oliaro, Alberto

    2009-04-01

    Solitary Fibrous Tumours (SFTs) of the pleura are rare neoplasms, with unpredictable biological behaviour. Although usually benign, malignant SFTs are described, and they are often associated with large, necrotic and locally invasive tumours. Radical resection represents the treatment of choice in all cases; recurrences are uncommon, and redo-surgery should be considered. The case of a giant, invasive, radically resected malignant SFT, is described. The role of postoperative radiotherapy, to reduce the risk of recurrence, is also discussed.

  9. Pancreatic Candidiasis That Mimics a Malignant Pancreatic Cystic Tumor on Magnetic Resonance Imaging: A Case Report in an Immunocompetent Patient.

    PubMed

    Seong, Minjung; Kang, Tae Wook; Ha, Sang Yun

    2015-01-01

    Candida is a commensal organism that is frequently found in the human gastrointestinal tract. It is the most common organism that causes pancreatic fungal infections. However, magnetic resonance imaging findings of Candida infection in the pancreas have not been described. We report imaging findings of pancreatic candidiasis in a patient in immunocompetent condition. It presented as a multi-septated cystic mass with a peripheral solid component in the background of pancreatitis and restricted diffusion on diffusion-weighted image that mimicked a malignant pancreatic cystic tumor.

  10. Giant cranionasal and cystic-solid craniopharyngioma associated with extensive bone erosion and ossification.

    PubMed

    Chen, Haifeng; Zhou, Liangxue; Luo, Linli; Jiang, Su; Guo, Gang; You, Chao

    2013-07-01

    Craniopharyngioma (CP), a rare benign and slow-growing epithelial tumor, is mainly located within the sellar/parasellar region. Primary CP involving the nasal cavity and the sellar region with extensive erosion of the skull base and ossification simultaneity has not been described previously. The authors report a 23-year-old man who presented to our institute with complaints of repeated nasal cavity bloodshed, liquid flow, and progressive visual loss. A neuroimaging examination showed a giant cranionasal and cystic-solid CP extending from the suprasellar region to the nasopharynx with inhomogeneous enhancement, which is associated with extensive erosion of the skull base and ossification. The patient underwent a transsphenoidal surgery to resect the nasopharyngeal component of CP and a subfrontal craniotomy with a total removal of intracranial component by grinding 3 months later. A histopathologic examination revealed characteristic features of adamantinomatous CP associated with ossification. The current study demonstrates that CP can exhibit cranionasal growth pattern and arise from residue of craniopharyngeal duct. Extensive erosion of the skull base, calcification, and ossification can present in tumor simultaneity. A 2-stage stratagem is important for its total removal because of the peculiar hardness. Postsurgical course is unevenly and should be dealt with carefully. PMID:23851734

  11. Successful Preoperative Chemoembolization in the Treatment of a Giant Malignant Phyllodes Tumor.

    PubMed

    Hashimoto, Kazuki; Mimura, Hidefumi; Arai, Yasunori; Doi, Masatomo; Kojima, Yasuyuki; Tsugawa, Koichiro; Nakajima, Yasuo

    2016-07-01

    The malignant phyllodes tumor is a relatively rare neoplasm and has not previously been a therapeutic target of interventional radiology. Herein, we report a successful case of preoperative chemoembolization of a giant malignant phyllodes tumor. The objective was to achieve sufficient tumor shrinkage before surgery to avoid the requirement for skin grafting after resection. Intra-arterial epirubicin infusion and subsequent embolization with Embosphere Microspheres (BioSphere Medical, Rockland, MA, USA) was undertaken three times over the course of 6 weeks and was well tolerated. The patient underwent surgery without skin grafting. Neither local recurrence nor distant metastasis was observed at 6 months after surgery. PMID:26916453

  12. Multiple malignant salivary gland neoplasms: mucoepidermoid carcinoma of palate and adenoid cystic carcinoma of floor of mouth.

    PubMed

    Whitt, Joseph C; Schafer, Duane R; Callihan, Michael D

    2008-03-01

    Salivary gland tumors usually occur as single lesions. To have more than one tumor is unusual. We report a case of an adult male who presented with a mucoepidermoid carcinoma involving the minor salivary glands of the palate at age 57 years, followed by an adenoid cystic carcinoma of the floor of mouth at age 63 years. The patient later succumbed to non-Hodgkin lymphoma at age 72 years. There are 31 acceptable cases of multiple malignant salivary gland neoplasms reported in the world literature. Multiple malignant tumors of the same histologic type are more common than those of different histologic type. Bilateral acinic cell adenocarcinoma was the most frequent combination of multiple salivary gland malignancy, accounting for 14 cases (10 synchronous and four metachronous). All involved the parotid glands bilaterally with the exception of one case that involved parotid and submandibular gland. Polymorphous low-grade adenocarcinoma accounted for three of the four cases of multiple malignant tumors involving minor salivary glands. Individuals with a history of malignancy are at risk for the development of additional malignant tumors and should receive appropriate clinical follow-up. PMID:20614341

  13. Malignant giant cell tumor in the carpal tunnel: a case report and review of literature.

    PubMed

    Theunissen, Carla I J M; Bras, Johannes; Lienden, Krijn P van; Obdeijn, Miryam C

    2013-08-01

    Background Malignant tenosynovial giant cell tumors (GCTs) are extremely rare, and their etiology is unknown. However, this type of malignancy is associated with high metastasis and mortality rates. Therefore, the treatment of choice is wide excision. Case Description A 66-year-old man complained of tingling and loss of sensation in the left hand, caused by a tumor that compressed the median nerve. The tumor was excised. Histopathologic examination revealed a ganglion cyst. Two years later, the patient visited our clinic with recurrent and similar complaints of the left hand. This time, however, the lesion turned out to be a malignant tenosynovial GCT and was treated by amputation of the forearm. Literature Review Since 1979, only 37 malignant tenosynovial GCTs have been reported in literature. Follow-up of these patients showed that 11 patients died of the disease, 4 patients were still living with the disease, and 14 patients had no evidence of disease after treatment. The other seven patients were lost to follow-up, and one patient died of other causes. In these 37 patients, a high incidence of lymph node metastasis (41%) and a high mortality rate (30%) were seen. Clinical Relevance Although this malignant tenosynovial GCT is very rare, high mortality rates have been observed because of the high incidence of lymph node metastases. Therefore, more awareness has to be created, to recognize and treat this tumor timely. PMID:24436827

  14. Giant malignant phyllodes tumor of the breast: A rare case report and literature review

    PubMed Central

    LIU, MIN; YANG, SHUO; LIU, BIN; GUO, LIANG; BAO, XUEYING; LIU, BAILONG; DONG, LIHUA

    2016-01-01

    Malignant phyllodes tumor of the breast (MPTB) is rarely encountered in clinical practice. Preoperative diagnosis is challenging due to nonspecific radiological and histological features, and the prognostic factors and optimal treatment remain controversial. The current report describes the case of a middle-aged female with giant MPTB who underwent multidisciplinary intervention, including surgery, postoperative chemotherapy and radiotherapy. To date, the disease-free survival (DFS) of the patient has reached 18 months. Furthermore, a related literature review summarize the clinicopathological characteristics and treatment progress regarding MPTB is presented, along with an analysis of the indications for therapeutic strategy in the current case. In the future, multi-center clinical trials must be initiated to identify the criteria for diagnosis and optimal treatment consensus for MPTB. In conclusion, the present case highlights that multidisciplinary management may contribute to DFS following the treatment of giant MPTB. PMID:27347111

  15. Synchronous clear cell renal cell carcinoma and multilocular cystic renal cell neoplasia of low malignant potential: A clinico-pathologic and molecular study.

    PubMed

    Raspollini, Maria Rosaria; Castiglione, Francesca; Cheng, Liang; Montironi, Rodolfo; Lopez-Beltran, Antonio

    2016-05-01

    We report a rare case of synchronous clear cell renal cell carcinoma and multilocular cystic renal cell neoplasia of low malignant potential in the same kidney. The tumors were seen incidentally in a 45-year-old man. Pathologic study revealed that the former tumor was nucleolar grade 2, and the multilocular cystic renal cell neoplasia of low malignant potential was nucleolar grade 1. At immunohistochemistry, the clear cells in both tumors were positive for CD10 and CA IX. Interestingly, these uncommon synchronous tumors showed a different KRAS/NRAS mutation analysis that was characterized by KRAS mutation at codon p.G12C in the clear cell renal cell carcinoma, while this mutation was not present in the case of multilocular cystic renal cell neoplasia of low malignant potential. NRAS mutation was not seen in any of the tumors. PMID:26874573

  16. Giant mucinous cystic neoplasms of pancreas and liver with unusual adipose tissue component: a case report.

    PubMed

    Ghatak, Supriyo; Ray, Sukanta; Sonar, Pankaj Kr; Das, Somak; Basu, Keya; Mridha, Asit Ranjan; Bhattacharyya, Avik; Sarkar, Rajib

    2012-12-01

    Simultaneous occurrence of pancreatic and hepatic mucinous cystic neoplasms is very rarely reported in the literature. We present a case with extensive fatty component of the pancreatic tumour arising from the head of the pancreas and attaining a huge size before being treated by Whipple's pancreatoduodenectomy and subsequently by a right hepatectomy for the hepatic tumour.

  17. Mutations of the KIT gene and loss of heterozygosity of the PTEN region in a primary malignant melanoma arising from a mature cystic teratoma of the ovary.

    PubMed

    Tate, Genshu; Tajiri, Takuma; Suzuki, Takao; Mitsuya, Toshiyuki

    2009-04-01

    A tumor suppressor gene at 10q23.3, designated PTEN, encoding a dual-specificity phosphatase with lipid and protein phosphatase activity, has been shown to play a pivotal role in the pathogenesis of a variety of human cancers. A frequent loss of heterozygosity (LOH) at 10q is found in melanoma; however, little is known about the role of PTEN in the pathogenesis of a primary malignant melanoma derived from ovarian mature cystic teratoma, which is an extremely rare melanoma. This study examined the genetic alterations involved in the mitogen-activated protein kinase and phosphatidylinositol 3 kinase pathways in an ovarian malignant melanoma. A LOH analysis revealed hemizygous deletion around and in the PTEN gene not only in the ovarian melanoma but also in a mature cystic teratoma. Another case of ovarian mature cystic teratomas in the absence of melanoma also showed allelic loss of the PTEN region. To date, mutations of BRAF, NRAS, and KIT genes have been reported in malignant melanomas. In the present study, D816H and K558E mutations of the KIT gene were revealed in the melanoma arising from a mature cystic teratoma, but not in a mature cystic teratoma. No mutations of the BRAF and NRAS genes were found in the melanoma. These results indicate that LOH of the PTEN region is one of the molecular alterations of an ovarian mature cystic teratoma and a KIT mutation is an additional promotional event associated with the oncogenesis of a melanoma arising from an ovarian mature cystic teratoma.

  18. Multiple malignant cylindromas of skin in association with basal cell adenocarcinoma with adenoid cystic features of minor salivary gland.

    PubMed

    Antonescu, C R; Terzakis, J A

    1997-08-01

    This unusual case is that of a middle-aged man exhibiting a tumor diathesis including a basal cell adenocarcinoma with features of adenoid cystic carcinoma arising in minor salivary gland of lip in association with multiple primary malignant cylindromas of skin. The labial lesion showed invasive tubules, solid epithelial sheets and cribriform structures. It did not exhibit PAS positive juxta-tubular basement membrane material. The skin lesions all showed features of a highly infiltrative cylindromatous carcinoma with two cell types, peripheral palisading and prominent PAS positive juxta-tubular basement membrane material. Immunocytochemical studies of the lip lesion and one of the skin lesions showed similarities, including positive staining for high and low molecular weight keratins and S-100 with negative staining for CEA. The precious descriptions of tumor diatheses involving dermal cylindromas and dermal analogue tumors of salivary glands and the distinctions with the present study are noted. If benign and even malignant cylindromas were described in the literature to be associated with basal cell adenocarcinoma of the major salivary glands, our case is unique by its association with this rare malignant tumor in a minor salivary gland.

  19. Reversal of severe cognitive impairment following medical treatment of cystic invasive giant prolactinoma

    PubMed Central

    Lois, K; Mathiopoulou, M; Grossman, A B; James, R A

    2016-01-01

    Summary Giant prolactinomas are rare tumours of the pituitary, which typically exceed 40 mm in their largest dimension. Impairment of higher cognitive function has been noted post-operatively after transcranial surgery and as a long-term consequence of the radiotherapy treatment. However, there has been little that is reported on such disturbances in relation to the tumour per se, and to our knowledge, there has been none in terms of responsivity to dopamine agonist therapy and shrinkage in these tumours. We present a case of successful restoration of severely impaired cognitive functions achieved safely after significant adenoma involution with medical treatment alone. Learning points Giant prolactinomas can be present with profound cognitive defects.Dopamine agonists remain in the mainstay first-line treatment of giant prolactinomas.Mechanisms of the reversible cognitive impairment associated with giant prolactinoma treatment appear to be complex and remain open to further studies.Young patients with giant prolactinomas mandate genetic testing towards familial predisposition. PMID:26904198

  20. The use of giant cell tumor conditioned media in cytogenetic studies of hematologic malignancies.

    PubMed

    Wason, D; Richkind, K E

    1992-07-15

    The use of conditioned media produced from solid tumor cell lines has been beneficial in the study of hematologic malignancies. Conditioned media from giant cell tumors (GCT), human lung adenocarcinoma, and human bladder carcinoma express growth factors that have been used to stimulate growth of bone marrow cells and improve the quality of the preparations. It has been reported that addition of Lu-CSF1-conditioned media from a lung adenocarcinoma cell line masks abnormalities in cases of acute leukemia [1.] Because we routinely use GCT-CM in bone marrow and leukemic blood cultures for chromosome analysis in our lab, we investigated this potential effect on our case analysis. We have performed a serial study of a 100 cases of hematologic malignancies received for analysis in our lab to determine the effect of the addition of GCT-CM to our culture media with respect to 1) mitotic index, 2) quality of preparation, and 3) differential selection of either chromosomally normal or abnormal cell lines. Our results indicate that the mitotic index and quality of metaphases is enhanced with the addition of GCT media and that there is no difference in the rate of abnormality detection with or without the addition of GCT media.

  1. Microsurgical management of giant malignant peripheral nerve sheath tumor of the scalp: two case reports and a literature review.

    PubMed

    Wang, Jun; Ou, Shao-wu; Guo, Zong-ze; Wang, Yun-jie; Xing, De-guang

    2013-10-10

    Malignant peripheral nerve sheath tumors of the scalp are rare lesions of the nervous system. Only 14 cases have been reported to date. The field of neurosurgery has struggled with diagnosing and treating these tumors. In this report, we present two cases of giant malignant peripheral nerve sheath tumors of the scalp and retrospectively analyze the clinical features, imaging findings, pathological features, and prognoses of these two patients. Each underwent microsurgery and radiotherapy. In addition, based on a literature review, we discuss the diagnostic and therapeutic strategies used to treat these unusual lesions.

  2. Malignant transformation in non-recurrent peritoneal cystic mesothelioma Our experience and review of the literature.

    PubMed

    Santangelo, Giuseppe; Accardo, Marina; De Vita, Ferdinando; Del Giudice, Santolo; Gallucci, Federica; Fabozzi, Alessio; De Falco, Massimo

    2016-01-29

    Il mesotelioma peritoneale cistico è un tumore raro. Si tratta di un tumore benigno, che tuttavia va incontro a recidive locali post-chirurgiche in oltre il 50% dei casi. È proprio a seguito di recidive ricorrenti che si possono determinare trasformazioni maligne di questa neoplasia, come descritto in Letteratura. Nel Giugno del 2004 un paziente di 73 anni è giunto presso la nostra struttura con una tumefazione addominale causa di dolore gravativo: si trattava di una massa di 16 cm, la cui struttura, agli esami strumentali (ecografia, TC con m.d.c. e RMN), appariva pluricistica. Attraverso una laparotomia mediana si è provveduto alla sua rimozione e il paziente è stato dimesso in VIII giornata post-operatoria senza complicanze. L’esame istologico, corredato da studio immunoistochimico e di microscopia elettronica, ha consentito di porre diagnosi di mesotelioma peritoneale pluricistico in trasformazione maligna. D’accordo con gli oncologi, in considerazione della scarsa efficacia dei trattamenti adiuvanti e della scarsità dei dati riportati in Letteratura sull’argomento, si è deciso di sottoporre il paziente unicamente ad uno stretto follow-up, con esecuzione di ecografia addominale ogni sei mesi e TC addominale ogni anno. Ad oggi, dieci anni dopo il trattamento, il paziente, di 83 anni, gode di un discreto stato di salute e non si sono verificate riprese o recidive di malattia, né locali, né a distanza. Oltre al fatto di essere risultato maligno alla sua prima manifestazione e non dopo ripetute recidive, il caso presenta altre particolarità: il mesotelioma peritoneale cistico, infatti, solitamente colpisce soggetti di sesso femminile (con un rapporto F:M di 4,5:1) ad un’età media di 60 anni, mentre il caso descritto riguarda un soggetto di sesso maschile di 73 anni. La nostra osservazione dimostra la possibilità che il mesotelioma peritoneale vada incontro a trasformazioni maligne già alla sua prima manifestazione e non esclusivamente dopo

  3. miR-24-3p Suppresses Malignant Behavior of Lacrimal Adenoid Cystic Carcinoma by Targeting PRKCH to Regulate p53/p21 Pathway

    PubMed Central

    Zhang, Hong; Tang, Hua

    2016-01-01

    MicroRNA (miRNA) may function as an oncogene or a tumor suppressor in tumorigenesis. However, the mechanism of miRNAs in adenoid cystic carcinoma (ACC) is unclear. Here, we provide evidence that miR-24-3p was downreglated and functions as a tumor suppressor in human lacrimal adenoid cystic carcinoma by suppressing proliferation and migration/invasion while promoting apoptosis. miR-24-3p down-regulated protein kinase C eta (PRKCH) by binding to its untranslated region (3’UTR). PRKCH increased the of the cell growth and migration/invasion in ACC cells and suppressed the expression of p53 and p21 in both mRNA and protein level. The overexpression of miR-24-3p decreased its malignant phenotype. Ectopic expression of PRKCH counteracted the suppression of malignancy induced by miR-24-3p, as well as ectopic expression of miR-24-3p rescued the suppression of PRKCH in the p53/p21 pathway. These results suggest that miR-24-3p promotes the p53/p21 pathway by down-regulating PRKCH expression in lacrimal adenoid cystic carcinoma cells. PMID:27351203

  4. Mediastinal epithelioid hemangioendothelioma with abundant spindle cells and osteoclast-like giant cells mimicking malignant fibrous histiocytoma

    PubMed Central

    2013-01-01

    Abstract Epithelioid hemangioendothelioma is a relatively uncommon lesion usually presenting in soft tissues. The occurrence in the mediastinum is exceptional rare. Histologically, this tumor is characterized by epithelioid cells with intracytoplasmic vacuoles in a hyalinized or mucinous stroma. Occasionally, spindle cells or osteoclast-like giant cells can be observed. Herein, we present a case of epithelioid hemangioendothelioma in a 38 year-old Chinese male. The tumor was predominantly composed of abundant spindle cells with marked atypia and scattered osteoclast-like giant cells reminiscent of malignant fibrous histiocytoma. The unusual histological appearance can pose a great diagnostic challenge. It may be easily misdiagnosed, especially if the specimen is limited or from fine-needle aspiration. Virtual slides http://www.diagnosticpathology.diagnomx.eu/vs/5804918529726307 PMID:23800015

  5. [Giant condyloma of the penis with malignant transformation associated with lichen sclerosus et atrophicus].

    PubMed

    Wessendorf, U; Bruch-Gerharz, D; Braunstein, S; Braunstein, R; Schulte, K-W; Reifenberger, J

    2009-10-01

    A 72-year old patient presented with a 6 months history of a rapidly growing tumor of the glans and foreskin. He had a long history of phimosis with lichen sclerosus et atrophicus-like lesions on the foreskin which had not been treated. The rest of the personal, family and sexual history was unremarkable. Treatment consists of circumcision and tumor excision. Histopathology confirmed a squamous cell carcinoma within a giant condyloma with a concomitant lichen sclerosus et atrophicus. CT- and ultrasound scans showed no metastases. Giant condylomas are a rare sexually transmitted disease usually caused by human papilloma virus subtypes 6, 11, but also by 16 and 18 among others. They are expansive, cauliflower-like destructive lesions that most frequently affect the anogenital region. In about 30 percent a giant condyloma progresses into a squamous cell carcinoma. Therapy of choice is the histopathologically controlled excision. Recurrences are often seen, so the patients should be monitored frequently after therapy. PMID:19756435

  6. 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 ...

  7. Giant tubular adenoma with malignancy clinical characteristics in a female teenager

    PubMed Central

    Mărginean, Cristina Oana; Mărginean, Maria Oana; Simu, Iunius; Horvath, Adrienne; Meliţ, Lorena Elena

    2016-01-01

    Abstract Background: Adenomas of the colon are usually benign tumors which carry a tendency for malignancy. These tumors can be villous, tubular, tubulovillous, or sessile serrated. Those with adenomatous structure can develop malignant characteristics in 1.5% to 9.4% of cases. Methods: We present a case report of a 16-year-old female adolescent with an adenoma of the descending colon. History revealed prolonged diarrheic syndrome for the past 6 months, repeated headache, and a weight loss of ∼5 kg in the past month. One week before the admission, the patient presented an episode of inferior digestive hemorrhage. Results: On admission laboratory tests revealed iron deficiency anemia, and a mildly increased erythrocyte sedimentation rate. The abdominal ultrasound revealed an inhomogeneous mass of the descending colon and 2 hyperechoic lesions in the liver. The colonoscopy showed a tumor of the descending colon, a tubular adenoma according to the pathological examination. Additionally, we noted an atypical presentation of the tumor and the signs of mild dysplasia identified at the pathological examination. Conclusion: Weight loss, bowel transit alterations, loss of appetite, and inferior hemorrhage in an adolescent can be symptoms of a benign or malignant tumor of the colon. PMID:27749532

  8. Complex histopathological and surgical aspects in a case of giant malignant gastric perforation.

    PubMed

    Serban, D; Branescu, C; Savlovschi, C; El-Khatib, A; Tudor, C; Nica, A; Kraft, A; Dascalu, A M

    2016-01-01

    We present the case of a 52-year-old male patient, hospitalized on an emergency basis in the University Emergency Hospital in Bucharest, after being diagnosed with pneumoperitoneum acute abdomen, for which emergency surgery was mandatory. A 3,5-4 cm malignant gastric perforation, ascitis and peritoneal carcinomatosis were found. The histopathological exam revealed infiltrative mucinous gastric carcinoma with epiploic metastasis. Due to the lack of available gastric material, an atypical surgical solution was performed: gastric packing with epiploic material by means of transgastric traction. The solution proved to be successful for short-term recovery. The underlying condition was not focused on, the patient being directed to the Oncology Department. Acute gastric perforation is a rare complication of gastric cancer, and the association with gastric linitis is uncommon. This specific histopathological condition made the classical surgical repair techniques unsuitable for the presented case and an atypical solution had to be performed. PMID:27453758

  9. Complex histopathological and surgical aspects in a case of giant malignant gastric perforation

    PubMed Central

    Serban, D; Branescu, C; Savlovschi, C; El-Khatib, A; Tudor, C; Nica, A; Kraft, A; Dascalu, AM

    2016-01-01

    We present the case of a 52-year-old male patient, hospitalized on an emergency basis in the University Emergency Hospital in Bucharest, after being diagnosed with pneumoperitoneum acute abdomen, for which emergency surgery was mandatory. A 3,5-4 cm malignant gastric perforation, ascitis and peritoneal carcinomatosis were found. The histopathological exam revealed infiltrative mucinous gastric carcinoma with epiploic metastasis. Due to the lack of available gastric material, an atypical surgical solution was performed: gastric packing with epiploic material by means of transgastric traction. The solution proved to be successful for short-term recovery. The underlying condition was not focused on, the patient being directed to the Oncology Department. Acute gastric perforation is a rare complication of gastric cancer, and the association with gastric linitis is uncommon. This specific histopathological condition made the classical surgical repair techniques unsuitable for the presented case and an atypical solution had to be performed. PMID:27453758

  10. 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

  11. The promoter of the human cystic fibrosis transmembrane conductance regulator gene directing SV40 T antigen expression induces malignant proliferation of ependymal cells in transgenic mice.

    PubMed

    Perraud, F; Yoshimura, K; Louis, B; Dalemans, W; Ali-Hadji, D; Schultz, H; Claudepierre, M C; Chartier, C; Danel, C; Bellocq, J P

    1992-05-01

    Transgenic mice bearing a human cystic fibrosis transmembrane conductance regulator (CFTR) promoter-SV40 T antigen fusion transgene were generated in order to localize in vivo the potential oncogenesis linked to the tissue-specific activity of the promoter for the CFTR gene. Surprisingly, the only site of tumors resulting from expression of the reporter onc gene was ependymal cells lining the brain ventricles. SV40 T antigen expression in these cells led to a consistent pathology in the first weeks of age: ependymoma and consequent hydrocephaly. Tumor-derived cell lines were established, characterized and shown to originate from SV40 T antigen-induced ependymoma. No pathological alterations were found in other organs, such as lungs and pancreas, in which cystic fibrosis is pathologically manifest in humans. Such transgenic mice and derived cell lines may represent valid models for analysing (1) the role of SV40 T antigen in ependymoma formation and (2) CFTR function in ependymal cells. PMID:1373882

  12. Cystic fibrosis from the gastroenterologist's perspective.

    PubMed

    Ooi, Chee Y; Durie, Peter R

    2016-03-01

    Cystic fibrosis is a life-limiting, recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Increased survival outcomes and the multisystem nature of the disease, including the involvement of hepatobiliary and gastrointestinal tracts, now require the need for more extensive knowledge and expertise in cystic fibrosis among gastroenterologists. Manifestations are either a direct consequence of the primary defect in cystic fibrosis or a secondary complication of the disease or therapy. Adult patients with cystic fibrosis also have an increased risk of malignancy in the gastrointestinal and pancreatico-biliary tracts compared with the general population. Novel treatments that target the basic defects in the CFTR protein have emerged, but to date not much is known about their effects on the gastrointestinal and hepatobiliary systems. The introduction of such therapies has provided new opportunities for the application of intestinal endpoints in clinical trials and the understanding of underlying disease mechanisms that affect the gut in cystic fibrosis.

  13. Cystic Echinococcosis

    PubMed Central

    Brunetti, Enrico; McCloskey, Cindy

    2015-01-01

    Echinococcosis is one of the 17 neglected tropical diseases (NTDs) recognized by the World Health Organization. The two major species of medical importance are Echinococcus granulosus and Echinococcus multilocularis. E. granulosus affects over 1 million people and is responsible for over $3 billion in expenses every year. In this minireview, we discuss aspects of the epidemiology, clinical manifestations, and diagnosis of cystic echinococcosis or cystic hydatid disease caused by E. granulosus. PMID:26677245

  14. Cystic Echinococcosis.

    PubMed

    Agudelo Higuita, Nelson Iván; Brunetti, Enrico; McCloskey, Cindy

    2016-03-01

    Echinococcosis is one of the 17 neglected tropical diseases (NTDs) recognized by the World Health Organization. The two major species of medical importance are Echinococcus granulosus and Echinococcus multilocularis. E. granulosus affects over 1 million people and is responsible for over $3 billion in expenses every year. In this minireview, we discuss aspects of the epidemiology, clinical manifestations, and diagnosis of cystic echinococcosis or cystic hydatid disease caused by E. granulosus. PMID:26677245

  15. 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- ...

  16. A novel NF1 mutation in a Chinese patient with giant café-au-lait macule in neurofibromatosis type 1 associated with a malignant peripheral nerve sheath tumor and bone abnormality.

    PubMed

    Tong, H-X; Li, M; Zhang, Y; Zhu, J; Lu, W-Q

    2012-08-29

    Neurofibromatosis type 1 (NF1; OMIM#162200) is a common neurocutaneous disorder that is characterized by multiple café-au-lait, skinfold freckling, Lisch nodules, and neurofibromas. Mutations in the NF1 gene, which encodes the neurofibromin protein, have been identified as the pathogenic gene of NF1. In this study, we present a clinical and molecular study of a Chinese patient with giant café-au-lait in NF1. The patient showed >6 café-au-lait spots on the body, axillary freckling, and multiple subcutaneous neurofibromas. He also had a malignant peripheral nerve sheath tumor and bone abnormalities. The germline mutational analysis of the NF1 gene revealed a novel missense mutation in exon 13. It is a novel heterozygous nucleotide G>A transition at position 2241 of the NF1 gene. We found no mutation in malignant peripheral nerve sheath tumor DNA from this patient. This expands the database for NF1 gene mutations in NF1. Its absence in the normal chromosomes suggests that it is responsible for the NF1 phenotype. To our knowledge, this is the first case of giant café-au-lait macule in NF1 associated with a malignant peripheral nerve sheath tumor and bone abnormality.

  17. [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.

  18. Giant endometrial cyst of the liver: a case report and review of the literature

    PubMed Central

    DE RIGGI, M.A.; FUSCO, F.; MARINO, G.; IZZO, A.

    2016-01-01

    Endometriosis is a benign condition described as the presence of endometrial-like tissue found outside the uterine cavity. Hepatic endometriosis is one of the rarest localization of extrapelvic endometriosis, only 22 cases have been reported in the literature. The preoperative diagnosis of hepatic endometriosis is rather difficult because in about the half of the patient affected they had no history of endometriosis. Moreover radiological images reveal no characteristic findings for hepatic endometriosis. It is often described as cystic mass with or without solid component, difficult to distinguish from hepatic abscess, hematoma, cystoadenoma or malignant neoplasia. We report a case of a 27-year-old female with a large cystic mass involving the left lobe of the liver. The patient underwent laparoscopic exploration and converted to laparotomy for resection of giant hepatic endometriosis. PMID:27381694

  19. 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.

  20. 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 and ...

  1. [Cystic angiomatosis].

    PubMed

    Fernández Jiménez, I; Alvarez Muñoz, V; Peláez Mata, D; Díaz Blanco, M; Galbe Sada, M; Antuña García, M J

    2000-04-01

    A case of cystic angiomatosis is presented in a patient whose clinical sign were the presence of an inguinoescrotal mass due to a retroperitoneal lymphangioma, visceral splenic lesions and diffuse skeletal lesions. This rare entity is discussed and a brief review of the literature is made.

  2. 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.

  3. Atypical presentation of a gastric stromal tumor masquerading as a giant intraabdominal cyst: A case report

    PubMed Central

    Sun, Ke-Kang; Xu, Song; Chen, Jinzhen; Liu, Gang; Shen, Xiaojun; Wu, Xiaoyang

    2016-01-01

    Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms that arise in the gastrointestinal tract, accounting for ~1% of gastric malignancies. The present study reports the case of a GIST of the stomach in a 75-year-old man who presented with abdominal distension and anorexia for 1 month. Gastroscopy was unremarkable. Ultrasound and computed tomography (CT) scans showed a giant intraabdominal cystic lesion of unknown origin. The lesion was initially believed to be a duplication cyst, a pancreatic pseudocyst or a liver cyst in the pre-operative diagnosis. Exploratory laparotomy revealed a cystic lesion of the lesser sac originating from the lesser curvature of the stomach. A distal gastrectomy with en bloc resection of the lesion was performed. The intraoperative frozen section showed a spindle-cell GIST and microscopically negative margins. The patient was treated with imatinib for 1 year. The latest CT scan at 14 months post-surgery did not show any recurrence. Although GISTs presenting as predominantly cystic lesions are very rare, they should be considered in the differential diagnosis of cystic lesions of the upper abdomen.

  4. Retroperitoneal bronchogenic cyst as a differential diagnosis of pancreatic mucinous cystic tumor.

    PubMed

    Andersson, Roland; Lindell, Gert; Cwikiel, Wojciech; Dawiskiba, Sigmund

    2003-01-01

    Cystic tumors of the pancreas where a pseudocyst has not been able to be excluded has been considered potentially proliferative and pre-malignant or malignant and thus aggressive surgical approach has been advocated. Retroperitoneal cystic tumors are rare and among these bronchogenic cysts are extremely infrequent. The present paper describes a case of bronchogenic cyst in association with the pancreas in which diagnostic work-up was not able to exclude a proliferative pancreatic cystic tumor.

  5. Giant cystadenoma of the floor of the mouth: A case report

    PubMed Central

    Castro, Victor Labres da Silva; Corrêa, Tiago Fernando Aires; Guimarães, Valeriana de Castro; Nery, Gustavo Vasconcelos; Ferreira, João Batista

    2013-01-01

    Summary Introduction: Cystadenoma is an uncommon epithelial neoplasia that arises from the salivary glands. The malignancy can affect structures such as the larynx, nasopharynx, buccal mucosa, and palate. Objective: To describe a case of a giant cystadenoma of the floor of the mouth treated at a public hospital in midwestern Brazil. Case report: The patient was a 46-year-old woman with complaints of difficulties in articulating words and swallowing solid food and vocal fatigue. The progression of the disease since the initial consultation, the results of clinical examinations, and the outcome of surgery are described. Finals Comments: Cystadenoma must be considered in the differential diagnosis of cystic injuries in the floor of the mouth if the patient's symptoms are suggestive of this malignancy. PMID:25992001

  6. Radiological description of cystic pancreatic tumors.

    PubMed

    Rodríguez Torres, C; Larrosa López, R

    2016-01-01

    Although most cystic pancreatic lesions are pseudocysts, it is important to do a thorough differential diagnosis with true cystic tumors because cystic tumors are potentially malignant. Sometimes computed tomography and magnetic resonance imaging cannot establish the definitive diagnosis, making it necessary to perform other imaging tests such as endoscopic ultrasound, which in addition to morphological information, can also enable cytologic and biochemical analysis of the lesion through puncture and aspiration of its contents. Combining all these findings nearly always provides enough diagnostic information to allow the appropriate approach in each case. This article describes the specific morphological characteristics for each cystic pancreatic tumor on computed tomography, magnetic resonance imaging, and endoscopic ultrasound and reviews the guidelines for managing these types of lesions.

  7. Endoscopic approach to the diagnosis of pancreatic cystic tumor

    PubMed Central

    Kawaguchi, Yoshiaki; Mine, Tetsuya

    2016-01-01

    Because of the aging of the population, prevalence of medical checkups, and advances in imaging studies, the number of pancreatic cystic lesions detected has increased. Once these lesions are detected, neoplastic cysts should be differentiated from non-neoplastic cysts. Furthermore, because of the malignant potential of some neoplastic pancreatic cysts, further differentiation between benign and malignant cysts should be made regardless of their size. Although endoscopic ultrasound (EUS) has a very high diagnostic performance for pancreatic cystic lesions among the various imaging modalities, EUS findings alone are insufficient for the differentiation of pancreatic cysts and diagnosis of malignancy. In addition, cytology by EUS-guided fine-needle aspiration (FNA) has a high specificity but a low sensitivity for diagnosing malignancy in pancreatic cystic tumors. The levels of amylase, lipase, and tumor markers in pancreatic cystic fluid are considered auxiliary parameters for diagnosis of benign and malignant cysts, and a definitive diagnosis of malignancy using these parameters is difficult. Thus, in addition to EUS, cytology by EUS-FNA, and cystic fluid analysis, new techniques based on EUS-guided through-the-needle imaging, such as confocal laser endomicroscopy and cystoscopy, have been explored in recent years. PMID:26909130

  8. 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

  9. 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. ...

  10. 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 ...

  11. Giant congenital melanocytic nevus.

    PubMed

    Viana, Ana Carolina Leite; Gontijo, Bernardo; Bittencourt, Flávia Vasques

    2013-01-01

    Giant congenital melanocytic nevus is usually defined as a melanocytic lesion present at birth that will reach a diameter ≥ 20 cm in adulthood. Its incidence is estimated in <1:20,000 newborns. Despite its rarity, this lesion is important because it may associate with severe complications such as malignant melanoma, affect the central nervous system (neurocutaneous melanosis), and have major psychosocial impact on the patient and his family due to its unsightly appearance. Giant congenital melanocytic nevus generally presents as a brown lesion, with flat or mammilated surface, well-demarcated borders and hypertrichosis. Congenital melanocytic nevus is primarily a clinical diagnosis. However, congenital nevi are histologically distinguished from acquired nevi mainly by their larger size, the spread of the nevus cells to the deep layers of the skin and by their more varied architecture and morphology. Although giant congenital melanocytic nevus is recognized as a risk factor for the development of melanoma, the precise magnitude of this risk is still controversial. The estimated lifetime risk of developing melanoma varies from 5 to 10%. On account of these uncertainties and the size of the lesions, the management of giant congenital melanocytic nevus needs individualization. Treatment may include surgical and non-surgical procedures, psychological intervention and/or clinical follow-up, with special attention to changes in color, texture or on the surface of the lesion. The only absolute indication for surgery in giant congenital melanocytic nevus is the development of a malignant neoplasm on the lesion.

  12. 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 ...

  13. Overview of the clinical problem: facts and current issues of mucinous cystic neoplasms of the pancreas.

    PubMed

    Jeurnink, S M; Vleggaar, F P; Siersema, P D

    2008-11-01

    Pancreatic cystic lesions are uncommon and consist of pseudocysts, congenital cysts and cystic neoplasms including mucinous cystic neoplasms, intraductal papillary mucinous neoplasms and serous cystic neoplasms. Mucinous cystic neoplasms are large septated cysts without connection to the ductal system, characterised by the presence of thick-walled ovarian-type stroma and mucin. They occur predominantly in women and often are malignant. Therefore, surgical resection is recommended. Intraductal papillary mucinous neoplasms are neoplasms with tall, columnar, mucin-containing epithelium involving the main pancreatic ducts or major side branches. Intraductal papillary mucinous neoplasms occur in men and women in their 60s and 70s and may differentiate into malignant neoplasms. Therefore, surgical resection is mandatory. Serous cystic neoplasms appear as multiple cysts lined with cubic flat epithelium containing glycogen-rich cells with clear cytoplasm. They mainly occur in women in their 50s and are generally benign. Therefore, a conservative approach is recommended. As both mucinous cystic neoplasm and intraductal papillary mucinous neoplasms have a high malignant potential, it is important to differentiate between the various pancreatic cystic lesions. Several imaging techniques and tumour markers have been evaluated. Nonetheless, definitive guidelines to differentiate between serous cystic neoplasms, mucinous cystic neoplasms and intraductal papillary mucinous neoplasms are still poorly defined. A number of management issues regarding these neoplasms are still under debate, for example which imaging technique to use, differentiation between malignant or benign lesions and the preferred treatment modality for each pancreatic cystic neoplasm. Further research may lead to a definitive guideline for the diagnosis and treatment of mucinous cystic neoplasms, intraductal papillary mucinous neoplasms and serous cystic neoplasms. PMID:18499541

  14. Surgical outcome in cystic vestibular schwannomas

    PubMed Central

    Nair, Suresh; Baldawa, Sachin S.; Gopalakrishnan, Chittur Viswanathan; Menon, Girish; Vikas, Vazhayil; Sudhir, Jayanand B.

    2016-01-01

    Background: Cystic vestibular schwannomas (VS) form a rare subgroup that differs from the solid variant clinically, radiologically, and histopathologically. These tumors also vary in their surgical outcome and carry a different risk of post-operative complications. We analyzed our series of 64 patients with cystic VS and discuss the technical difficulties related to total excision of these tumors and focus on complication avoidance. Materials and Methods: A retrospective review of cystic VS surgically managed over a span of 11 years. The case records were evaluated to record the clinical symptoms and signs, imaging findings, surgical procedure, complications, and follow-up data. Post-operative facial nerve palsy was analyzed with respect to tumor size and tumor type. Results: Progressive hearing impairment was the most common initial symptom (76.6%). Atypical initial symptoms were present in 15 patients (23.4%). Preoperatively, 78% patients had good facial nerve function (HB grade 1, 2) and 22% had intermediate (HB grade 3, 4) to poor (HB grade 5 and 6) function. Mean tumor size was 4.1 cm. Complete tumor removal was achieved in 53 patients (83%). The facial nerve was anatomically intact but thinned out after tumor excision in 38 patients (59.4%). Ninety percent patients had either intermediate or poor facial nerve function at follow-up. Poor facial nerve outcome was associated with giant tumors and peripherally located, thin-walled cystic tumors. Conclusion: Resection of cystic VS is complicated by peritumoral adhesions of the capsule to the nerve. Extensive manipulation of the nerve in order to dissect the tumor–nerve barrier results in worse facial nerve outcome. The outcome is worse in peripherally located, thin-walled cystic VS as compared to centrally located, thick-walled cystic tumors. Subtotal excision may be justified, especially in tumors with dense adhesion of the cyst wall to the facial nerve in order to preserve nerve integrity. PMID:27366248

  15. 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 ...

  16. [Cystic tumors of the pancreas - - our experience with diagnostics].

    PubMed

    Krechler, T; Ulrych, J; Dvořák, M; Hoskovec, D; Macášek, J; Švestka, T; Hořejš, J

    2013-07-01

    Number of newly diagnosed cystic pancreatic tumors is permanently increasing. This fact is primarily related to the development of new diagnostic methods. The main representative ones are: serous cystadenoma, mucinous cystic neoplasm, intraductal papillary mucinous neoplasm and solid pseudopapillar tumor. Because of the malignant potential of these lesions, proper indication of surgical treatment is extremely important. The article highlights and describes our experience in diagnostics and therapy of cystic pancreatic tumors diagnosed in the General Teaching Hospital Prague in the period: 1/ 2008- 12/ 2012. All patients were investigated by computerised tomography and endoscopic ultrasound with fine -  needle aspiration biopsy. Thirty seven patients in total were diagnosed with cystic pancreatic tumors: 19 with serous cystadenoma, 5 with mucinous cystic neoplasm, 5 with mucinous cystadenocarcinoma, 5 with intraductal papillary mucinous neoplasm and 3 with solid pseudopapillar tumor. In 14 cases patients were indicated for surgery, in 1 case signs of malignant transformation were found. Determination of the optimal strategy for diagnostic and therapeutic procedures in patients with cystic pancreatic tumors requires the dia-gnosis, treatment and followup observation in adequately equiped specialized centers. PMID:23909261

  17. 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

  18. A Huge Mature Cystic Teratoma in a Nulliparous Patient

    PubMed Central

    Gunduz, Melih; Pata, Ozlem; Unlu, Cihat

    2015-01-01

    Here we report a case of a giant mature cystic teratoma of the ovary in a 38-year-old nullipaous woman. The patient presented with abdominal distension and abdominal pain. Laparotomy and cystectomy yielded satisfactory results. Histologic evaluation confirmed a benign cystic teratoma of the ovary. The patient underwent surgery for rupture of corpus luteum six years ago and no gross lesion was seen at the operation. This case demonstrates that dermoid cysts can grow to enormous sizes within a short duration. PMID:26500962

  19. Preoperative cytological diagnosis of papillary cystic variant of acinic cell carcinoma: A key consideration in patient management.

    PubMed

    Datar, Sonali S; Poflee, Sandhya V; Pande, Nandu P; Umap, Pradeep S

    2015-01-01

    Preoperative diagnosis of malignant salivary gland tumors is difficult as radiological imaging procedures have low sensitivity rate for detecting malignancy in parotid gland tumors. With careful and detailed analysis of cytological features, guided fine needle aspiration cytology can prove to be a reliable diagnostic modality that can help in differential diagnosis of cystic parotid malignancies from cystic benign tumors and non-neoplastic lesions. Papillary cystic variant of acinic cell carcinoma is a rare, cystic, primary neoplasm of salivary gland that occurs commonly in parotid. The tumor shows high local recurrence rate and has poorer prognosis compared to classic acinic cell carcinoma. Preoperative cytological diagnosis of papillary cystic variant of acinic cell carcinoma of parotid, in the case described here was the basis for adequate and timely surgical management with good results.

  20. Conservative surgical treatment for a giant thoracoabdominal benign teratoma.

    PubMed

    Catania, A; D'Andrea, V; Panzironi, G; Biancari, F; De Antoni, E

    1997-06-01

    The authors describe an adult patient with a giant, cystic teratoma (33 x 22 x 18 cm) involving the posterior mediastinum and retroperitoneum. A conservative surgical approach consisting of a partial endocystectomy plus injections of tetracycline into the residual cavity, was performed. Two months after surgery, a CT scan showed a 6 x 5 x 3 cm residual cyst. The authors believe that the excision of the inner surface of the cystic lesion and the intracystic administration of tetracycline, may successfully prevent the accumulation of fluid which is the main cause of the progressive enlargement of such benign cystic teratomas.

  1. 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

  2. Lapatinib in Treating Patients With Recurrent and/or Metastatic Adenoid Cystic Cancer or Other Salivary Gland Cancers

    ClinicalTrials.gov

    2013-10-10

    High-grade Salivary Gland Carcinoma; High-grade Salivary Gland Mucoepidermoid Carcinoma; Low-grade Salivary Gland Carcinoma; Low-grade Salivary Gland Mucoepidermoid Carcinoma; Recurrent Adenoid Cystic Carcinoma of the Oral Cavity; Recurrent Salivary Gland Cancer; Salivary Gland Acinic Cell Tumor; Salivary Gland Adenocarcinoma; Salivary Gland Adenoid Cystic Carcinoma; Salivary Gland Malignant Mixed Cell Type Tumor

  3. Diabetes in cystic fibrosis.

    PubMed

    Bridges, Nicola

    2013-05-01

    Cystic fibrosis related diabetes (CFRD) is a common complication of cystic fibrosis, caused by a fall in insulin secretion with age in individuals with pancreatic insufficiency. CFRD is associated with worse clinical status and increased mortality. Treatment of CFRD with insulin results in sustained improvements in lung function and nutrition. While clinical experience with insulin treatment in CF has increased, the selection of who to treat and glycaemic targets remain unclear.

  4. Magnetic resonance imaging of cystic pancreatic lesions in adults: an update in current diagnostic features and management.

    PubMed

    Barral, Matthias; Soyer, Philippe; Dohan, Anthony; Laurent, Valérie; Hoeffel, Christine; Fishman, Elliot K; Boudiaf, Mourad

    2014-02-01

    Magnetic resonance (MR) imaging has become a widespread diagnostic solving tool for the detection and characterization of a large range of pancreatic cystic lesions. Benign and malignant cystic lesions of the pancreas including serous microcystic adenoma, mucinous cystic tumor, intraductal papillary mucinous tumor, solid pseudopapillary tumor, and also the less common lesions such as cystic endocrine tumors, cystic metastases, and lymphangiomas have suggestive MR imaging presentation that allows them to be differentiated from each other. Knowledge of MR imaging findings of cystic pancreatic lesions is critical to help suggest the diagnosis and chose the best therapeutic approach. The purpose of this review is to discuss and illustrate MR imaging features that are helpful for pancreatic cystic lesion detection and characterization and to provide an update in current MR imaging diagnostic features and management. PMID:24263208

  5. European experts consensus statement on cystic tumours of the pancreas.

    PubMed

    Del Chiaro, Marco; Verbeke, Caroline; Salvia, Roberto; Klöppel, Gunter; Werner, Jens; McKay, Colin; Friess, Helmut; Manfredi, Riccardo; Van Cutsem, Eric; Löhr, Matthias; Segersvärd, Ralf

    2013-09-01

    Cystic lesions of the pancreas are increasingly recognized. While some lesions show benign behaviour (serous cystic neoplasm), others have an unequivocal malignant potential (mucinous cystic neoplasm, branch- and main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm). European expert pancreatologists provide updated recommendations: diagnostic computerized tomography and/or magnetic resonance imaging are indicated in all patients with cystic lesion of the pancreas. Endoscopic ultrasound with cyst fluid analysis may be used but there is no evidence to suggest this as a routine diagnostic method. The role of pancreatoscopy remains to be established. Resection should be considered in all symptomatic lesions, in mucinous cystic neoplasm, main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm as well as in branch duct intraductal papillary mucinous neoplasm with mural nodules, dilated main pancreatic duct >6mm and possibly if rapidly increasing in size. An oncological partial resection should be performed in main duct intraductal papillary mucinous neoplasm and in lesions with a suspicion of malignancy, otherwise organ preserving procedures may be considered. Frozen section of the transection margin in intraductal papillary mucinous neoplasm is suggested. Follow up after resection is recommended for intraductal papillary mucinous neoplasm, solid pseudo-papillary neoplasm and invasive cancer.

  6. Pancreatic Cystic Neoplasms

    PubMed Central

    Limaiem, Faten; Khalfallah, Tahar; Farhat, Leila Ben; Bouraoui, Saâdia; Lahmar, Ahlem; Mzabi, Sabeh

    2014-01-01

    Background: Cystic neoplasms of the pancreas are rare and constitute approximately 0.5% of all pancreatic neoplasms. Aims: The study was to describe clinicopathological features of pancreatic cystic tumors. Patients and Methods: In our retrospective study, we reviewed 10 cases of pancreatic cystic neoplasms that were diagnosed at the pathology department of Mongi Slim hospital over a 14-year period (2000-2013). We adopted the latest World Health Organization (WHO) classification (2010) in grouping all tumors. Results: There were one male and nine female patients (sex ratio M/F = 1:9) aged between 21 and 68 years (mean = 37.5 years). The most common clinical presentation was epigastric and abdominal pain (n = 6) followed by vomiting (n = 3). Abdominal computed tomography (CT) scan disclosed a cystic lesion of the pancreas ranging in size between 2 and 10 cm (mean = 6.75 cm). All patients underwent surgical treatment. Histopathological examination of the surgical specimen established the diagnosis of solid pseudopapillary neoplasm (n = 2), serous cystic neoplasm (n = 2), mucinous cystadenoma (n = 4), mucinous cystadenocarcinoma (n = 1), and intraductal papillary mucinous neoplasm with invasive carcinoma (n = 1). Conclusion: Better understanding of pancreatic cystic neoplasms is essential for clinicians to make accurate diagnosis and to provide the best management for patients. PMID:25210676

  7. 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 ...

  8. Expression of KIT (CD117) in neoplasms of the head and neck: an ancillary marker for adenoid cystic carcinoma.

    PubMed

    Mino, M; Pilch, B Z; Faquin, W C

    2003-12-01

    Adenoid cystic carcinoma is an indolent salivary gland malignancy that is associated with a poor long-term prognosis. The distinction of adenoid cystic carcinoma from other head and neck neoplasms can occasionally be problematic, particularly in small biopsies. Recent studies suggest that KIT (CD117) might be useful as an ancillary marker for adenoid cystic carcinoma; however, the expression of KIT in other benign and malignant head and neck neoplasms, including those that might mimic adenoid cystic carcinoma, has not been well studied. Here we use two different antibodies against KIT to evaluate its expression in a series of 66 adenoid cystic carcinomas compared with its expression in 98 other neoplasms of the head and neck. Overall, 94% (n = 62) of adenoid cystic carcinomas from various anatomic sites and of various histologic subtypes were positive for at least one of the KIT antibodies, and 77% (n = 50) of adenoid cystic carcinoma cases were positive for both antibodies. This contrasted with only 8% (n = 8) of other head and neck neoplasms that were positive for both KIT antibodies (P <.001). It was of note that certain neoplasms, including pleomorphic adenoma, basal cell adenoma, polymorphous low-grade adenocarcinoma, and basal cell carcinoma, that can show histologic overlap with adenoid cystic carcinoma had significantly less KIT immunoreactivity than did adenoid cystic carcinoma (P <.001). In contrast, KIT expression did not reliably distinguish adenoid cystic carcinoma from basal cell adenocarcinoma and basaloid squamous carcinoma (P >.05). The overall sensitivity of the two KIT antibodies for adenoid cystic carcinoma was 82-89%, and the specificity was 87-88%. The findings in this study support the potential use of KIT immunoexpression for distinguishing adenoid cystic carcinoma from many other benign and malignant head and neck neoplasms.

  9. A case of congenital giant gallbladder with massive hydrops mimicking celiac cyst

    PubMed Central

    ZONG, LIANG; CHEN, PING; WANG, LEI; HE, CHUNLAN; WANG, GUANGYAO; JIANG, JIAN; WANG, HAO

    2013-01-01

    We present a case of a 55-year-old female who suffered from a mass in the right upper abdomen, which had been present for over six months. Pre-operative blood examinations, including tumor markers, were normal. Initially, the admitting diagnosis was a giant celiac cyst, due to its liquid cystic appearance and large size (approximately 30.0×18.0 cm), visible in the hepatic region under ultrasound. Following the discovery of a cystic duct during surgery, the diagnosis was corrected to be a giant gallbladder. As no obstructive matter was observed, the giant gallbladder was considered to be congenital. PMID:23255925

  10. Recurrent malignant salivary gland neoplasms.

    PubMed

    Rodriguez-Bigas, M A; Sako, K; Razack, M S; Shedd, D P; Bakamjian, V Y; Castillo, N B; Rao, U

    1989-10-01

    Recurrent salivary gland malignancies present difficult therapeutic decisions and poor prognosis in many instances, and treatment becomes of a palliative nature only. As many of the salivary gland malignancies we see are of the recurrent type, the following study was done to determine the efficacy of a vigorous attempt at retreatment. During the period January 1, 1960, through December 31, 1984, 352 patients with major and minor salivary gland tumors were evaluated at our institution. There were 149 benign lesions and 203 patients with malignant tumors. Of these, 99 patients had recurrent and metastatic tumors that had been treated initially elsewhere. Thirty-three of these patients were able to be treated with curative intent: surgery, 21; surgery plus radiation, 9; radiation therapy alone, 2; and radiation plus chemotherapy, 1. The 5 year survival with no evidence of disease was achieved in three patients with surgery alone and two patients with surgery plus radiation therapy. The group of five patients was comprised of two patients with adenoid cystic carcinomas of the parotid, one with intermediate grade mucoepidermoid carcinoma of the parotid, one, sebaceous cell carcinoma of the parotid, and one, adenoid cystic carcinoma of an accessory salivary gland. The results of this study serve to re-emphasize the relative poor yield of attempts at retreatment of loco-regional recurrence of salivary gland tumors.

  11. Floret-like multinucleated giant cells in neurofibroma

    PubMed Central

    Shaktawat, Sameer Singh; Golka, Dariusz

    2007-01-01

    This short report discusses a case of neurofibroma containing floret-like multinucleated giant cells. This being the second such case in the literature. Floret-like multinucleated giant cells have been reported in gynaecomastia and neurofibroma in neurofibromatosis type 1. These cells have been reported in uncommon soft tissue tumours including pleomorphic lipoma, giant cell collagenoma, giant cell fibroblastoma and giant cell angiofibroma. We recommend these cells to be interpreted carefully keeping in mind the rare malignant change in neurofibromas. Immunohistochemistry would help in defining the nature of such cells. PMID:18067673

  12. Floret-like multinucleated giant cells in neurofibroma.

    PubMed

    Shaktawat, Sameer Singh; Golka, Dariusz

    2007-12-08

    This short report discusses a case of neurofibroma containing floret-like multinucleated giant cells. This being the second such case in the literature. Floret-like multinucleated giant cells have been reported in gynaecomastia and neurofibroma in neurofibromatosis type 1. These cells have been reported in uncommon soft tissue tumours including pleomorphic lipoma, giant cell collagenoma, giant cell fibroblastoma and giant cell angiofibroma. We recommend these cells to be interpreted carefully keeping in mind the rare malignant change in neurofibromas. Immunohistochemistry would help in defining the nature of such cells.

  13. Giant Planets

    NASA Astrophysics Data System (ADS)

    Lunine, J. I.

    Beyond the inner solar system's terrestrial planets, with their compact orbits and rock -metal compositions, lies the realm of the outer solar system and the giant planets. Here the distance between planets jumps by an order of magnitude relative to the spacing of the terrestrial planets, and the masses of the giants are one to two orders of magnitude greater than Venus and Earth - the largest terrestrial bodies. Composition changes as well, since the giant planets are largely gaseous, with inferred admixtures of ice, rock, and metal, while the terrestrial planets are essentially pure rock and metal. The giant planets have many more moons than do the terrestrial planets, and the range of magnetic field strengths is larger in the outer solar system. It is the giant planets that sport rings, ranging from the magnificent ones around Saturn to the variable ring arcs of Neptune. Were it not for the fact that only Earth supports abundant life (with life possibly existing, but not proved to exist, in the martian crust and liquid water regions underneath the ice of Jupiter's moon Europa), the terrestrial planets would pale in interest next to the giant planets for any extraterrestrial visitor.

  14. [Atypical intraperitoneal cystic masses].

    PubMed

    Domínguez-Pérez, S T; Baeza-Herrera, C; Villalobos-Castillejos, A; González-Mateos, T; Aguilar-Venegas, M

    2010-01-01

    Omental, mesenteric and retroperitoneal cystic masses are very rare in pediatric population. They usually present as asymptomatic abdominal tumors and only occasionally a preoperative diagnosis is made. We presented 4 cases presented to our hospital during a 2-year period. All patients were male with an age ranged from 5 months to 7 years. Three patients had previous diagnosis of cystic mass by abdominal ultrasound. Three patients presented with acute abdomen and one patient with bowel obstruction. All patients underwent successful resection of the mass with no perioperative mortality.

  15. Giant frontal sinus mucocoele. Report of two cases.

    PubMed

    Adekeye, E O; Ord, R A

    1984-08-01

    The giant frontal sinus mucocoele is best approached surgically using a bi-coronal flap to give an ample view of orbital and intra-cranial extensions. The size of the cystic cavity necessitates packing rather than obliteration with autografts. Reconstruction of the frontal bone may be carried out as a secondary procedure.

  16. Unusual renal tumour: multilocular cystic renal cell carcinoma.

    PubMed

    Palmeiro, Marta Morna; Niza, João Luz; Loureiro, Ana Luisa; Conceição e Silva, João Paulo

    2016-01-01

    Multilocular cystic renal cell carcinoma (MCRCC) is a rare presentation of renal cell carcinoma. Most patients are asymptomatic and frequently MCRCCs are detected incidentally. MCRCCs have good prognosis because of their low malignant potential. We report a case of a 39-year-old woman who presented with mild right flank pain and normal laboratory data. On imaging examinations, a Bosniak III cystic lesion was detected in the lower third of the right kidney. She underwent right partial nephrectomy and histopathology showed a multilocular cystic renal cell carcinoma Fuhrman grade 1. In this article, we also present a review of the literature on MCRCC, highlight the correlation of the pathological and imaging characteristics of these low aggressive renal lesions, and underscore the importance of their recognition to prevent unnecessary radical surgery. PMID:26957035

  17. [Perinephric liposarcoma mimicking cystic renal tumor].

    PubMed

    Horiguchi, Akio; Oyama, Masafumi

    2002-03-01

    Liposarcoma is one of the most common primary retroperitoneal neoplasms, and the perinephric region is a frequent location for them. Liposarcomas show a variety of radiographic features in terms of histological types and tumor sizes, so the specific diagnosis of liposarcoma is often difficult. We present a unique case of perinephric dedifferentiated liposarcoma mimicking cystic renal tumor. A 71-year-old man presented himself at our hospital with a palpable mass in his upper right abdomen. Abdominal computerized tomography (CT) revealed a well-defined cystic mass at the lower pole of the right kidney that contained heterogeneous solid components and small foci of fat. There were no signs of lymphadenopathy or tumor thrombus in the renal vein. Metastatic evaluation by chest x-ray and bone scan was negative. The probable diagnosis was cystic renal cell carcinoma or atypical angiomyolipoma. Because we could not exclude the possibility of cystic malignancy, a right radical nephrectomy was performed. Grossly, the tumor was predominantly encapsulated by a unilocular fibrous capsule and was filled with bloody fluid and debris. The anterior portion of the tumor was composed of various-sized soft and rubbery masses covered with necrotic tissue. The histological diagnosis was dedifferentiated liposarcoma arising in the perinephric retroperitoneum with extensive necrosis, and the cyst wall was composed of a necrotic tumor with a well differentiated liposarcoma and a fibrous capsule. Although the tumor widely covered the right kidney, there was no microscopic invasion of the kidney. No signs of tumor recurrence were noted six months after the operation.

  18. A correlation study of diagnostic fine-needle aspiration with histologic diagnosis in cystic neck lesions.

    PubMed

    Moatamed, Neda A; Naini, Bita V; Fathizadeh, Payman; Estrella, Julie; Apple, Sophia K

    2009-10-01

    The clinical diagnosis of a mass in the neck region encompasses a wide spectrum of differential diagnosis. Fine-needle aspiration is a quick and safe technique, which can provide useful information for initial assessment and further therapeutic measures. The aim of this retrospective study was to evaluate the performance characteristics of the fine-needle aspiration (FNA) in cystic neck lesions. Of 142 patients with FNA for cystic neck masses during 2002-2007, 92 cases were selected with a follow-up histologic diagnosis, excluding the cystic colloid nodule of the thyroid. The cases were divided into salivary gland cystic neck (37 patients) and non-salivary cystic neck (55 patients) mass groups. False-positive and false-negative diagnoses were applied only to the malignant lesions after confirmation by histopathology. In the first group, nine malignant and 28 benign diagnoses were made by FNA; of which three were false-negative. In the second group, there were nine malignant and 46 benign diagnoses with three false negatives. The overall performance of the FNA showed 76% sensitivity and 100% specificity. In conclusion, FNA of the cystic neck lesions offers an invaluable and highly specific initial diagnostic approach for the management of the patients.

  19. Prolonged Air Leakage Caused by Mesenchymal Cystic Hamartoma of the Lung

    PubMed Central

    Lee, Young Uk; Lee, Jang Hoon; Baek, Jong Hyun

    2016-01-01

    A 16-year-old girl was transferred to the department of thoracic and cardiovascular surgery because of a spontaneous pneumothorax with prolonged air leakage. Chest computed tomography demonstrated a cystic lesion measuring 2×3 cm and involving the left upper lobe. Left upper lobectomy was performed via video-assisted thoracoscopic surgery. A pathologic examination of the specimen revealed a mesenchymal cystic hamartoma. Despite the rarity of pulmonary mesenchymal cystic hamartoma, it should be considered a potential cause of pneumothorax for patients with a large pulmonary cyst. Further, surgical resection must be considered because serious complications such as hemothorax, hemoptysis, and malignant transformation have been reported. PMID:27525242

  20. Malignant adenolymphoma.

    PubMed

    Moosavi, H; Ryan, C; Schwartz, S; Donnelly, J A

    1980-01-01

    Adenolymphoma (Warthin's tumor) is a well studied benign tumor of the salivary gland. Malignant transformation of such a tumor is rare and not well documented in the literature. The light microscopic and ultrastructural features of an undifferentiated carcinoma arising in an adenolymphoma in the parotid gland of a middle aged male are described, and the relevant literature is reviewed. Similarities between the benign adenolymphoma and the undifferentiated malignant tumor, such as the presence of interstitial lymphoplasmacytic cell infiltrates, dark and light epithelial cells, similar cytoplasmic organelles, and nuclear morphology, suggest a malignant transformation of a previously existing benign adenolymphoma.

  1. Pathologic review of cystic and cavitary lung diseases.

    PubMed

    Kim, Na Rae; Han, Joungho

    2012-10-01

    Pulmonary cystic and cavitary lesions caused by diverse etiologies are commonly encountered in chest imaging. The terms "cyst" and "cavity" are used to describe air-filled regions in the center of a nodule or consolidation of the lung. To date, only radiologic aspects of these lesions have been addressed. The morphologies of pulmonary cystic and cavitary lesions exhibit a broad spectrum, ranging from benign to malignant pulmonary diseases of acquired or congenital origin, including variable infectious diseases. In this review, we summarized the differential diagnosis of pathological entities to provide pathologists and radiologists with an overview of the diseases most commonly associated with pulmonary cystic and cavitary lesions in adults and children. The results showed slightly different patterns in the distribution of the diseases in the two groups. The most common causes of cavitary lesions include malignancy and infection in adults, and congenital malformation in children. Therefore, identification of pathologic entities correlating with the radiologic findings, clinical course, and location of the lesion is important in the evaluation of cystic and cavitary lung lesions in order to avoid unnecessary surgical procedures or delayed treatment. PMID:23136566

  2. Advanced MRI Techniques in the Evaluation of Complex Cystic Breast Lesions

    PubMed Central

    Popli, Manju Bala; Gupta, Pranav; Arse, Devraj; Kumar, Pawan; Kaur, Prabhjot

    2016-01-01

    OBJECTIVE The purpose of this research work was to evaluate complex cystic breast lesions by advanced MRI techniques and correlating imaging with histologic findings. METHODS AND MATERIALS In a cross-sectional design from September 2013 to August 2015, 50 patients having sonographically detected complex cystic lesions of the breast were included in the study. Morphological characteristics were assessed. Dynamic contrast-enhanced MRI along with diffusion-weighted imaging and MR spectroscopy were used to further classify lesions into benign and malignant categories. All the findings were correlated with histopathology. RESULTS Of the 50 complex cystic lesions, 32 proved to be benign and 18 were malignant on histopathology. MRI features of heterogeneous enhancement on CE-MRI (13/18), Type III kinetic curve (13/18), reduced apparent diffusion coefficient (18/18), and tall choline peak (17/18) were strong predictors of malignancy. Thirteen of the 18 lesions showed a combination of Type III curve, reduced apparent diffusion coefficient value, and tall choline peak. CONCLUSIONS Advanced MRI techniques like dynamic imaging, diffusion-weighted sequences, and MR spectroscopy provide a high level of diagnostic confidence in the characterization of complex cystic breast lesion, thus allowing early diagnosis and significantly reducing patient morbidity and mortality. From our study, lesions showing heterogeneous contrast enhancement, Type III kinetic curve, diffusion restriction, and tall choline peak were significantly associated with malignant complex cystic lesions of the breast. PMID:27330299

  3. Malignant hyperthermia.

    PubMed

    Brockhouse, R T

    1979-04-01

    A case has been presented that illustrates successful managment of a patient with suspected malignant hyperthermia. The causes of this disorder are uncertain. If screening procedures identify a patient as susceptible to this disorder, careful planning in the preoperative stage is indicated. Preparedness during the operative procedure for any emergency is mandatory. Early and effective treatment seems to be the only method of preventing mortality with patients experiencing malignant hyperthermia. PMID:285135

  4. Malignant oncocytoma.

    PubMed

    Laurian, N; Zohar, Y; Kende, L

    1977-09-01

    A case of malignant oncocytoma of the parotid gland in a 32-year-old male is presented. Ten months after parotidectomy an undifferentiated carcinoma, in which oncocytes still could be recognized, developed in the operated area. According to the literature available to us, this is the second reported case in which malignant transformation in a benign oncocytoma of the salivary gland has been observed.

  5. Adenoid cystic carcinoma of the nasopharynx after previous adenoid irradiation

    SciTech Connect

    Sofferman, R.A.; Heisse, J.W. Jr.

    1985-04-01

    In 1978, Pratt challenged the otolaryngology community to identify an incidence of malignancy in individuals who have previously received radium therapy to the nasopharyngeal lymphoid tissues. This case report is a direct response to that quest and presents a well documented adenoid cystic carcinoma evolving 23 years after radium applicator treatment to the fossa of Rosenmuller. Although a cause-and-effect relationship cannot be scientifically proven, the case history raises several important questions concerning the stimulating effects of radiation on the later onset of frank malignancy.

  6. Congenital cystic choristoma mimicking cervical lymphangioma.

    PubMed

    Moon, Suk-Bae; Park, Kwi-Won; Yun, Woong-Jae; Patten, Phillip P; Jung, Sung-Eun

    2008-09-01

    Choristoma is a tissue or mass with a normal histology at an abnormal location, and cystic choristoma has rarely been reported in the head and neck region. Cervical cystic masses in neonates are usually diagnosed as cystic lymphangioma. The authors present a case of a congenital laterocervical cystic mass that appeared to be lymphangioma, but which turned out to be a cystic choristoma.

  7. 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

  8. 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

  9. Malignant Melanoma With Osteoclast-Like Differentiation.

    PubMed

    Wasserman, Jason K; Sekhon, Harmanjatinder S; Ayroud, Yasmine

    2015-09-01

    Osteoclast-like giant cells are frequently encountered in nonskeletal malignancies; however, the evidence to date suggests that they represent a tissue response to the lesion rather than neoplastic differentiation. We describe a case of metastatic melanoma demonstrating osteoclast-like differentiation in the lung. The lung nodule was diagnosed as a metastatic melanoma by histological features and confirmed by immunohistochemistry. Resection specimen showed numerous multinucleated giant cells exhibiting osteoclast-like morphology dispersed throughout the lesion. Both the neoplastic melanocytes and giant cells were reactive for HMB-45, Melan-A, and S100. In addition, the multinucleated neoplastic giant cells were also reactive for the monocyte/macrophage lineage markers CD68 and CD163, and alkaline phosphatase, an enzyme present in normal osteoclasts. The neoplastic melanocytes and the multinucleated neoplastic giant cells were also reactive for microphthalmia-associated transcription factor, a protein required for the development of both melanocytes and osteoclasts. Collectively, a co-expression of monocyte/macrophage markers along with melanocytic markers and alkaline phosphatase in the multinucleated neoplastic giant cells in metastatic melanoma suggest that malignant melanocytes are capable of differentiating into osteoclast-like cells and consequently aid invasion into various structures and eliciting the aggressive behavior.

  10. Pleural malignancies.

    PubMed

    Vargas, F S; Teixeira, L R

    1996-07-01

    Carcinoma of the lung, metastatic breast carcinoma, and lymphoma are responsible for approximately 75% of all malignant pleural effusions. The presence of malignant cells in the pleural fluid or in the parietal pleura confirms the diagnosis. Recently, several authors have proposed the combination of morphometric procedures and quantitative analysis of nucleolar organizer regions stained by silver nitrate. Videothoracoscopy is recommended for patients suspected of having a malignant pleural effusion in whom the diagnosis is not established after two cytologic studies of the fluid and one needle biopsy. The standard treatment is the intrapleural instillation of a chemical agent to produce a pleurodesis. The recommended sclerosant is talc, a tetracycline derivative, or Corynebacterium parvum where it is available. When a patient is not an ideal candidate for chemical pleurodesis, the options include symptomatic treatment, serial thoracentesis, implantation of a pleuroperitoneal shunt, and pleurectomy. PMID:9363162

  11. Molecular Diagnosis of Cystic Fibrosis.

    PubMed

    Deignan, Joshua L; Grody, Wayne W

    2016-01-01

    This unit describes a recommended approach to identifying causal genetic variants in an individual suspected of having cystic fibrosis. An introduction to the genetics and clinical presentation of cystic fibrosis is initially presented, followed by a description of the two main strategies used in the molecular diagnosis of cystic fibrosis: (1) an initial targeted variant panel used to detect only the most common cystic fibrosis-causing variants in the CFTR gene, and (2) sequencing of the entire coding region of the CFTR gene to detect additional rare causal CFTR variants. Finally, the unit concludes with a discussion regarding the analytic and clinical validity of these approaches.

  12. A Challenging Giant Dermatofibrosarcoma Protuberans on the Face.

    PubMed

    Pérez, Gimena Castro; Arias, Cintia; Luna, Paula; Sorín, Irene; Mazzuoccolo, Luis Daniel

    2016-01-01

    Dermatofibrosarcoma protuberans (DFSP) is a malignant fibrohistiocytic tumor that appears exclusively on the skin. It is a low-grade malignant soft tissue tumor of subcutaneous tissues that has a propensity for local recurrence but seldom metastasizes. It may rarely occur on the head and neck accounting for less than one percent of total head and neck malignancies. We present a man with a giant DFSP on the face. Oncological, functional, and aesthetic aspects are set forth. PMID:27437151

  13. Hematologic malignancies

    SciTech Connect

    Hoogstraten, B.

    1986-01-01

    The principle aim of this book is to give practical guidelines to the modern treatment of the six important hematologic malignancies. Topics considered include the treatment of the chronic leukemias; acute leukemia in adults; the myeloproliferative disorders: polycythemia vera, essential thrombocythemia, and idiopathic myelofibrosis/agnogenic myeloid metaplasia; Hodgkin's Disease; non-Hodgkin's lymphoma; and Multiple Myeloma.

  14. Malignant hyperthermia.

    PubMed

    Taiclet, L

    1985-01-01

    Despite numerous reviews and clinical reports, much remains to be learned about the cause, treatment, and prevention of malignant hyperthermia.Among the most worrisome concerns of the clinician administering anesthesia is the malignant hyperthermia crisis. When it arises, it is always frightening-and sometimes fatal. Usually occurring very suddenly and without warning, malignant hyperthermia is considered to be a hypercatabolic crisis; the condition is known to affect humans and certain breeds of pigs. The exact triggering mechanisms of malignant hyperthermia (MH) in humans are not known, but a crisis can be initiated by volatile general anesthetics, neuromuscular blocking agents, and amide local anesthetics. Although a history of an MH crisis is a diagnostic aid, previous uneventful exposure to anesthesia does not guarantee the safety of the patient in subsequent anesthetic procedures.(1) For these reasons, it is important for the anesthesiologist to be aware of the initial signs of MH and to be prepared to provide immediate treatment to reverse such a crisis. PMID:3865561

  15. Malignant hyperthermia.

    PubMed Central

    Ben Abraham, R.; Adnet, P.; Glauber, V.; Perel, A.

    1998-01-01

    Malignant hyperthermia is a rare autosomal dominant trait that predisposes affected individuals to great danger when exposed to certain anaesthetic triggering agents (such as potent volatile anaesthetics and succinylcholine). A sudden hypermetabolic reaction in skeletal muscle leading to hyperthermia and massive rhabdomyolysis can occur. The ultimate treatment is dantrolene sodium a nonspecific muscle relaxant. Certain precautions should be taken before anaesthesia of patients known to be susceptible to malignant hyperthermia. These include the prohibition of the use of triggering agents, monitoring of central body temperature and expired CO2, and immediate availability of dantrolene. In addition, careful cleansing of the anaesthesia machine of vapours of halogenated agents is recommended. If these measures are taken, the chances of an MH episode are greatly reduced. When malignant hyperthermia-does occur in the operating room, prompt recognition and treatment usually prevent a potentially fatal outcome. The most reliable test to establish susceptibility to malignant hyperthermia is currently the in vitro caffeine-halothane contracture test. It is hoped that in the future a genetic test will be available. PMID:9538480

  16. Fine needle aspiration biopsy of cystic lesions of the head and neck, excluding the thyroid.

    PubMed

    Dejmek, A; Lindholm, K

    1990-01-01

    The occurrence of cystic lesions of the head and neck region (excluding the thyroid gland) and their diagnosis by fine needle aspiration (FNA) biopsy were reviewed for a two-year period. Of the 967 total aspirates of the region, 98 were cystic. The frequency of cysts was thus 10% in the total material, including 23% in parotid gland aspirates and 3% in lymph node aspirates. Salivary gland cysts occurred more often on the right side. The overall malignancy rate was the same for cysts and solid lesions (16%); however, 81% of cystic lymph node lesions were malignant. There was no difference in the frequency of nondiagnostic FNA material between solid and cystic lesions. A histologically correct diagnosis of benign or malignant was rendered by cytology in 85% of the cystic cases, with 4% false negatives and 2% false positives. This diagnostic accuracy was similar to that of the solid lesions. The FNA diagnostic problems were concentrated in the salivary gland lesions, with the false diagnoses illustrating the difficulties of interpreting atypical oxyphilic epithelium. The results suggest that a true neoplastic lesion should be seriously considered when a single population of oxyphilic epithelium is identified, even at the risk of overdiagnosing benign lesions.

  17. Giant Axonal Neuropathy

    MedlinePlus

    ... Diversity Find People About NINDS NINDS Giant Axonal Neuropathy Information Page Table of Contents (click to jump ... done? Clinical Trials Organizations What is Giant Axonal Neuropathy? Giant axonal neuropathy (GAN) is a rare inherited ...

  18. 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

  19. Retroperitoneal cystic lymphangioma.

    PubMed

    Nuzzo, G; Lemmo, G; Marrocco-Trischitta, M M; Boldrini, G; Giovannini, I

    1996-03-01

    Two cases of retroperitoneal cystic lymphangioma (CL) are presented; the current literature on this rare, benign neoplasm of the lymphatic system is reviewed. This tumor consists of various numbers of cyst-like cavities filled with a serous, serosanguineous or chylous fluid. The histogenesis of CL is still uncertain. Most commonly CL occurs in the neck and in the axillary region, whereas it is rare in the retroperitoneum. Although retroperitoneal CL is a benign lesion, it may cause significant morbidity due to its large size, and its often invasive character with a strong tendency to secondary infection. The treatment of choice is surgical excision.

  20. Sebaceous carcinoma arising in benign cystic teratoma of the ovary. Case report.

    PubMed

    Betta, P G; Cosimi, M F

    1984-01-01

    A case of sebaceous carcinoma in a benign cystic teratoma of the ovary is reported. Histogenesis is from adnexal sebaceous glands. Sebaceous metaplasia of either a squamous cell carcinoma or a salivary-gland malignant variant are excluded on histological ground.

  1. 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

  2. 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

  3. Diffuse malignant biphasic peritoneal mesothelioma with cystic areas.

    PubMed

    Cabibi, Daniela; Tutino, Roberta; Salamone, Giuseppe; Cocorullo, Gianfranco; Agrusa, Antonino; Gulotta, Gaspare

    2016-06-20

    Il mesotelioma peritoneale maligno è una patologia rara la cui diagnosi è resa particolarmente difficile dalla molteplicità delle possibili presentazioni cliniche e morfologiche. A differenza della patologia pleurica, questa sembra avere una minore correlazione con l’esposizione all’asbesto. La prognosi è sfavorevole con una sopravvivenza media di 5.4 mesi. Riportiamo un caso di mesotelioma peritoneale in un paziente di 73 anni con esposizione professionale all’asbesto. La diagnosi è verosimilmente avvenuta dopo diversi inquadramenti clinici, che hanno compreso addominalgie attribuite ad IBD, poi non accertate istologicamente, e ad un’appendicite acuta. Per ultimo il paziente è giunto alla nostra osservazione con un quadro subocclusivo che l’indagine TC attribuiva ad una verosimile carcinosi peritoneale a primitivo sconosciuto. La laparoscopia esplorativa, poi convertita in approccio laparotomico, ha mostrato un quadro di peritonite incapsulante che coinvolgeva estesamente l’intestino tenue. È stata eseguita una resezione intestinale del tenue coinvolto ed una asportazione di un’area nodulare peri-splenica. L’esame istologico con l’integrazione di indagini immunoistochimiche ha permesso la diagnosi di mesotelioma peritoneale cistico maligno con istotipo bifasico che risulta essere il primo descritto in letteratura. Le aree cistiche avevano inizialmente fatto propendere per una diagnosi di mesotelioma multicistico benigno o di mesotelioma cistico con secondaria proliferazione miofibroblastica pseudosarcomatosa. Negli ultimi anni la prognosi di questa patologia ha trovato nell’utilizzo della chirurgia citoriduttiva e della chemioterapia intraperitoneale un miglioramento prognostico, queste nuove tecniche sono disponibili in centri di riferimento e trovano ad oggi indicazione solo in casi selezionati e in istotipi poco aggressivi. Il nostro paziente a causa dell’estensione della malattia, dell’istotipo non favorevole e dell’età avanzata è stato riferito al servizio di oncologia per il prosieguo delle cure. E’ stato sottoposto a chemioterapia con Pemetrexed e Cisplatino e dopo sei mesi presenta soddisfacenti condizioni cliniche generali e la TC di controllo non ha mostrato progressione di malattia.

  4. [Retroperitoneal cystic lymphangioma].

    PubMed

    Waisberg, J; Pezzolo, S; Henrique, A C; Kerr, L M; Speranzini, M B

    1999-01-01

    The lymphangioma is a rare disease, more frequently reported in children and just occasionally in the adult patient. The lymphangioma is considered a benign neoplasm of embryonic origin of the lymphatic vessels. Its habitual location is in the cervical and axillary area; it is rarely found in the abdominal cavity and exceptionally in the retroperitonio. In this latter location, the lesion habitually is asymptomatic. The clinical diagnosis of the retroperitoneal cystic lymphangioma is not often due to its rarity and the absence of clinical expression. The size of the lesion is more important than its location to the symptomatology development. The findings of the abdominal ultrasonography and computerized tomography of the abdomen usually show a cystic lesion and its location. The treatment is surgical and it consists of the resection of the cyst or group of cysts once the liquid accumulation in its interior may be responsible for the development of some important complications of this disease. The cure is obtained when the lesion is completely resected also with the resection of eventual adhesive structures. The relapse may take place when the resection is incomplete. A case of retroperitoneal lymphangioma in a female adult patient as incidental finding of abdominal ultra-sonography is described. It is discussed the clinical picture, the radiologic diagnosis, the treatment and the prognostic of this unusual disease.

  5. 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

  6. Laparoscopic resection of a giant mesenteric cyst.

    PubMed

    Saw, E C; Ramachandra, S

    1994-02-01

    Laparoscopic resection of a giant retroperitoneal, mesenteric cyst in a 38-year-old man who presented with abdominal distension and pedal edema is described. The diagnosis was made by abdominal computed tomography, which revealed a large cystic mass that was causing extrinsic compression of the inferior vena cava and the right ureter. The hospital course was uneventful, and the patient was discharged 2 days postoperatively. This new endoscopic approach offers a useful alternative to the traditional transabdominal excision of a mesenteric cyst and may have some theoretical advantages, including less postoperative pain and shorter convalescence.

  7. Fine-needle aspiration study of cystic papillary thyroid carcinoma: Rare cytological findings

    PubMed Central

    Mokhtari, Maral; Kumar, Perikala Vijayananda; Hayati, Kamran

    2016-01-01

    Background: Cystic papillary thyroid carcinoma (CPTC) is a variant of papillary carcinoma that has many mimickers in cytological grounds. Aim: To study the cytomorphologic features of CPTC and compare them to those of other cystic thyroid lesions using fine-needle aspiration cytology (FNAC). We also aimed to identify the cytomorphologic features that distinguish CPTC from other cystic thyroid lesions. Materials and Methods: Seventy-three cases of CPTC were included in the study. The cytomorphologic features of these cases were analyzed. The FNA smears of other thyroid lesions with cystic changes (300 colloid goiters, 290 adenomatoid nodules, 11 follicular neoplasms, and 9 hurtle cell neoplasm) were also studied. Results: The smears in CPTC revealed isolated follicular cells, small groups of cells with scalloped margins, cell swirls, small clusters with a cartwheel pattern, papillary clusters, intranuclear inclusions, nuclear grooves, sticky colloid, intracellular colloids, psammoma bodies, multinucleated giant cells, and foamy and hemosiderin laden macrophages. Small groups of cells with scalloped borders, cellular swirls, and small clusters with a cartwheel pattern were seen in CPTC, but not in other cystic lesions. Interestingly, mesothelial-like cells and hemophagocytic cells were seen in five and three cases of CPTC, respectively, but not in other cystic lesions. Conclusion: Mesothelial-like cells and hemophagocytic cells were observed in five and three cases of CPTC, respectively. Similar finding have not been previously reported in the literature.

  8. Nutritional Issues in Cystic Fibrosis.

    PubMed

    Solomon, Missale; Bozic, Molly; Mascarenhas, Maria R

    2016-03-01

    The importance of maintaining adequate nutrition in patients with cystic fibrosis has been well known for the past 3 decades. Achieving normal growth and maintaining optimal nutrition is associated with improved lung function. Comprehensive and consistent nutritional assessments at regular intervals can identify those at risk of nutritional failure and uncover micronutrient deficiencies contributing to malnutrition. Management of malnutrition in cystic fibrosis should follow a stepwise approach to determine the causes and comorbidities and to develop a nutritional plan. Nutritional management is crucial at every stage in a person's life with cystic fibrosis and remains a cornerstone of management.

  9. Paraovarian cystic endosalpingiosis in association with tamoxifen therapy

    PubMed Central

    McCluggage, W; Weir, P

    2000-01-01

    This report describes a case of macroscopically visible cystic endosalpingiosis involving the paraovarian region in a woman who had been taking tamoxifen for breast cancer. A 2.5 cm multicystic lesion was seen on the external surface of the right ovary and histological examination showed a mass of dilated glands lined by ciliated tubal-type epithelium and set in a fibrovascular stroma. Cystic endosalpingiosis resulting in a tumour-like mass is a rarely described entity which is probably not well recognised by histopathologists. Although unlikely to be mistaken for malignancy, the lesion may result in diagnostic confusion. The role of tamoxifen in the development of the lesion in the present case is not clear but the oestrogenic effects of this drug may have contributed to its formation. Key Words: ovary • paraovarian region • endosalpingiosis, tamoxifen PMID:10767837

  10. Rare Case of Ovarian Cystic Lymphangioma Managed at Laparoscopy.

    PubMed

    Naik, Sejal Arunbhai

    2011-01-01

    Lymphangiomas are rare, usually benign tumors of the lymphatic system. Lymphangiomas may arise in any part of the body. Lymphangioma of the ovary is very rare tumor, which is usually slow-growing, remains asymptomatic for a long time, and identified incidentally at histopathological examination after excision. It is advisable to excise the lesion with microscopically clear margins. Many times, diagnosis becomes difficult because of confusion with malignant ovarian mass, especially in post-menopausal woman. A 72-year-old post-menopausal woman had a symptomatic pelvic mass. Imaging studies demonstrated a complex right ovarian cyst. Laparoscopic total hysterectomy with bilateral salpingo-oophorectomy was performed successfully. Cystic Lymphangioma should be included in the differential diagnosis of an ovarian cystic mass, and laparoscopic excision may be the method of treatment. PMID:26085753

  11. Rare Case of Ovarian Cystic Lymphangioma Managed at Laparoscopy

    PubMed Central

    Naik, Sejal Arunbhai

    2011-01-01

    Lymphangiomas are rare, usually benign tumors of the lymphatic system. Lymphangiomas may arise in any part of the body. Lymphangioma of the ovary is very rare tumor, which is usually slow-growing, remains asymptomatic for a long time, and identified incidentally at histopathological examination after excision. It is advisable to excise the lesion with microscopically clear margins. Many times, diagnosis becomes difficult because of confusion with malignant ovarian mass, especially in post-menopausal woman. A 72-year-old post-menopausal woman had a symptomatic pelvic mass. Imaging studies demonstrated a complex right ovarian cyst. Laparoscopic total hysterectomy with bilateral salpingo-oophorectomy was performed successfully. Cystic Lymphangioma should be included in the differential diagnosis of an ovarian cystic mass, and laparoscopic excision may be the method of treatment. PMID:26085753

  12. Giant Lipoma of Posterior Cervical Region

    PubMed Central

    Kumar, Lovekesh; Karande, Snehal K.; Kolhe, Yuvraj

    2014-01-01

    Lipomas are the slow growing soft tissue tumors of benign nature. They commonly grow on torso and extremities but may also develop in head and neck region. Rarely lipomas can grow to acquire gigantic proportions, turning into an entity termed as giant lipoma. Such lipomas are entitled to immediate attention as they have a relatively high malignant potential. We report a rare case of giant cervical lipoma in an elderly gentleman, followed by a brief discussion on diagnosis and management of the disorder. PMID:25349767

  13. Malignant hyperthermia.

    PubMed

    Cantin, R Y; Poole, A; Ryan, J F

    1986-10-01

    The increasing use of intravenous and inhalation sedation in the dental office has the potential of increasing the incidence of malignant hyperthermia (MH) in susceptible subjects. The object of this article is to present two cases of MH and to discuss its pathophysiology, its clinical picture, and its management in the light of the current literature. Stringent screening procedures should be adopted and maintained in order to channel suspected cases to appropriate centers for expert consultation and management. It is further advocated that a program of education for patients and their families be instituted, as it is an essential prerequisite of effective prophylaxis. PMID:2946013

  14. Pneumothorax in cystic fibrosis

    PubMed Central

    Kioumis, Ioannis P.; Zarogoulidis, Konstantinos; Huang, Haidong; Li, Qiang; Dryllis, Georgios; Pitsiou, Georgia; Machairiotis, Nikolaos; Katsikogiannis, Nikolaos; Papaiwannou, Antonis; Lampaki, Sofia; Porpodis, Konstantinos; Zaric, Bojan; Branislav, Perin; Mpoukovinas, Ioannis; Lazaridis, George

    2014-01-01

    Pneumothorax is recognized as a common and life-threatening complication in cystic fibrosis (CF) patients, especially in those who are infected with P. aeruginosa, B. cepacia or Aspergillus, need enteral feeding, are diagnosed as suffering from allergic bronchopulmonary aspergillosis (ABPA), developed massive hemoptysis, and their respiratory function is seriously compromised. Structural impairment and altered airflow dynamics in the lungs of CF patients are considered as the main predisposing factors, but also inhaled medications and non-invasive positive pressure ventilation (NIPPV) could increase the risk of pneumothorax. Clinical presentation could range from dramatic to very mild. Management of spontaneous pneumothorax occurring to patients with CF is essentially similar to that for non-CF patients. Therapeutic options include intercostal tube drainage, video-assisted thoracoscopic surgery (VATS), and medical or surgical pleurodesis. Pneumothorax increases both short- and long-term morbidity and mortality in CF patients and causes significant deterioration of their quality of life. PMID:25337406

  15. Malignant hyperthermia

    PubMed Central

    2012-01-01

    Malignant hyperthermia (MH) is an uncommon, life-threatening pharmacogenetic disorder of the skeletal muscle. It presents as a hypermetabolic response in susceptible individuals to potent volatile anesthetics with/without depolarizing muscle relaxants; in rare cases, to stress from exertion or heat stress. Susceptibility to malignant hyperthermia (MHS) is inherited as an autosomally dominant trait with variable expression and incomplete penetrance. It is known that the pathophysiology of MH is related to an uncontrolled rise of myoplasmic calcium, which activates biochemical processes resulting in hypermetabolism of the skeletal muscle. In most cases, defects in the ryanodine receptor are responsible for the functional changes of calcium regulation in MH, and more than 300 mutations have been identified in the RYR1 gene, located on chromosome 19q13.1. The classic signs of MH include increase of end-tidal carbon dioxide, tachycardia, skeletal muscle rigidity, tachycardia, hyperthermia and acidosis. Up to now, muscle contracture test is regarded as the gold standard for the diagnosis of MHS though molecular genetic test is used, on a limited basis so far to diagnose MHS. The mortality of MH is dramatically decreased from 70-80% to less than 5%, due to an introduction of dantrolene sodium for treatment of MH, early detection of MH episode using capnography, and the introduction of diagnostic testing for MHS. This review summarizes the clinically essential and important knowledge of MH, and presents new developments in the field. PMID:23198031

  16. Adenoid cystic carcinoma presenting as an ulcer on the floor of the mouth: a rare case report

    PubMed Central

    Agwani, Khalid; Bhargava, Puneet; Kumar, Sreeja P.

    2014-01-01

    Adenoid cystic carcinoma is a rare epithelial tumour, and comprises about 1% of all malignant tumours of the oral and maxillofacial region. It is a malignant tumour which may develop in the trachea, bronchus, lungs or mammary glands, in addition to the head and neck region. Occurrences in the head and neck are mostly detected in the major salivary gland, oral cavity, pharynx and paranasal sinus where it presents as a slow growing firm nodular swelling. The aim of the article is to highlight the unique presentation of adenoid cystic carcinoma as a solitary ulcer on the floor of the mouth. PMID:25368840

  17. [Cystic testicular lesions in infancy].

    PubMed

    Calleja Escudero, J; Pascual Samaniego, M; Garrido Redondo, M; Matas Gómez, V; Fernández Domínguez, L; Fernández del Busto, E

    2004-09-01

    The present article reports a case 11 month-old infant with a right intratesticular cyst. We analyze the etiology, differential diagnosis and management off all cystic lesions of the pediatric testis. Patient age at presentation, examination features, tumor markers and sonographic appearance may assist in making a presumptive and occasionally definitive diagnosis preoperatively. The differential diagnosis include intratesticular simple cyst, epidermoid cyst, tunica albuginea cyst, testicular teratoma, juvenil granulosa cell tumor-gonadal stromal tumor, cystic dysplasia of the rete testis, cystic lymphangioma, and testicular torsion. Usually enucleation is the best treatment. A thorough understanding of potentially cystic testis lesions in children leads to the best management choices and often to preservation of a substantial portion of the affected testis.

  18. 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 ...

  19. Giant Pyogenic Granuloma in a Patient with Chronic Lymphocytic Leukemia

    PubMed Central

    Plovanich, Molly; Tsibris, Hillary C.; Lian, Christine G.; Mostaghimi, Arash

    2014-01-01

    Pyogenic granuloma, also known as lobular capillary hemangioma, is a common benign vascular proliferative lesion that can present at any age on the skin or mucous membranes. Most lesions do not exceed 2 cm, but there are a handful of giant cutaneous pyogenic granulomas that have been reported, often in individuals with underlying immune dysfunction. Here, we report the first giant pyogenic granuloma in a patient with a hematological malignancy, chronic lymphocytic leukemia. PMID:25408651

  20. Aggressive Metaplastic Carcinoma of the Breast with Osteoclastic Giant Cells.

    PubMed

    Khong, Kathleen; Zhang, Yanhong; Tomic, Mary; Lindfors, Karen; Aminololama-Shakeri, Shadi

    2015-09-01

    Metaplastic carcinoma of the breast is an uncommon type of malignancy that is aggressive but can mimic other benign breast neoplastic processes on imaging. We present a case of a young female patient who presented with a rapidly progressing metaplastic carcinoma with osteoclastic giant cells subtype. There have been only very rare published reports of this pathologic subtype of metaplastic carcinoma containing osteoclastic giant cells.

  1. Aggressive Metaplastic Carcinoma of the Breast with Osteoclastic Giant Cells

    PubMed Central

    Khong, Kathleen; Zhang, Yanhong; Tomic, Mary; Lindfors, Karen; Aminololama-Shakeri, Shadi

    2015-01-01

    Metaplastic carcinoma of the breast is an uncommon type of malignancy that is aggressive but can mimic other benign breast neoplastic processes on imaging. We present a case of a young female patient who presented with a rapidly progressing metaplastic carcinoma with osteoclastic giant cells subtype. There have been only very rare published reports of this pathologic subtype of metaplastic carcinoma containing osteoclastic giant cells. PMID:26629304

  2. Giant Magnons Meet Giant Gravitons

    SciTech Connect

    Hofman, Diego M.

    2008-07-28

    We study the worldsheet reflection matrix of a string attached to a D-brane in AdS{sub 5}xS{sup 5}. The D-brane corresponds to a maximal giant graviton that wraps an S{sup 3} inside S{sup 5}. In the gauge theory, the open string is described by a spin chain with boundaries. We focus on open strings with a large SO(6) charge and define an asymptotic boundary reflection matrix. Using the symmetries of the problem, we review the computation of the boundary reflection matrix, up to a phase. We also discuss weak and strong coupling computations where we obtain the overall phase factor and test our exact results.

  3. Malignant mesothelioma

    PubMed Central

    Ahmed, Ishtiaq; Ahmed Tipu, Salman; Ishtiaq, Sundas

    2013-01-01

    Malignant Mesothelioma (MM) is a rare but rapidly fatal and aggressive tumor of the pleura and peritoneum with limited knowledge of its natural history. The incidence has increased in the past two decades but still it is a rare tumor. Etiology of all forms of mesothelioma is strongly associated with industrial pollutants, of which asbestos is the principal carcinogen. Mesothelioma is an insidious neoplasm arising from mesothelial surfaces i.e., pleura (65%-70%), peritoneum (30%), tunica vaginalis testis, and pericardium (1%-2%). The diagnosis of peritoneal and Pleural mesothelioma is often delayed, due to a long latent period between onset and symptoms and the common and nonspecific clinical presentation. The definite diagnosis can only be established by diagnostic laparoscopy or open surgery along with biopsy to obtain histological examination and immunocytochemical analysis. Different treatment options are available but Surgery can achieve a complete or incomplete resection and Radical resection is the preferred treatment. Chemotherapy has an important role in palliative treatment. Photodynamic therapy is also an option under trial. Patients who successfully underwent surgical resection had a considerably longer median survival as well as a significantly higher 5-year survival. Source of Data/Study Selection: The data were collected from case reports, cross-sectional studies, Open-label studies and phase –II trials between 1973-2012. Data Extraction: Web sites and other online resources of American college of surgeons, Medline, NCBI and Medscape resource centers were used to extract data. Conclusion: Malignant Mesothelioma (MM) is a rare but rapidly fatal and aggressive tumor with limited knowledge of its natural history. The diagnosis of peritoneal and Pleural mesothelioma is often delayed, so level of index of suspicion must be kept high. PMID:24550969

  4. Giant pleomorphic adenoma of the parotid gland.

    PubMed

    Takahama, Ademar; da Cruz Perez, Danyel Elias; Magrin, José; de Almeida, Oslei Paes; Kowalski, Luiz Paulo

    2008-01-01

    Pleomorphic adenoma is the most common type of all benign and malignant salivary gland tumors, involving more frequently the parotid gland. It is a benign tumor with a slow and continuous growth that without treatment can reach an enormous size. We present a case of a giant pleomorphic adenoma in a 78-year-old man with a history of more than 30 years of a growing lesion in the parotid gland. Clinical examination revealed a giant mass on the right side of the face, however without any sign of facial nerve damage. The tumor was completely resected by total parotidectomy and preservation of the facial nerve. Macroscopically, the tumor measured 28 cm and weighed 4.0 Kg. On the histological examination there was a predominance of epithelial and myoepithelial cells in a hyaline and myxoid stroma. It was not found any area of malignant transformation. In the post-operatory the aesthetic and functional results were excellent.

  5. [Therapy-resistant aggressive cystic lesion of the mandible].

    PubMed

    Ziegler, C M; Bergstrand, S; Lund, J-A; Viset, T

    2011-10-01

    Several extensive surgical interventions of a cystic lesion in the left mandible were followed by recurrences. The lesion extended from the primary mandibular region into the area of pterygopalatine fossa finally infiltrating the orbital region and the skull base. Histological results could never demonstrate a malignancy with certainty. Due to the patient's poor general condition, the refusal for further surgical inventions and due to the malignoma-like growth pattern radiation treatment was performed. However, this had no effect on tumor progression. PMID:21845481

  6. [Clinical and histological studies of adenoid cystic carcinoma].

    PubMed

    Semba, O; Sato, T; Miyahara, H; Yoshino, K; Umatani, K; Ishiguro, S

    1983-04-01

    Forty-four salivary gland tumors were seen at The Center for Adult Diseases, Osaka, between 1979 and 1981; 30 were benign and 14 malignant. Of the latter, 7 (50%) were adenoid cystic carcinoma (ACC). Six of 7 ACC were reviewed with special attention to their histologic pattern, atypism, connective tissue around the cell nest, tumor cell nest, tumor cell invasion into lymphatics(ly) and blood vessels(v), and mitosis. ACC was divided into 3 histological groups. Patients with ly and/or v develop cervical and/or distant metastases at the early stage or during the follow-up period. Ly and v were closely correlated with metastasis.

  7. MR imaging features of giant reservoir formation in the orbit: an unusual complication of Ahmed glaucoma valve implantation.

    PubMed

    Jeon, T Y; Kim, H-J; Kim, S T; Chung, T-Y; Kee, C

    2007-09-01

    We report the MR imaging findings of an unusually large fluid collection, so-called giant reservoir, around an Ahmed glaucoma valve implant that caused progressive proptosis in a patient with posttraumatic glaucoma. Although other cystic lesions of the juxtalacrimal area of the orbit, such as dermoid cyst, can present similar imaging findings, the characteristic morphology and location should lead the radiologist to the correct nature of this cystic lesion.

  8. Adenoid cystic carcinoma of the palate: case report and review of literature.

    PubMed

    Yaga, Uday Shankar; Gollamudi, Nishanth; Mengji, Ashwini Kumar; Besta, Radhika; Panta, Prashanth; Prakash, Bhanu; Rajashekar, Edunuri

    2016-01-01

    Adenoid Cystic Carcinoma (ACC) is a rare tumor constitutes for less than 1% of head and neck malignancies and 10% of all salivary gland tumors. Palate is the most common site to be involved in the oral cavity followed by parotid gland and submandibular gland. They are usually asymptomatic, slow growing, characteristically shows infiltrative growth and perineural invasion. This paper reports a case of Adenoid Cystic Carcinoma in a 35 year old female man reported with a swelling on the left side of palate involving the hard and soft palate since 8 months which was diagnosed histopathologically and review of literature of the peculiar clinical, and histopathological features. PMID:27642445

  9. Recurrent renal giant leiomyosarcoma.

    PubMed

    Öziş, Salih Erpulat; Gülpınar, Kamil; Şahlı, Zafer; Konak, Baha Burak; Keskin, Mete; Özdemir, Süleyman; Ataoğlu, Ömür

    2016-01-01

    Primary renal leiomyosarcomas are rare, aggressive tumors. They constitute 1-2% of adult malignant renal tumors. Although leiomyosarcomas are the most common histological type (50-60%) of renal sarcomas, information on renal leiomyosarcoma is limited. Local or systemic recurrences are common. The radiological appearance of renal leiomyosarcomas is not specific, therefore renal leiomyosarcoma cannot be distinguished from renal cell carcinoma by imaging methods in all patients. A 74-year-old female patient presented to our clinic complaining of a palpable mass on the right side of her abdomen in November 2012. The abdominal magnetic resonance imaging revealed a mass, 25 × 24 × 23 cm in size. Her past medical history revealed that she has undergone right radical nephrectomy in 2007, due to a 11 × 12 × 13 cm renal mass that was then reported as renal cell carcinoma on abdominal magnetic resonance imaging, but the pathological diagnosis was low-grade renal leiomyosarcoma. The most recent follow-up of the patient was in 2011, with no signs of local recurrence or distant metastases within this four-year period. The patient underwent laparotomy on November 2012, and a 35 cm retroperitoneal mass was excised. The pathological examination of the mass was reported as high-grade leiomyosarcoma. The formation of this giant retroperitoneal mass in 1 year can be explained by the transformation of the lesion's pathology from low-grade to a high-grade tumor. PMID:27436926

  10. A popliteal giant synovial osteochondroma mimicking a parosteal osteosarcoma

    PubMed Central

    2013-01-01

    Both giant synovial osteochondroma and parosteal osteosarcoma are rare musculo-skeletal tumors, often localized in the vicinity of the knee. Misdiagnosis of a malignant bone tumor can entail fatal consequences. Etiology of giant synovial osteochondroma is widely unsolved but is believed to originate from synovial chondromatosis, a mostly benign metaplasia of the synovial membrane. Parosteal osteosarcoma is a low-grade surface osteosarcoma with a propensity of local recurrence and the potential of distant metastasis and therefore requiring a different therapeutical approach. We report the case of a popliteal giant osteochondroma mimicking a parosteal osteosarcoma. Relevant facts of this rare entity regarding pathogenesis, treatment, and differential diagnoses will be discussed. PMID:24066980

  11. 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

  12. 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

  13. 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

  14. [Malignant hyperthermia].

    PubMed

    Metterlein, T; Schuster, F; Graf, B M; Anetseder, M

    2014-12-01

    Malignant hyperthermia (MH) is a rare hereditary, mostly subclinical myopathy. Trigger substances, such as volatile anesthetic agents and the depolarizing muscle relaxant succinylcholine can induce a potentially fatal metabolic increase in predisposed patients caused by a dysregulation of the myoplasmic calcium (Ca) concentration. Mutations in the dihydropyridine ryanodine receptor complex in combination with the trigger substances are responsible for an uncontrolled release of Ca from the sarcoplasmic reticulum. This leads to activation of the contractile apparatus and a massive increase in cellular energy production. Exhaustion of the cellular energy reserves ultimately results in local muscle cell destruction and subsequent cardiovascular failure. The clinical picture of MH episodes is very variable. Early symptoms are hypoxia, hypercapnia and cardiac arrhythmia whereas the body temperature rise, after which MH is named, often occurs later. Decisive for the course of MH episodes is a timely targeted therapy. Following introduction of the hydantoin derivative dantrolene, the previously high mortality of fulminant MH episodes could be reduced to well under 10 %. An MH predisposition can be detected using the invasive in vitro contracture test (IVCT) or mutation analysis. Few elaborate diagnostic procedures are in the developmental stage. PMID:25384957

  15. Mucinous Cystic Neoplasms of Pancreas

    PubMed Central

    Naveed, Shah; Qari, Hasina; Banday, Tanveer; Altaf, Asma; Para, Mah

    2014-01-01

    The purpose of this study was to investigate the actual management of mucinous cystic neoplasm (MCN) of the pancreas. A systematic review was performed in December 2009 by consulting PubMed MEDLINE for publications and matching the key words “pancreatic mucinous cystic neoplasm”, “pancreatic mucinous cystic tumor”, “pancreatic mucinous cystic mass”, “pancreatic cyst” and “pancreatic cystic neoplasm” to identify English language articles describing the diagnosis and treatment of the MCN of the pancreas. In total, 16,322 references ranging from January 1969 to December 2009 were analyzed and 77 articles were identified. No articles published before 1996 were selected because MCNs were not previously considered to be a completely autonomous disease. Definition, epidemiology, anatomopathological findings, clinical presentation, preoperative evaluation, treatment and prognosis were reviewed. MCNs are pancreatic mucin-producing cysts with a distinctive ovarian-type stroma localized in the body-tail of the gland and occurring in middle-aged females. The majority of MCNs are slow growing and asymptomatic. The prevalence of invasive carcinoma varies between 6% and 55%. Preoperative diagnosis depends on a combination of clinical features, tumor markers, computed tomography (CT), magnetic resonance imaging, endoscopic ultrasound with cyst fluid analysis and positron emission tomography-CT. Surgery is indicated for all MCNs.

  16. Transforming giants.

    PubMed

    Kanter, Rosabeth Moss

    2008-01-01

    Large corporations have long been seen as lumbering, inflexible, bureaucratic--and clueless about global developments. But recently some multinationals seem to be transforming themselves: They're engaging employees, moving quickly, and introducing innovations that show true connection with the world. Harvard Business School's Kanter ventured with a research team inside a dozen global giants--including IBM, Procter & Gamble, Omron, CEMEX, Cisco, and Banco Real--to discover what has been driving the change. After conducting more than 350 interviews on five continents, she and her colleagues came away with a strong sense that we are witnessing the dawn of a new model of corporate power: The coordination of actions and decisions on the front lines now appears to stem from widely shared values and a sturdy platform of common processes and technology, not from top-down decrees. In particular, the values that engage the passions of far-flung workforces stress openness, inclusion, and making the world a better place. Through this shift in what might be called their guidance systems, the companies have become as creative and nimble as much smaller ones, even while taking on social and environmental challenges of a scale that only large enterprises could attempt. IBM, for instance, has created a nonprofit partnership, World Community Grid, through which any organization or individual can donate unused computing power to research projects and see what is being done with the donation in real time. IBM has gained an inspiring showcase for its new technology, helped business partners connect with the company in a positive way, and offered individuals all over the globe the chance to contribute to something big.

  17. 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.

  18. Giant congenital nevus

    MedlinePlus

    ... pigmented nevus; Giant hairy nevus; Giant pigmented nevus; Bathing trunk nevus; Congenital melanocytic nevus - large ... baby grows in the womb. In some families bathing trunk nevi may be inherited. The condition may ...

  19. Giant Cell Arteritis

    MedlinePlus

    Giant cell arteritis is a disorder that causes inflammation of your arteries, usually in the scalp, neck, and arms. ... arteries, which keeps blood from flowing well. Giant cell arteritis often occurs with another disorder called polymyalgia ...

  20. A Case of Bilateral Cystic Partially Differentiated Nephroblastoma vs Cystic Wilms' Tumor: Highlighting a Diagnostic Dilemma.

    PubMed

    Stout, Thomas E; Au, Jason K; Hicks, J M; Gargollo, Patricio C

    2016-06-01

    Cystic partially differentiated nephroblastoma (CPDN) is a rare multicystic renal tumor along the spectrum of cystic nephroma and cystic Wilms' tumor. There have only been two previously reported cases of bilateral CPDN in the literature. We present here a case of bilateral CPDN vs cystic Wilms' tumor treated with neoadjuvant and adjuvant chemotherapy in addition to a bilateral partial nephrectomy. We also review the relevant literature regarding CPDN in an effort to aid in diagnosis and management of these rare cystic renal tumors.

  1. [Abdominal cystic tumor revealing lymphangioleiomyomatosis].

    PubMed

    Barbier, L; Ebbo, M; Andrac-Meyer, L; Schneilitz, N; Le Treut, Y-P; Reynaud-Gaubert, M; Hardwigsen, J

    2009-02-01

    We report the case of a 39 year-old woman with many years of intermittent abdominal pain who was found to have cystic masses evocative of cystic lymphangioma involving the posterior mediastinal and retroperitoneum. Worsening abdominal pain led to a recommendation for laparoscopic unroofing and decompression of the cysts. During the postoperative period, hemorrhagic shock required reintervention with excision of the tumoral mass. Pathologic examination revealed lymphangioleiomyomatosis (LAM). On the 15th postoperative day, the patient developed a chylopneumothorax which required prolonged chest tube drainage. The presence of multiple polycystic lesions in the pulmonary parenchyma supported the diagnosis of diffuse LAM with primary extrapulmonary presentation. This diagnosis should be considered preoperatively since it modifies the treatment: a complete excision of the cystic lesions seems to be necessary in order to prevent bleeding and lymphatic extravasation.

  2. Acquired cystic kidney disease.

    PubMed

    Levine, E

    1996-09-01

    ACKD is characterized by the development of many fluid-filled renal cysts and sometimes neoplasms in the kidneys of individuals with chronic renal failure but without a history of hereditary cystic disease. The condition is seen mainly in dialysis patients, but often begins in patients with ESRD before dialysis is started. Most patients with ACKD are asymptomatic, but the disorder may be associated with such serious complications as retroperitoneal hemorrhage and metastatic renal cell carcinoma. The diagnosis of ACKD and its complications is best achieved by CT scanning, although US and MR imaging may be useful in evaluation, particularly in patients not treated with dialysis. Cyst hemorrhage is common in ACKD and may cause flank pain and hematuria. Hemorrhagic cysts may be recognized by their CT scan, sonographic, or MR imaging features. Hemorrhagic cysts may rupture into the perinephric space causing large perinephric hematomas. These can usually be treated-conservatively. Patients with ACKD, particularly those treated with dialysis, have an increased risk of renal cell carcinoma. Renal cell carcinoma may also develop in the native kidneys of renal transplant recipients with good graft function many years after transplantation. Annual imaging of the native kidneys of all dialysis patients or of transplant recipients for the development of carcinoma is not justified, however, because it has not been shown to have a significant effect on patient outcome. Screening may, however, be useful in selected dialysis patients with good general medical condition and who have known risk factors for renal cell carcinoma including prolonged dialysis, large kidneys, ACKD, and male gender. Screening of the native kidneys of transplant recipients may be performed when they are referred for US evaluation of the renal allograft.

  3. Smell in cystic fibrosis.

    PubMed

    Lindig, J; Steger, C; Beiersdorf, N; Michl, R; Beck, J F; Hummel, T; Mainz, J G

    2013-03-01

    In cystic fibrosis (CF), the most frequent life threatening inherited disease in Caucasians, sinonasal mucosa is regularly affected by defective mucociliary clearance. This facilitates pathogen colonization into CF airways and causes frequent symptoms of rhinosinusitis, including an impaired sense of smell. Despite probable effects on nutrition and overall health, CF-rhinosinusitis is little understood: CF-associated smelling deficiencies reported in literature vary between 12 and 71 %. The aim of this study was to assess olfactory and gustatory function in relation to sinonasal symptoms and sinonasal colonization, and lung function and nutrition. Thirty-five CF patients of different ages were compared to 35 age-matched healthy controls. Olfactory function was assessed by 'Sniffin'Sticks', gustatory qualities by "Taste-strips", and symptoms by sino-nasal outcome test 20 (SNOT-20). Normosmia was found in 62.8 % of healthy controls but only in 28.6 % of CF patients. In contrast the majority of CF patients exhibited a smell loss; almost 62.9 % of them were hyposmic, and 8.6 % functionally anosmic. Importantly, reduced olfactory function only affected odor thresholds, which were significantly increased in CF, not odor identification. This suggests that the olfactory dysfunction in CF results from the olfactory periphery due to either problems in conduction and/or a functional lesion due to the inflammatory process. SNOT-20 scores increased continuously from normosmic to hyposmic and anosmic CF patients (means 7.2/11.1/28.3 points). Neither sinonasal pathogen colonization, gender, pulmonary function, nor allergy or sinonasal surgery appeared to have significant effects on olfactory function and taste. Olfactory disorders are considerably more frequent in CF patients than in age-matched healthy controls. Assessing these parameters within CF-routine care should be considered because of their importance to nutrition and, thus, overall therapy outcome.

  4. [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.

  5. Newborn intraabdominal cystic lymphatic malformations.

    PubMed

    Lin, J I; Fisher, J; Caty, M G

    2000-08-01

    Cystic lymphatic malformations are rare causes of abdominal masses in the newborn. Also known as mesenteric, omental, or retroperitoneal cysts, they can present in a variety of ways including, intestinal obstruction, volvulus, nonspecific abdominal pain, intracystic hemorrhage, or as an asymptomatic abdominal mass. Abdominal ultrasound scan provides a definitive diagnosis in most suspected cases. Complete resection is possible in most patients except those with extensive retroperitoneal involvement. Recurrence is unusual when complete resection is accomplished. Because most case series with complete data suggest that these entities are lymphatic malformations, the authors suggest the more specific term, cystic lymphatic malformations, be used to describe these lesions.

  6. [Adrenal cystic masses. Our experience].

    PubMed

    Costantino, V; Petrin, P; Da Lio, C; Zaramella, D; Pedrazzoli, S

    1993-10-01

    Cystic masses of the adrenal gland are clinically and pathologically rare findings and few cases have been reported up to now in the medical literature. In the present work 5 new cases are reported: 3 adrenal pseudocysts, 1 lymphangioma, 1 cystic pheochromocytoma. In 3 cases there were clinical symptoms of retroperitoneal mass (lumbar pain, palpable mass, digestive symptoms); in 3 cases conventional radiology was helpful; ultrasonography was used for diagnosis in 1, CT scan in 2. In the pheochromocytoma case the real nature of the mass was determined through fluid hormone determination after fine needle puncture. All cases were treated by surgery.

  7. [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

  8. Giant impacts on giant planets

    NASA Astrophysics Data System (ADS)

    de Pater, Imke

    2014-10-01

    The 2009 impact and recent superbolides on Jupiter caught the world by surprise and cast doubt on impactor flux estimates for the outer solar system. Enhanced amateur planetary imaging techniques yield both high spatial resolution (enabling the 2009 impact debris field detection) and rapid frame rates (enabling the 2010/2012 impact flash detections and lightcurve measurements).We propose a ToO program to image future impacts on Jupiter and Saturn. To remove the possibility of impact cloud non-detections, the program will be triggered only if an existing impact debris field is seen, an object on a collision course with Jupiter or Saturn is discovered, or an impact light curve is measured with an estimated total energy large enough to generate an impact cloud in a giant planet atmosphere (10^20 J).HST provides the only way to image these events in the ultraviolet, providing information on aerosol altitudes and on smaller particles that are less visible to ground-based infrared observations. High-resolution imaging with proper timing (not achievable from the ground) is required to measure precisely both the velocity fields of impact sites and the optical spectrum of impact debris. HST observations of past impacts on Jupiter have also served both as cornerstones of science investigations at other wavelengths and as vehicles for effective public outreach.Large outer solar system impacts are governed by the same physics as in the terrestrial events that dominate the impact threat to humans. Studying the behavior of impactors of various sizes and compositions, as they enter the atmosphere at varying angles and speeds, will better quantify terrestrial impact hazards.

  9. Giant impacts on giant planets

    NASA Astrophysics Data System (ADS)

    de Pater, Imke

    2013-10-01

    The 2009 impact and recent superbolides on Jupiter caught the world by surprise and cast doubt on impactor flux estimates for the outer solar system. Enhanced amateur planetary imaging techniques yield both high spatial resolution {enabling the 2009 impact debris field detection} and rapid frame rates {enabling the 2010/2012 impact flash detections and lightcurve measurements}.We propose a ToO program to image future impacts on Jupiter and Saturn. To remove the possibility of impact cloud non-detections, the program will be triggered only if an existing impact debris field is seen, an object on a collision course with Jupiter or Saturn is discovered, or an impact light curve is measured with an estimated total energy large enough to generate an impact cloud in a giant planet atmosphere {10^20 J}.HST provides the only way to image these events in the ultraviolet, providing information on aerosol altitudes and on smaller particles that are less visible to ground-based infrared observations. High-resolution imaging with proper timing {not achievable from the ground} is required to measure precisely both the velocity fields of impact sites and the optical spectrum of impact debris. HST observations of past impacts on Jupiter have also served both as cornerstones of science investigations at other wavelengths and as vehicles for effective public outreach.Large outer solar system impacts are governed by the same physics as in the terrestrial events that dominate the impact threat to humans. Studying the behavior of impactors of various sizes and compositions, as they enter the atmosphere at varying angles and speeds, will better quantify terrestrial impact hazards.

  10. Mucinous cystic tumor of the retroperitoneum. A report of two cases.

    PubMed

    Motoyama, T; Chida, T; Fujiwara, T; Watanabe, H

    1994-01-01

    Two cases of retroperitoneal mucinous cystic tumors and some diagnostic problems on aspiration cytology are presented. The first was a mixed benign, borderline and malignant mucinous tumor, and the second was a mixed benign and borderline mucinous tumor. Our knowledge of the cytology of ovarian mucinous cystic tumors was not helpful in making a correct diagnosis in these cases. Based on our experience, we emphasize the following. First, consider methods of collecting enough cells to make a diagnosis. Second, consider the possibility of mixed histologic features. Direct aspiration, if possible, from papillary lesions inside the cyst will probably lead to an accurate diagnosis. Measurement of carcinoembryonic antigen levels in the cystic fluid is useful in checking for underdiagnosis of such tumors.

  11. Gross cystic disease fluid protein-15 in salivary gland tumors.

    PubMed

    Swanson, P E; Pettinato, G; Lillemoe, T J; Wick, M R

    1991-02-01

    Gross cystic disease fluid protein-15 (GCDFP-15) is a 15-kd glycoprotein that is expressed by normal apocrine epithelia and in a majority of breast carcinomas. However, recent studies have demonstrated that this substance is also present in tumors of the salivary glands, sweat glands, and prostate gland. To determine whether the expression of CGDFP-15 might aid in the differential diagnosis of salivary gland lesions, the anti-GCDFP-15 monoclonal antibody D6 was applied to paraffin sections of 133 such neoplasms. Benign tumors (76% reactive) were more often labeled than malignant lesions (28% reactive) by this antibody; overall, 53 (41%) of 133 cases were positive for GCDFP-15. Notably, the tubuloglandular components in 17 (81%) of 21 pleomorphic adenomas were reactive, but no example of either adenoid cystic carcinoma or polymorphous low-grade adenocarcinoma were labeled. In contrast, 24% of adenocarcinomas stained with this antibody. The apparent expression of GCDFP-15 by a spectrum of salivary gland tumors supports their biologic relationship to lesions of the cutaneous apocrine glands and breast. Furthermore, the demonstration of this determinant may be of use in suggesting the salivary gland nature of poorly differentiated carcinomas of the head and neck, and it may facilitate the separation of pleomorphic adenoma from histologically similar malignant neoplasms in the salivary glands themselves.

  12. [A case of retroperitoneal mucinous cystadenoma of borderline malignancy].

    PubMed

    Nagata, J; Yamauchi, M; Terabe, K; Watanabe, T; Ichihara, H; Takagi, H; Nakashima, N

    1987-04-01

    Surgical experience of a rare case of malignant retroperitoneal cyst is reported. A 41-year-old female was admitted on Feb. 26, 1986, complaining of left lower abdominal tumor and mild abdominal pain. She underwent complete removal of an abdominal tumor located at the left flank lateral to the sigmoid colon on March 5. The tumor was well encapsulated, cystic and oval, 12 X 10 X 9 cm in size. Histologic feature of the tumor is classified as mucinous cystadenoma of low grade malignancy by WHO classification.

  13. 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

  14. Cystic mesothelioma of the peritoneum.

    PubMed

    O'Neil, J D; Ros, P R; Storm, B L; Buck, J L; Wilkinson, E J

    1989-02-01

    Cystic mesothelioma (CM) of the peritoneum is a rare, benign neoplasm that occurs predominantly in women and tends to recur locally. It has received little attention (to our knowledge, a single case report) in the radiology literature. Five cases of CM are presented. Computed tomography (CT) was performed in three cases, ultrasound (US) in four cases, and magnetic resonance (MR) imaging in one case. Twenty-eight cases reported in the literature are reviewed for comparison. CM shows a clear predilection for the surfaces of the pelvic viscera but is seen in other areas of the peritoneum and retroperitoneum. The neoplasm was intraperitoneal in three cases and primarily retroperitoneal in the other two cases. In all CT and US studies performed, a single large, multilocular cystic mass was demonstrated. MR imaging, performed in one case, showed that the lesion had signal characteristics typical of a watery collection low in solute concentration. It is concluded that CM of the peritoneum has a nonspecific multilocular cystic appearance on images, which does not permit it to be differentiated from other cystic lesions.

  15. [Giant phyllodes tumor of the prostate].

    PubMed

    Kawamorita, Naoki; Inaba, Yasuo; Soma, Fumihiko; Katayama, Yousei; Mikami, Yoshiki

    2007-09-01

    A 55 year-old man complained dysuria and visited to our hospital. Physical examination showed firm large mass occupying whole abdomen. Computed tomography (CT) demonstrated a huge retroperitoneal tumor which compressed intestine, liver, kidney, and urinary bladder. We performed extirpation of the tumor (8.6 kg, largest diameter 60 cm) which was composed of myxoid stromal region associated with cystic pattern. Histological examination revealed that the epithelium of the cystic region was positive for prostate specific antigen (PSA) immunostaining. The tumor was diagnosed phyllodes tumor of the prostate (prostatic stromal proliferation of uncertain malignancy, PSTUMP). Serum PSA was declined 3.9 ng/ml to 0.9 ng/ml; however, magnetic resonance imaging (MRI) demonstrated a residual (recurrent?) tumor in the pelvis one month after the operation. We carried out total prostatectomy and residual tumor resection. Phyllodes tumor of the prostate is histologically characterized with biphasic pattern of hyperplastic epithelial cysts and variably cellular spindle stroma. The tumor is considered to have malignant potential and several histological factors including cellularity, atypia, etc. are utilized to assess it. However diagnostic criteria and subsequent treatment modalities are not established thus far. Previous reports showed efficacy of total surgical removal rather than partial resection and that we performed radical extirpation of the entire tumor. Close follow up is needed against this frequently recurrent disease.

  16. [Prevalence and clinicopathological characteristics of giant cell tumors].

    PubMed

    Estrada-Villaseñor, E G; Linares-González, L M; Delgado-Cedillo, E A; González-Guzmán, R; Rico-Martínez, G

    2015-01-01

    The frequency of giant cell tumors reported in the literature is very variable. Considering that our population has its own features, which distinguish it from the Anglo-Saxon and Asian populations, we think that both the frequency and the clinical characteristics of giant cell tumors in our population are different. The major aim of this paper was to determine the frequency and clinicopathological characteristics of giant cell tumors of the bone. A cross-sectional descriptive study was conducted of the cases diagnosed at our service as giant cell tumors of the bone from January to December 2013. The electronic clinical records, radiologic records and histologic slides from each case were reviewed. Giant cell tumors represented 17% of total bone tumors and 28% of benign tumors. Patients included 13 females and 18 males. The most frequent locations of giant cell tumors were: the proximal tibia, 9 cases (29%), and the distal femur, 6 cases (19%). Forty-five percent of giant cell tumors were associated with aneurysmal bone cyst (ABC) (14 cases) and one case (3%) was malignant. The frequency of giant cell tumors in this case series was intermediate, that is, higher than the one reported in Anglo-Saxon countries (usually low), but without reaching the frequency rates reported in Asian countries (high).

  17. [Prevalence and clinicopathological characteristics of giant cell tumors].

    PubMed

    Estrada-Villaseñor, E G; Linares-González, L M; Delgado-Cedillo, E A; González-Guzmán, R; Rico-Martínez, G

    2015-01-01

    The frequency of giant cell tumors reported in the literature is very variable. Considering that our population has its own features, which distinguish it from the Anglo-Saxon and Asian populations, we think that both the frequency and the clinical characteristics of giant cell tumors in our population are different. The major aim of this paper was to determine the frequency and clinicopathological characteristics of giant cell tumors of the bone. A cross-sectional descriptive study was conducted of the cases diagnosed at our service as giant cell tumors of the bone from January to December 2013. The electronic clinical records, radiologic records and histologic slides from each case were reviewed. Giant cell tumors represented 17% of total bone tumors and 28% of benign tumors. Patients included 13 females and 18 males. The most frequent locations of giant cell tumors were: the proximal tibia, 9 cases (29%), and the distal femur, 6 cases (19%). Forty-five percent of giant cell tumors were associated with aneurysmal bone cyst (ABC) (14 cases) and one case (3%) was malignant. The frequency of giant cell tumors in this case series was intermediate, that is, higher than the one reported in Anglo-Saxon countries (usually low), but without reaching the frequency rates reported in Asian countries (high). PMID:27403516

  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.

  19. Giant Cornu Cutaneum Superimposed on Basal Cell Carcinoma.

    PubMed

    Agirgol, S; Mansur, A T; Bozkurt, K; Azakli, H N; Babacan, A; Dikmen, A

    2015-09-01

    Cornu cutaneum (CC) is a clinical term that describes the horn-like keratotic lesions extending vertically from the skin. Benign, premalignant or malignant lesions may be present at the base of CC. Seborrhoeic keratosis and squamous cell carcinoma (SCC) are the most commonly reported benign and malignant forms, respectively. Basal cell carcinoma (BCC) at the base is rare. Here, we report on an 85-year old female patient having multiple CC lesions, one being giant on her face and two of the lesions diagnosed with BCC at the base. This case is of significance due to the presence of giant and multiple CC and detection of BCC at the base of more than one lesion. This present case indicates the need for the treatment of possible malignant lesions underlying CC in the elderly by total surgical excision.

  20. Giant Cornu Cutaneum Superimposed on Basal Cell Carcinoma

    PubMed Central

    Agirgol, S; Mansur, AT; Bozkurt, K; Azakli, HN; Babacan, A; Dikmen, A

    2015-01-01

    ABSTRACT Cornu cutaneum (CC) is a clinical term that describes the horn-like keratotic lesions extending vertically from the skin. Benign, premalignant or malignant lesions may be present at the base of CC. Seborrhoeic keratosis and squamous cell carcinoma (SCC) are the most commonly reported benign and malignant forms, respectively. Basal cell carcinoma (BCC) at the base is rare. Here, we report on an 85-year old female patient having multiple CC lesions, one being giant on her face and two of the lesions diagnosed with BCC at the base. This case is of significance due to the presence of giant and multiple CC and detection of BCC at the base of more than one lesion. This present case indicates the need for the treatment of possible malignant lesions underlying CC in the elderly by total surgical excision. PMID:26624603

  1. Dasatinib in Treating Patients With Recurrent or Metastatic Malignant Salivary Gland Tumors

    ClinicalTrials.gov

    2016-05-26

    High-grade Salivary Gland Mucoepidermoid Carcinoma; Low-grade Salivary Gland Mucoepidermoid Carcinoma; Recurrent Salivary Gland Cancer; Salivary Gland Acinic Cell Tumor; Salivary Gland Adenocarcinoma; Salivary Gland Adenoid Cystic Carcinoma; Salivary Gland Anaplastic Carcinoma; Salivary Gland Malignant Mixed Cell Type Tumor; Salivary Gland Poorly Differentiated Carcinoma; Salivary Gland Squamous Cell Carcinoma; Stage IV Salivary Gland Cancer

  2. [A case of giant hemorrhagic adrenal pseudocystectomy with a flank incision].

    PubMed

    Takeuchi, Yasuharu; Ozaki, Yumi; Suyama, Taisuke; Sawada, Yoshitomo; Kuroda, Kanami; Nakajima, Kouichi; Oharaseki, Toshiaki; Wakayama, Megumi

    2011-06-01

    The patient was a 43-year-old woman who underwent detailed examinations for a retroperitoneal cystic lesion that was incidentally found during orthopedic treatment. Although the tumor was a non-functioning tumor, and diagnostic imaging was negative for malignancy, the tumor was surgically resected with a flank incision. The histopathological diagnosis was adrenal pseudocyst.

  3. Giant adrenal pseudocyst harbouring adrenocortical cancer

    PubMed Central

    Wilkinson, Michael; Fanning, Deirdre Mary; Moloney, James; Flood, Hugh

    2011-01-01

    The authors report a very rare case of adreno-cortical carcinoma arising in a giant adrenal pseudocyst. A 64-year-old woman presented to the emergency department with a 6 week history of progressively worsening severe left abdominal pain, anorexia, anergia and constipation. On examination, she was cachectic with tenderness over the left abdomen and flank. Medical history was significant for gastritis and anaemia. During her investigation, a well-defined para-renal 12×6 centimetre multi-loculated cyst, of uncertain origin was identified on CT. Ultrasound-guided biopsy was not diagnostic. MRI showed the cyst to be likely adrenal in origin. Serum and urinary catecholamines were unremarkable. At laparotomy an unresectable large, tense, fixed, cystic mass was seen to occupy the left side of the abdomen. The cyst was de-roofed. Pathology showed a high-grade poorly differentiated adreno-cortical carcinoma with a pseudo-capsule. She died 2 months postoperatively. PMID:22679267

  4. Biomarkers in Paediatric Cystic Fibrosis Lung Disease.

    PubMed

    Ramsey, Kathryn A; Schultz, André; Stick, Stephen M

    2015-09-01

    Biomarkers in cystic fibrosis are used i. for the measurement of cystic fibrosis transmembrane regulator function in order to diagnose cystic fibrosis, and ii. to assess aspects of lung disease severity (e.g. inflammation, infection). Effective biomarkers can aid disease monitoring and contribute to the development of new therapies. The tests of cystic fibrosis transmembrane regulator function each have unique strengths and weaknesses, and biomarkers of inflammation, infection and tissue destruction have the potential to enhance the management of cystic fibrosis through the early detection of disease processes. The development of biomarkers of cystic fibrosis lung disease, in particular airway inflammation and infection, is influenced by the challenges of obtaining relevant samples from infants and children for whom early detection and treatment of disease might have the greatest long term benefits.

  5. 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

  6. Non-cystic fibrosis bronchiectasis.

    PubMed

    Al Subie, Haya; Fitzgerald, Dominic A

    2012-05-01

    Non-cystic fibrosis (CF) bronchiectasis is said to be a declining problem in developed countries, although its prevalence in indigenous communities in Australia and New Zealand is among the highest reported in the world. Early childhood pneumonia and underlying conditions such as immunodeficiency and primary ciliary dyskinesia need to be considered in the aetiology. A high-resolution computerised tomography scan is the key investigation in children with a chronic wet cough in whom bronchiectasis is suspected. Regardless of the cause, the treatment of bronchiectasis is centred upon facilitating the clearance of airway secretions and the treatment of pulmonary exacerbations. This review aims to provide general paediatricians with an update on the presenting features, investigation and management of non-cystic fibrosis bronchiectasis. PMID:21040075

  7. Adenoid cystic carcinoma of trachea.

    PubMed

    Vigg, Ajit; Mantri, Sumant; Vigg, Avanti; Vigg, Arul

    2004-01-01

    A 20-year-old male, presented with cough, haemoptysis, breathlessness and wheezing for the past one month. Contrast enhanced computerised tomographic (CECT), scan of chest and fibreoptic bronchoscopy revealed an endotracheal mass that on histopathological examination showed adenoid cystic carcinoma of trachea. Magnetic resonance imaging (MRI) scan of chest confirmed involvement of adjacent prevertebral, para-oesophageal and subcarinal lymph nodes rendering the tumour inoperable. PMID:15515830

  8. [Retroperitoneal cystic mesothelioma and lymphangioma].

    PubMed

    Segura Martín, M; Lorenzo Romero, J G; Hernández Millán, I; Pastor Guzmán, J M; Salinas Sánchez, A S; Ruiz Mondéjar, R; Virseda Rodríguez, J A

    1998-03-01

    Retroperitoneal cysts are uncommon entities of difficult diagnosis because of their insidious symptomatology. Urinary apparatus involvement is quite often the mode of presentation and the reason for calling on the urologist. The origin of many of these retroperitoneal cysts remains practically unknown. Surgery with exeresis is the choice management method. Follow-up is necessary for cystic mesothelioma because of the highly frequent relapses. The outlook of hormonal conservative therapy for relapses appears as a future alternative to treatment.

  9. Non-cystic fibrosis bronchiectasis.

    PubMed

    McShane, Pamela J; Naureckas, Edward T; Tino, Gregory; Strek, Mary E

    2013-09-15

    There is renewed interest in non-cystic fibrosis bronchiectasis, which is a cause of significant morbidity in adults and can be diagnosed by high-resolution chest computed tomography scan. No longer mainly a complication after pulmonary infection with Mycobacterium tuberculosis, diverse disease processes and mechanisms have been demonstrated to result in the chronic cough, purulent sputum production, and airway dilation that characterize this disease. Improved understanding of the role of mucus stasis in causing bacterial colonization has led to increased emphasis on the use of therapies that enhance airway clearance. Inhalational antibiotics reduce the bacterial burden associated with a worse outcome. Low-dose, chronic macrolide therapy has been shown to decrease exacerbation frequency and airway inflammation. For the first time, a number of therapies for non-cystic fibrosis bronchiectasis are undergoing testing in clinical research trials designed specifically for this population. This concise clinical review focuses on the major etiologies, diagnostic testing, microbiology, and management of patients with adult non-cystic fibrosis bronchiectasis. Systematic evaluation identifies a specific cause in the majority of patients and may affect subsequent treatment. We outline current therapies and review the data that support their use. PMID:23898922

  10. Pediatric Salivary Gland Malignancies.

    PubMed

    Ord, Robert A; Carlson, Eric R

    2016-02-01

    Pediatric malignant salivary gland tumors are extremely rare. The percentage of malignant tumors is higher than that seen in adults, although the outcomes in terms of survival are better in pediatric patients. The mainstay of treatment is surgical excision with negative margins. This article reviews current concepts in demographics, etiology, management, and outcomes of malignant salivary tumors in children.

  11. Giant intradural extramedullary spinal hydatid cyst--a rare presentation.

    PubMed

    Rashid, Muddassir; Kirmani, Sanna; Rashid, Mubashir

    2012-01-01

    The hydatidosis, or echinococcosis, has a characteristic geographic distribution, occurring most frequently in sheep-raising regions in Mediterranean, Central Asian, and South American countries and in Australia. Spinal hydatidosis is very rare, and intradural location is a rarer category of spinal hydatidosis. We report a case of intradural extramedullary spinal hydatid cyst in a 9-year-old boy. On magnetic resonance imaging, an intradural extramedullary giant cystic lesion was seen mimicking an arachnoid cyst. However, endemic origin of the patient and positive serology helped to make the diagnosis of hydatid cyst, which was confirmed on postoperative histopathology.

  12. Large pancreatic mucinous cystic neoplasm during pregnancy: what should be done?.

    PubMed

    Tica, Andrei Adrian; Tica, Oana Sorina; Saftoiu, Adrian; Camen, Dragos; Tica, Vlad Iustin

    2013-01-01

    Pancreatic mucinous cystic neoplasms are uncommon and their occurrence in pregnancy is extremely rare. The authors report the unique case of a newborn weighing 3,620 g, delivered vaginally with no complications by a patient with a large 'silent' pancreatic mucinous cystic neoplasms, and analyze the very few other reports. With no available protocol, this case highlights an interesting dilemma on the management of pregnancy and delivery as well on the timing of pancreatic surgery. Despite its limitations, MRI remains the most accurate investigation either for differentiating the mucinous from nonmucinous cysts or for evaluating the malignancy, but echography is also very useful. Without symptoms, all low-grade malignant potential tumors, independent of the moment of their diagnosis during pregnancy, should be resected 2-3 months after delivery and we believe that the best option is a term vaginal birth, even in the presence of a large cyst and large fetus. On the contrary, all high-grade malignant potential tumors, discovered in the first two trimesters of pregnancy should be resected during the second trimester, and followed by a vaginal delivery at term. If high-grade malignant potential tumor is diagnosed in the third trimester, an early vaginal delivery followed by surgery is recommended. Finally, the patient's preference is crucial.

  13. Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis

    PubMed Central

    Hirche, T. O.; Knoop, C.; Hebestreit, H.; Shimmin, D.; Solé, A.; Elborn, J. S.; Ellemunter, H.; Aurora, P.; Hogardt, M.; Wagner, T. O. F.; ECORN-CF Study Group

    2014-01-01

    There are no European recommendations on issues specifically related to lung transplantation (LTX) in cystic fibrosis (CF). The main goal of this paper is to provide CF care team members with clinically relevant CF-specific information on all aspects of LTX, highlighting areas of consensus and controversy throughout Europe. Bilateral lung transplantation has been shown to be an important therapeutic option for end-stage CF pulmonary disease. Transplant function and patient survival after transplantation are better than in most other indications for this procedure. Attention though has to be paid to pretransplant morbidity, time for referral, evaluation, indication, and contraindication in children and in adults. This review makes extensive use of specific evidence in the field of lung transplantation in CF patients and addresses all issues of practical importance. The requirements of pre-, peri-, and postoperative management are discussed in detail including bridging to transplant and postoperative complications, immune suppression, chronic allograft dysfunction, infection, and malignancies being the most important. Among the contributors to this guiding information are 19 members of the ECORN-CF project and other experts. The document is endorsed by the European Cystic Fibrosis Society and sponsored by the Christiane Herzog Foundation. PMID:24800072

  14. Cytologic diagnosis of adenoid cystic carcinoma of salivary glands.

    PubMed

    Nagel, H; Hotze, H J; Laskawi, R; Chilla, R; Droese, M

    1999-06-01

    The cytomorphologic features in fine-needle aspiration (FNA) biopsies from 31 primary and 33 recurrent adenoid cystic carcinomas (ACC) were investigated. The correct FNA diagnosis was established in 24 of 31 primary ACC (77%). The diagnostic clue in aspirates from ACC are large globules of extracellular matrix, partially surrounded by basaloid tumor cells. In FNAs with predominance of basaloid tumor cells, but lacking characteristic globules, all other benign and malignant salivary gland tumors of epithelial-myoepithelial differentiation should be considered in the cytologic diagnosis. Pleomorphic adenoma is most frequently confused with ACC, and therefore, the cytologic findings in FNAs from 50 pleomorphic adenomas were compared with those diagnosed as ACC. Furthermore, rare neoplasms of salivary glands with epithelial-myoepithelial cell differentiation, including basal-cell adenoma and carcinoma, epithelial-myoepithelial carcinoma, and polymorphous low-grade adenocarcinoma, as well as some nonsalivary gland neoplasms presenting an adenoid cystic pattern, must be considered. The cytologic features of these entities are discussed in detail with respect to the cytologic diagnostic criteria of ACC.

  15. Adenoid cystic carcinoma of the upper trachea: a rare neoplasm.

    PubMed

    Choudhury, Binoy Kumar; Barman, Geetanjali; Singh, Shobhit; Ahmed, Kuddush

    2013-01-01

    Primary malignant tracheal tumors are not common and adenoid cystic carcinoma (ACC) of trachea is rare. We report an extremely rare case of ACC of proximal trachea, which was diagnosed in a 42-year-old male who presents with 6-month history of dyspnea. Lateral skiagram of neck, computed tomography, and magnetic resonance imaging revealed a broad-based polypoidal soft tissue mass arising from posterior wall of the proximal trachea. Biopsy confirmed the diagnosis of ACC. The patient was treated by surgical resection followed by radiotherapy and is on regular follow-up. Follow-up at 18 months post-treatment showed no local recurrence or distant metastases. The literature on tracheal ACC is reviewed. Image findings are briefly discussed. PMID:24228208

  16. Adenoid Cystic Carcinoma Mimicking an Oroantral Fistula: A Case Report

    PubMed Central

    Monteiro, Bárbara Vanessa de Brito; Grempel, Rafael Grotta; Gomes, Daliana Queiroga de Castro; Godoy, Gustavo Pina; Miguel, Márcia Cristina da Costa

    2013-01-01

    Introduction Adenoid cystic carcinoma (ACC) is one of the most frequent malignant salivary gland tumors, which commonly affects the minor salivary glands of the mouth and is rare in the nose and paranasal sinuses. In the maxillary sinus, ACC can mimic inflammatory diseases and has a poor prognosis. Objective To report a case of a 50-year-old man with ACC of the maxillary sinus whose clinical findings in the alveolar ridge mimicked an oroantral fistula. Case Report An excisional biopsy was performed and histopathologic analysis revealed ACC. Lung metastases and residual tumor in the maxillary sinus were detected by imaging methods. In view of the poor general health of the patient, no new surgical intervention was performed and he was only treated by radiotherapy and follow-up. Conclusion Although rare in the maxillary sinus, ACC should be included in the differential diagnosis of lesions affecting this site. PMID:25992095

  17. Cystic glioblastoma multiforme masquerading as a cerebral tuberculoma

    PubMed Central

    Hasan, Mahboob; Siddiqui, Bushra; Qadri, Shagufta; Faridi, Shahbaz

    2014-01-01

    Glioblastoma multiforme (GBM) is by far the most common and most aggressive malignant neoplasm of the primary brain tumours. It arises from the astrocytes and classified as WHO grade 4 astrocytoma. Diagnosis of GBM is sometimes difficult as radiological picture sometimes mimic with cerebral tuberculoma. In both the cases contrast-enhanced CT may show similar finding of a mass lesion with a hypodense centre surrounded by a ring of enhancement and any cyst if present. In the present case, a 45-year-old male patient presented with seizures and headache, a provisional diagnosis of tuberculoma was made on the basis of clinical and CT findings. However, on grounds of suspicion the patient was operated and fluid from the cyst was sent peroperatively for cytopathological examination which suggested the diagnosis of cystic GBM. This helped the surgeon to do maximum debulking of the tumour. Diagnosis was further confirmed by histopathology. PMID:25326570

  18. A combination therapy for cystic fibrosis.

    PubMed

    Brodsky, Jeffrey L; Frizzell, Raymond A

    2015-09-24

    The most prevalent form of cystic fibrosis arises from an amino acid deletion in the cystic fibrosis transmembrane conductance regulator, CFTR. A recently approved treatment for individuals homozygous for this mutation combines a chemical corrector, which helps CFTR fold, and a potentiator that increases CFTR channel activity. PMID:26406363

  19. A combination therapy for cystic fibrosis.

    PubMed

    Brodsky, Jeffrey L; Frizzell, Raymond A

    2015-09-24

    The most prevalent form of cystic fibrosis arises from an amino acid deletion in the cystic fibrosis transmembrane conductance regulator, CFTR. A recently approved treatment for individuals homozygous for this mutation combines a chemical corrector, which helps CFTR fold, and a potentiator that increases CFTR channel activity.

  20. Psychosocial impact of cystic fibrosis in adolescence.

    PubMed

    Harrop, Michele

    2007-12-01

    Over 8,000 children, young people and adults in the UK are affected by cystic fibrosis and although no cure exists, comprehensive therapy started early and administered consistently delays disease progression. This article explores three aspects of the psychosocial effects of cystic fibrosis on the adolescent/young adult: the effect on the family, the effect on relationships and adherence to treatment. Much of the early research on the psychosocial impact of cystic fibrosis on the adolescent and the family presented a dismal picture of dysfunction. More recent studies indicate that cystic fibrosis patients generally lead active, age-appropriate social lives, that good information and support can reduce negative effects on families and that treatment regimes continue to cause difficulties for young people. Further research is needed into the psychosocial impact of cystic fibrosis on the increasing numbers of young people and adults with the disease.

  1. Cystic Lesions of the Mediastinum.

    PubMed

    Vargas, Daniel; Suby-Long, Thomas; Restrepo, Carlos S

    2016-06-01

    Cystic lesions are commonly seen in the mediastinum, and they may arise from virtually any organ. The vast majority of these lesions are benign and result in no symptoms. When large, cysts may produce symptoms related to compression of adjacent structures. The most common mediastinal cysts are pericardial and foregut duplication cysts. Both computed tomography and magnetic resonance are routinely used to evaluate these lesions. Although computed tomography offers superior spatial resolution, magnetic resonance is useful in differentiating cysts that contain proteinaceous material from solid lesions. Occasionally, cysts arise from solid lesions, such as thymoma or teratoma. Although cysts are alike in appearance, location helps narrowing the differential diagnoses.

  2. Endocrine Disorders in Cystic Fibrosis.

    PubMed

    Blackman, Scott M; Tangpricha, Vin

    2016-08-01

    Cystic fibrosis is frequently complicated by endocrine disorders. Diabetes can be expected to affect most with CF and pancreatic insufficiency and varies widely in age of onset, but early identification and treatment improve morbidity and mortality. Short stature can be exacerbated by relative delay of puberty and by use of inhaled corticosteroids. Bone disease in CF causes fragility fractures and should be assessed by monitoring bone mineral density and optimizing vitamin D status. Detecting and managing endocrine complications in CF can reduce morbidity and mortality in CF. These complications can be expected to become more common as the CF population ages. PMID:27469183

  3. An unusual retroperitoneal cystic tumor.

    PubMed

    Salinas Sánchez, A S; Lorenzo Romero, J G; Segura Martín, M; Hernández Millán, I R; Pastor Guzmán, J M; Virseda Rodríguez, J A

    2000-01-01

    This report describes a case of textiloma (term given to an inflammatory swelling caused by a retained textile foreign body) in a 60-year-old patient who had undergone surgery for a perforated gastroduodenal ulcer 17 years earlier. On examination the presence of a large mass was detected on the left hypochondria. A CT scan confirmed a cystic tumor with images suggesting detritus or necrosis in its lower part, as well as calcifications. Transperitoneal midline laparotomy was performed with extension by thoracophrenolaparotomy. Splenectomy was necessary. Macroscopic examination showed a spherical mass. After opening the tumor, retained surgical gauze was found. The histopathological diagnosis was granulomatosis reaction to a foreign body.

  4. Unstable giant gravitons

    SciTech Connect

    Mello Koch, Robert de; Ives, Norman; Smolic, Jelena; Smolic, Milena

    2006-03-15

    We find giant graviton solutions in Frolov's three parameter generalization of the Lunin-Maldacena background. The background we study has {gamma}-tilde{sub 1}=0 and {gamma}-tilde{sub 2}={gamma}-tilde{sub 3}={gamma}-tilde. This class of backgrounds provides a nonsupersymmetric example of the gauge theory/gravity correspondence that can be tested quantitatively, as recently shown by Frolov, Roiban, and Tseytlin. The giant graviton solutions we find have a greater energy than the point gravitons, making them unstable states. Despite this, we find striking quantitative agreement between the gauge theory and gravity descriptions of open strings attached to the giant.

  5. Carcinoid tumor arising in a mature cystic teratoma: A case report

    PubMed Central

    TOSUNER, ZEYNEP; SÖNMEZ, FATMA CAVİDE; ARICI, DİLEK SEMA; DANSUK, RAMAZAN

    2015-01-01

    Mature cystic teratomas are common benign tumors of the ovary. The most common form of malignant transformation is squamous cell carcinoma, however, malignant transformation is rarely observed and cases are usually asymptomatic. Carcinoid tumors are rare tumors of the neuroendocrine system. A number of the carcinoids result in clinical syndromes due to the secretion of vasoactive substances produced by the tumor. A 75-year-old woman suffering from groin pain was admitted to the Faculty of Medicine at Bezmialem Vakıf University (Istanbul, Turkey). An adnexal mass was detected during the physical examination. Magnetic resonance imaging scans exhibited an 8×7-cm mass in the right ovary. Surgical excision of the mass was performed and histopathological examination revealed a mature cystic teratoma with three germ layers. An area consisting of nests and trabeculae was evident in the cyst wall. The pathological diagnosis was of an insular and trabecular variant of carcinoid tumor arising in a mature cystic teratoma. The patient did not present with carcinoid syndrome, and no recurrence was observed after 11 months of follow-up examinations. PMID:26137048

  6. Rheumatic Diseases and Malignancies

    PubMed Central

    BOJINCA, Violeta; JANTA, Iustina

    2012-01-01

    ABSTRACT There are many studies which demonstrate a higher risk for malignancy in patients with rheumatic diseases. There have been a number of possible explanations for the differences in the risk of certain malignancies in patients with rheumatic disease, compared with general population, but a clear mechanism is difficult to identify. Rheumatoid syndromes may be associated with malignancy as paraneoplastic conditions, which can antedate the neoplasm diagnosis. On the other hand, autoimmune rheumatic diseases have a higher risk of malignancy by themselves or because of the immunosuppressant treatments. PMID:23482881

  7. Cystic Fibrosis: Brazilian ENT Experience

    PubMed Central

    Sih, Tania; Godinho, Ricardo; Franco, Leticia Paiva; Piltcher, Otávio

    2012-01-01

    Most published studies about Cystic Fibrosis (CF) are European or North American. There are still few publications about the characteristics of fibrocystic populations in developing countries. The incidence of cystic fibrosis (CF) in Brazil varies among different regions (1 : 10,000 in Minas Gerais, 1 : 9,500 in Paraná, 1 : 8,700 in Santa Catarina, and 1 : 1600 in Rio Grande do Sul). The prevalence of the DF508 mutation also varies according to population: 33% in Sao Paulo, 49% in Rio Grande do Sul, 27% in Santa Catarina, and 52% in Minas Gerais. Cough and nasal obstruction are the most common symptoms. The variation in nasal polyposis prevalence may be explained by population genotypic characteristics in a country that spans a continent. Findings on nasal endoscopy and computed tomography (CT) have better correlation than do this information compared with surgical and clinical history. Microbiologic studies suggest a high level of early contamination of the airways. Sensorineural hearing loss (SNHL) occurs in these patients as a result of ototoxic antibiotics. The data compiled in this paper is useful, but also lead to the general agreement that more research would be welcome due to the unique characteristics of this country. PMID:22611403

  8. Aspergillus infections in cystic fibrosis.

    PubMed

    King, Jill; Brunel, Shan F; Warris, Adilia

    2016-07-01

    Patients with cystic fibrosis (CF) suffer from chronic lung infection and airway inflammation. Respiratory failure secondary to chronic or recurrent infection remains the commonest cause of death and accounts for over 90% of mortality. Bacteria as Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia complex have been regarded the main CF pathogens and their role in progressive lung decline has been studied extensively. Little attention has been paid to the role of Aspergillus spp. and other filamentous fungi in the pathogenesis of non-ABPA (allergic bronchopulmonary aspergillosis) respiratory disease in CF, despite their frequent recovery in respiratory samples. It has become more apparent however, that Aspergillus spp. may play an important role in chronic lung disease in CF. Research delineating the underlying mechanisms of Aspergillus persistence and infection in the CF lung and its link to lung deterioration is lacking. This review summarizes the Aspergillus disease phenotypes observed in CF, discusses the role of CFTR (cystic fibrosis transmembrane conductance regulator)-protein in innate immune responses and new treatment modalities. PMID:27177733

  9. The Giant Cell.

    ERIC Educational Resources Information Center

    Stockdale, Dennis

    1998-01-01

    Provides directions for the construction of giant plastic cells, including details for building and installing the organelles. Also contains instructions for preparing the ribosomes, nucleolus, nucleus, and mitochondria. (DDR)

  10. The Next Giant Step

    NASA Video Gallery

    Artist Robert McCall painted "The Next Giant Step" in 1979 to commemorate the heroism and courage of spaceflight pioneers. Located in the lobby of Johnson's building 2, the mural depicts America's ...

  11. Intraabdominal cystic lymphangiomas obscured by marked superimposed reactive changes: clinicopathological analysis of a series.

    PubMed

    Hornick, Jason L; Fletcher, Christopher D M

    2005-04-01

    lymphatic spaces contained either clear fluid or large numbers of foamy macrophages. The lymphatic endothelial cells lining the cystic spaces were generally attenuated with no cytological atypia. One case showed features of a complex vascular malformation with a predominant component of cavernous lymphangioma. By immunohistochemistry, in all cases, the endothelial cells lining the dilated lymphatic spaces were positive for CD31 and D2-40, 4 of 7 were positive for CD34, and all were negative for keratin. Clinical follow-up information was available for 4 patients (median, 26 months; range, 22-36 months): 3 patients had no evidence of recurrence and 1 patient was asymptomatic with radiographic evidence of minimal persistent disease. In summary, some intraabdominal lymphangiomas have a tendency to induce marked reactive and inflammatory changes in the surrounding tissues, often obscuring their nature and occasionally leading to the clinical impression of a malignant tumor. Awareness of this unusual occurrence will lead to the correct diagnosis.

  12. Anaplastic giant cell thyroid carcinoma.

    PubMed

    Wallin, G; Lundell, G; Tennvall, J

    2004-01-01

    Anaplastic (giant cell) thyroid carcinoma (ATC), is one of the most aggressive malignancies in humans with a median survival time after diagnosis of 3-6 months. Death from ATC was earlier seen because of local growth and suffocation. ATC is uncommon, accounting for less than 5 % of all thyroid carcinomas. The diagnosis can be established by means of multiple fine needle aspiration biopsies, which are neither harmful nor troublesome for the patient. The cytological diagnosis of this high-grade malignant tumour is usually not difficult for a well trained cytologist. The intention to treat patients with ATC is cure, although only few of them survive. The majority of the patients are older than 60 years and treatment must be influenced by their high age. We have by using a combined modality regimen succeeded in achieving local control in most patients. Every effort should be made to control the primary tumour and thereby improve the quality of remaining life and it is important for patients, relatives and the personnel to know that cure is not impossible. Different treatment combinations have been used since 30 years including radiotherapy, cytostatic drugs and surgery, when feasible. In our latest combined regimen, 22 patients were treated with hyper fractionated radiotherapy 1.6Gy x 2 to a total target dose of 46 Gy given preoperatively, 20 mg doxorubicin was administered intravenously once weekly and surgery was carried out 2-3 weeks after the radiotherapy. 17 of these 22 patients were operated upon and none of these 17 patients got a local recurrence. In the future we are awaiting the development of new therapeutic approaches to this aggressive type of carcinoma. Inhibitors of angiogenesis might be useful. Combretastatin has displayed cytotoxicity against ATC cell lines and has had a positive effect on ATC in a patient. Sodium iodide symporter (NIS) genetherapy is also being currently considered for dedifferentiated thyroid carcinomas with the ultimate aim of

  13. Unexpected Malignant Diagnosis in Colonic Biopsies: Malignant Transformation of Ovarian Mature Teratomas—Two Case Reports and Review of the Literature

    PubMed Central

    Rojas, Claudia P.; Ganjei-Azar, Parvin; Garcia-Buitrago, Monica T.

    2015-01-01

    Colorectal adenocarcinoma is the second cause of cancer-related deaths in the United States. The occurrence of squamous cell carcinoma in the colorectum is extremely unusual. Malignant transformation from mature cystic teratoma of the ovary is a rare event. The most common transformation is squamous cell carcinoma, followed by adenocarcinoma. It occurs more often in elderly patients, who usually present with advance disease. We report two unusual cases of postmenopausal women diagnosed with squamous cell carcinoma in colon biopsies. After surgical resections, the carcinoma was proven to be the result of malignant transformation of ovarian mature cystic teratomas. Since squamous cell carcinoma of the colorectum is extremely rare, the presence of squamous cell carcinoma in a colonic biopsy in a female patient should alert the clinicians to other possible primary sites, as seen in these cases. PMID:26881165

  14. Massive pneumatic expansion of lymphatic vessel resulting in cystic lesions in the pulmonary parenchyma: a rare case of persistent interstitial pulmonary emphysema in a non-ventilated infant.

    PubMed

    Fujishiro, Jun; Komuro, Hiroaki; Ono, Kentaro; Urita, Yasuhisa; Shinkai, Toko; Minami, Yuko; Kawabata, Yoshinori; Kishimoto, Hiroshi; Masumoto, Kouji

    2012-12-01

    We report the case of 2-week-old female infant with cystic lung disease who presented with mild tachypnea and had no history of mechanical ventilation. Chest CT demonstrated multiple air-filled cystic lesions in right upper lobe, and the patient subsequently underwent a right upper lobectomy. Histology revealed cystic lesions located in the pulmonary parenchyma and showed that the lesions were lined by lymphatic endothelium and were communicating with dilated lymphatic vessels in the interstitium. Additionally, multinucleated foreign body giant cells were attached to the lumen of the cyst. On the basis of these findings, we considered this a case of persistent interstitial pulmonary emphysema (PIPE) with massive pneumatic expansion of the lymphatic vessels, resulting in cystic lesions with lymphatic endothelium in the pulmonary parenchyma. While PIPE is extremely rare in term non-ventilated infants, our case demonstrated that this disease should be added to the differential diagnosis of cystic lung diseases with lymphatic endothelium even in infants without mechanical ventilation. When cystic lesions and symptoms persist despite conservative treatment, open or thoracoscopic resection is an appropriate option for diagnosis and treatment.

  15. RO4929097, Temozolomide, and Radiation Therapy in Treating Patients With Newly Diagnosed Malignant Glioma

    ClinicalTrials.gov

    2015-09-28

    Acoustic Schwannoma; Adult Anaplastic (Malignant) Meningioma; Adult Anaplastic Astrocytoma; Adult Anaplastic Ependymoma; Adult Brain Stem Glioma; Adult Choroid Plexus Neoplasm; Adult Craniopharyngioma; Adult Diffuse Astrocytoma; Adult Ependymoblastoma; Adult Ependymoma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Grade I Meningioma; Adult Grade II Meningioma; Adult Medulloblastoma; Adult Mixed Glioma; Adult Myxopapillary Ependymoma; Adult Oligodendroglioma; Adult Papillary Meningioma; Adult Pilocytic Astrocytoma; Adult Pineal Gland Astrocytoma; Adult Pineoblastoma; Adult Pineocytoma; Adult Primary Melanocytic Lesion of Meninges; Adult Subependymal Giant Cell Astrocytoma; Adult Subependymoma; Adult Supratentorial Primitive Neuroectodermal Tumor; Malignant Adult Intracranial Hemangiopericytoma

  16. The ultrastructure of malignant fibrous histiocytoma (case report).

    PubMed

    Lombardi, L; Pilotti, S; Carbone, A; Rilke, F

    1977-01-01

    A soft tissue malignant fibrous histiocytoma was studied by light and electron microscopy. The presence of fibroblast-like cells, histiocyte-like cells, undifferentiated stem cells, and xanthomatous cells was confirmed. Fibroblast- and histiocyte-like multinucleated giant cells were also observed. The observation of lysosomes in some fibroblast-like cells, the features of undifferentiated stem cells, and the presence of cells and intermediate characteristics common to xanthomatous, histiocytic, and stem cells suggest a strict relationship among these cellular types.

  17. Benign retroperitoneal schwannoma mimicking a pancreatic cystic tumor: case report and literature review.

    PubMed

    Hsiao, W C; Lin, P W; Chang, K C

    1998-01-01

    A rare case of benign retroperitoneal schwannoma mimicking a pancreatic cystic tumor is reported herein. The tumor mass, based on a computed tomography scan and an abdominal echo examination, was initially suspected to be a mucinous cystadenoma or cystadenocarcinoma of the pancreas. However, the surgical and pathohistological findings made a final diagnosis of benign pancreatic schwannoma. The patient is doing well at a 2-year follow-up. The tumor, 18x17x15 cm in size, represented the largest among reported pancreatic schwannomas. Furthermore, a comprehensive review of reported cases of pancreatic schwannoma was carried out to summarize corresponding findings, including benign or malignant forms, adherence to other tissues, cystic change of the tumor, as well as relation to von Recklinghausen's neurofibromatosis.

  18. Primary malignant melanoma

    PubMed Central

    Mısır, A. Ferhat; Durmuşlar, Mustafa C.; Zerener, Tamer; Gün, Banu D.

    2016-01-01

    Malignant melanomas (MM) of the oral cavity are extremely rare, accounting for 0.2% to 8.0% of all malignant melanomas. Malignant melanomas is more frequently seen at the level of the hard palate and gingiva. Early diagnosis and treatment are important for reducing morbidity. Malignant melanoma cells stain positively with antibodies to human melanoma black 45, S-100 protein, and vimentin; therefore, immunohistochemistry can play an important role in evaluating the depth of invasion and the location of metastases. A 76-year-old man developed an oral malignant melanoma, which was originally diagnosed as a bluish reactive denture hyperplasia caused by an ill-fitting lower denture. The tumor was removed surgically, and histopathological examination revealed a nodular-type MM. There was no evidence of recurrence over a 4-year follow-up period. PMID:27052289

  19. Laparoscopic resection of a huge retroperitoneal cystic lymphangioma after successful reduction of tumor size with a double balloon catheter

    PubMed Central

    Ishibashi, Yusuke; Tsujimoto, Hironori; Kouzu, Keita; Horiguchi, Hiroyuki; Nomura, Shinsuke; Ito, Nozomi; Kanematsu, Kyohei; Yamazaki, Kenji; Hiraki, Shuichi; Aosasa, Suefumi; Noro, Takuji; Yamamoto, Junji; Hase, Kazuo

    2015-01-01

    Introduction Retroperitoneal cystic lymphangiomas are rare. We report a case of retroperitoneal huge cystic lymphangioma that was successfully aspirated the cyst’s contents with double balloon catheter and excised laparoscopically. Presentation of case A 34-year-old man was admitted to our hospital with low-grade fever and abdominal pain that had lasted for 1 week. Abdominal computed tomography and magnetic resonance imaging showed a fluid-filled multilocular mass measuring 13.5 cm in diameter around the tail of the pancreas, which was diagnosed as a retroperitoneal cystic lymphangioma. We successfully excised the tumor by laparoscopic distal pancreatosplenectomy. We punctured and aspirated the tumor with a double-balloon catheter to decrease the tumor’s size without spilling the tumor content. Cytology showed no malignant cells, and histopathological examination confirmed cystic lymphangioma. No recurrence was noted on radiographic imaging 10 months postoperatively. Discussion and conclusion Laparoscopic treatment for retroperitoneal huge cystic lymphangioma is feasible, and the double balloon catheter is useful for reducing the tumor volume. PMID:25898335

  20. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

    PubMed

    Labombarda, Fabien; Saloux, Eric; Brouard, Jacques; Bergot, Emmanuel; Milliez, Paul

    2016-09-01

    Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis.

  1. Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

    PubMed

    Labombarda, Fabien; Saloux, Eric; Brouard, Jacques; Bergot, Emmanuel; Milliez, Paul

    2016-09-01

    Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis. PMID:27578468

  2. Asymptomatic peritoneal carcinomatosis originating from benign cystic peritoneal mesothelioma

    PubMed Central

    Iacoponi, S; Calleja, J; Hernandez, G; de la Cuesta, R Sainz

    2015-01-01

    Benign multicystic mesothelioma is a rare tumour that originates from the abdominal peritoneum with a predisposition to the pelvic peritoneum. It typically affects women of reproductive age. There have been less than 200 cases of this rare neoplasia reported to date. We present the case of a 35-year-old woman who was referred to our centre because of the detection of a peritoneal carcinomatosis during a gynaecological exam. A diagnostic laparoscopy was performed. The findings included multiple cysts appearing as ‘a bunch of grapes’ occupying the omentum. Biopsies were taken during the surgery and the results showed benign multicystic peritoneal mesothelioma. Benign multicystic mesothelioma can simulate other conditions, such as malignant ovarian tumours or cystic lymphangioma. It is often diagnosed accidentally during surgery performed for another reason. The diagnosis is interoperative, observing multicystic structures grouped as a ‘bunch of grapes’ containing clear fluid with thin walls made of connective tissue. Immunohistochemistry confirmed mesothelial origin. Surgery is considered the treatment of choice and is based on the removal of the cysts from the abdominal cavity. Hyperthermic intraperitoneal chemotherapy can be considered as a primary treatment in patients with recurrences or even as a part of primary treatment associated with surgery. Survival at 5 years is 100% and invasive or malignant progression is extraordinary. The treatment approach should be multidisciplinary, and the patient should be referred to a referral centre. PMID:26715942

  3. Chronic pancreatitis and cystic fibrosis

    PubMed Central

    Witt, H

    2003-01-01

    Recent discoveries of trypsinogen and trypsin inhibitor mutations in patients with chronic pancreatitis (CP) support the hypothesis that an inappropriate activation of pancreatic zymogens to active enzymes within the pancreatic parenchyma starts the inflammatory process. Current data suggest that CP may be inherited dominant, recessive, or complex as a result of mutations in the above mentioned or yet unidentified genes. Evaluation of patients with CP should include genetic testing. Cystic fibrosis (CF) is an autosomal recessive inherited disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) gene and is characterised by pancreatic insufficiency and chronic bronchopulmonary infection. The progression and severity of pulmonary disease differs considerably between people with identical CFTR mutations and does not seem to correlate with the type or class of the CFTR mutation. The identification of further disease modifying genetic factors will increase the pathophysiological understanding and may help to identify new therapeutic targets. PMID:12651880

  4. Cystic echinococcosis in southern Brazil.

    PubMed

    de la Rue, Mario L

    2008-01-01

    Cystic echinococcosis (CE) is very common in the southern part of Rio Grande do Sul State where sheep and cattle raising is the most important economical activity. Prevalence in intermediate hosts is well known due to reports in slaughterhouses while human reports are of discussed value. This is due to underreporting of cases and few epidemiological studies make it difficult to assess the situation of echinococcosis in the population. Whereas cattle infection rate is more or less stable around 12% of the slaughter animals, in sheep there has been an increasing number in the last five years. Some efforts have been done to control the zoonosis but no effective results were obtained so far. Probably educational efforts to change human practices (feeding dogs with raw viscera), periodic treatment of dogs with praziquantel and joined actions with slaughterhouses could bring more attention to improve some control measures.

  5. Drugs Approved for Malignant Mesothelioma

    MedlinePlus

    ... Professionals Questions to Ask about Your Treatment Research Drugs Approved for Malignant Mesothelioma This page lists cancer ... in malignant mesothelioma that are not listed here. Drugs Approved for Malignant Mesothelioma Alimta (Pemetrexed Disodium) Pemetrexed ...

  6. Giant localized fibrous tumours of the pleura: report of three subsequent cases.

    PubMed

    Breda, Cristiano; Zuin, Andrea; Marulli, Giuseppe; Galligioni, Alessandra; Rea, Federico

    2006-05-01

    Localized fibrous tumours of the pleura (LFTP) represent clinical entities rarely encountered, especially in giant forms. We report of three cases of giant localized fibrous tumours of the pleura found last year in a short time. The patients underwent surgery by thoracotomy. All tumours arose from visceral pleura and were radically excised by a wedge resection of the lung. The patients discharged from the hospital after a short postoperative period without complications. Two of three giant localized fibrous tumours were classified as benign forms; in one case a malignant characteristic was found. PMID:16580088

  7. Laparoscopic observation of retroperitoneal cystic lymphangioma.

    PubMed

    Takeuchi, Y; Fujinami, S; Kitagawa, S; Shiro, T; Fujii, T; Fukui, Y; Kawashima, Y; Kubota, Y; Shiozaki, Y; Inoue, K

    1994-01-01

    A case of retroperitoneal cystic lymphangioma observed laparoscopically is reported. In a 60 year old asymptomatic male patient, an 8 cm multi-loculated cystic lesion was detected incidentally near the splenic hilum with ultrasonography. Endosonography, computed tomography and magnetic resonance imaging revealed the lesion with thin wall and clear fluid. Laparoscopy showed a thin-walled cyst with smooth surface, and straw-coloured clear fluid was observed through the wall. These findings suggested benign aetiology, and seemed to be characteristic of cystic lymphangioma. The tumour was resected, and microscopic examination showed proliferated lymph channels intespersed by lymph follicles. Diagnosis of cystic lymphangioma was established. Laparoscopy seems a useful pre-operative method.

  8. Caveolin-1 overexpression in benign and malignant salivary gland tumors.

    PubMed

    Jaafari-Ashkavandi, Zohreh; Ashraf, Mohammad Javad; Nazhvani, Ali Dehghani; Azizi, Zahra

    2016-02-01

    Caveolin-1, a tyrosine-phosphorylated protein, is supposed to have different regulatory roles as promoter or suppressor in many human cancers. However, no published study concerned its expression in benign and malignant salivary gland tumors. The aim of this study was to evaluate and compare the expression of Cav-1 in the most common benign and malignant salivary gland tumors and evaluate its correlation with proliferation activity. In this cross-sectional retrospective study, immunohistochemical expression of caveolin-1 and Ki67 were evaluated in 49 samples, including 11 normal salivary glands, 15 cases of pleomorphic adenoma (PA), 13 adenoid cystic carcinomas (AdCC), and 10 mucoepidermoid carcinomas (MEC). The expression of Cav-1 was seen in 18 % of normal salivary glands and 85 % of tumors. The immunoreaction in the tumors was significantly higher than normal tissues (P = 0.001), but the difference between benign and malignant tumors was not significant (P = 0.07). Expression of Cav-1 was correlated with Ki67 labeling index in PAs, but not in malignant tumors. Cav-1 expression was not in association with tumor size and stage. Overexpression of Cav-1 was found in salivary gland tumors in comparison with normal tissues, but no significant difference was observed between benign and malignant tumors. Cav-1 was inversely correlated with proliferation in PA. Therefore, this marker may participate in tumorigenesis of salivary gland tumors and may be a potential biomarker for cancer treatments.

  9. Giant osteomas of the ethmoid and frontal sinuses: Clinical characteristics and review of the literature

    PubMed Central

    CHENG, KE-JIA; WANG, SHEN-QING; LIN, LIN

    2013-01-01

    Giant osteomas of the ethmoid and frontal sinuses ary very rare, with only a few dozen cases reported in the literature. Given their rarity, the clinical characteristics and treatment of this disease remain controversial. In this study, the clinical presentation and surgical methods used to treat three patients with giant osteomas of the ethmoid and frontal sinuses are described, combined with a review of the literature from 1975 to 2011. In total, 45 patients with giant osteomas arising from the ethmoid and frontal sinuses (including the present cases) have been reported in 41 articles. Headache and ocular signs are the most common symptoms. This disease often leads to intracranial or intraorbital complications. The main treatment for giant osteoma is surgery via an external approach. The outcome of surgery for giant osteoma is good, with rare recurrence, no malignant transformation and few persistent symptoms. PMID:23759920

  10. Correlative Imaging in a Patient with Cystic Thymoma: CT, MR and PET/CT Comparison

    PubMed Central

    Romeo, Valeria; Esposito, Alfredo; Maurea, Simone; Camera, Luigi; Mainenti, Pier Paolo; Palmieri, Giovannella; Buonerba, Carlo; Salvatore, Marco

    2015-01-01

    Summary Background Cystic thymoma is a rare variant of thymic neoplasm characterized by almost complete cystic degeneration with mixed internal structure. We describe a case of a 60 year-old woman with a cystic thymoma studied with advanced tomographic imaging stydies. CT, MRI and PET/CT with 18F-FDG were performed; volumetric CT and MRI images provided better anatomic evaluation for pre-operative assessment, while PET/CT was helpful for lesion characterization based on 18F-FDG uptake. Although imaging studies are mandatory for pre-operative evaluation of cystic thymoma, final diagnosis still remains surgical. Case Report A 60-year-old woman with recent chest pain and no history of previous disease was admitted to our departement to investigate the result of a previous chest X-ray that showed bilateral mediastinal enlargement; for this purpose, enhanced chest CT scan was performed using a 64-rows scanner (Toshiba, Aquilion 64, Japan) before and after intravenous bolus administration of iodinated non ionic contrast agent; CT images demonstrated the presence of a large mediastinal mass (11×8 cm) located in the anterior mediastinum who extended from the anonymous vein to the cardio-phrenic space, compressing the left atrium and causing medium lobe atelectasis; bilateral pleural effusion was also present. Conclusions In conclusion, correlative imaging plays a foundamental role for the diagnostic evaluation of patient with cystic thymoma. In particular, volumetric CT and MRI studies can provide better anatomic informations regarding internal structure and local tumor spread for pre-operative assessment. Conversely, metabolic imaging using 18F-FDG PET/CT is helpful for lesion characterization differentiating benign from malignant lesion on the basis of intense tracer uptake. The role of PET/MRI is still under investigation. However, final diagnosis still remains surgical even though imaging studies are mandatory for pre-operative patient management. PMID:25593635

  11. Giant supratentorial neurenteric cyst.

    PubMed

    Junaid, Muhammad; Kalsoom, Anisa; Khalid, Muhammad; Bukhari, Syed Sarmad

    2014-11-01

    Intracranial neurenteric cysts are rare congenital lesions that may be mistaken for other cystic neoplastic and nonneoplastic lesions. A 35 years old right handed man presented with a one year history of progressive left hemiparesis, headache, personality changes and seizures. Clinical examination revealed a confused patient with left hemiparesis (power 4/5 in both left upper and lower limbs), upper motor neuron type signs in left half of the body (up going plantar reflex and exaggerated deep tendon reflexes at 3+), left facial weakness of upper motor neuron type. CT scan head with contrast revealed a non-enhancing spherical cystic lesion in the frontotemporoparietal region with minimal to moderate mass effect. The cyst was removed using a combination of hydrodissection and excision. Recovery was complete with no evidence of recurrence or residual disease at 3 months.

  12. Giant supratentorial neurenteric cyst.

    PubMed

    Junaid, Muhammad; Kalsoom, Anisa; Khalid, Muhammad; Bukhari, Syed Sarmad

    2014-11-01

    Intracranial neurenteric cysts are rare congenital lesions that may be mistaken for other cystic neoplastic and nonneoplastic lesions. A 35 years old right handed man presented with a one year history of progressive left hemiparesis, headache, personality changes and seizures. Clinical examination revealed a confused patient with left hemiparesis (power 4/5 in both left upper and lower limbs), upper motor neuron type signs in left half of the body (up going plantar reflex and exaggerated deep tendon reflexes at 3+), left facial weakness of upper motor neuron type. CT scan head with contrast revealed a non-enhancing spherical cystic lesion in the frontotemporoparietal region with minimal to moderate mass effect. The cyst was removed using a combination of hydrodissection and excision. Recovery was complete with no evidence of recurrence or residual disease at 3 months. PMID:25518778

  13. Precision Genomic Medicine in Cystic Fibrosis.

    PubMed

    Chang, Eugene H; Zabner, Joseph

    2015-10-01

    The successful application of precision genomic medicine requires an understanding of how a person's genome can influence his or her disease phenotype and how medical therapies can provide personalized therapy to one's genotype. In this review, we highlight advances in precision genomic medicine in cystic fibrosis (CF), a classic autosomal recessive genetic disorder. We discuss genotype-phenotype correlations in CF, genetic and environmental modifiers of disease, and pharmacogenetic therapies that target specific genetic mutations thereby addressing the primary defect of cystic fibrosis.

  14. Cervical Cystic Hygroma in an Adult

    PubMed Central

    Derin, Serhan; Şahan, Murat; Dere, Yelda; Çullu, Neşat; Şahan, Leyla

    2014-01-01

    Cystic hygromas/lymphangiomas are extremely rare malformations in adults. They are usually seen in infants and children under 2 years of age. En bloc resection is difficult due to the adhesive characteristics of the tumors. Inadequate surgical intervention often leads to recurrent disease. We report herein the case of a cystic hygroma/lymphangioma that presented as an uncommon mass on the cervical region in an adult, together with its histopathological, radiologic, and operative features. PMID:25548704

  15. Cervical cystic hygroma in an adult.

    PubMed

    Derin, Serhan; Şahan, Murat; Dere, Yelda; Çullu, Neşat; Şahan, Leyla

    2014-01-01

    Cystic hygromas/lymphangiomas are extremely rare malformations in adults. They are usually seen in infants and children under 2 years of age. En bloc resection is difficult due to the adhesive characteristics of the tumors. Inadequate surgical intervention often leads to recurrent disease. We report herein the case of a cystic hygroma/lymphangioma that presented as an uncommon mass on the cervical region in an adult, together with its histopathological, radiologic, and operative features. PMID:25548704

  16. [Cystic degeneration of autonomous adenomas (author's transl)].

    PubMed

    Galvan, G; Pohl, G B

    1976-01-01

    Follow-up examinations in four patients with autonomous adenomas showed cystic degeneration in the autonomous adenomas 20 to 45 months after the first examination, confirmed by fine needle biopsy. Clinical improvement occurred three times with scintigraphic compensation, decompensation occurred once without clinical deterioration. In particular cases a therapeutic policy of wait and see is justified in patients with autonomous adenomas because they may remain clinically inconspicuous for a long time; on the other hand there is a possibility of a cystic degeneration.

  17. Malignant cause of ventriculoperitoneal shunt 'pseudocyst': a case report.

    PubMed

    Awori, Jonathan; Wu, Chris Y; Maher, Cormac O

    2015-01-01

    Abdominal pseudocysts are an uncommon complication of ventriculoperitoneal (VP) shunts. We present the case of a 4-year-old boy with a history of complicated hydrocephalus managed with a VP shunt due to sequelae of prematurity. The patient presented with abdominal distention, and a pseudocyst was diagnosed. Despite shunt externalization and aspiration, the pseudocyst continued to produce up to 1 liter of serosanguineous fluid per day. After MRI revealed malignant features within the pseudocyst, laparotomy was performed and the pseudocyst was partially excised. Pathology reports suggested sarcoma. The cystic mass grew back aggressively, accompanied by distant metastasis. The patient's condition deteriorated and he died from his disease. To our knowledge, this represents the first report of an abdominal malignancy mimicking a pseudocyst and causing VP shunt failure.

  18. Retroperitoneal malignant fibrous histiocytoma can mimic a hydatid cyst.

    PubMed

    Erbay, Gurcan; Ulusan, Serife; Koc, Zafer; Canpolat, Emine Tuba; Calıskan, Kenan

    2011-01-01

    Malignant fibrous histiocytoma is the second most common soft-tissue sarcoma in adults. After the extremities, the retroperitoneal space is the second most common site of this tumor. A 50-year-old man presented with a right retroperitoneal, thick-walled, cystic multilocular mass measuring 10 × 10 cm that was thought to be a type CE 5 hydatid cyst preoperatively. However, the postoperative histopathology did not agree with the radiological findings and instead showed a malignant fibrous histiocytoma. The computed tomography and ultrasound/Doppler ultrasound findings of this retroperitoneal mass mimicked a type CE 5 hydatid cyst. We present this case because the surgical management of these two lesions differs and misdiagnosis can be problematic.

  19. Panuveal malignant mesenchymoma.

    PubMed

    Pe'er, J; Neudorfer, M; Ron, N; Anteby, I; Lazar, M; Rosenmann, E

    1995-09-01

    Intraocular malignant mesenchymal tumors are very rare, and only a few case reports of such primary and metastatic tumors have been reported. We report a case of a malignant mesenchymoma involving the entire uveal tract. A 21-year-old woman presented with a tumor on the whole iris of the right eye, which caused intractable glaucoma. Upon enucleation of the eye, a very anaplastic tumor was found to occupy the whole uveal tract; its features were compatible with a tumor of mesenchymal origin, including rhabdomyosarcomatous and liposarcomatous characteristics. Choroidal osteoma was a coincidental finding. The histologic findings of the tumor were of two types of malignant mesenchymal tumors, and therefore the diagnosis of malignant mesenchymoma was made. This is to our knowledge the first tumor of its kind to be reported intraocularly. PMID:7668945

  20. Diffuse cystic lung disease due to pulmonary metastasis of colorectal carcinoma

    PubMed Central

    Fielli, Mariano; Avila, Fabio; Saino, Agustina; Seimah, Deborah; Fernández Casares, Marcelo

    2016-01-01

    The diffuse cystic lung diseases (DCLDs) are a pathophysiologically heterogeneous processes characterized by the presence of multiple thin-walled, air-filled spaces within the pulmonary parenchyma. The most common causes of DCLD are lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH). DCLD develops rarely as a result of malignancy, typically secondary to metastases from peripheral sarcomas and mesenchymal tumors. DCLD have also been reported in a variety of other metastatic disease such as adenocarcinoma. Our case describes a patient with DCLD as a result of metastatic colorectal adenocarcinoma. PMID:27222791

  1. Giant colon diverticulum.

    PubMed

    Chater, C; Saudemont, A; Zerbib, P

    2015-11-01

    Giant colonic diverticulum is defined by a diverticulum whose diameter is greater than 4 cm. This is a rare entity, arising mainly in the sigmoid colon. The diagnosis is based on abdominal computed tomography that shows a gas-filled structure communicating with the adjacent colon, with a smooth, thin diverticular wall that does not enhance after injection of contrast. Surgical treatment is recommended even in asymptomatic diverticula, due to the high prevalence and severity of complications. The gold standard treatment is segmental colectomy. Some authors propose a diverticulectomy when the giant diverticulum is unique.

  2. The Malignant Protein Puzzle.

    PubMed

    Walker, Lary C; Jucker, Mathias

    2016-01-01

    When most people hear the words malignant and brain, cancer immediately comes to mind. But our authors argue that proteins can be malignant too, and can spread harmfully through the brain in neurodegenerative diseases that include Alzheimer's, Parkinson's, CTE, and ALS. Studying how proteins such as PrP, amyloid beta, tau, and others aggregate and spread, and kill brain cells, represents a crucial new frontier in neuroscience. PMID:27408676

  3. 18F-FDG PET/CT Brain Imaging on a Patient With Paraneoplastic Opsoclonus-Myoclonus Syndrome Arising out of a Mature Cystic Teratoma.

    PubMed

    Na, Chang Ju; Jeong, Young Jin; Lim, Seok Tae; Sohn, Myung-Hee; Jeong, Hwan-Jeong

    2016-02-01

    Opsoclonus-myoclonus syndrome (OMS) is an involuntary multidirectional eye movement accompanied by myoclonic jerks and a subtype of paraneoplastic neurological syndromes. Clinical features of OMS include opsoclonus with myoclonic jerks and cerebellar ataxia. Although there have been a few studies on brain FDG PET in paraneoplastic neurological syndrome associated with some kinds of malignancies such as lung and gastric cancer, brain FDG PET of patients with OMS caused by a mature cystic teratoma has not been reported. Here, we described a case of brain FDG PET/CT studies performed in a woman with OMS provoked from a mature cystic teratoma.

  4. Clinical evaluation of cytological diagnosis of nasopharyngeal malignancies.

    PubMed

    Molinari, R; Pilotti, S; Rilke, F

    1978-01-01

    Between 1970 and 1975 cytological examination was applied to the diagnosis of nasopharyngeal malignancies in a series of 216 consecutive patients who had either a tumour in the nasopharynx or clinical signs of nasopharyngeal carcinoma, or who were locally asymptomatic but had enlarged cervical lymph nodes. Smears were taken by introducing a small rough pad of compressed gauze through the mouth into the nasopharynx with an upward-angled forceps. In each case the cytological smear was taken immediately before biopsy; often, a lymph node was removed subsequently. When morphological diagnoses were doubtful and histological findings were at variance with positive cytological findings, the patients were reexamined clinically, and diagnosis was postponed. The case material was made up of 90 nasopharyngeal carcinomas, 24 lymphomas, one malignant melanoma, one adenoid cystic carcinoma and 100 patients without malignancies. Cytological findings from the first smear were positive in 77.8% of nasopharyngeal carcinomas, in 66.6% of lymphomas and in the cases of melanoma and adenoid cystic carcinoma. There were no false-positive results. When the nasopharyngeal carcinomas were subdivided into undifferentiated carcinomas of the nasopharyngeal type and squamous-cell carcinomas, cytological findings were positive in ,0% and 73%, respectively. Positivity of histological findings was distributed as follows: 91.7% for malignant lymphomas, 86.6% for undifferentiated carcinomas and 86.6% for squamous-cell carcinomas. With respect to clinical suspicion of malignancy, positive cytological findings were obtained in 50% of clinically occult cases and in 84.6% of patients with obvious malignancies; intermediate figures were found for clinically doubtful (64.3%) and for highly suspicious (77.8%) cases. Cyto-histological concordance was shown in 70% of cases; false-negative histological results were obtained in 7.8% and false-negative cytological results in 16.6% of cases. Combined cyto

  5. Histopathologic and immunohistochemical study in one case of cystadenoma of parotid gland becoming malignant.

    PubMed

    Simionescu, C; Florescu, M; Margaritescu, C; Marinescu, M

    Cystadenoma and cystadenocarcinoma of the salivary glands are rare tumors, approximately half of cases being diagnosed at the level of parotid. The studied tumor associated both benign and malign zones, with deep infiltrating character in the adjacent structures. In both areas the growing pattern was predominantly cystic and papillary, and the proliferated neoplastic cells were majority cubical or columnar, with low grade of nuclear pleomorphism in the becoming malign areas, discordant with the infiltrative character of the neoplasm. Immunohistochemically, we investigated the tumor with AE1-AE3, CEA, SMA, S-100 and PCNA.

  6. Expression and mutation patterns of p53 in benign and malignant salivary gland tumors.

    PubMed

    Nordkvist, A; Röijer, E; Bang, G; Gustafsson, H; Behrendt, M; Ryd, W; Thoresen, S; Donath, K; Stenman, G

    2000-03-01

    The expression and mutation patterns of p53 were studied in a series of 68 benign pleomorphic adenomas and 237 malignant salivary gland tumors. p53 overexpression (nuclear staining exceeding 10%) was detected in 20% of the malignant salivary gland tumors, with the highest prevalence observed in polymorphous low grade adenocarcinoma, squamous cell carcinoma, and carcinoma ex pleomorphic adenoma and the lowest in adenoid cystic carcinoma and acinic cell carcinoma. In contrast, none of the 68 benign pleomorphic adenomas had nuclear staining exceeding 10%. SSCP and nucleotide sequence analysis of exons 4 to 9 of p53 in 19 malignant tumors revealed 9 mutations in 7 tumors. Our findings indicate that p53 may be a useful marker to help discriminate between benign and malignant salivary gland tumors.

  7. Molecular epidemiology of cystic echinococcosis.

    PubMed

    McManus, D P; Thompson, R C A

    2003-01-01

    Echinococcus granulosus exhibits substantial genetic diversity that has important implications for the design and development of vaccines, diagnostic reagents and drugs effective against this parasite. DNA approaches that have been used for accurate identification of these genetic variants are presented here as is a description of their application in molecular epidemiological surveys of cystic echinococcosis in different geographical settings and host assemblages. The recent publication of the complete sequences of the mitochondrial (mt) genomes of the horse and sheep strains of E. granulosus and of E. multilocularis, and the availability of mt DNA sequences for a number of other E. granulosus genotypes, has provided additional genetic information that can be used for more in depth strain characterization and taxonomic studies of these parasites. This very rich sequence information has provided a solid molecular basis, along with a range of different biological, epidemiological, biochemical and other molecular-genetic criteria, for revising the taxonomy of the genus Echinococcus. This has been a controversial issue for some time. Furthermore, the accumulating genetic data may allow insight to several other unresolved questions such as confirming the occurrence and precise nature of the E. granulosus G9 genotype and its reservoir in Poland, whether it is present elsewhere, why the camel strain (G6 genotype) appears to affect humans in certain geographical areas but not others, more precise delineation of the host and geographic ranges of the genotypes characterised to date, and whether additional genotypes of E. granulosus remain to be identified. PMID:15042999

  8. Respiratory Conditions Update: Cystic Fibrosis.

    PubMed

    Pritchard, Lyle L

    2016-09-01

    Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction. Pulmonary system dysfunction causes the most morbidity and mortality. Pulmonary function testing is the primary modality used to monitor CF progression. Therapies include chest physiotherapy, mucolytics, antibiotics, anti-inflammatory drugs, targeted therapies, and vaccines. Dysfunction of the exocrine pancreas and gastrointestinal tract leads to malabsorption, malnutrition, and intestinal obstruction. Nutrition should be optimized with adequate calories, pancreatic enzymes, and appropriate dietary supplements. Complications, including acute pulmonary exacerbations, gastrointestinal conditions, chronic rhinosinusitis, CF-related diabetes, osteoporosis, infertility, and psychosocial issues, must be managed. At the appropriate time, lung transplantation and end-of-life issues must be addressed. PMID:27576234

  9. Lung Transplantation for Cystic Fibrosis

    PubMed Central

    Adler, Frederick R.; Aurora, Paul; Barker, David H.; Barr, Mark L.; Blackwell, Laura S.; Bosma, Otto H.; Brown, Samuel; Cox, D. R.; Jensen, Judy L.; Kurland, Geoffrey; Nossent, George D.; Quittner, Alexandra L.; Robinson, Walter M.; Romero, Sandy L.; Spencer, Helen; Sweet, Stuart C.; van der Bij, Wim; Vermeulen, J.; Verschuuren, Erik A. M.; Vrijlandt, Elianne J. L. E.; Walsh, William; Woo, Marlyn S.; Liou, Theodore G.

    2009-01-01

    Lung transplantation is a complex, high-risk, potentially life-saving therapy for the end-stage lung disease of cystic fibrosis (CF). The decision to pursue transplantation involves comparing the likelihood of survival with and without transplantation as well as assessing the effect of wait-listing and transplantation on the patient's quality of life. Although recent population-based analyses of the US lung allocation system for the CF population have raised controversies about the survival benefits of transplantation, studies from the United Kingdom and Canada have suggested a definite survival advantage for those receiving transplants. In response to these and other controversies, leaders in transplantation and CF met together in Lansdowne, Virginia, to consider the state of the art in lung transplantation for CF in an international context, focusing on advances in surgical technique, measurement of outcomes, use of prognostic criteria, variations in local control over listing, and prioritization among the United States, Canada, the United Kingdom, and The Netherlands, patient adherence before and after transplantation and other issues in the broader context of lung transplantation. Finally, the conference members carefully considered how efforts to improve outcomes for lung transplantation for CF lung disease might best be studied. This Roundtable seeks to communicate the substance of our discussions. PMID:20008865

  10. Evaluation of hepatic cystic lesions

    PubMed Central

    Lantinga, Marten A; Gevers, Tom JG; Drenth, Joost PH

    2013-01-01

    Hepatic cysts are increasingly found as a mere coincidence on abdominal imaging techniques, such as ultrasonography (USG), computed tomography (CT) and magnetic resonance imaging (MRI). These cysts often present a diagnostic challenge. Therefore, we performed a review of the recent literature and developed an evidence-based diagnostic algorithm to guide clinicians in characterising these lesions. Simple cysts are the most common cystic liver disease, and diagnosis is based on typical USG characteristics. Serodiagnostic tests and microbubble contrast-enhanced ultrasound (CEUS) are invaluable in differentiating complicated cysts, echinococcosis and cystadenoma/cystadenocarcinoma when USG, CT and MRI show ambiguous findings. Therefore, serodiagnostic tests and CEUS reduce the need for invasive procedures. Polycystic liver disease (PLD) is arbitrarily defined as the presence of > 20 liver cysts and can present as two distinct genetic disorders: autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (PCLD). Although genetic testing for ADPKD and PCLD is possible, it is rarely performed because it does not affect the therapeutic management of PLD. USG screening of the liver and both kidneys combined with extensive family history taking are the cornerstone of diagnostic decision making in PLD. In conclusion, an amalgamation of these recent advances results in a diagnostic algorithm that facilitates evidence-based clinical decision making. PMID:23801855

  11. [Azithromycin therapy in cystic fibrosis].

    PubMed

    Máiz Carro, Luis; Cantón Moreno, Rafael

    2004-03-01

    Progressive lung disease, caused by chronic endobronchial colonization, is the major cause of morbidity and mortality in patients with cystic fibrosis (CF). Several pathogens, including Staphylococcus aureus and Pseudomonas aeruginosa are responsible for this effect. The steadily improving prognosis of CF has been attributed to the use of antibiotics with activity against these organisms. Despite a significant increase in the amount of published material demonstrating the potential role of macrolide antibiotics as antiinflammatory agents and their effects on bacterial virulence, their mechanism of action in CF patients is still unknown. Although there is a limited number of clinical trials assessing the efficacy and safety of azithromycin (AZM) in CF, increasing evidence suggests that 3 to 6-month AZM treatment in CF patients is safe and well tolerated. This treatment results in clinical improvement, decreasing the number of pulmonary exacerbations and increasing pulmonary function. Therefore, chronic treatment with AZM should be considered in CF patients added to conventional therapy. Clinical experience with macrolides other than AZM in CF patients is very limited. PMID:15030744

  12. Dual FISH analysis of benign and malignant tumors of the salivary glands and paranasal sinuses.

    PubMed

    Götte, Karl; Ganssmann, Stefan; Affolter, Annette; Schäfer, Carsten; Riedel, Frank; Arens, Norbert; Finger, Sonja; Hörmann, Karl

    2005-11-01

    To date, the underlying genomic changes in benign and malignant tumors of salivary-gland and paranasal-sinus origin are poorly understood. This is due in part to the low incidence of these tumors and the enormous histological variety of tumors within this head and neck region. We examined 58 of these tumors (14 adenoid cystic carcinomas, 9 adenocarcinomas, 5 cylindrical carcinomas, 11 pleomorphic adenomas, and 19 inverted papillomas) by dual fluorescence in situ hybridization (FISH) with centromere-specific probes on six chromosomes (3, 7, 9, 11, 17, and 18) for numerical changes. In adenoid cystic carcinomas, monosomy of chromosome 17 and polysomy of chromosomes 3, 9 and 11 were most frequently encountered. In adenocarcinomas, monosomy of chromosome 17 and polysomy of chromosomes 7 and 11 were most frequent. In cylindrical cell carcinomas, polysomy of chromosomes 7, 9, 11 and 17 was present in the majority of tumors. Disomy is rare, even in benign tumors. Polysomy is more frequent in malignant tumors than in benign. Tetrasomy is found almost only in malignant tumors. In summary, the occurrence of polysomy might reflect a step towards malignancy in tumors of the salivary glands and paranasal mucosa. Polysomy of chromosome 11 could be defined as typical for all investigated histological types of malignant tumor in this region of the head and neck. PMID:16211271

  13. CXCR4+ granulocytes reflect fungal cystic fibrosis lung disease.

    PubMed

    Carevic, Melanie; Singh, Anurag; Rieber, Nikolaus; Eickmeier, Olaf; Griese, Matthias; Hector, Andreas; Hartl, Dominik

    2015-08-01

    Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy control subjects cross-sectionally and longitudinally and correlated these findings with lung function parameters.Cystic fibrosis patients with chronic fungal colonisation by Aspergillus fumigatus were characterised by an accumulation of a distinct granulocyte subset, expressing the HIV coreceptor CXCR4. Percentages of airway CXCR4(+) granulocytes correlated with lung disease severity in patients with cystic fibrosis.These studies demonstrate that chronic fungal colonisation with A. fumigatus in cystic fibrosis patients is associated with CXCR4(+) airway granulocytes, which may serve as a potential biomarker and therapeutic target in fungal cystic fibrosis lung disease. PMID:25929952

  14. Genetics Home Reference: medullary cystic kidney disease type 1

    MedlinePlus

    ... disease type 1 medullary cystic kidney disease type 1 Enable Javascript to view the expand/collapse boxes. ... Close All Description Medullary cystic kidney disease type 1 (MCKD1) is an inherited condition that affects the ...

  15. Rehabilitation with Cystic Fibrosis: From Utopia to Reality.

    ERIC Educational Resources Information Center

    Goldberg, Richard T.; And Others

    1980-01-01

    The paper dispels some of the myths regarding cystic fibrosis (a genetic metabolism disorder), provides information on the latest developments in rehabilitation, summarizes research in the field, and projects future needs of the patient with cystic fibrosis. (SBH)

  16. CXCR4+ granulocytes reflect fungal cystic fibrosis lung disease.

    PubMed

    Carevic, Melanie; Singh, Anurag; Rieber, Nikolaus; Eickmeier, Olaf; Griese, Matthias; Hector, Andreas; Hartl, Dominik

    2015-08-01

    Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy control subjects cross-sectionally and longitudinally and correlated these findings with lung function parameters.Cystic fibrosis patients with chronic fungal colonisation by Aspergillus fumigatus were characterised by an accumulation of a distinct granulocyte subset, expressing the HIV coreceptor CXCR4. Percentages of airway CXCR4(+) granulocytes correlated with lung disease severity in patients with cystic fibrosis.These studies demonstrate that chronic fungal colonisation with A. fumigatus in cystic fibrosis patients is associated with CXCR4(+) airway granulocytes, which may serve as a potential biomarker and therapeutic target in fungal cystic fibrosis lung disease.

  17. Adenoid cystic carcinoma of the parotid gland associated with salivary calculi: An unusual presentation.

    PubMed

    Shenoy, Vijendra S; Kamath, M Panduranga; Sreedharan, Suja; Suhas, S S

    2015-01-01

    Adenoid cystic carcinomas (ACC) of the head and neck are relatively rare tumors, consisting of approximately 10-15% of all salivary gland neoplasms. ACC, a slow-growing aggressive malignant tumor of salivary gland commonly seen in the submandibular, sublingual, minor salivary glands is seldom found in the parotid. Calculus, the common cause of salivary gland dysfunction is usually identified in submandibular salivary gland because of its duct anatomy and physiochemical characteristic serous secretion. We report an unusual case of co-existent presentation of ACC with salivary calculi in the parotid gland which is never been reported in the literature. Co-existence of ductal calculi and ACC is rare. Presence of parotid calculus could be due to long standing ductal obstruction by the slow-growing ACC of the parotid or other possibility is that the malignancy could have developed because of chronic irritation by parotid calculi. Confirmatory studies are required to understand its mutual pathological association. PMID:26458630

  18. Metastatic Bladder Cancer Presenting with Persistent Hematuria in Young Man with Cystic Fibrosis

    PubMed Central

    Patel, Premal; Rabin, Harvey R.; Vickers, Michael M.; Parkins, Michael D.

    2013-01-01

    We report a case of metastatic bladder cancer developing in a young man with cystic fibrosis (CF) that was initially diagnosed as ureterolithiasis and managed as renal colic. With the improved survival of patients with CF, an increasing burden of extrapulmonary disease manifestations is apparent. Renal colic is observed at an increased frequency in patients with CF relative to the general population and is a commonly recognized cause of hematuria. However, CF patients harboring a malignancy are recognized to be at increased risk of delayed identification owing to atypical symptoms and lack of demographic risk factors. This case illustrates how investigations to rule out malignancy are warranted in those CF patients not responding to therapies directed towards presumptive diagnoses. PMID:23762727

  19. Inflammatory malignant fibrous histiocytoma of the retroperitoneum.

    PubMed

    Cvjetko, Ivan; Kocman, Branko; Sitić, Sanda

    2011-03-01

    The authors present a case of inflammatory malignant fibrous histiocytoma located in the left retroperitoneum. The tumor was resected enblock with kidney and suprarenal gland. During the resection the system of retractors called the pillars of Kocman was used which allowed wide exposure of the abdominal cavity. The tumor measured 23 x 17 x 10 cm with the left kidney and suprarenal incorporated. The tumor was centrally pseudocystic made of xanthomatous cells, foamy cells and rare giant cells with storiform formations and infiltrated with neutrophils. Imunohistochemically, the tumor cells were vimentin and CD 68 positive and CD 20, CD3, EMA, S-100, HMB 45, CD 34 and CD 1a negative. Neutrophils were CD 15 positive.

  20. Primary Malignant Tumours of Bone Following Previous Malignancy

    PubMed Central

    Patton, J. T.; Sommerville, S. M. M.; Grimer, R. J.

    2008-01-01

    Destructive bone lesions occurring in patients who have previously had a malignancy are generally assumed to be a metastasis from that malignancy. We reviewed 60 patients with a previous history of malignancy, who presented with a solitary bone lesion that was subsequently found to be a new and different primary sarcoma of bone. These second malignancies occurred in three distinct groups of patients: (1) patients with original tumours well known to be associated with second malignancies (5%); (2) patients whose second malignancies were likely to be due to the previous treatment of their primary malignancy (40%); (3) patients in whom there was no clearly defined association between malignancies (55%). The purpose of this study is to emphasise the necessity for caution in assuming the diagnosis of a metastasis when a solitary bone lesion is identified following a prior malignancy. Inappropriate biopsy and treatment of primary bone sarcomas compromises limb salvage surgery and can affect patient mortality. PMID:18414590

  1. Ovarian Carcinosarcoma and Its Association with Mature Cystic Teratoma and Primary Tubal Carcinoma

    PubMed Central

    Satabongkoch, Nopporn; Suprasert, Prapaporn; Khunamornpong, Surapan

    2016-01-01

    Introduction. Carcinosarcoma is an uncommon form of ovarian cancers, classified as being part of the group of mixed epithelial and mesenchymal tumors. The occurrence of carcinosarcoma in association with a mature cystic teratoma and synchronous tubal carcinoma is very rare. Case Report. A 69-year-old woman presented with a pelvic mass. An abdominal computerized tomographic scan detected a 15 cm right pelvic mass which was suggestive of malignant transformation of a dermoid cyst. Intraoperative, bilateral ovarian masses (left 10 cm and right 12 cm) with diffuse peritoneal metastatic nodules were identified. Histologically, the left ovarian mass was composed of 2 components including carcinosarcoma and mature cystic teratoma, whereas the right ovarian mass represented a mature cystic teratoma with serosal surface involvement of high-grade serous adenocarcinoma. The left fallopian tube was macroscopically unremarkable but contained a 5.0 mm focus of high-grade serous adenocarcinoma in the distal part, with adjacent serous tubal intraepithelial carcinoma. Conclusion. As the fallopian tube has recently been proposed to be an origin for a majority of pelvic or ovarian high-grade serous adenocarcinomas, tubal carcinoma may be the origin for ovarian carcinosarcomas through an epithelial-mesenchymal transition. The coexistence of ovarian carcinosarcoma and teratoma in the present case should represent a collision tumor. PMID:27807494

  2. Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1

    PubMed Central

    Alina, Basnet; Sebastian, Jofre A.; Gerardo, Capo

    2015-01-01

    Malignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence of MTTs in two sisters. The first patient is a 36-year-old female who presented with left sided chest wall swelling. She also had clinical features consistent with neurofibromatosis type 1 (NF-1). Debulking of the mass showed high-grade malignant peripheral nerve sheath tumor with skeletal muscle differentiation (MTT). The patient was treated with ifosfamide and adriamycin along with radiation. Four years after treatment, she still has no evidence of disease recurrence. Her sister subsequently presented to us at the age of 42 with left sided lateral chest wall pain. Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement. The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT). The patient was started on chemotherapy and radiation as described above. PMID:26114002

  3. Paranasal sinuses malignancies: A 12-year review of clinical characteristics

    PubMed Central

    Sarafraz, Alireza; Chamani, Mojtaba; Derakhshandeh, Hossein

    2016-01-01

    Background Inadequate epidemiologic investigations of the paranasal sinuses malignancies prompted this retrospective study with special emphasis on a major group of 111 tumors. Material and Methods Clinical records of 111 patients with histologically confirmed malignant tumors of the paranasal sinuses were investigated retrospectively from April 2000 to January 2012. Collection of data included demographic information, clinical manifestations, treatment plans, and histopathology of the tumor. Results There were 69 (62.16%) male and 42 (37.83%) female patients (male-to-female ratio of 1.6:1), with a median age of 49±12.2 years (range 21 to 88 years). A high level of occurrence was noticed in the fifth (26.3%) decade of life. The most frequent histological types were squamous cell carcinoma (43.5%) and adenoid cystic carcinoma (19%). Among clinical manifestations, nasal obstruction was the most frequent followed by diplopia, and facial swelling. Fifty three patients (47.74%) were treated with combined approach of surgery and radiation therapy. Conclusions Paranasal sinuses malignancies are rare conditions with nonspecific symptoms which make early diagnosis of the lesions more challenging. The optimal therapeutic protocol for patients suffering from these tumors is still a somewhat controversial entity and requires further studies. Key words:Paranasal sinuses, malignancy, surgery,radiotherapy. PMID:27475693

  4. Endoscopic transnasal management of sinonasal malignancies – our initial experience

    PubMed Central

    Osuch-Wójcikiewicz, Ewa; Held-Ziółkowska, Marta; Kużmińska, Magdalena; Niemczyk, Kazimierz

    2014-01-01

    Introduction Malignant tumors of the paranasal sinuses are traditionally managed through external approaches. Advances in endoscopic transnasal surgery have allowed for the endoscopic treatment of some of these tumors. Aim To present the results of treatment of a series of patients with paranasal sinus malignancies treated with an endoscopic approach at a single institution. Material and methods The data on tumor type, operative technique, perioperative complications and postoperative course were analyzed. Results Eleven patients meeting the inclusion criteria were identified. The histopathology was as follows: malignant melanoma in 3 patients, squamous cell carcinoma in 2, adenocarcinoma in 2, poorly differentiated carcinoma in 1, hemangiopericytoma in 1, adenoid cystic carcinoma in 1 and fibrosarcoma in 1. There were no severe perioperative complications with the exception of 1 case of cerebrospinal fluid leak, which was successfully closed. The mean observation period was 13.5 months. One of the patients died of disease, another was lost to follow-up, and one was reoperated on due to recurrence. The remaining 8 patients are alive with no signs of recurrence. Conclusions Our initial experience seems to confirm results obtained by other authors indicating that in selected cases endoscopic surgery of sinonasal malignancies is similarly effective as external approach surgery. PMID:25097677

  5. Nucleolar organizer regions in malignant salivary gland tumors.

    PubMed

    Fujita, S; Takahashi, H; Okabe, H

    1992-10-01

    Proliferative activity of carcinomas arising from salivary glands was analyzed by enumeration of argyrophilic nucleolar organizer regions (AgNORs). The mean numbers of AgNORs in the various tumors were as follows: mucoepidermoid carcinoma, 2.20; acinic cell carcinoma, 2.51; adenoid cystic carcinoma (ACC), 2.57; carcinoma in pleomorphic adenoma, 1.00 (benign component) and 3.99 (cancer-bearing area); salivary duct carcinoma, 4.49; polymorphous low-grade adenocarcinoma, 3.37; sebaceous carcinoma, 2.57; oncocytic carcinoma, 4.63; adenocarcinoma, 4.53. Cells of most tumors showed heterogeneous activity within the same tumor. In mucoepidermoid carcinoma, the mucous cells had suppressed activity in comparison with the epidermoid cells and intermediate cells. In ACC, the activity of the tumor cells increased according to growth pattern in the order tubular, glandular and solid. In carcinoma in pleomorphic adenoma, vigorous proliferative activity was observed in the malignant component, whereas less active cells were seen in the myxoid or chondroid matrix. AgNOR staining was useful for distinguishing benign from malignant regions in carcinoma in pleomorphic adenoma. Our results suggest that mucoepidermoid carcinoma, acinic cell carcinoma and ACC, except for that with a solid growth pattern, may be considered as low-grade malignancies, whereas solid-type ACC, the cancer component in carcinoma in pleomorphic adenoma and some of the other carcinomas have high-grade malignant behavior.

  6. Multidisciplinary approach to giant paratesticular liposarcoma

    PubMed Central

    Sopeña-Sutil, Raquel; Silan, Francesco; Butron-Vila, Maria Teresa; Guerrero-Ramos, Felix; Lagaron-Comba, Emilio; Passas-Martinez, Juan

    2016-01-01

    Primary paratesticular tumours are very rare and the spermatic cord (SCT) is the most frequent site of origin, with 20% of malignancy. Although liposarcoma is the most frequent histotype (46.6 %), less than 200 cases have been reported in the literature. We report the case of a 56-year-old man who presented a giant scrotal mass of 25 years of evolution and measuring 40 × 40 cm. It could be considered the greatest paratesticular liposarcoma described to date. Computed tomogaphy (CT) revealed mass features consistent with liposarcoma and the simultaneous presence of bilateral inguinal hernia with bladder involvement. A multidisciplinary approach was taken to remove the mass, solve the hernia, and provide functional results.

  7. Radionuclide bone scanning in giant cell tumor

    SciTech Connect

    Van Nostrand, D.; Madewell, J.E.; McNiesh, L.M.; Kyle, R.W.; Sweet, D.

    1986-03-01

    Radionuclide bone scan findings are described and correlated with pathology in 23 patients with giant cell tumor (GCT) of the bone. The degree of radionuclide activity was markedly increased in 20 (87%), minimally increased in three (13%), and decreased in none of the patients. Of the 23 patients with increased radioactivity, the pattern was diffuse in 11 (48%) and doughnut in 12 (52%). Extended patterns of radioactivity were present in 19 of 22 patients; however, none were associated with true tumor extension. Bone scanning did not aid in the detection of GCT, was nonspecific, and did not differentiate benign from malignant GCT. Although radioactivity extended beyond the radiographic abnormality in the majority of patients, this was most likely secondary to other bony abnormalities or local and/or regional hyperemia, and caution should be taken in ascribing this extension to either tumor or metastasis.

  8. Multidisciplinary approach to giant paratesticular liposarcoma

    PubMed Central

    Sopeña-Sutil, Raquel; Silan, Francesco; Butron-Vila, Maria Teresa; Guerrero-Ramos, Felix; Lagaron-Comba, Emilio; Passas-Martinez, Juan

    2016-01-01

    Primary paratesticular tumours are very rare and the spermatic cord (SCT) is the most frequent site of origin, with 20% of malignancy. Although liposarcoma is the most frequent histotype (46.6 %), less than 200 cases have been reported in the literature. We report the case of a 56-year-old man who presented a giant scrotal mass of 25 years of evolution and measuring 40 × 40 cm. It could be considered the greatest paratesticular liposarcoma described to date. Computed tomogaphy (CT) revealed mass features consistent with liposarcoma and the simultaneous presence of bilateral inguinal hernia with bladder involvement. A multidisciplinary approach was taken to remove the mass, solve the hernia, and provide functional results. PMID:27695588

  9. Living with Cystic Fibrosis: A Guide for the Young Adult.

    ERIC Educational Resources Information Center

    Cystic Fibrosis Foundation, Atlanta, GA.

    Intended for the young adult with cystic fibrosis, the booklet provides information on dealing with problems and on advances in treatment and detection related to the disease. Addressed are the following topics: description of cystic fibrosis; inheritance of cystic fibrosis; early diagnosis; friends, careers, and other matters; treatment;…

  10. 78 FR 26681 - Medical Criteria for Evaluating Cystic Fibrosis

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-05-07

    ... ADMINISTRATION RIN 0960-AF58 Medical Criteria for Evaluating Cystic Fibrosis AGENCY: Social Security....04 to evaluate claims involving cystic fibrosis in adults and children under titles II and XVI of the... information on the disability program. 2. Information for individuals with cystic fibrosis who apply...

  11. Arthroscopic Decompression for a Giant Meniscal Cyst.

    PubMed

    Ohishi, Tsuyoshi; Suzuki, Daisuke; Matsuyama, Yukihiro

    2016-01-01

    The authors report the case of a giant medial meniscal cyst in an osteoarthritic knee of an 82-year-old woman that was successfully treated with only arthroscopic cyst decompression. The patient noticed a painful mass on the medial side of the right knee that had been gradually growing for 5 years. Magnetic resonance imaging showed an encapsulated large medial cystic mass measuring 80×65×40 mm that was adjacent to the medial meniscus. An accompanying horizontal tear was also detected in the middle and posterior segments of the meniscus. The medial meniscus was resected up to the capsular attachment to create bidirectional flow between the joint and the cyst with arthroscopic surgery. Magnetic resonance imaging performed 14 months postoperatively showed that the cyst had completely disappeared, and no recurrence was observed during a 2-year follow-up period. An excellent result could be obtained by performing limited meniscectomy to create a channel leading to the meniscal cyst, even though the cyst was large. Among previously reported cases of meniscal cysts, this case is the largest to be treated arthroscopically without open excision.

  12. Hemostasis and malignancy.

    PubMed

    Francis, J L; Biggerstaff, J; Amirkhosravi, A

    1998-01-01

    There is considerable evidence that the hemostatic system is involved in the growth and spread of malignant disease. There is an increased incidence of thromboembolic disease in patients with cancers and hemostatic abnormalities are extremely common in such patients. Antihemostatic agents have been successfully used to treat a variety of experimental tumors, and several clinical trials in humans have been initiated. Although metastasis is undoubtedly multifactorial, intravascular coagulation activation and peritumor fibrin deposition seem to be important. The mechanisms by which hemostatic activation facilitates the malignant process remain to be completely elucidated. Of central importance may be the presence on malignant cells of tissue factor and urokinase receptor. Recent studies have suggested that these proteins, and others, may be involved at several stages of metastasis, including the key event of neovascularization. Tissue factor, the principal initiator of coagulation, may have additional roles, outside of fibrin formation, that are central to the biology of some solid tumors.

  13. Hemostasis and malignancy.

    PubMed

    Francis, J L; Biggerstaff, J; Amirkhosravi, A

    1998-01-01

    There is considerable evidence that the hemostatic system is involved in the growth and spread of malignant disease. There is an increased incidence of thromboembolic disease in patients with cancers and hemostatic abnormalities are extremely common in such patients. Antihemostatic agents have been successfully used to treat a variety of experimental tumors, and several clinical trials in humans have been initiated. Although metastasis is undoubtedly multifactorial, intravascular coagulation activation and peritumor fibrin deposition seem to be important. The mechanisms by which hemostatic activation facilitates the malignant process remain to be completely elucidated. Of central importance may be the presence on malignant cells of tissue factor and urokinase receptor. Recent studies have suggested that these proteins, and others, may be involved at several stages of metastasis, including the key event of neovascularization. Tissue factor, the principal initiator of coagulation, may have additional roles, outside of fibrin formation, that are central to the biology of some solid tumors. PMID:9579631

  14. Malignancy-Associated Dyslipidemia

    PubMed Central

    Bielecka-Dąbrowa, Agata; Hannam, Simon; Rysz, Jacek; Banach, Maciej

    2011-01-01

    Cholesterol and triglycerides, important lipid constituents of cell, are essential to carry out several vital physiological functions. Lipids might be associated with cancers because they play a key role in the maintenance of cell integrity. The pathway for cholesterol synthesis may also produce various tumorigenic compounds and cholesterol serves as a precursor for the synthesis of many sex hormones linked to increased risk of various cancers. In some malignant diseases, blood cholesterol undergoes early and significant changes. The mechanism for the link between cancer and cholesterol remains controversial. The dates from studies are confusing because both hypolipidemia and hypercholesterolemia might be connected with malignancy. Not only cancers but also antineoplastic therapies have an influence on lipid profile. There are also dates suggesting that antihyperlipemic drugs might nfluenced malignancy. PMID:21660223

  15. Do brine shrimp diagnose cystic fibrosis?

    PubMed

    Hodes, M E; Thomas, J; Morgan, S; Merritt, A D

    1975-11-01

    The nauplii of the brine shrimp Artemia salina are dependent upon the function of their salt gland to maintain osmotic pressure within narrow limits. A number of drugs interfere with this function and are lethal to the nauplii. Saliva and serum from normal persons, patients with cystic fibrosis, and obligate heterozygotes were tested for lethal effect against brine shrimp nauplii. At salt concentrations between 100 mM and 2.5 no difference was found among the phenotypes. At lower concentrations a difference was noted occasionally between some normal subjects and some individuals carrying one or two genes for cystic fibrosis. Data from an independent series of experiments indicate that the naupliar deaths result from distorted ratios of Na+/K+ and not from a specific gene product. No difference was noted in the O2 uptake of nauplii treated with saliva or serum obtained from normal subjects, patients with cystic fibrosis, or obligate heterozygotes. PMID:1187245

  16. Florid cystic endosalpingiosis of the uterus

    PubMed Central

    Heatley, M; Russell, P

    2001-01-01

    A 73 year old woman presented with a right sided adnexal cystic mass. At laparotomy, this proved to be a benign serous ovarian cyst and an aggregation of thin walled subserosal and soft tissue cysts and spongy nodules up to 16 mm in diameter involving the side wall of the uterus and adjacent parametrium. These were removed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histologically, the cystic spaces and smaller acini were lined by benign tubo-endometrioid epithelium, with smaller areas typical of serous differentiation and rare microfoci of endocervical-type mucinous epithelium. These features indicated multidirectional Mullerian differentiation in a process that, overall, was consistent with so called florid cystic endosalpingiosis. This lesion is to be distinguished from other benign conditions including multicystic mesothelioma, endometriosis, endocervicosis, florid deep glands of the uterine cervix, and deep Nabothian cysts of the uterine cervix. Key Words: uterus • endosalpingiosis • cysts PMID:11328842

  17. Surveillance for gastrointestinal malignancies

    PubMed Central

    Tiwari, Ashish K; Laird-Fick, Heather S; Wali, Ramesh K; Roy, Hemant K

    2012-01-01

    Gastrointestinal (GI) malignancies are notorious for frequently progressing to advanced stages even in the absence of serious symptoms, thus leading to delayed diagnoses and dismal prognoses. Secondary prevention of GI malignancies through early detection and treatment of cancer-precursor/premalignant lesions, therefore, is recognized as an effective cancer prevention strategy. In order to efficiently detect these lesions, systemic application of screening tests (surveillance) is needed. However, most of the currently used non-invasive screening tests for GI malignancies (for example, serum markers such as alpha-fetoprotein for hepatocellular carcinoma, and fecal occult blood test, for colon cancer) are only modestly effective necessitating the use of highly invasive endoscopy-based procedures, such as esophagogastroduodenoscopy and colonoscopy for screening purposes. Even for hepatocellular carcinoma where non-invasive imaging (ultrasonography) has become a standard screening tool, the need for repeated liver biopsies of suspicious liver nodules for histopathological confirmation can’t be avoided. The invasive nature and high-cost associated with these screening tools hinders implementation of GI cancer screening programs. Moreover, only a small fraction of general population is truly predisposed to developing GI malignancies, and indeed needs surveillance. To spare the average-risk individuals from superfluous invasive procedures and achieve an economically viable model of cancer prevention, it’s important to identify cohorts in general population that are at substantially high risk of developing GI malignancies (risk-stratification), and select suitable screening tests for surveillance in these cohorts. We herein provide a brief overview of such high-risk cohorts for different GI malignancies, and the screening strategies that have commonly been employed for surveillance purpose in them. PMID:22969223

  18. Genetics of Cystic Fibrosis: Clinical Implications.

    PubMed

    Egan, Marie E

    2016-03-01

    Cystic fibrosis (CF) is a common life-shortening autosomal recessive genetic disorder caused by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). Almost 2000 variants in the CFTR gene have been identified. The mutational classes are based on the functional consequences on CFTR. New therapies are being developed to target mutant CFTR and restore CFTR function. Understanding specific CF genotypes is essential for providing state-of-the art care to patients. In addition to the variation in CFTR genotype, there are several modifier genes that contribute to the respiratory phenotype.

  19. Diagnosis of Adult Patients with Cystic Fibrosis.

    PubMed

    Nick, Jerry A; Nichols, David P

    2016-03-01

    The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis.

  20. Cystic dilation of the ventriculus terminalis

    PubMed Central

    Kawanishi, Masahiro; Tanaka, Hidekazu; Yokoyama, Kunio; Yamada, Makoto

    2016-01-01

    The ventriculus terminalis (VT) is a virtual cavity of the conus medullaris that appears during embryonic life. We presented a case with the cystic dilation of the ventriculus terminalis (CDVT) in a symptomatic patient. A 66-year-old female suffered from disturbance while walking evolving for the past 2 years. An MR image revealed a cystic dilatation of ventriculus terminalis. The patient experienced marked improvement of lower extremity strength by a fenestration of cyst and cyst-subarachnoid shunt. Treatment for asymptomatic patients seems to be the best conducted conservatively, whereas patients with focal neurological deficits seem to be best handled surgically. PMID:27695242

  1. Cystic dilation of the ventriculus terminalis

    PubMed Central

    Kawanishi, Masahiro; Tanaka, Hidekazu; Yokoyama, Kunio; Yamada, Makoto

    2016-01-01

    The ventriculus terminalis (VT) is a virtual cavity of the conus medullaris that appears during embryonic life. We presented a case with the cystic dilation of the ventriculus terminalis (CDVT) in a symptomatic patient. A 66-year-old female suffered from disturbance while walking evolving for the past 2 years. An MR image revealed a cystic dilatation of ventriculus terminalis. The patient experienced marked improvement of lower extremity strength by a fenestration of cyst and cyst-subarachnoid shunt. Treatment for asymptomatic patients seems to be the best conducted conservatively, whereas patients with focal neurological deficits seem to be best handled surgically.

  2. Idiopathic cystic artery aneurysm complicated with hemobilia.

    PubMed

    Anand, Utpal; Thakur, Sanjeev Kumar; Kumar, Sanjay; Jha, Achyutanand; Prakash, Vijay

    2011-01-01

    Aneurysm of the cystic artery is not common, and it is a rare cause of hemobilia. Most of reported cases are pseudoaneurysms resulting from either an inflammatory process in the abdomen or abdominal trauma. We report a healthy individual who developed hemobilia associated with cystic artery aneurysm. The patient was managed with cholecystectomy and concomitant aneurysm repair. Visceral artery aneurysms are rare and can rupture with potentially grave outcome due to excessive bleeding. Angiographic embolization is a common method of treatment for visceral artery aneurysms. Open cholecystectomy and aneurysm repair was performed in our patient due to radiological evidence of associated cholecystitis.

  3. Giant Solitary Fibrous Tumor of Orbit.

    PubMed

    Tenekeci, Goktekin; Sari, Alper; Vayisoglu, Yusuf; Serin, Onur

    2015-07-01

    Solitary fibrous tumors (SFTs) have been reported in various locations in the body. Solitary fibrous tumors are extremely rare tumors, especially when located in the orbit. Diagnosis of SFT cannot be made based on histopathology only because it exhibits a variable microscopic appearance, and necessitates immunohistochemistry to confirm the diagnosis. A 51-year-old man was admitted to our clinic for the evaluation of a mass bulging in his left eye. Clinical examination revealed a painless mass extruding out of the orbital cavity with dimensions of 8 × 7  cm. Exenteration of the left eye including the upper and lower eyelid and reconstruction of the orbital cavity using a temporoparietal fascia flap and a temporal muscle flap was performed. SFT of orbital region is known as a slow growing and painless tumor. Based on previous studies, increased mitotic rate of the tumor gives the impression that the tumor has a malignant nature. Until now a small number or orbital SFTs were reported and none of them presented with a giant mass protruding out of the orbital cavity. We present a unique case of orbital SFT filling the whole orbital cavity and protruding outward as a giant mass. This case has been reported to expand our knowledge in this debated entity. PMID:26102546

  4. Giant Solitary Fibrous Tumor of Orbit.

    PubMed

    Tenekeci, Goktekin; Sari, Alper; Vayisoglu, Yusuf; Serin, Onur

    2015-07-01

    Solitary fibrous tumors (SFTs) have been reported in various locations in the body. Solitary fibrous tumors are extremely rare tumors, especially when located in the orbit. Diagnosis of SFT cannot be made based on histopathology only because it exhibits a variable microscopic appearance, and necessitates immunohistochemistry to confirm the diagnosis. A 51-year-old man was admitted to our clinic for the evaluation of a mass bulging in his left eye. Clinical examination revealed a painless mass extruding out of the orbital cavity with dimensions of 8 × 7  cm. Exenteration of the left eye including the upper and lower eyelid and reconstruction of the orbital cavity using a temporoparietal fascia flap and a temporal muscle flap was performed. SFT of orbital region is known as a slow growing and painless tumor. Based on previous studies, increased mitotic rate of the tumor gives the impression that the tumor has a malignant nature. Until now a small number or orbital SFTs were reported and none of them presented with a giant mass protruding out of the orbital cavity. We present a unique case of orbital SFT filling the whole orbital cavity and protruding outward as a giant mass. This case has been reported to expand our knowledge in this debated entity.

  5. Cystic renal cell carcinoma and acquired renal cystic disease associated with consumption of chaparral tea: a case report.

    PubMed

    Smith, A Y; Feddersen, R M; Gardner, K D; Davis, C J

    1994-12-01

    Nordihydroguaiaretic acid is an antioxidant used experimentally to induce cystic renal disease in rats. It may be extracted from the leaves of the creosote bush, which are consumed as chaparral tea in the southwestern United States. We report a case of cystic renal disease and cystic adenocarcinoma of the kidney associated with a history of protracted consumption of chaparral tea.

  6. Giant cell tumor of lower end of tibia.

    PubMed

    Bami, Monish; Nayak, Ashok R; Kulkarni, Shreepad; Kulkarni, Avinash; Gupta, Rupali

    2013-01-01

    Introduction. Giant cell tumor of bones is an unusual neoplasm that accounts for 4% of all primary tumors of bone, and it represents about 10% of malignant primary bone tumors with its different grades from borderline to high grade malignancy. Case Report. A 35-year-old patient presented with complains of pain and swelling in left ankle since 1 year following a twisting injury to his left ankle. On examination, swelling was present over the distal and anterior part of leg and movements of ankle joint were normal. All routine blood investigations were normal. X-ray and CT ankle showed morphology of subarticular well-defined expansile lytic lesion in lower end of left tibia suggestive of giant cell tumor. Histopathology of the tissue shows multinucleated giant cells with uniform vesicular nucleus and mononuclear cells which are spindle shaped with uniform vesicular nucleus suggestive of GCT. The patient was treated by excision, curettage, and bone cement to fill the defect. Conclusion. The patient at 12-month followup is doing well and walking without any pain comfortably and with full range of motion at ankle joint with articular congruity maintained and no signs of recurrences. PMID:23844298

  7. Giant intrathyroidal parathyroid adenoma

    PubMed Central

    Vilallonga, Ramon; Zafón, Carlos; Migone, Raul; Baena, Juan Antonio

    2012-01-01

    Primary hyperparathyroidism (PHPT) is not an uncommon endocrine disorder. However, acute primary hyperparathyroidism, or parathyroid crisis (PC), is a rare clinical entity characterized by life-threatening hypercalcemia of a sudden onset in patients with PHPT. We describe a patient with PC who presented with acute worsening of depressive symptoms, nausea and vomiting, and required emergency surgery. Serum calcium, alkaline phosphatase, and parathyroid hormone were elevated and serum phosphorus was low. An emergency hemithyroidectomy was performed because of none medical control of hypercalcemia. A giant intrathyroidal parathyroid adenoma was diagnosed. PHTP can be a life-threatening situation for patients, requiring immediate surgical treatment. A giant intrathyroidal parathyroid adenoma is an uncommon cause of PC. PMID:22787355

  8. Giant cell arteritis

    PubMed Central

    Calvo-Romero, J

    2003-01-01

    Giant cell arteritis (GCA), temporal arteritis or Horton's arteritis, is a systemic vasculitis which involves large and medium sized vessels, especially the extracranial branches of the carotid arteries, in persons usually older than 50 years. Permanent visual loss, ischaemic strokes, and thoracic and abdominal aortic aneurysms are feared complications of GCA. The treatment consists of high dose steroids. Mortality, with a correct treatment, in patients with GCA seems to be similar that of controls. PMID:13679546

  9. Giant thymic carcinoid.

    PubMed

    John, L C; Hornick, P; Lang, S; Wallis, J; Edmondson, S J

    1991-05-01

    Thymic carcinoid is a rare tumour. It may present with ectopic endocrine secretion or with symptoms of compression as a result of its size. A case is reported which presented with symptoms of compression where the size of the tumour was uniquely large such as to warrant the term giant thymic carcinoid. The typical histological features are described, together with its possible origin and its likely prognosis.

  10. Ice Giant Exploration

    NASA Astrophysics Data System (ADS)

    Rymer, A. M.; Arridge, C. S.; Masters, A.; Turtle, E. P.; Simon, A. A.; Hofstadter, M. D.; Turrini, D.; Politi, R.

    2015-12-01

    The Ice Giants in our solar system, Uranus and Neptune, are fundamentally different from their Gas Giant siblings Jupiter and Saturn, from the different proportions of rock and ice to the configuration of their planetary magnetic fields. Kepler space telescope discoveries of exo-planets indicate that planets of this type are among the most ubiquitous universally and therefore a future mission to explore the nature of the Ice Giants in our own solar system will provide insights into the nature of extra-solar system objects in general. Uranus has the smallest self- luminosity of all the planets, potentially related to catastrophic events early in the planet's history, which also may explain Uranus' large obliquity. Uranus' atmosphere is subject to extreme seasonal forcing making it unique in the Solar System. Neptune is also unique in a number of ways, notably its large moon Triton which is likely a captured Kuiper Belt Object and one of only two moons in the solar system with a robustly collisional atmosphere. Similar to Uranus, the angle between the solar wind and the magnetic dipole axis is subject to large-amplitude variations on both diurnal and seasonal timescales, but peculiarly it has one of the quietest magnetospheres of the solar system, at least according to Voyager 2, the only spacecraft to encounter Neptune to date. A comprehensive mission, as advocated in the Decadal Survey, would provide enormous science return but is also challenging and expensive. In this presentation we will discuss mission scenarios and suggest how collaboration between disciplines and internationally can help us to pursue a mission that includes Ice Giant exploration.

  11. Rare biliary cystic tumors: a case series of biliary cystadenomas and cystadenocarcinoma.

    PubMed

    Banerjee, Abhirup; Shah, Sudeep R; Singh, Abhiyutthan; Joshi, Anand; Desai, Devendra

    2016-01-01

    Cystic lesions of the liver are common and a major proportion is formed by parasitic cysts and simple cysts. Biliary cystic tumors (BCTs), namely biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC), are rare tumors which usually arise from the intrahepatic biliary tree. BCAs have malignant potential and are difficult to differentiate from BCAC pre-operatively on radiological imaging. Here we have presented 4 patients with BCTs and reviewed the literature pertaining to them.The data of four patients with BCA/BCAC diagnosed and treated at our institute were retrieved from our database and records were reviewed for age, sex, history, imaging, surgery, pathology and follow-up. Mean age of the patients was 53.5 years (range 30-71 years). Two male and two female patients presented with abdominal pain, of which one male patient had pancreatitis at diagnosis. Characteristic features were seen on pre-operative imaging (cystic lesions with internal septations) and biliary communication was identified in the patient with pancreatitis. Three patients were diagnosed with a BCA on final histology, while one patient had a BCAC. Following surgical resection, all the patients are asymptomatic and disease free with a mean follow-up of 24 months (range 10-40 months). In conclusion, BCTs should be suspected in the presence of a well-encapsulated, cystic hepatic lesion with internal septations. Although pre-operative distinction between BCA and BCAC is difficult, the lesion, whenever possible, should be completely resected as long-term outcomes are good, especially with BCA. PMID:27049501

  12. Detection of cystic structures using pulsed ultrasonically induced resonant cavitation

    NASA Technical Reports Server (NTRS)

    Bar-Cohen, Yoseph (Inventor); Kovach, John S. (Inventor)

    2002-01-01

    Apparatus and method for early detection of cystic structures indicative of ovarian and breast cancers uses ultrasonic wave energy at a unique resonance frequency for inducing cavitation in cystic fluid characteristic of cystic structures in the ovaries associated with ovarian cancer, and in cystic structures in the breast associated with breast cancer. Induced cavitation bubbles in the cystic fluid implode, creating implosion waves which are detected by ultrasonic receiving transducers attached to the abdomen of the patient. Triangulation of the ultrasonic receiving transducers enables the received signals to be processed and analyzed to identify the location and structure of the cyst.

  13. Malignant Pigmented Villonodular Synovitis-A Rare Entity

    PubMed Central

    Sistla, Radha; J.V.S, Vidyasagar; Afroz, Tameem

    2014-01-01

    Introduction: Malignant pigmented villonodular synovitis (PVNS) or Malignant giant cell tumour tendon sheath (MGCTTS) is a controversial and debatable lesion. Few case reports have indicated the potential for metastasis.1These aggressive cases are designated malignant giant cell tumour tendon sheath or malignant PVNS. Less than 20 cases are described in literature. We report a case of 65 year old lady who was diagnosed eight years back as pigmented villonodular synovitis. She had multiple local recurrences and now presented with lymphnodal metastases, which is extremely rare. Case Report: Sixty five year old lady presented with swelling in left inguinal region of six months duration. She gave a past history of swelling around medial condyle of left femur eight years back. Swelling was excised three times. At the time of third recurrence, swelling was extensive, infiltrating surrounding tissues and underlying bone, encasing femoral and popliteal vessels for which she underwent an above knee amputation. She now presented with inguinal swelling measuring 5.7×3.0 cms. Positron Emission Tomography Scan (PET-CT) revealed multiple enlarged left common iliac, internal and external iliac nodes, largest measuring 7.0 cms. Both the inguinal and pelvic nodes were excised. Lesion was diagnosed as metastatic deposits of Malignant pigmented villonodular synovitis based on morphological and Immunohistochemical findings. Conclusion: It is important to have a high index of clinical suspicion because these lesions can have an aggressive behaviour even with bland cytological features. Our experience suggests that in a recurrent lesion for GCTTS. A wide surgical excision with safe surgical margins and close follow up with radiological evaluation might help to diagnose these lesions early and be amenable to limb salvage surgeries. PMID:27298991

  14. "Malignant Cutaneous Ulcer".

    PubMed

    Sundriyal, Deepak; Kotwal, Sumedha

    2016-09-01

    Renal cell carcinoma (RCC) is an aggressive malignancy and the rich vascular supply enables it to metastasize early via haematogenous route. Skin lesions are a late manifestation of the disease. Clinicians should be aware of cutaneous presentation of RCC while evaluating a case of unknown primary with skin lesions. PMID:27651705

  15. Immunotherapy for malignant glioma.

    PubMed

    Suryadevara, Carter M; Verla, Terence; Sanchez-Perez, Luis; Reap, Elizabeth A; Choi, Bryan D; Fecci, Peter E; Sampson, John H

    2015-01-01

    Malignant gliomas (MG) are the most common type of primary malignant brain tumor. Most patients diagnosed with glioblastoma (GBM), the most common and malignant glial tumor, die within 12-15 months. Moreover, conventional treatment, which includes surgery followed by radiation and chemotherapy, can be highly toxic by causing nonspecific damage to healthy brain and other tissues. The shortcomings of standard-of-care have thus created a stimulus for the development of novel therapies that can target central nervous system (CNS)-based tumors specifically and efficiently, while minimizing off-target collateral damage to normal brain. Immunotherapy represents an investigational avenue with the promise of meeting this need, already having demonstrated its potential against B-cell malignancy and solid tumors in clinical trials. T-cell engineering with tumor-specific chimeric antigen receptors (CARs) is one proven approach that aims to redirect autologous patient T-cells to sites of tumor. This platform has evolved dramatically over the past two decades to include an improved construct design, and these modern CARs have only recently been translated into the clinic for brain tumors. We review here emerging immunotherapeutic platforms for the treatment of MG, focusing on the development and application of a CAR-based strategy against GBM.

  16. Giant Cell Tumor of the Uterus: A Report of 3 Cases With a Spectrum of Morphologic Features.

    PubMed

    Bennett, Jennifer A; Sanada, Sakiko; Selig, Martin K; Hariri, Lida P; Nielsen, Gunnlaugur P; Oliva, Esther

    2015-07-01

    Giant cell tumors, a well-recognized neoplasm of bone, can rarely be found in the uterus. Such tumors are characterized by a dual population of mononuclear and osteoclast-like giant cells that lack epithelial and specific mesenchymal differentiation. In this study, the clinicopathologic features of 3 giant cell tumors of the uterus were reviewed. Immunohistochemistry for CD68, CD163, h-caldesmon, desmin, SMA, AE1/AE3, CD10, ER, PR, cyclin D1, CD1a, CD34, CD30, S100, myogenin/myoglobin, and Ki-67 was performed in all tumors, along with ultrastructural analysis in one. The patients were 47, 57, and 59 yr and the tumors measured 2.5, 7.5, and 16.0 cm. One neoplasm was confined to the endometrium, whereas the other 2 were myometrial. All 3 tumors showed a nodular growth comprised of mononuclear and osteoclast-like giant cells. The endometrial-confined tumor consisted of histologically benign mononuclear cells, whereas the others exhibited marked atypia. Mitotic activity was up to 5/10 HPF in the benign tumor and up to 22/10 HPF in the malignant. No cytologic atypia or mitoses were observed in the giant cells. CD68 and CD10 were strongly and diffusely expressed in both components of 3 and 2 neoplasms, respectively. Cyclin D1 was focal in the mononuclear cells and focal to diffuse in the giant cells. CD163 was diffuse in the mononuclear cells, but absent to focal in the giant cells. Ultrastructural analysis lacked diagnostic features of epithelial or specific mesenchymal differentiation. Both malignant tumors demonstrated an aggressive behavior. In summary, although rare, giant cell tumor of the uterus should be included in the differential diagnosis of benign or malignant tumors containing osteoclast-like giant cells. PMID:25851705

  17. Lung Transplantation for Cystic Fibrosis: Results, Indications, Complications, and Controversies

    PubMed Central

    Lynch, Joseph P.; Sayah, David M.; Belperio, John A.; Weigt, S. Sam

    2016-01-01

    Survival in patients with cystic fibrosis (CF) has improved dramatically over the past 30 to 40 years, with mean survival now approximately 40 years. Nonetheless, progressive respiratory insufficiency remains the major cause of mortality in CF patients, and lung transplantation (LT) is eventually required. Timing of listing for LT is critical, because up to 25 to 41% of CF patients have died while awaiting LT. Globally, approximately 16.4% of lung transplants are performed in adults with CF. Survival rates for LT recipients with CF are superior to other indications, yet LT is associated with substantial morbidity and mortality (~50% at 5-year survival rates). Myriad complications of LT include allograft failure (acute or chronic), opportunistic infections, and complications of chronic immunosuppressive medications (including malignancy). Determining which patients are candidates for LT is difficult, and survival benefit remains uncertain. In this review, we discuss when LT should be considered, criteria for identifying candidates, contraindications to LT, results post-LT, and specific complications that may be associated with LT. Infectious complications that may complicate CF (particularly Burkholderia cepacia spp., opportunistic fungi, and nontuberculous mycobacteria) are discussed. PMID:25826595

  18. 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.

  19. The limitations of gallium scintigraphy in the differentiation between benign and malignant salivary gland mass lesions.

    PubMed

    Yoshikai, T; Ishimaru, J; Satoh, T; Inokuchi, A; Kudo, S

    2003-06-01

    The aim of this study was to evaluate the ability of gallium scintigraphy to differentiate between benign and malignant salivary gland mass lesions and to identify what types of lesions surpass its diagnostic utility. By considering the uptake of 67Ga, 193 salivary gland masses were graded visually as negative, weakly positive, moderately positive or strongly positive in comparison to the uptake in the nasal cavity and the liver. The uptake was compared with histopathological findings. Among 39 malignant tumours, uptake was positive in 31 (79%) (strongly positive in 18, moderately positive in seven and weakly positive in six) and uptake was negative in eight (21%). Adenoid cystic carcinoma was the most common malignant tumour in our study (11/39), and uptake was negative in five (45%) of these tumours. Malignant tumours did not differ significantly in size despite differences in uptake. Among 154 benign lesions, uptake was negative in 101 (66%) and positive in 53 (34%) (strongly positive in 12, moderately positive in 19 and weakly positive in 22). Out of 88 pleomorphic adenomas, 41 (47%) showed positive uptake. Sensitivity, specificity and accuracy for gallium study were 80%, 66% and 68%, respectively, when the malignancy criterion was weakly positive uptake. Accuracy was greatest (83%) when the criterion was strongly positive uptake, but this criterion failed to detect more than a half of malignant tumours (46% sensitivity). In conclusion, gallium scintigraphy had limitations in differentiating between benign and malignant salivary gland mass lesions. Adenoid cystic carcinomas and pleomorphic adenomas were the principal sources of false negative and false positive results, respectively.

  20. Ten-year follow-up of a giant prolactinoma.

    PubMed

    Fernandes, Vera; Santos, Maria Joana; Almeida, Rui; Marques, Olinda

    2015-11-20

    Giant prolactinomas are rare pituitary tumours of which management can be a challenge. A 28-year-old man presented with headaches, visual impairment and behavioural changes. Clinically, the patient was found to have hypogonadism and bitemporal hemianopsia. A MRI demonstrated a pituitary tumour 76 mm in diameter and blood tests revealed a serum prolactin of 158,700 µU/mL (reference range 58-254). Initially, a craniotomy was performed. Immunohistochemistry of the tumour identified a prolactinoma with a high proliferative index and the patient was started on treatment with a dopamine agonist. A year later, neurological symptoms worsened due to regrowth of the lesion's cystic component, and so further surgery was performed. After 10 years of treatment with dopamine agonists, the prolactin levels decreased by 96.8%, there was an effective reduction in tumour size, and the neurological signs and symptoms resolved.

  1. Malignant Melanoma of the Foot

    MedlinePlus

    ... Javascript in your browser. Malignant Melanoma of the Foot What is Malignant Melanoma? Melanoma is a cancer ... age groups, even the young. Melanoma in the Foot Melanoma that occurs in the foot or ankle ...

  2. Zinc supplementation in children with cystic fibrosis

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Cystic fibrosis (CF) leads to malabsorption of macro- and micronutrients. Symptomatic zinc deficiency has been reported in CF but little is known about zinc homeostasis in children with CF. Zinc supplementation (Zn suppl) is increasingly common in children with CF but it is not without theoretcial r...

  3. Nutritional assessment in children with cystic fibrosis

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Optimal nutrition, including consuming 35–40% of calories (kcal) as fat, is a vital part of the management of cystic fibrosis (CF), and involves accurate assessment of dietary intake. We compared 3 methods of nutritional assessment in 8– to 14-year-old children (n=20) with CF: 1) a 24-h Dietary Reca...

  4. Cystic lesion around the hip joint

    PubMed Central

    Yukata, Kiminori; Nakai, Sho; Goto, Tomohiro; Ikeda, Yuichi; Shimaoka, Yasunori; Yamanaka, Issei; Sairyo, Koichi; Hamawaki, Jun-ichi

    2015-01-01

    This article presents a narrative review of cystic lesions around the hip and primarily consists of 5 sections: Radiological examination, prevalence, pathogenesis, symptoms, and treatment. Cystic lesions around the hip are usually asymptomatic but may be observed incidentally on imaging examinations, such as computed tomography and magnetic resonance imaging. Some cysts may enlarge because of various pathological factors, such as trauma, osteoarthritis, rheumatoid arthritis, or total hip arthroplasty (THA), and may become symptomatic because of compression of surrounding structures, including the femoral, obturator, or sciatic nerves, external iliac or common femoral artery, femoral or external iliac vein, sigmoid colon, cecum, small bowel, ureters, and bladder. Treatment for symptomatic cystic lesions around the hip joint includes rest, nonsteroidal anti-inflammatory drug administration, needle aspiration, and surgical excision. Furthermore, when these cysts are associated with osteoarthritis, rheumatoid arthritis, and THA, primary or revision THA surgery will be necessary concurrent with cyst excision. Knowledge of the characteristic clinical appearance of cystic masses around the hip will be useful for determining specific diagnoses and treatments. PMID:26495246

  5. Intra-mandibular adenoid cystic carcinoma.

    PubMed

    Bouaichi, A; Aimad-Eddine, S; Mommers, X-A; Ella, B; Zwetyenga, N

    2014-04-01

    Intra-mandibular localization of adenoid cystic carcinoma is rare. This tumor is characterized by progressive local, regional, and distant aggressiveness. We reviewed the latest data on this rare type of cancer with a small number of reported cases, alack of consensus for its treatment, and its bad prognosis.

  6. A cystic fibrosis handbook for teachers.

    PubMed

    Ryan, L L; Williams, J K

    1996-01-01

    The purposes of this project were to (1) develop a handbook on cystic fibrosis for elementary school teachers and to (2) pilot this handbook with a group of teachers and school nurses. The project used a descriptive survey design in which parents, teachers, and school nurses of 14 elementary-age children with cystic fibrosis were recruited from one cystic fibrosis clinic. Interest in using the handbook with their child's teachers was elicited from parents; also, interest in using the handbook was obtained by open-ended questions in a mailed survey sent to teachers and school nurses. Levels of teacher and school nurse knowledge were measured with a true/false pretest and posttest instrument. All parents expressed a desire to use the handbook with their child's teachers. Sixty-seven percent of the teachers and 89% of the school nurses returned the survey, and all endorsed the use of the handbook in their classrooms or schools. Comparison of the pretest and posttest scores from the teachers revealed an increase in teachers' knowledge. Scores on pretest and posttest measures from school nurses were high at each testing time. Results support the use of a printed handbook to promote knowledge of cystic fibrosis in teachers and to support communication among nurses, parents, and teachers.

  7. Diabetes mellitus in patients with cystic fibrosis.

    PubMed

    Alves, Crésio de Aragão Dantas; Aguiar, Renata Arruti; Alves, Ana Cláudia S; Santana, Maria Angélica

    2007-01-01

    Cystic fibrosis-related diabetes (CFRD) is the principal extra-pulmonary complication of cystic fibrosis, occurring in 15-30% of adult cystic fibrosis patients. The number of cystic fibrosis patients who develop diabetes is increasing in parallel with increases in life expectancy. The aim of this study was to review the physiopathology, clinical presentation, diagnosis and treatment of CFRD. A bibliographic search of the Medline and Latin American and Caribbean Health Sciences Literature databases was made. Articles were selected from among those published in the last twenty years. Insulin deficiency, caused by reduced beta-cell mass, is the main etiologic mechanism, although insulin resistance also plays a role. Presenting features of type 1 and type 2 diabetes, CFRD typically affects individuals of approximately 20 years of age. It can also be accompanied by fasting, non-fasting or intermittent hyperglycemia. Glucose intolerance is associated with worsening of nutritional status, increased morbidity, decreased survival and reduced pulmonary function. Microvascular complications are always present, although macrovascular complications are rarely seen. An oral glucose tolerance test is recommended annually for patients > or = 10 years of age and for any patients presenting unexplained weight loss or symptoms of diabetes. Patients hospitalized with severe diseases should also be screened. If fasting hyperglycemia persists for more than 48 h, insulin therapy is recommended. Insulin administration remains the treatment of choice for diabetes and fasting hyperglycemia. Calories should not be restricted, and patients with CFRD should be managed by a multidisciplinary team. PMID:17724542

  8. [A case of retroperitoneal cystic lymphangioma].

    PubMed

    Perini, F; Metelli, F; Bazzana, M

    1979-06-30

    A case of retroperitoneal cystic lymphangioma in a 62-yr-old woman presented as symptom-free abdominal swelling. The intraoperative diagnosis was confirmed histologically. Radical management consisted of stripping of the cyst from its adjacent structures. The postoperative course was uneventful.

  9. Giant primary angiosarcoma of the small intestine showing severe sepsis.

    PubMed

    Takahashi, Mizuna; Ohara, Masanori; Kimura, Noriko; Domen, Hiromitsu; Yamabuki, Takumi; Komuro, Kazuteru; Tsuchikawa, Takahiro; Hirano, Satoshi; Iwashiro, Nozomu

    2014-11-21

    Primary malignant tumors of the small intestine are rare, comprising less than 2% of all gastrointestinal tumors. An 85-year-old woman was admitted with fever of 40 °C and marked abdominal distension. Her medical history was unremarkable, but blood examination showed elevated inflammatory markers. Abdominal computed tomography showed a giant tumor with central necrosis, extending from the epigastrium to the pelvic cavity. Giant gastrointestinal stromal tumor of the small intestine communicating with the gastrointestinal tract or with superimposed infection was suspected. Because no improvement occurred in response to antibiotics, surgery was performed. Laparotomy revealed giant hemorrhagic tumor adherent to the small intestine and occupying the peritoneal cavity. The giant tumor was a solid tumor weighing 3490 g, measuring 24 cm × 17.5 cm × 18 cm and showing marked necrosis. Histologically, the tumor comprised spindle-shaped cells with anaplastic large nuclei. Immunohistochemical studies showed tumor cells positive for vimentin, CD31, and factor VIII-related antigen, but negative for c-kit and CD34. Angiosarcoma was diagnosed. Although no postoperative complications occurred, the patient experienced enlargement of multiple metastatic tumors in the abdominal cavity and died 42 d postoperatively. The prognosis of small intestinal angiosarcoma is very poor, even after volume-reducing palliative surgery.

  10. [Giant retroperitoneal hydatid cyst].

    PubMed

    Massoud, W; Saheb, N; Iliescu, B; Kreitmann, L; Chabenne, J; Campeggi, A; Molinie, V; Baumert, H

    2009-06-01

    Hydatidosis is an endemic, widely distributed anthropozoonosis, which involves the liver, lung and other organs [Int J Urol 13 (2006) 76-9]. We reported the case of a large retroperitoneal hydatid cyst, which is a rare situation [Hepatogastroenterology 48 (2001) 1037-9; Int Urol Nephrol 32 (2000) 41-6; J Urol (Paris) 94 (1988) 445-8]. Diagnosis was suspected with blood tests and radiological examinations. A wide incision in the right iliac fossa (such in renal graft) was performed. The cyst wall was excised partially. Before, during and following the operation, the patient was given albendazole tablets (15mg/kg per day) for 3 weeks (1 week before and 2 weeks after the surgery) with blood count and liver enzyme monitorization. Especially in the endemic areas, hydatid cyst should be remembered when evaluating cystic masses in the retroperitoneum. It can be treated successfully with surgery.

  11. Nutrient Status of Adults with Cystic Fibrosis

    PubMed Central

    GORDON, CATHERINE M.; ANDERSON, ELLEN J.; HERLYN, KAREN; HUBBARD, JANE L.; PIZZO, ANGELA; GELBARD, RONDI; LAPEY, ALLEN; MERKEL, PETER A.

    2011-01-01

    Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This cross-sectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants’ mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%–25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were normal despite a relatively high fat intake. Mean protein consumption was adequate, but the range of intake was concerning, as both inadequate or excessive intake may have deleterious skeletal effects. These findings call into question the applicability of established nutrient thresholds for patients with cystic fibrosis. PMID:18060897

  12. Fat-poor angiomyolipoma with cyst-like changes mimicking a cystic renal cell carcinoma: a case report.

    PubMed

    Kobari, Yuki; Takagi, Toshio; Kondo, Tsunenori; Tachibana, Hidekazu; Iida, Shoichi; Nishina, Yu; Omae, Kenji; Morita, Satoru; Yamamoto, Tomoko; Iizuka, Junpei; Nagashima, Yoji; Tanabe, Kazunari

    2015-01-01

    Angiomyolipoma is a common benign renal tumor. It is typically composed of adipose tissue and hence is easily diagnosed by using imaging methods such as ultrasonography, computed tomography, and magnetic resonance imaging. However, it is difficult to differentiate an atypical angiomyolipoma such as a fat-poor angiomyolipoma from a malignant tumor by using these imaging methods. We report a case of a fat-poor angiomyolipoma with cyst-like changes in a 35-year-old man. The angiomyolipoma was initially suspected to be a cystic renal cell carcinoma according to preoperative imaging studies. A 5-cm cystic tumor with an enhanced septal wall and exophytic formation was present in the middle section of the left kidney. The patient underwent partial nephrectomy. Pathological findings showed necrosis and hematoma in almost the entire lesion, with a small amount of adipose and muscle tissue. Finally, a fat-poor angiomyolipoma was diagnosed.

  13. Congenital Giant Keratinous Cyst Mimicking Lipoma: Case Report and Review

    PubMed Central

    Sabhlok, Samrat; Kalele, Ketki; Phirange, Asmita; Kheur, Supriya

    2015-01-01

    Epidermal cysts represent the most common cutaneous cysts. They arise following a localized inflammation of the hair follicle and occasionally after the implantation of the epithelium, following a trauma or surgery. Conventional epidermal cysts are about 5 cm in diameter; however, rare reports of cysts more than 5 cm are reported in the literature and are referred as “Giant epidermal cysts.” Epidermal cysts although common, can mimic other common benign lesions in the head and neck area. A thorough clinico-pathologic investigation is needed to diagnose these cutaneous lesions as they differ in their biologic behavior, treatment, and prognosis. We report a case of a giant epidermoid cyst in the scalp area of a young female patient which mimicked lipoma on clinical, as well as cyotological examination. We also present a brief review of epidermal cysts, their histopathological differential diagnosis, and their malignant transformation. PMID:26677303

  14. Elevated serum tumor markers in patients with testicular cancer after induction chemotherapy due to a reservoir of markers in cystic differentiated mature teratoma.

    PubMed

    van der Gaast, A; Hoekstra, J W; Croles, J J; Splinter, T A

    1991-04-01

    Elevated serum tumor markers in patients with testicular cancer after induction chemotherapy indicate in most instances the presence of residual malignant disease. We describe 2 patients with elevated tumor markers after chemotherapy and before retroperitoneal lymph node dissection who did not prove to have residual malignant disease but cystic differentiated mature teratoma with a high content of alpha-fetoprotein and beta-human chorionic gonadotropin, respectively, in the cysts. It is postulated that leakage of the contents of these cysts to the plasma compartment was responsible for maintaining elevated serum tumor marker levels. Recognition of such entities is of consequence since unnecessary salvage chemotherapy in these patients may be avoided.

  15. Hyaluronan in human malignancies

    SciTech Connect

    Sironen, R.K.; Tammi, M.; Tammi, R.; Auvinen, P.K.; Anttila, M.; Kosma, V-M.

    2011-02-15

    Hyaluronan, a major macropolysaccharide in the extracellular matrix of connective tissues, is intimately involved in the biology of cancer. Hyaluronan accumulates into the stroma of various human tumors and modulates intracellular signaling pathways, cell proliferation, motility and invasive properties of malignant cells. Experimental and clinicopathological evidence highlights the importance of hyaluronan in tumor growth and metastasis. A high stromal hyaluronan content is associated with poorly differentiated tumors and aggressive clinical behavior in human adenocarcinomas. Instead, the squamous cell carcinomas and malignant melanomas tend to have a reduced hyaluronan content. In addition to the stroma-cancer cell interaction, hyaluronan can influence stromal cell recruitment, tumor angiogenesis and epithelial-mesenchymal transition. Hyaluronan receptors, hyaluronan synthases and hyaluronan degrading enzymes, hyaluronidases, are involved in the modulation of cancer progression, depending on the tumor type. Furthermore, intracellular signaling and angiogenesis are affected by the degradation products of hyaluronan. Hyaluronan has also therapeutic implications since it is involved in multidrug resistance.

  16. Metastatic malignant chondroblastoma.

    PubMed

    Rodgers, W B; Mankin, H J

    1996-12-01

    A case of malignant chondroblastoma with metastases is reported. The patient initially presented with a lytic lesion in his left pubic ramus. He was treated with curettage, but the lesion recurred 3 years later. After repeated curettage, radiation therapy, and the late development of multiple bone and soft-tissue metastases, he succumbed to his disease 13 years after diagnosis. The surgical pathology from each of his several procedures was reviewed. Although no definite malignant transformation was apparent, a metastatic deposit curetted 3 months prior to death showed some increase in mitotic activity. Flow cytometry of specimens from the patient's first local recurrence and a late distant metastasis was performed and revealed the interval development of a minor aneuploid peak between the two samples. This fatal chondroblastoma is the only one in our series of 80 patients treated over the past 25 years. PMID:9001683

  17. Nicotinamide Phosphoribosyltransferase in Malignancy

    PubMed Central

    Shackelford, Rodney E.; Mayhall, Kim; Maxwell, Nicole M.; Kandil, Emad

    2013-01-01

    Nicotinamide phosphoribosyltransferase (Nampt) catalyzes the rate-limiting step of nicotinamide adenine dinucleotide (NAD) synthesis. Both intracellular and extracellular Nampt (iNampt and eNampt) levels are increased in several human malignancies and some studies demonstrate increased iNampt in more aggressive/invasive tumors and in tumor metastases. Several different molecular targets have been identified that promote carcinogenesis following iNampt overexpression, including SirT1, CtBP, and PARP-1. Additionally, eNampt is elevated in several human cancers and is often associated with a higher tumor stage and worse prognoses. Here we review the roles of Nampt in malignancy, some of the known mechanisms by which it promotes carcinogenesis, and discuss the possibility of employing Nampt inhibitors in cancer treatment. PMID:24386506

  18. Lymphoscintigraphy in malignant melanoma

    SciTech Connect

    Berman, C.G.; Norman, J.; Cruse, C.W.; Reintgen, D.S.; Clark, R.A. )

    1992-01-01

    The development and rationale for the use of lymphoscintigraphy in the preoperative evaluation of patients with malignant melanoma being considered for elective lymph node dissection is reviewed. This overview is updated by an analysis of 135 patients with early stage malignant melanoma involving the head, neck, shoulders, and trunk at Moffitt Cancer Center and Research Institute at the University of South Florida (Tampa, FL). High discordancy rates (overall, 41%) were seen between drainage patterns predicted from historical anatomical guidelines and those revealed by the lymphoscintigraphic examination. The high discordancy rate was most pronounced in the head (64%) and the neck (73%). Surgical management was changed in 33% of the patients, overall. A preoperative lymphoscintigram is recommended for all patients with melanoma with head, neck, and truncal lesions evaluated for elective lymph node dissection as the lymphatic drainage patterns are often unpredictable and variable.

  19. Asbestos-related malignancy.

    PubMed

    Talcott, J A; Antman, K H

    1988-01-01

    Asbestos-associated malignancies have received significant attention in the lay and medical literature because of the increasing frequency of two asbestos-associated tumors, lung carcinoma and mesothelioma; the wide distribution of asbestos; its status as a prototype environmental carcinogen; and the many recent legal compensation proceedings, for which medical testimony has been required. The understanding of asbestos-associated carcinogenesis has increased through study of animal models, human epidemiology, and, recently, the application of modern molecular biological techniques. However, the detailed mechanisms of carcinogenesis remain unknown. A wide variety of malignancies have been associated with asbestos, although the strongest evidence for a causal association is confined to lung cancer and mesothelioma. Epidemiological studies have provided evidence that both the type of asbestos fiber and the industry in which the exposure occurs may affect the rates of asbestos-associated cancers. It has been shown that asbestos exerts a carcinogenic effect independent of exposure to cigarette smoking that, for lung cancers, is synergistically enhanced by smoking. Other questions remain controversial, such as whether pulmonary fibrosis necessarily precedes asbestos-associated lung cancer and whether some threshold level of exposure to asbestos (including low-dose exposures that may occur in asbestos-associated public buildings) may be safe. Mesothelioma, the most closely asbestos-associated malignancy, has a dismal natural history and has been highly resistant to therapy. However, investigational multi-modality therapy may offer benefit to some patients. A description of the processes through which compensation claims for asbestos-associated malignancies are evaluated illustrates for physicians the legal system's approach to possible injury from toxic substances. The differences between scientific and legal reasoning about the causes of diseases with long latency

  20. Imaging Extrasolar Giant Planets

    NASA Astrophysics Data System (ADS)

    Bowler, Brendan P.

    2016-10-01

    High-contrast adaptive optics (AO) imaging is a powerful technique to probe the architectures of planetary systems from the outside-in and survey the atmospheres of self-luminous giant planets. Direct imaging has rapidly matured over the past decade and especially the last few years with the advent of high-order AO systems, dedicated planet-finding instruments with specialized coronagraphs, and innovative observing and post-processing strategies to suppress speckle noise. This review summarizes recent progress in high-contrast imaging with particular emphasis on observational results, discoveries near and below the deuterium-burning limit, and a practical overview of large-scale surveys and dedicated instruments. I conclude with a statistical meta-analysis of deep imaging surveys in the literature. Based on observations of 384 unique and single young (≈5-300 Myr) stars spanning stellar masses between 0.1 and 3.0 M ⊙, the overall occurrence rate of 5-13 M Jup companions at orbital distances of 30-300 au is {0.6}-0.5+0.7 % assuming hot-start evolutionary models. The most massive giant planets regularly accessible to direct imaging are about as rare as hot Jupiters are around Sun-like stars. Dividing this sample into individual stellar mass bins does not reveal any statistically significant trend in planet frequency with host mass: giant planets are found around {2.8}-2.3+3.7 % of BA stars, <4.1% of FGK stars, and <3.9% of M dwarfs. Looking forward, extreme AO systems and the next generation of ground- and space-based telescopes with smaller inner working angles and deeper detection limits will increase the pace of discovery to ultimately map the demographics, composition, evolution, and origin of planets spanning a broad range of masses and ages.

  1. Asbestos-related malignancy

    SciTech Connect

    Talcott, J.A.; Antman, K.H.

    1988-05-01

    Asbestos-associated malignancies have received significant attention in the lay and medical literature because of the increasing frequency of two asbestos-associated tumors, lung carcinoma and mesothelioma; the wide distribution of asbestos; its status as a prototype environmental carcinogen; and the many recent legal compensation proceedings, for which medical testimony has been required. The understanding of asbestos-associated carcinogenesis has increased through study of animal models, human epidemiology, and, recently, the application of modern molecular biological techniques. However, the detailed mechanisms of carcinogenesis remain unknown. A wide variety of malignancies have been associated with asbestos, although the strongest evidence for a causal association is confined to lung cancer and mesothelioma. Epidemiological studies have provided evidence that both the type of asbestos fiber and the industry in which the exposure occurs may affect the rates of asbestos-associated cancers. It has been shown that asbestos exerts a carcinogenic effect independent of exposure to cigarette smoking that, for lung cancers, is synergistically enhanced by smoking. Other questions remain controversial, such as whether pulmonary fibrosis necessarily precedes asbestos-associated lung cancer and whether some threshold level of exposure to asbestos (including low-dose exposures that may occur in asbestos-associated public buildings) may be safe. Mesothelioma, the most closely asbestos-associated malignancy, has a dismal natural history and has been highly resistant to therapy. However, investigational multi-modality therapy may offer benefit to some patients. 179 references.

  2. GIANT INTRACANALICULAR FIBROADENOMA

    PubMed Central

    Smith, Clyn; Parsons, Robert J.; Bogart, William M.

    1951-01-01

    Five cases of giant intracanalicular fibroadenoma (“cystosarcoma phylloides”) were observed at one hospital in a period of three years. In a search of the literature, additional reports of breast tumors of this kind, not included in previous reviews, were noted. As there is record of 229 cases, it would appear that this rapidly growing benign tumor should be kept in mind in the diagnosis of masses in the breast. If removal is incomplete, there may be recurrence. Simple mastectomy is the treatment of choice. Radical mastectomy should be avoided. ImagesFigure 1Figure 2.Figure 3Figure 4Figure 5 PMID:14848732

  3. Epigenetics in the hematologic malignancies

    PubMed Central

    Fong, Chun Yew; Morison, Jessica; Dawson, Mark A.

    2014-01-01

    A wealth of genomic and epigenomic data has identified abnormal regulation of epigenetic processes as a prominent theme in hematologic malignancies. Recurrent somatic alterations in myeloid malignancies of key proteins involved in DNA methylation, post-translational histone modification and chromatin remodeling have highlighted the importance of epigenetic regulation of gene expression in the initiation and maintenance of various malignancies. The rational use of targeted epigenetic therapies requires a thorough understanding of the underlying mechanisms of malignant transformation driven by aberrant epigenetic regulators. In this review we provide an overview of the major protagonists in epigenetic regulation, their aberrant role in myeloid malignancies, prognostic significance and potential for therapeutic targeting. PMID:25472952

  4. MTA1 Expression in Benign and Malignant Salivary gland Tumors

    PubMed Central

    Andisheh-Tadbir, Azadeh; Dehghani-Nazhvani, Ali; Ashraf, Mohammad Javad; Khademi, Bijan; Mirhadi, Hosein; Torabi-Ardekani, Shima

    2016-01-01

    Introduction: Salivary gland tumors (SGTs) are important parts of human neoplasms. The most common SGT is pleomorphic adenoma and the most common malignant SGTs are mucoepidermoid carcinoma and adenoid cystic carcinoma (ACC). Metastasis-associated genes 1 (MTA1), a member of the nucleosome remodeling and histone deacetylation complex, is one newly discovered gene which recruits histone deacetylation, causing ATP-dependent chromosome remodeling, and regulating transcription. MTA1 had been shown to be overexpressed in malignant tumors with the enhancement of invasion and metastasis. Materials and Methods: Fifty-six samples of salivary gland tumors from the Khalili Hospital archive, including 20 cases of pleomorphic adenoma, 17 cases of mucoepidermoid carcinoma, 19 cases of ACC, and 23 cases of normal salivary gland tissues were chosen for immunohistochemical analysis of MTA1. Results: MTA1 expression in the malignant tumors was significantly higher than that in pleomorphic adenoma (P<0.001), and higher in pleomorphic adenoma than the normal salivary glands(P< 0.001). In total, 69.6% of normal salivary gland tissues showed MTA1, but all cases of salivary gland tumors were positive for MTA1. High nuclear expression of MTA1 was detected in 83.3% (30/36) of the malignant salivary gland tumors and 45% (9/20) of pleomorphic adenoma, while low MTA1 expression was seen in all of the normal salivary gland tissues. No statistically significant correlation was found between MTA1 protein expression and any clinicopathological features (P>0.05). Conclusion: Our findings demonstrate that MTA1 was significantly overexpressed in malignant salivary gland neoplasm in comparison to a lower level in benign pleomorphic adenoma, suggesting that MTA1 protein might be involved in carcinogenesis. PMID:26878004

  5. Salivary gland-type tumors of the breast: a spectrum of benign and malignant tumors including "triple negative carcinomas" of low malignant potential.

    PubMed

    Foschini, Maria P; Krausz, Thomas

    2010-02-01

    Salivary gland-type neoplasms of the breast are uncommon and comprise numerous entities analogous to that more commonly seen in salivary glands. The clinicopathologic spectrum ranges from benign to malignant but there are important differences as compared with those of their salivary counterpart. In the breast, benign adenomyoepithelioma is recognized in addition to malignant one, whereas in the salivary gland a histologically similar tumor is designated as epithelial-myoepithelial carcinoma without a separate benign subgroup. Mammary adenoid cystic carcinoma is a low-grade neoplasm compared with its salivary equivalent. It is also important to appreciate that in contrast to "triple negative" conventional breast carcinomas with aggressive course, most salivary-type malignant breast neoplasms behave in a low-grade manner. Most of these tumors are capable of differentiating along both epithelial and myoepithelial lines, but the amount of each lineage-component varies from case to case, contributing to diagnostic difficulties. Well established examples of this group include pleomorphic adenoma, adenomyoepithelioma, and adenoid cystic carcinoma. Another family of salivary gland-type mammary epithelial neoplasms is devoid of myoepithelial cells. Key examples include mucoepidermoid carcinoma and acinic cell carcinoma. The number of cases of salivary gland-type mammary neoplasms in the published data is constantly increasing but some of the rarest subtypes like polymorphous low-grade adenocarcinoma and oncocytic carcinoma are "struggling" to become clinically relevant entities in line with those occurring more frequently in salivary glands.

  6. Giant appendiceal mucocele: a case report and review of the literature.

    PubMed

    Ghumman, S; Muguti, G I

    2006-01-01

    Appendiceal mucocele is a rare condition characterised by cystic dilatation of the lumen of the appendix. Whenever possible it is essential to identify the disease pre-operatively by full clinical assessment and thorough investigations including abdominal CT scan and colonoscopy or barium enema. Accurate diagnosis helps to plan for a careful resection to remove the mass without spillage of the cyst contents, which can lead to the most dreaded complication of pseudomyxoma peritonei. In this paper we report on a rare case of giant mucocele of the appendix and review the relevant literature. PMID:18254458

  7. Midkine expression in malignant salivary gland tumors and its role in tumor angiogenesis.

    PubMed

    Ota, Tomoko; Ota, Kazutoshi; Jono, Hirofumi; Fujimori, Hiromi; Ueda, Mitsuharu; Shinriki, Satoru; Sueyoshi, Takanao; Shinohara, Masanori; Ando, Yukio

    2010-09-01

    The aims of this study were to investigate midkine (MK) expression patterns in salivary gland tumors (SGTs) and to evaluate the correlation between MK expression and the degree of malignancy. We performed immunohistochemistry to examine MK expression in specimens of adenoid cystic carcinoma (ACC), mucoepidermoid carcinoma (MEC), and pleomorphic adenoma (PA). In addition, we performed immunohistochemistry for CD31 and measured microvessel density (MVD), which is an indicator of angiogenesis. Immunohistochemistry showed that MK protein expression was significantly higher in specimens of malignant SGTs (ACC [P<0.01] and MEC [P<0.001]) than in benign SGT (PA) samples. Furthermore, MVD values tended to be higher in cases that exhibited high expression of MK, which indicated a significant correlation between the degree of MK expression and MVD (P<0.001). These results suggest that MK may play important roles in malignant transformation and tumor angiogenesis in SGTs.

  8. Gas Giants Form Quickly

    NASA Technical Reports Server (NTRS)

    2007-01-01

    This is an artist's concept of a hypothetical 10-million-year-old star system. The bright blur at the center is a star much like our sun. The other orb in the image is a gas-giant planet like Jupiter. Wisps of white throughout the image represent traces of gas.

    Astronomers using NASA's Spitzer Space Telescope have found evidence showing that gas-giant planets either form within the first 10 million years of a sun-like star's life, or not at all. The lifespan for sun-like stars is about 10 billion years.

    The scientists came to this conclusion after searching for traces of gas around 15 different sun-like stars, most with ages ranging from 3 million to 30 million years. With the help of Spitzer's Infrared Spectrometer instrument, they were able to search for relatively warm gas in the inner regions of these star systems, an area comparable to the zone between Earth and Jupiter in our own solar system. They also used ground-based radio telescopes to search for cooler gas in the outer regions of these systems, an area comparable to the zone around Saturn and beyond.

  9. Giant solitary trichoepithelioma

    PubMed Central

    Teli, Bhavuray; Thrishuli, P. B.; Santhosh, R.; Amar, D. N.; Rajpurohit, Shravan

    2015-01-01

    Adnexal tumors like giant solitary trichoepitheliomas are uncommon to most of us to permit a ready familiarity with them. Information regarding the genesis, clinical profile, behavior, and management options for this tumor is limited. There are 18 cases reported in the world literature till date. This review attempts to provide insight to this rare tumor. Our search included indexed literature from Pubmed, Directory of Open Access Journals, Health Inter Network Access to Research Initiative and Google databases in addition to standard dermatology texts. Giant solitary trichoepithelioma is a rare trichogenic tumor with potential for local recurrence. It has predilection for the older age, but may present at any age including at birth. It has close resemblance to basal cell carcinoma and other skin adnexal tumors - clinically, cytologically, and histologically. CD10, CD 34, PHLDA1 but not p75NTR are useful adjunct markers. Surgical excision is the standard treatment. Recurrence and possible transformation into BCC cautions follow up at regular intervals. PMID:25839021

  10. Giant papillary conjunctivitis.

    PubMed Central

    Donshik, P C

    1994-01-01

    Giant papillary conjunctivitis is a syndrome found frequently as a complication of contact lenses. Many variables can affect the onset and severity of the presenting signs and symptoms. Rigid gas permeable contact lenses appear to result in less severe signs and symptoms, with a longer time before the development of giant papillary conjunctivitis. Nonionic, low-water-content soft contact lenses tend to produce less severe signs and symptoms than ionic, low-water-content soft contact lenses. Enzymatic treatment appears to lessen the severity of signs and symptoms. The association of an allergy appears to play a role in the onset of the severity of the signs and symptoms but does not appear to affect the final ability of the individual to wear contact lenses. Using multiple treatment options, such as changing the polymer to a glyceryl methyl methacrylate or a rigid lens, or utilizing a soft lens on a frequent-replacement basis, can result in a success rate of over 90%. In individuals who still have a return of symptoms, the use of topical mast cell stabilizers or a nonsteroidal anti-inflammatory drug as an adjunctive therapy offers the added possibility of keeping these patients in contact lenses. Images FIGURE 1 FIGURE 2 FIGURE 3 FIGURE 4 FIGURE 5 FIGURE 6 FIGURE 7 FIGURE 11 A FIGURE 11 B FIGURE 11 C FIGURE 11 D PMID:7886881

  11. Rheology of giant micelles

    NASA Astrophysics Data System (ADS)

    Cates, M. E.; Fielding, S. M.

    2006-12-01

    Giant micelles are elongated, polymer-like objects created by the self-assembly of amphiphilic molecules (such as detergents) in solution. Giant micelles are typically flexible, and can become highly entangled even at modest concentrations. The resulting viscoelastic solutions show fascinating flow behaviour (rheology) which we address theoretically in this article at two levels. First, we summarize advances in understanding linear viscoelastic spectra and steady-state nonlinear flows, based on microscopic constitutive models that combine the physics of polymer entanglement with the reversible kinetics of self-assembly. Such models were first introduced two decades ago, and since then have been shown to explain robustly several distinctive features of the rheology in the strongly entangled regime, including extreme shear thinning. We then turn to more complex rheological phenomena, particularly involving spatial heterogeneity, spontaneous oscillation, instability and chaos. Recent understanding of these complex flows is based largely on grossly simplified models which capture in outline just a few pertinent microscopic features, such as coupling between stresses and other order parameters such as concentration. The role of ‘structural memory’ (the dependence of structural parameters such as the micellar length distribution on the flow history) in explaining these highly nonlinear phenomena is addressed. Structural memory also plays an intriguing role in the little-understood shear thickening regime, which occurs in a concentration regime close to but below the onset of strong entanglement, and which is marked by a shear-induced transformation from an inviscid to a gelatinous state.

  12. Solid variant of aneurysmal bone cyst presenting as a giant cervical mass: A clinical, radiological, histopathological dilemma

    PubMed Central

    Savardekar, Amey R.; Patra, Deviprasad; Chatterjee, Debajyoti; Ahuja, Chirag K.; Salunke, Pravin

    2015-01-01

    Background: Typical aneurysmal bone cysts (ABCs) are osteolytic, multicystic lesions with parietal sclerosis and blood-filled cysts. In rare instances, the cystic components may be completely absent. Such solid variants in ABC (s-ABC) exhibit a solid architecture; making the clinical, radiological, and histological differentiation from other solid bone tumors like osteosarcoma (especially giant cell rich osteosarcoma) and giant cell tumor, a difficult task. Case Report: We report the case of a 45–year-old male presenting with a giant solid cervical spine lesion. Histopathology revealed solid variant of ABC, even though the radiological and fine needle aspiration cytology studies pointed toward a giant cell tumor. Conclusion: We aim to discuss the clinical, radiological, and histological findings of solid ABC (a rare benign entity) vis-à-vis the common neoplastic entities of osteosarcoma and giant cell tumor. The histopathological nuisances in making the diagnosis of s-ABC are put forth, along with its impact on management of such giant bony spinal lesions. PMID:26005581

  13. Allometry indicates giant eyes of giant squid are not exceptional

    PubMed Central

    2013-01-01

    Background The eyes of giant and colossal squid are among the largest eyes in the history of life. It was recently proposed that sperm whale predation is the main driver of eye size evolution in giant squid, on the basis of an optical model that suggested optimal performance in detecting large luminous visual targets such as whales in the deep sea. However, it is poorly understood how the eye size of giant and colossal squid compares to that of other aquatic organisms when scaling effects are considered. Results We performed a large-scale comparative study that included 87 squid species and 237 species of acanthomorph fish. While squid have larger eyes than most acanthomorphs, a comparison of relative eye size among squid suggests that giant and colossal squid do not have unusually large eyes. After revising constants used in a previous model we found that large eyes perform equally well in detecting point targets and large luminous targets in the deep sea. Conclusions The eyes of giant and colossal squid do not appear exceptionally large when allometric effects are considered. It is probable that the giant eyes of giant squid result from a phylogenetically conserved developmental pattern manifested in very large animals. Whatever the cause of large eyes, they appear to have several advantages for vision in the reduced light of the deep mesopelagic zone. PMID:23418818

  14. 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

  15. Diffuse Cystic Lung Disease. Part II.

    PubMed

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

    2015-07-01

    The diffuse cystic lung diseases have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Obtaining an accurate diagnosis can be challenging, and management approaches are highly disease dependent. Unique imaging features, genetic tests, serum studies, and clinical features provide invaluable clues that help clinicians distinguish among the various etiologies, but biopsy is often required for definitive diagnosis. In part II of this review, we present an overview of the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic mutations, or aberrant lung development and provide an approach to aid in their diagnosis and management.

  16. Cystic fibrosis and physiological responses to exercise.

    PubMed

    Williams, Craig A; Saynor, Zoe L; Tomlinson, Owen W; Barker, Alan R

    2014-12-01

    Cardiopulmonary exercise testing is underutilized within the clinical management of patients with cystic fibrosis. But within the last 5 years, there has been considerable interest in its implementation, which has included deliberations by the European Cystic Fibrosis Society about incorporating this method within the clinical assessment of patients. This review examines the current use of cardiopulmonary exercise testing in assessing the extent and cause(s) of exercise limitation from a pediatric perspective. Examples of the measured parameters and their interpretation are provided. Critical synthesis of recent work in the oxygen uptake (VO2) kinetics response to and following exercise is also discussed, and although identified more as a research tool, its utilization advances researchers understanding of the cardiovascular, respiratory and muscular limitations to exercise tolerance. Finally, exercise and its application in therapeutic interventions are highlighted and a number of recommendations made about the utility of exercise prescription.

  17. Calcium-mediated mechanisms of cystic expansion

    PubMed Central

    Abdul-Majeed, Shakila; Nauli, Surya M.

    2010-01-01

    In this review, we will discuss several well-accepted signaling pathways toward calcium-mediated mechanisms of cystic expansion. The second messenger calcium ion has contributed to a vast diversity of signal transduction pathways. We will dissect calcium signaling as a possible mechanism that contributes to renal cyst formation. Because cytosolic calcium also regulates an array of signaling pathways, we will first discuss cilia-induced calcium fluxes, followed by Wnt signaling that has attributed to much-discussed planar cell polarity. We will then look at the relationship between cytosolic calcium and cAMP as one of the most important aspects of cyst progression. The signaling of cAMP on MAPK and mTOR will also be discussed. We infer that while cilia-induced calcium fluxes may be the initial signaling messenger for various cellular pathways, no single signaling mediator or pathway is implicated exclusively in the progression of the cystic expansion. PMID:20932898

  18. Acquired Cystic Fibrosis Transmembrane Conductance Regulator Deficiency.

    PubMed

    Cho, Do-Yeon; Woodworth, Bradford A

    2016-01-01

    In the genetic airway disease cystic fibrosis (CF), deficiency or dysfunction of the cystic fibrosis membrane conductance regulator (CFTR) alters anion transport in respiratory epithelium and consequently disrupts mucociliary clearance. An enriched understanding of the role of CFTR in the maintenance of normal epithelial function has revealed that mild and variable CFTR mutations play a causative role in a number of diseases not classically associated with CF. Furthermore, recent evidence indicates that acquired defects in wild-type CFTR protein processing, endocytic recycling and function can contribute to the pathogenesis of airway diseases, such as chronic obstructive pulmonary disease. In this chapter, we discuss emerging findings implicating acquired CFTR dysfunction in the pathogenesis of chronic rhinosinusitis and propose a new and leading edge approach to future CRS therapy using CFTR potentiators. PMID:27466849

  19. Inflammation and its genesis in cystic fibrosis.

    PubMed

    Nichols, David P; Chmiel, James F

    2015-10-01

    The host inflammatory response in cystic fibrosis (CF) lung disease has long been recognized as a central pathological feature and an important therapeutic target. Indeed, many believe that bronchiectasis results largely from the oxidative and proteolytic damage comprised within an exuberant airway inflammatory response that is dominated by neutrophils. In this review, we address the longstanding argument of whether or not the inflammatory response is directly attributable to impairment of the cystic fibrosis transmembrane conductance regulator or only secondary to airway obstruction and chronic bacterial infection and challenge the importance of this distinction in the context of therapy. We also review the centrality of neutrophils in CF lung pathophysiology and highlight more recent data that suggest the importance of other cell types and signaling beyond NF-κB activation. We discuss how protease and redox imbalance are critical factors in CF airway inflammation and end by reviewing some of the more promising therapeutic approaches now under development.

  20. Cystic fibrosis and physiological responses to exercise.

    PubMed

    Williams, Craig A; Saynor, Zoe L; Tomlinson, Owen W; Barker, Alan R

    2014-12-01

    Cardiopulmonary exercise testing is underutilized within the clinical management of patients with cystic fibrosis. But within the last 5 years, there has been considerable interest in its implementation, which has included deliberations by the European Cystic Fibrosis Society about incorporating this method within the clinical assessment of patients. This review examines the current use of cardiopulmonary exercise testing in assessing the extent and cause(s) of exercise limitation from a pediatric perspective. Examples of the measured parameters and their interpretation are provided. Critical synthesis of recent work in the oxygen uptake (VO2) kinetics response to and following exercise is also discussed, and although identified more as a research tool, its utilization advances researchers understanding of the cardiovascular, respiratory and muscular limitations to exercise tolerance. Finally, exercise and its application in therapeutic interventions are highlighted and a number of recommendations made about the utility of exercise prescription. PMID:25395018

  1. [Dry powder inhalers in cystic fibrosis].

    PubMed

    Steinkamp, G

    2014-06-01

    Inhaled medications play an important role in the daily treatment of patients with cystic fibrosis (CF). The classic route of administration was nebulisation via jet nebulisers. Respiratory delivery of fluid particles should loosen the viscid respiratory secretions, making airway clearance via cough or physiotherapy more efficient. Until recently, only jet nebulisers allowed to administer high doses of aerosolised antipseudomonal antibiotics. Powder inhalers for the treatment of cystic fibrosis have recently been made available. The newly developed powders and inhalers differ considerably from conventional dry powder inhalers used for the treatment of chronic obstructive airway disease. The present article will review two inhaled antibiotics, i. e. tobramycin and colistin, and the hyperosmotic agent mannitol, which increases the hydration of the airways. Topics are particle engineering, efficacy and tolerability results from clinical trials, as well as functional and practical aspects related to these new drugs. PMID:24664997

  2. Spontaneous regression of a cystic retroperitoneal tumour in young women postpartum. Report of two cases.

    PubMed

    de Zeeuw, Sharonne; Schouten van der Velden, Arjan P; Eggink, Alex J; Strijk, Simon; Wobbes, Theo

    2011-01-01

    Retroperitoneal cystic tumours are rarely found, and of these, the most common lesion is a cystic lymphangioma. We present two postpartum patients with a cystic retroperitoneal tumour which showed spontaneous regression and a review of the literature.

  3. [New prospects in cystic fibrosis treatment].

    PubMed

    Prados, C; Serrano, S; Alvarez-Sala, R; Villamor, J

    1997-04-01

    Only a few years ago, cystic fibrosis (CF) was considered the most frequent genetic disease in childhood, although survival has increased considerably in recent years owing to improved treatment. We discuss treatments that are still relevant as well as others that are under investigation now, aiming for better understanding of the disease and the therapies that have improved quality of life for CF patients. PMID:9280562

  4. Malignant testicular tumours

    PubMed Central

    Vecchio, Pierre Del; Tawil, Elie; Béland, Gilles

    1974-01-01

    A series of 71 patients with malignant testicular tumours treated primarily by orchiectomy and irradiation is reviewed with respect to pathological and clinical features and modes of treatment. The three-year crude survival rate in 36 patients with seminoma was 86% and in 24 patients with carcinoma it was 41.7%. There were no survivors among patients with choriocarcinoma. Our results are comparable with those of other series. A prospective study is proposed of the value of irradiation and subsequent limited lymph node dissection following orchiectomy in cases of carcinoma of the testis. PMID:4855670

  5. Intraoral malignant melanoma

    PubMed Central

    Babburi, Suresh; Subramanyam, R. V.; Aparna, V.; Sowjanya, P.

    2013-01-01

    Primary oral mucosal melanoma is a rare aggressive neoplasm and accounts for only 0.2-8% of all reported melanomas. It is a malignant neoplasm of melanocytes that may arise from a benign melanocytic lesion or de novo from melanocytes within normal skin or mucosa. It is considered to be the most deadly and biologically unpredictable of all human neoplasms, having the worst prognosis. In this article, we report a case of oral melanoma in a 52-year-old female patient with a chief complaint of black discolouration of the maxillary gingiva and palate. PMID:24249959

  6. Progress in therapies for cystic fibrosis.

    PubMed

    De Boeck, Kris; Amaral, Margarida D

    2016-08-01

    Standard follow-up and symptomatic treatment have allowed most patients with cystic fibrosis to live to young adulthood. However, many patients still die prematurely from respiratory insufficiency. Hence, further investigations to improve these therapies are important and might have relevance for other diseases-eg, exploring how to increase airway hydration, how to safely downscale the increased inflammatory response in the lung, and how to better combat lung infections associated with cystic fibrosis. In parallel, development of modulators that target the underlying dysfunction in the cystic fibrosis transmembrane conductance regulator (CFTR) is fast moving forward. Existing treatments are specific to certain mutations, or mutation class, in CFTR. An effective, although not yet entirely corrective, treatment is available for patients with class III mutations, and a treatment with modest effectiveness is available for patients who are homozygous for Phe508del, albeit at a very high cost. Corrective treatments that are non-specific to mutation class and thus applicable to all patients-eg, gene therapy, cell-based therapies, and activation of alternative ion channels that bypass CFTR-are being explored, but they are still in early stages of development. In view of the large number of patients with very rare mutations, a plan to advance personalised biomarkers to predict treatment effect is also being investigated and validated. PMID:27053340

  7. [Molecular epidemiology of cystic fibrosis in Tunisia].

    PubMed

    Messaoud, T; Bel Haj Fredj, S; Bibi, A; Elion, J; Férec, C; Fattoum, S

    2005-01-01

    Cystic fibrosis is the most frequent autosomal recessive genetic disease in North European population. This pathology seems to not be rare in Tunisia. On another hand, development of molecular biology techniques has largely contributed to implement the study of the different mutations in the CFTR gene where over 1,300 mutations were reported. Herein, we describe the strategy used to detect molecular defects responsible of cystic fibrosis on 390 children (383 families) in Tunisian population. Several techniques were performed for genotype diagnosis: DNA extraction was from peripheral blood. Polymerase chain reaction (PCR) and polyacylamide gel electrophoresis, and reverse dot blot procedures were used to detect known point mutations. Denaturant gradient gel electrophoresis (DGGE) were used in a next step searching for the unknown point mutations that are later identified by automated sequencing on ABIprism 310. This strategy allowed us to detect 17 different mutations located on the different exons of the CFTR gene. The most frequent was the F508del (50.74%) followed by three other mutations (G542X, W1282X and N1303K) known to be common in the Mediterranean area. For mutations (T665S, 2766 del8, F1166C, L1043R) were exclusively found, up to now, in the Tunisian population. Our results permitted to establish cystic fibrosis mutations and their distribution in Tunisia and to implement an appropriate prevention program of these diseases through the genetic council and prenatal diagnosis.

  8. Different manifestations of calcifying cystic odontogenic tumor.

    PubMed

    Utumi, Estevam Rubens; Pedron, Irineu Gregnanin; da Silva, Leopoldo Penteado Nucci; Machado, Gustavo Grothe; Rocha, André Caroli

    2012-01-01

    The calcifying cystic odontogenic tumor normally presents as a painless, slow-growing mass, involving both maxilla and mandible, primarily the anterior segment (incisor/canine area). It generally affects young adults in the third to fourth decades, with no gender predilection. Computerized tomography images revealed important characteristics that were not detected by panoramic radiography, such as fenestration, calcification and tooth-like structures. The typical microscopic feature of this lesion is the presence of variable amounts of aberrant epithelial cells, without nuclei, which are named "ghost cells". In addition, dysplastic dentine can be found and occasionally the cyst can be associated with an area of dental hard tissue formation resembling an odontoma. The treatment for calcifying cystic odontogenic tumor involves simple enucleation and curettage. The purpose of this article is to present two different manifestation of calcifying cystic odontogenic tumor in which computerized tomography, associated to clinical features, served as an important tool for diagnosis, adequate surgical planning and follow-up of patients.

  9. Giant magnetostrictive composites

    NASA Astrophysics Data System (ADS)

    Duenas, Terrisa Ann

    The limitation of magnetostrictive composites has been in their low magnetostrictive response when compared to their monolithic counterparts. In this dissertation research is presented describing the methods and analysis used to create a giant magnetostrictive composite (GMC) producing giant strains at low fields, exhibiting magnetization ``jumping'' and the ΔE effect. This composite combines the giant magnetostrictive material, Terfenol-D (Tb0.3Dy0.7Fe2) in particle form, with a nonmetallic binder and is capable of producing strains (at room temperature) exceeding 1000 ppm at a nominal field of 1.5 kOe mechanically unloaded and 1200 ppm at 8 MPa preload (2.5 kOe). Several studies leading to the high response of this composite are presented. A connectivity study shows that a [1-3] connected composite produces 50% more strain than a [0-3] composite. A resin study indicates that the lower the viscosity of the resin, the greater the magnetostrictive response; this is attributed to the removal of voids during degassing. A void study correlates the increase in voids to the decrease in strain response. A model is used to correlate analysis with experimental results within 10% accuracy and shows that an optimal volume fraction exists based on the properties of the binder. Using a Polyscience Spurr low- viscosity (60 cps) binder this volume fraction is nominally 20%; this optimum is attributed to the balance of epoxy contracting on the particle (built-in preload) and the actuation delivered by the magnetostrictive material. In addition to the connectivity, resin, void, and volume-fraction study, particle size and gradation studies are presented. Widely dispersed (<106, <212, <300 μm), narrowly dispersed (<45, (90-106), (275-300) μm), and an optimized bimodal (18.7% of (45-90) μm with 81.3% of (250-300) μm) particle distributions are studied. Results show that the larger the particle size, the higher the magnetostrictive response; this is attributed to the reduction of

  10. Giant vesicles: preparations and applications.

    PubMed

    Walde, Peter; Cosentino, Katia; Engel, Helen; Stano, Pasquale

    2010-05-01

    There is considerable interest in preparing cell-sized giant unilamellar vesicles from natural or nonnatural amphiphiles because a giant vesicle membrane resembles the self-closed lipid matrix of the plasma membrane of all biological cells. Currently, giant vesicles are applied to investigate certain aspects of biomembranes. Examples include lateral lipid heterogeneities, membrane budding and fission, activities of reconstituted membrane proteins, or membrane permeabilization caused by added chemical compounds. One of the challenging applications of giant vesicles include gene expressions inside the vesicles with the ultimate goal of constructing a dynamic artificial cell-like system that is endowed with all those essential features of living cells that distinguish them from the nonliving form of matter. Although this goal still seems to be far away and currently difficult to reach, it is expected that progress in this and other fields of giant vesicle research strongly depend on whether reliable methods for the reproducible preparation of giant vesicles are available. The key concepts of currently known methods for preparing giant unilamellar vesicles are summarized, and advantages and disadvantages of the main methods are compared and critically discussed. PMID:20336703

  11. [Pleomorphic adenoma of salivary glands: diagnostic pitfalls and mimickers of malignancy].

    PubMed

    Skálová, A; Andrle, P; Hostička, L; Michal, M

    2012-10-01

    Pleomorphic adenoma is the most common salivary gland tumor, characterized by a complex biphasic proliferation of epithelial and myoepithelial cells intermingled with a mezenchymal component with frequent metaplastic changes and protean histomorphology of the cells. This review describes several unusual histological findings in pleomorphic adenoma that may mimic malignancy, and therefore they represent a diagnostic pitfall. Intravascular invasion of tumor cells is generally suspicious of malignancy; however, intravascular tumor deposits may be rarely found within the capsule of clinically benign salivary pleomorphic adenomas. It is important not to render a malignant diagnosis in such neoplasms, in the absence of other evidence of malignancy. Pleomorphic adenomas, particularly of minor glands of palate, may contain large areas of squamous and mucinous metaplasia suspicious of mucoepidermoid carcinoma (MEC). In contrast to MEC, metaplastic pleomorphic adenomas do not harbour the distinctive translocations t(11;19) and t(11;15), they are not invasive, in contrast they reveal at least focally myxochondroid stroma. Cribriform structures in pleomorphic adenoma may mimic adenoid cystic carcinoma. Oncocytic metaplasia in cellular rich pleomorphic adenoma/myoepithelioma may be associated with significant nuclear polymorphism and hyperchromasia suspicious of malignancy. The most common pitfall in diagnosis of pleomorphic adenoma is so called "atypical PA" that must be distinguished from early malignant transformation to in situ-carcinoma ex pleomorphic adenoma.

  12. Chloride and potassium channels in cystic fibrosis airway epithelia

    NASA Astrophysics Data System (ADS)

    Welsh, Michael J.; Liedtke, Carole M.

    1986-07-01

    Cystic fibrosis, the most common lethal genetic disease in Caucasians, is characterized by a decreased permeability in sweat gland duct and airway epithelia. In sweat duct epithelium, a decreased Cl- permeability accounts for the abnormally increased salt content of sweat1. In airway epithelia a decreased Cl- permeability, and possibly increased sodium absorption, may account for the abnormal respiratory tract fluid2,3. The Cl- impermeability has been localized to the apical membrane of cystic fibrosis airway epithelial cells4. The finding that hormonally regulated Cl- channels make the apical membrane Cl- permeable in normal airway epithelial cells5 suggested abnormal Cl- channel function in cystic fibrosis. Here we report that excised, cell-free patches of membrane from cystic fibrosis epithelial cells contain Cl- channels that have the same conductive properties as Cl- channels from normal cells. However, Cl- channels from cystic fibrosis cells did not open when they were attached to the cell. These findings suggest defective regulation of Cl- channels in cystic fibrosis epithelia; to begin to address this issue, we performed two studies. First, we found that isoprenaline, which stimulates Cl- secretion, increases cellular levels of cyclic AMP in a similar manner in cystic fibrosis and non-cystic fibrosis epithelial cells. Second, we show that adrenergic agonists open calcium-activated potassium channels, indirectly suggesting that calcium-dependent stimulus-response coupling is intact in cystic fibrosis. These data suggest defective regulation of Cl- channels at a site distal to cAMP accumulation.

  13. Mesenteric calcified cystic lymphangioma in an adult patient.

    PubMed

    Albayrak, Yavuz; Albayrak, Fatih; Arslan, Serdar; Calik, Ilknur

    2011-06-01

    Abdominal cystic lymphangiomas are rare congenital benign malformations of the lymphatic system. To the best of our knowledge, only 6 mesenteric calcified cystic lymphangiomas have ever been reported. We herein describe a woman who presented to our hospital with stomachache that had been continuous for approximately 8 months. An abdominal computed tomography showed a cystic lesion. In the exploration, the cyst was totally excised. Based on the histomorphological data, a case of "calcified cystic lymphangioma" was diagnosed. Although mesenteric lymphangiomas are rare, especially in adults, they should be considered as a possible cause of abdominal pain. Treatment is surgical with resection of the mass, sometimes including resection of adjacent bowel.

  14. Spontaneous malignant craniopharyngioma in an aged Wistar rat

    PubMed Central

    Heinrichs, Martin; Ernst, Heinrich

    2016-01-01

    Craniopharyngiomas are extremely rare epithelial tumors of the sellar region in human beings and domestic and laboratory animals. A craniopharyngioma, 0.6 cm in diameter, was observed grossly in the sellar and parasellar regions of an untreated 23-month-old male Wistar-derived rat sacrificed moribund. The tumor was composed of cords, columns, and nests of neoplastic stratified squamous epithelium with marked hyperkeratosis and parakeratosis. Neoplastic cells formed solid or cystic areas, infiltrating the base of the skull, brain, and pituitary gland. Immunocytochemical evaluation revealed a strong cytoplasmic reaction for pan-cytokeratin in all tumor cells. Malignant craniopharyngioma should be considered a differential diagnosis in the rat when a tumor with stratified squamous epithelial features and a locally aggressive growth pattern is observed in the sellar or suprasellar region. PMID:27559246

  15. Spontaneous malignant craniopharyngioma in an aged Wistar rat.

    PubMed

    Heinrichs, Martin; Ernst, Heinrich

    2016-07-01

    Craniopharyngiomas are extremely rare epithelial tumors of the sellar region in human beings and domestic and laboratory animals. A craniopharyngioma, 0.6 cm in diameter, was observed grossly in the sellar and parasellar regions of an untreated 23-month-old male Wistar-derived rat sacrificed moribund. The tumor was composed of cords, columns, and nests of neoplastic stratified squamous epithelium with marked hyperkeratosis and parakeratosis. Neoplastic cells formed solid or cystic areas, infiltrating the base of the skull, brain, and pituitary gland. Immunocytochemical evaluation revealed a strong cytoplasmic reaction for pan-cytokeratin in all tumor cells. Malignant craniopharyngioma should be considered a differential diagnosis in the rat when a tumor with stratified squamous epithelial features and a locally aggressive growth pattern is observed in the sellar or suprasellar region. PMID:27559246

  16. Systemic therapy for selected skull base sarcomas: Chondrosarcoma, chordoma, giant cell tumour and solitary fibrous tumour/hemangiopericytoma.

    PubMed

    Colia, Vittoria; Provenzano, Salvatore; Hindi, Nadia; Casali, Paolo G; Stacchiotti, Silvia

    2016-01-01

    This review highlights the data currently available on the activity of systemic therapy in chondrosarcoma, chordoma, giant cell tumour of the bone (GCTB) and solitary fibrous tumour, i.e., four rare sarcomas amongst mesenchymal malignancy arising from the skull base.

  17. Systemic therapy for selected skull base sarcomas: Chondrosarcoma, chordoma, giant cell tumour and solitary fibrous tumour/hemangiopericytoma.

    PubMed

    Colia, Vittoria; Provenzano, Salvatore; Hindi, Nadia; Casali, Paolo G; Stacchiotti, Silvia

    2016-01-01

    This review highlights the data currently available on the activity of systemic therapy in chondrosarcoma, chordoma, giant cell tumour of the bone (GCTB) and solitary fibrous tumour, i.e., four rare sarcomas amongst mesenchymal malignancy arising from the skull base. PMID:27330421

  18. Multinuclear giant cell formation is enhanced by down-regulation of Wnt signaling in gastric cancer cell line, AGS

    SciTech Connect

    Kim, Shi-Mun; Kim, Rockki; Ryu, Jae-Hyun; Jho, Eek-Hoon; Song, Ki-Joon; Jang, Shyh-Ing; Kee, Sun-Ho . E-mail: keesh@korea.ac.kr

    2005-08-01

    AGS cells, which were derived from malignant gastric adenocarcinoma tissue, lack E-cadherin-mediated cell adhesion but have a high level of nuclear {beta}-catenin, which suggests altered Wnt signal. In addition, approximately 5% of AGS cells form multinuclear giant cells in the routine culture conditions, while taxol treatment causes most AGS cells to become giant cells. The observation of reduced nuclear {beta}-catenin levels in giant cells induced by taxol treatment prompted us to investigate the relationship between Wnt signaling and giant cell formation. After overnight serum starvation, the shape of AGS cells became flattened, and this morphological change was accompanied by decrease in Myc expression and an increase in the giant cell population. Lithium chloride treatment, which inhibits GSK3{beta} activity, reversed these serum starvation effects, which suggests an inverse relationship between Wnt signaling and giant cell formation. Furthermore, the down-regulation of Wnt signaling caused by the over-expression of ICAT, E-cadherin, and Axin enhanced giant cell formation. Therefore, down-regulation of Wnt signaling may be related to giant cell formation, which is considered to be a survival mechanism against induced cell death.

  19. [Giant adrenal myelolipoma].

    PubMed

    El Mejjad, Amine; Fekak, Hamid; Dakir, Mohamed; Sarf, Ismail; Manni, Ahmed; Meziane, Fethi

    2004-02-01

    Adrenal myelolipoma is a rare, benign, non-secreting tumour composed of adipose and haematopoietic tissue. The authors report a rare case of giant adrenal myelolipoma in a 53-year-old patient presenting with low back pain and a palpable flank mass on examination. CT scan suggested the diagnosis and surgical resection was indicated in view of the size and symptomatic nature of this mass. Histological examination confirmed the diagnosis. The outcome was favourable without recurrence after a follow-up of one year. The diagnosis of adrenal myelolipoma is based on radiology. Conservative management is generally sufficient for small asymptomatic tumours, but resection is required for large (> 5 cm) and/or symptomatic tumours.

  20. Two giant stellar complexes

    NASA Astrophysics Data System (ADS)

    Efremov, Yu. N.; Efremov, E. Yu.

    Common star complexes are huge (0.3-1 kpc in diameter) groups of relatively young stars, associations and clusters. The complexes usually form regular chains along spiral arms of grand design galaxies, being evidently formed and supported by magneto- gravitational instability developing along an arm. Special attention is given to a few large complexes which have signatures of gravitational boundness, such as round shape and high central density. Concentrations of stars and clusters in such a complex in M51 galaxy were found in this paper; we concluded it is possible to suggest that the complex is gravitationally bound. It is also stressed that some properties of the giant complex in NGC 6946 (such as its semicircular and sharp Western edge) are still enigmatic.

  1. Pleural malignancies including mesothelioma.

    PubMed

    Hillerdal, G

    1995-07-01

    Malignant mesothelioma is caused almost exclusively by occupational exposure to asbestos. During the past few years, however, increasing evidence has mounted that background exposure to asbestos could be sufficient to cause mesothelioma. Treatment of malignant mesothelioma remains a big problem. Some new approaches are on their way, and the most exciting ones are local immunotherapy in very early cases. Some success has been reported with local interferon treatment. As for treatment of metastatic pleural disease, the main purpose is symptomatic relief of dyspnea caused by fluid accumulation. The best way to achieve a lasting palliation is pleurodesis, and the most common way to do this, is by chemical means. The drug of choice in the United States has for many years been tetracycline, but since injectable tetracycline is no longer available, some substitute must be found. The substance that will "win" is not yet clear, but the two leading contestants are talc and doxycycline. Bleomycin also has its supporters, and a dark horse is quinacrine, which although not easily available in the United States, has been used in many European centers for decades. PMID:9363074

  2. Malignant pleural mesothelioma.

    PubMed

    Boutin, C; Schlesser, M; Frenay, C; Astoul, P

    1998-10-01

    The incidence of malignant pleural mesothelioma (MPM) has risen for some decades and is expected to peak between 2010 and 2020. Up to now, no single treatment has been proven to be effective and death usually occurs within about 12-17 months after diagnosis. Perhaps because of this poor prognosis, early screening has incited little interest. However, certain forms may have a better prognosis when diagnosed early and treated by multimodal therapy or intrapleural immunotherapy. Diagnosis depends foremost on histological analysis of samples obtained by thoracoscopy. This procedure allows the best staging of the pleural cavity with an attempt to detect visceral pleural involvement, which is one of the most important prognostic factors. Although radiotherapy seems necessary and is efficient in preventing the malignant seeding after diagnostic procedures in patients, there has been no randomized phase III study showing the superiority of any treatment compared with another. However, for the early-stage disease (stage I) a logical therapeutic approach seems to be neoadjuvant intrapleural treatment using cytokines. For more advanced disease (stages II and III) resectability should be discussed with the thoracic surgeons and a multimodal treatment combining surgery, radiotherapy and chemotherapy should be proposed for a randomized controlled study. Palliative treatment is indicated for stage IV. In any case, each patient should be enrolled in a clinical trial.

  3. Nonconventional papillary thyroid carcinomas with pleomorphic tumor giant cells: a diagnostic pitfall with anaplastic carcinoma.

    PubMed

    Hommell-Fontaine, Juliette; Borda, Angela; Ragage, Florence; Berger, Nicole; Decaussin-Petrucci, Myriam

    2010-06-01

    The presence of pleomorphic tumor giant cells in thyroid carcinomas of follicular cell origin is always worrisome for the pathologist as they first of all refer to anaplastic carcinoma, one of the most aggressive human malignancies. However, non-anaplastic pleomorphic giant cells are well described in other thyroid diseases, most often benign. In this paper, we describe four cases of papillary thyroid carcinoma displaying pleomorphic tumor giant cells with features that differ from those of anaplastic carcinoma. Pleomorphic giant cells were admixed with the underlying thyroid carcinoma and constituted from 5% to 25% of the tumor. Cytologically, they had an abundant eosinophilic cytoplasm with large and irregular nuclei. Compared to pleomorphic giant cells of anaplastic carcinoma, they reproduced the growth pattern of the underlying carcinoma, had a low mitotic index without necrosis or inflammation, and were reactive with thyroglobulin and thyroid-specific transcription factor-1 and strongly and diffusely positive for cytokeratin AE1/AE3. After 16-84 months of follow-up, patients are relapse-free and still alive. These cases show that pleomorphic tumor giant cells arising in papillary thyroid carcinomas do not always represent dedifferentiation and progression to anaplastic carcinoma. Distinction among these processes is critical as their treatment and prognosis are very different.

  4. Atmospheres of Extrasolar Giant Planets

    NASA Technical Reports Server (NTRS)

    Marley, Mark

    2006-01-01

    The next decade will almost certainly see the direct imaging of extrasolar giant planets around nearby stars. Unlike purely radial velocity detections, direct imaging will open the door to characterizing the atmosphere and interiors of extrasola planets and ultimately provide clues on their formation and evolution through time. This process has already begun for the transiting planets, placing new constraints on their atmospheric structure, composition, and evolution. Indeed the key to understanding giant planet detectability, interpreting spectra, and constraining effective temperature and hence evolution-is the atmosphere. I will review the universe of extrasolar giant planet models, focusing on what we have already learned from modeling and what we will likely be able to learn from the first generation of direct detection data. In addition to these theoretical considerations, I will review the observations and interpretation of the - transiting hot Jupiters. These objects provide a test of our ability to model exotic atmospheres and challenge our current understanding of giant planet evolution.

  5. Landscape of the lost giants

    NASA Astrophysics Data System (ADS)

    2013-09-01

    The Pleistocene megafauna extinction erased a group of remarkable animals. Whether humans had a prominent role in the extinction remains controversial, but it is emerging that the disappearance of the giants has markedly affected the environment.

  6. Pharma giants swap research programs.

    PubMed

    2014-07-01

    Pharmaceutical giants Novartis and GlaxoSmithKline (GSK) agreed in late April to swap some assets, with Novartis handing off its vaccine business to GSK and getting most of the British company's cancer portfolio in return.

  7. Cystic Fibrosis below the Diaphragm: Abdominal Findings in Adult Patients.

    PubMed

    Lavelle, Lisa P; McEvoy, Sinead H; Ni Mhurchu, Elaine; Gibney, Robert G; McMahon, Colm J; Heffernan, Eric J; Malone, Dermot E

    2015-01-01

    Cystic fibrosis (CF) is the most common lethal autosomal recessive disease in the white population. Mutation of the CF transmembrane conductance regulator gene on chromosome 7 results in production of abnormally viscous mucus and secretions in the lungs of patients with CF. A similar pathologic process occurs in the gastrointestinal tract, pancreas, and hepatobiliary system. Inspissated mucus causes luminal obstruction and resultant clinical and radiologic complications associated with the disease process. Pancreatic involvement can result in exocrine and endocrine insufficiency, pancreatic atrophy, fatty replacement, or lipomatous pseudohypertrophy. Acute and chronic pancreatitis, pancreatic calcification, cysts, and cystosis also occur. Hepatic manifestations include hepatic steatosis, focal biliary and multilobular cirrhosis, and portal hypertension. Biliary complications include cholelithiasis, microgallbladder, and sclerosing cholangitis. The entire digestive tract can be involved. Distal ileal obstruction syndrome, intussusception, appendicitis, chronic constipation, colonic wall thickening, fibrosing colonopathy, pneumatosis intestinalis, gastroesophageal reflux, and peptic ulcer disease have been described. Renal manifestations include nephrolithiasis and secondary amyloidosis. The educational objectives of this review are to reveal the abdominal manifestations of CF to facilitate focused analysis of cross-sectional imaging in adult patients. Life expectancy in patients with CF continues to improve because of a combination of aggressive antibiotic treatment, improved emphasis on nutrition and physiotherapy, and development of promising new CF transmembrane conductance regulator modulators. As lung function and survival improve, extrapulmonary conditions, including hepatic and gastrointestinal malignancy, will be an increasing cause of morbidity and mortality. Awareness of the expected abdominal manifestations of CF may assist radiologists in identifying

  8. Acinic-cell carcinoma, papillary-cystic variant: a diagnostic dilemma in salivary gland aspiration.

    PubMed

    Ali, Syed Z

    2002-10-01

    Fine-needle aspiration (FNA) plays a pivotal role as the initial diagnostic modality of choice when dealing with salivary gland (SG) lesions. Due to the heterogeneous nature of most SG neoplasms, cytologic interpretation on FNA can be quite challenging. This is particularly valid when dealing with rare tumor types, such as the papillary-cystic variant of acinic-cell carcinoma (ACC-PCV), resulting in a high rate of false-negative cytologic diagnoses. Seven cases of ACC-PCV diagnosed on tissue resection with a prior FNA performed in cytology were studied. Material consisted of Diff-Quik and Papanicolaou stained cytologic smears, as well as hematoxylin-eosin stained cell block and histopathologic sections. In no case did the FNA performed prior to the surgical resection eventuate in a cytopathologic diagnosis of cancer. A retrospective morphologic review of the smears disclosed several characteristics common to all 7 cases, i.e., mostly tightly cohesive fragments of neoplastic epithelium seen as monolayered sheets or with a prominent papillary architecture, high nuclear:cytoplasmic ratio ductal-type epithelium, cystic material and degenerated cellular debris, histiocytes, cells with squamoid and metaplastic oncocytic changes, vacuolated and pigmented histiocyte-like tumor cells, and lack of a predominant single-cell component or naked neoplastic cell nuclei. ACC-PCV represents a rare yet significant cause of false-negative results for cancer on SG aspirations. An erroneous interpretation may occur due to lack of experience in dealing with this tumor type, the rarity of published literature on ACC-PCV, and a predominantly cystic, somewhat variegated appearance of the tumor mimicking other benign and malignant SG lesions.

  9. Neuroleptic malignant syndrome.

    PubMed

    Ingall, T J; Tennant, C

    1986-11-01

    The neuroleptic malignant syndrome is a relatively rare but potentially fatal complication of the use of major tranquillizers; mortality may be as high as 20%. The syndrome is manifest by the onset of hyperpyrexia, muscular rigidity and tremor, impaired consciousness and autonomic dysfunction. The pathophysiology is thought to be by way of dopamine receptor blockade. The syndrome is managed by cessation of the neuroleptic medication, by supportive measures and by instituting treatment with one or more of a number of specific drugs whose use is based on theoretical considerations rather than empirical evidence of efficacy; these drugs include anticholinergics, L-dopa, bromocriptine amantidine and dantrolene sodium. Although not proven, early recognition and treatment may reduce both the mortality and the longer term morbidity of this syndrome. PMID:3773831

  10. Primary pineal malignant melanoma

    PubMed Central

    Cedeño Diaz, Oderay Mabel; Leal, Roberto García; La Cruz Pelea, Cesar

    2011-01-01

    Primary pineal malignant melanoma is a rare entity, with only thirteen cases reported in the world literature to date. We report a case of a 70-year-old man, who consulted with gait disturbance of six months duration, associated in the last month with dizziness, visual abnormalities and diplopia. No other additional melanocytic lesions were found elsewhere. The magnetic resonance showed a 25 mm expansive mass in the pineal gland that was associated with hydrocephaly, ventricular and transependimary oedema. The lesion was partially excised by a supracerebellar infratentorial approach. The histological examination revealed a melanoma. The patient received radiation therapy, but died of disease 16 weeks later. We herein review the literature on this rare tumour and comment on its clinical, radiological and histopathological features and differential diagnosis. PMID:24765293

  11. Anorectal giant condyloma acuminatum (Buschke-Loewenstein tumor): CT and radiographic manifestations

    SciTech Connect

    Balthazar, E.J.; Streiter, M.; Megibow, A.J.

    1984-03-01

    Giant condyloma acuminatum (Buschke-Loewenstein tumor) is an unusual variety of venereal wart characterized by a large size, cauliflower-like appearance, and locally invasive nature. CT examination of two patients with anorectal giant condylomata revealed the papillomatous and invasive nature of the lesion and the extent of tumor; there was infiltration of the subcutaneous tissue, perirectal fascial planes, and luminal narrowing with marked thickening of the rectal wall. The pathologic specimens showed benign histologic finding in one case and a mixed pattern with areas of malignant degeneration in the other. CT accurately demonstrated the exact location and extent of the lesions. The presence of malignant degeneration in this inherently invasive lesion can be established only on histologic examination.

  12. The malignant primate?

    PubMed

    de Grouchy, J

    1991-01-01

    Speciation and carcinogenesis result from genomic instability at the gametic or at the somatic levels. After an infinity of trials they occur, by chromosome rearrangements, in single individuals or in single cells and evolve by similar chromosomal or clonal evolutions. Loss of heterozygosity for the first event is essential in both processes: in evolution, a chromosomal rearrangement, a pericentric inversion or a Robertsonian fusion, must become homozygous to ensure a reproductive barrier for a new species; Knudson's two-event sequence is a similar situation in cancer. Position effect is equally important: we have shown overexpression of the SOD1 gene in the orangutan phylum probably by an intrachromosomal rearrangement; the t(9;22) in CML acts by typical position effect. Parental imprinting underlies the evolution of genome function and the unset of certain cancers. Evolution and malignancy are interweaved by viruses and oncogenes since the dawn of life. Cancer uses its intelligence to expand and to destroy the other tissues, using subtle metabolic pathways and a variety of tricks to metastasize other cells. It always wins but saws the branch on which it sits. Mankind also grows exponentially, killing thousands of other species, poisoning the oceans and soft waters, polluting the atmosphere, all for his egoistic needs. Man also travels and metastasizes other Earths. He modifies his genome or that of other species, and develops new technologies for his reproduction. He can destroy the planet in an eyeblink. To be or not to be the malignant primate, that will be the dilemma for the 21st Century. PMID:1809219

  13. An Adolescent Girl with Giant Fibroadenoma - A Case Report.

    PubMed

    Ferdousee, Ishrat; Arafat, S M Yasir; Ahmed, Zuhayer

    2016-09-01

    Though fibroadenoma is the most common benign tumor of the breast and is more common under the age of 30, giant fibroadenoma is rare representing less than 4% of all fibroadenomas. A 12 years old girl presented with rapidly enlarging well-circumscribed firm, non-tender mass in right breast for 2 months which was painless and with no history of trauma, nipple discharge, fever, anorexia, weight loss or axillary lymphadenopathy. There was no family history of neoplasms. Clinically, the lump was about 12 × 12 cm and not fixed to skin or underlying structures with the absence of nipple retraction or discharge. There was no axillary lymphadenopathy. Fine needle aspiration cytology showed a benign proliferative breast disease. Total excision of mass was done preserving nipple and areola having weight of 535 gm with histopathological features suggestive of giant fibroadenoma. Giant fibroadenoma is a benign breast disease that may mimic rare malignant lesion. So, breast and nipple conserving surgery should always be performed irrespective of size of tumor as in this case.

  14. An Adolescent Girl with Giant Fibroadenoma - A Case Report.

    PubMed

    Ferdousee, Ishrat; Arafat, S M Yasir; Ahmed, Zuhayer

    2016-09-01

    Though fibroadenoma is the most common benign tumor of the breast and is more common under the age of 30, giant fibroadenoma is rare representing less than 4% of all fibroadenomas. A 12 years old girl presented with rapidly enlarging well-circumscribed firm, non-tender mass in right breast for 2 months which was painless and with no history of trauma, nipple discharge, fever, anorexia, weight loss or axillary lymphadenopathy. There was no family history of neoplasms. Clinically, the lump was about 12 × 12 cm and not fixed to skin or underlying structures with the absence of nipple retraction or discharge. There was no axillary lymphadenopathy. Fine needle aspiration cytology showed a benign proliferative breast disease. Total excision of mass was done preserving nipple and areola having weight of 535 gm with histopathological features suggestive of giant fibroadenoma. Giant fibroadenoma is a benign breast disease that may mimic rare malignant lesion. So, breast and nipple conserving surgery should always be performed irrespective of size of tumor as in this case. PMID:27648624

  15. Lacrimal gland adenoid cystic carcinoma: intracranial and extracranial en bloc resection.

    PubMed

    Marsh, J L; Wise, D M; Smith, M; Schwartz, H

    1981-10-01

    Adenoid cystic carcinoma of the lacrimal gland is a rare tumor, although it is the malignancy most frequently arising in the gland. Treatment has been unsuccessful generally, with a 15-year survival of less than 20 percent. Our experience with this tumor in a 61-year-old woman has led to a proposal for therapeutic management based on awareness of the lesion's natural history, an understanding of regional anatomy, and familiarity with therapies reported in the literature. The feasibility of adequate tumor ablation is determined from preoperative evaluation, including CT scan, initial exploratory craniotomy, and frozen-section examination of the cranial nerves transversing the orbit. Once resectability is confirmed, "curative" intracranial and extracranial en bloc resection is performed, including the tumor, the lacrimal gland, and all contiguous structures. The defect is immediately resurfaced with and "ice cream cone" forehead flap in anticipation of adjuvant radiotherapy. An orbital prosthesis is fitted as soon as the radiation reaction subsides, and a postablative CT scan is obtained as the baseline for follow-up. It remains to be seen whether this application of the technology of CT scanning and the techniques of craniofacial surgery will improve the prognosis for adenoid cystic carcinoma arising in the lacrimal gland. PMID:6269133

  16. Differential diagnosis between adenoid cystic carcinoma and pleomorphic adenoma of the minor salivary glands of palate.

    PubMed

    Cerulli, Giulio; Renzi, Giancarlo; Perugini, Maurizio; Becelli, Roberto

    2004-11-01

    Tumors arising from minor salivary glands of the palate may exhibit an overlap of clinical and biologic features that may produce diagnostic and therapeutic dilemmas. Surgical treatment can be very different, depending on the dimensions and malignant or benign nature of the tumors, and therefore should be planned on the basis of an accurate differential diagnosis. A retrospective analysis in 24 patients with pleomorphic adenoma and adenoid cystic carcinoma of minor salivary glands of the palate was performed to investigate the accuracy of fine needle aspiration cytology (FNAC) and biopsy with histology in the preoperative diagnosis. Preoperative diagnoses obtained with FNAC and biopsy were compared with findings of the definitive histopathologic examination performed on the resected mass. Correspondence between the preoperative diagnoses determined by FNACs and the definitive histopathologic results was observed in 22 of 24 cases, whereas a complete equivalence was found with regards to histology. In the analysis, FNAC was associated with 91.6% accuracy and an error rate of 8.4% in the diagnosis of pleomorphic adenoma and adenoid cystic carcinoma of the palate. From the results of the analysis, histologic examination is still the most accurate diagnostic tool in such tumors. FNAC can be considered in tumors of the head and neck regions that are difficult to reach by means of a common biopsy.

  17. Mucoepidermoid carcinoma of the parotid presenting as periauricular cystic nodules: a series of four cases.

    PubMed

    Lehmer, Larisa M; Ragsdale, Bruce D; Crawford, Richard I; Bukachevsky, Roman; Hannah, Lauren A

    2012-07-01

    Mucoepidermoid carcinoma is a relatively common neoplasm of the major and minor salivary glands that can secondarily involve skin. In the vicinity of the ear lobe, mimicry of a benign cyst, both clinically and histopathologically is a diagnostic pitfall to avoid. The clinical manifestations, diagnostic histopathology, and clinical course of mucoepidermoid carcinoma of the parotid gland presenting as a clinically benign periauricular cystic nodule in four patients ranging in age from 11 to 63 years, are analyzed in the present report. Illustrating the challenge of accurate diagnosis, three of the four cases were initially misinterpreted on biopsy as benign cystic lesions. Multiple biopsies displayed foamy histiocytes around mucinous extravasations into dermis that mimicked ruptured epithelial cysts in two cases before malignancy was ascertained. This series demonstrates the need to include parotid tumor in the differential diagnosis of odd periauricular cyst-like expansions and adenosquamous proliferations. Mucoepidermoid carcinoma in particular can explain indolent, infra-auricular 'mucinous cysts'. Familiarity with this syndrome should arouse suspicion of parotid carcinoma when a 'cyst' or nodule is located near the earlobe. Delay in diagnosis results in larger surgical procedures than are otherwise necessary.

  18. Bilateral Calcifying Cystic Odontogenic Tumour of Mandible: A Rare Case Report and Review of Literature

    PubMed Central

    Khandelwal, Pragun; Mhapuskar, Amit

    2015-01-01

    Calcifying cystic odontogenic tumour (CCOT) is a relatively rare lesion of oral and maxillofacial region and forms only 2% of all odontogenic tumours. It was previously known as Calcifying odontogenic cyst and only recently has been classified as a tumour by WHO. The controversy regarding its origin can be owed to its diverse clinical and histopathological presentation and variation in reported malignant potential. It was first reported by Gorlin in 1962 and since then conundrum regarding its true nature has persisted. It is seen in association with other lesions like odontoma, ameloblastoma and ameloblastic fibroma. Both intra-osseous and extra-osseous forms of CCOT have been reported. It commnoly occurs in anterior region with equal preponderance in maxilla and mandible. Here we present a rare case of bilateral CCOT in the posterior mandible of a 16-year-old male patient which was discovered incidentally during a radiographic examination. PMID:26673837

  19. Bilateral Calcifying Cystic Odontogenic Tumour of Mandible: A Rare Case Report and Review of Literature.

    PubMed

    Khandelwal, Pragun; Aditya, Amita; Mhapuskar, Amit

    2015-11-01

    Calcifying cystic odontogenic tumour (CCOT) is a relatively rare lesion of oral and maxillofacial region and forms only 2% of all odontogenic tumours. It was previously known as Calcifying odontogenic cyst and only recently has been classified as a tumour by WHO. The controversy regarding its origin can be owed to its diverse clinical and histopathological presentation and variation in reported malignant potential. It was first reported by Gorlin in 1962 and since then conundrum regarding its true nature has persisted. It is seen in association with other lesions like odontoma, ameloblastoma and ameloblastic fibroma. Both intra-osseous and extra-osseous forms of CCOT have been reported. It commnoly occurs in anterior region with equal preponderance in maxilla and mandible. Here we present a rare case of bilateral CCOT in the posterior mandible of a 16-year-old male patient which was discovered incidentally during a radiographic examination.

  20. Axillary lymph node metastases in adenoid cystic carcinoma of the breast. A rare finding

    PubMed Central

    SPILIOPOULOS, D.; MITSOPOULOS, G.; KAPTANIS, S.; HALKIAS, C.

    2015-01-01

    Adenoid cystic carcinoma (ACC) of the breast is a rare malignant salivary-type neoplasm that has a good prognosis and represents less than 1% of all breast cancers. It is a triple negative carcinoma that presents as a painful mass. The mean age at the time of diagnosis is 50–60 years old. The solid variant of this type of tumour with basaloid features and presence of nodal metastases is very rare and considered to have a more aggressive clinical course. We present a case with presence of axillary lymph node metastases that was successfully treated with no evidence of recurrence one year after the diagnosis and review the literature. PMID:26712257

  1. Papillary cystic variant of acinic cell carcinoma presenting as parotid tail tumor.

    PubMed

    Khan, Sabina; Pujani, Mukta; Hassan, Mohd Jaseem; Jetley, Sujata

    2015-01-01

    Acinic cell carcinoma (ACC) is an uncommon low-grade tumor of the salivary glands that constitutes 2.5-4% of parotid gland tumors. Papillary cystic variant (PCV) of ACC is even rarer and can be diagnosed on histopathological examination only. It is important to diagnose this variant as it carries a poor prognosis when compared with other variants of ACC and is known to be universally fatal in 10 years. The present case describes ACC-PCV in a 20-year-old male, which presented as a slow growing parotid tail tumor and was misdiagnosed as a benign lesion both cytologically and radiologically. This case emphasizes the importance of histopathological examination in parotid masses as well as the need to consider malignant lesion in the differential diagnosis of a parotid tail tumor.

  2. Clinical evolution of cystic teratoma during treatment with combination chemotherapy.

    PubMed

    Taylor, H G; Tell, D T; Skoog, S J; McLeod, D G

    1986-09-01

    Progressive, cystic tumor enlargement in the abdomen developed in a patient with teratocarcinoma during treatment with systemic chemotherapy. Tumor markers were elevated in the cyst fluid and negative in serum. Further, the patient underwent a successful surgical debulking of large amounts of cystic teratoma.

  3. Iron overload and hematologic malignancies.

    PubMed

    Franchini, Massimo; Veneri, Dino

    2004-01-01

    Although iron is essential for cell replication and survival, an increase of body iron stores has been implicated in the development of cancer. However, while the association between iron overload and hepatocellular carcinoma is well documented, the relationship with nonhepatocellular malignancies remains ill-defined. In this review, we briefly report the present knowledge regarding the association between iron overload and hematologic malignancies.

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

    PubMed

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

    2015-06-01

    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.

  5. Pseudomonal infection in cystic fibrosis: the battle continues.

    PubMed

    Elkin, Sarah; Geddes, Duncan

    2003-12-01

    Pseudomonas aeruginosa lung infection is the major cause of morbidity and mortality in patients with cystic fibrosis. Infection usually begins in childhood and is responsible for respiratory failure and death in most patients with cystic fibrosis. The organism triggers an exuberant chronic inflammatory reaction which damages the airways and leads to progressive loss of lung function. Over the last decade significant advances have been made in the understanding of the pathophysiology of cystic fibrosis airways disease. These should assist the development of new and better therapies to treat this pathogen. This review provides an overview of pseudomonal infection in cystic fibrosis, including mechanisms by which the bacteria may colonize the cystic fibrosis airway, persistence of pseudomonal infection and the biofilm mode of growth. Available treatments and possible novel approaches to therapy will be discussed. PMID:15482158

  6. New insights into cystic fibrosis-related diabetes in children.

    PubMed

    Ode, Katie L; Moran, Antoinette

    2013-09-01

    Cystic fibrosis-related diabetes (CFRD) is the most common complication of cystic fibrosis. It is associated with significantly increased morbidity and mortality in adults and children. Adolescents with cystic fibrosis have a much higher prevalence of diabetes than any other similar age population. Glucose abnormalities that precede diabetes are even more common, especially in children younger than 10 years. The pathophysiology of glucose metabolic abnormalities is poorly understood, but insulin insufficiency is clearly the main component. Findings from animal studies have provided insight into the pathophysiology of CFRD, and imply that carbohydrate metabolic abnormalities might begin at much younger ages than was previously thought in patients with cystic fibrosis, and might be related to the basic cystic fibrosis chloride channel defect. In this Review we explore present knowledge of CFRD in children and adolescents, and new data that indicate that the pathophysiology of CFRD begins in very young patients.

  7. Cystic Fibrosis Diagnosis and Newborn Screening.

    PubMed

    Rosenfeld, Margaret; Sontag, Marci K; Ren, Clement L

    2016-08-01

    The diagnosis of cystic fibrosis (CF) has evolved over the past decade as newborn screening has become universal in the United States and elsewhere. The heterogeneity of phenotypes associated with CF transmembrane conductance regulator (CFTR) dysfunction and mutations in the CFTR gene has become clearer, ranging from classic pancreatic-insufficient CF to manifestations in only 1 organ system to indeterminate diagnoses identified by newborn screening. The tools available for diagnosis have also expanded. This article reviews the newest diagnostic criteria for CF, newborn screening, prenatal screening and diagnosis, and indeterminate diagnoses in newborn-screened infants and symptomatic adults. PMID:27469178

  8. The Changing Microbial Epidemiology in Cystic Fibrosis

    PubMed Central

    LiPuma, John J.

    2010-01-01

    Summary: Infection of the airways remains the primary cause of morbidity and mortality in persons with cystic fibrosis (CF). This review describes salient features of the epidemiologies of microbial species that are involved in respiratory tract infection in CF. The apparently expanding spectrum of species causing infection in CF and recent changes in the incidences and prevalences of infection due to specific bacterial, fungal, and viral species are described. The challenges inherent in tracking and interpreting rates of infection in this patient population are discussed. PMID:20375354

  9. Cystic fibrosis diagnosed in an elderly man.

    PubMed

    Gilljam, Marita; Björck, Eva

    2004-01-01

    Although most patients with cystic fibrosis (CF) are diagnosed in early childhood, the diagnosis may be delayed for patients with mild symptoms or single-organ disease. We describe a man with known infertility and a history of productive cough diagnosed with CF at 61 years of age. The patient carries two mutations and one variant in the CF transmembrane conductance regulator gene: 394delTT, P99L and R75Q. During a 15-year follow-up time he has developed significant pulmonary disease.

  10. Nutrition and Growth in Cystic Fibrosis.

    PubMed

    Lusman, Sarah; Sullivan, Jillian

    2016-08-01

    Close attention to nutrition and growth is essential in caring for children with cystic fibrosis (CF). Growth and nutritional status should be monitored as part of routine CF care. Children with CF should achieve growth and nutritional status comparable with that of well-nourished children without CF. Children with CF are at risk for nutritional deficiencies. Optimal nutritional and growth status may be difficult to attain in this population given risk of insufficient caloric intake and likelihood of increased caloric expenditure. Various methods to attain optimal nutritional status may be used, including oral supplementation, behavioral treatment, pharmacotherapy, and enteral nutrition. PMID:27469181

  11. Congenital cystic adenomatoid malformation type I

    PubMed Central

    Ribeiro, Frederico Becker; Schultz, Regina

    2015-01-01

    Congenital cystic adenomatoid malformation (CCAM) is an hamartomatous congenital pulmonary airway malformation with incidence ranging between 1:10,000 and 1:35,000 newborns. Currently CCAM is classified into five groups according to clinical and pathological features. The clinical outcome varies depending on the subtype and the extent of involvement. The authors report the case of a premature male newborn with the prenatal diagnosis of CCAM Type 1 associated with cardiac right axis deviation, who died 67 hours after birth due to respiratory failure. In addition to the autopsy report of this rare entity, the authors present its classification and prognosis. PMID:26558243

  12. Cystic retroperitoneal lymphangioma in infants and children.

    PubMed

    Leonidas, J C; Brill, P W; Bhan, I; Smith, T H

    1978-04-01

    Retroperitoneal lymphangiomas are uncommon benign tumors usually presenting in early life. They may cause significant pressure on adjacent structures, most often with superior and medial displacement of the kidney and medial deviation of the ureter. Relative radiolucency on total body opacification during excretory urography, and features of a cystic structure on ultrasonography and computed tomography should further suggest the possibility of a retroperitoneal lymphangioma. Vascular stretching and displacement without neovascularity on angiography and contrast entry into the cysts on lower extremity lymphography are additional features, but such procedures are usually unnecessary.

  13. [Acquired cystic renal disease. Association with hypernephroma].

    PubMed

    Comesaña, E; Pesqueira, D; Tardáguila, F; De la Fuente, A; Antón, I; Vidal, L; Zungri, E

    1992-02-01

    Emergence of multiple bilateral renal cysts observed in patients undergoing periodic haemodialysis is 40%. The pathology, known as Acquired Cystic Renal Disease (A.C.R.D.) presents a high association to renal cancer. Two cases of A.C.R.D. and their association with hypernephroma, one resulting in secondary retroperitoneal haemorrhage and the other in intracystic haemorrhage, are presented. Forms and diagnosis are analyzed, insisting upon the need of monitoring the patients in haemodialysis from the point of view of tumour emergence.

  14. Retroperitoneal cystic neuroendocrine tumor. A case report.

    PubMed

    Scapinello, A; D'Amore, E S; Cavazzana, A O; Gramegna, V; Ninfo, V

    1995-10-01

    A 21 cm retroperitoneal cystic mass was excised from a 71 year old woman. The cyst was filled with a hemorrhagic fluid and contained a 5 cm parietal hemorrhagic nodule. On histology, the nodule was composed of a uniform population of round cells arranged in trabeculae and nests. The neoplastic cells were immunoreactive to cytokeratin, EMA, NSE, chromogranin A, pancreatic polypeptide (PP) and Gastrin (G). Ultrastructural observation of neurosecretory granules confirmed the neuroendocrine nature of the tumor. No other lesions were detected and a diagnosis of primary epithelial neuroendocrine tumor was rendered. The histogenesis of the tumor including the possibility of a paraganglionic origin is discussed.

  15. [Nutrition, cystic fibrosis and the digestive tract].

    PubMed

    Olveira, Gabriel; Olveira, Casilda

    2008-05-01

    The prevalence of hyponutrition in cystic fibrosis is high although it may vary according to the different studies. Detection of hyponutrition should be done by combining different methods, depending on their availability. However, the simplest and most validated criterion is to measure at each visit the weight (and height in children) in order to calculate the body mass index and categorizing hyponutrition according to absolute criteria: in adults < 18.5 kg/m(2), and in children as percentiles of the body mass index. Worsening of the nutritional status is directly related with the decrease in lung function parameters and it has been proposed as a morbidity (and even mortality) predictive factor in people with cystic fibrosis, independently of the level of pulmonary dysfunction. Exocrine pancreatic insufficiency is present is approximately 70-90% of the patients with cystic fibrosis and the genotype-phenotype correlation is high. Most of the patients with exocrine pancreatic insufficiency tolerate a high-fat diet provided that they are treated with pancreatic enzymes at appropriate doses. The prevalence of diabetes increases with age, reaching up 40% of the cases in patients older than 30 years. Clinical liver involvement is less prevalent (it approximately affects 1/3 of the patients). Other intestinal complications such as meconial ileus, gastroesophageal reflux, obstruction of the distal intestine, or fibrosing colon disease may also condition malnourishment. In patients with cystic fibrosis, a usual high-fat diet providing 120%-150% of the recommended calories is advised. If the nutritional goals are not achieved or maintained with diet modifications, artificial supplements may be added, although the recommendation for their use has not been endorsed by solid scientific evidences. The most frequently used preparations usually are polymeric or hypercaloric. The indications for enteral (through a tube, especially gastrostomy) or parenteral nutritional support are

  16. A unique advantage for giant eyes in giant squid.

    PubMed

    Nilsson, Dan-Eric; Warrant, Eric J; Johnsen, Sönke; Hanlon, Roger; Shashar, Nadav

    2012-04-24

    Giant and colossal deep-sea squid (Architeuthis and Mesonychoteuthis) have the largest eyes in the animal kingdom [1, 2], but there is no explanation for why they would need eyes that are nearly three times the diameter of those of any other extant animal. Here we develop a theory for visual detection in pelagic habitats, which predicts that such giant eyes are unlikely to evolve for detecting mates or prey at long distance but are instead uniquely suited for detecting very large predators, such as sperm whales. We also provide photographic documentation of an eyeball of about 27 cm with a 9 cm pupil in a giant squid, and we predict that, below 600 m depth, it would allow detection of sperm whales at distances exceeding 120 m. With this long range of vision, giant squid get an early warning of approaching sperm whales. Because the sonar range of sperm whales exceeds 120 m [3-5], we hypothesize that a well-prepared and powerful evasive response to hunting sperm whales may have driven the evolution of huge dimensions in both eyes and bodies of giant and colossal squid. Our theory also provides insights into the vision of Mesozoic ichthyosaurs with unusually large eyes.

  17. Giant gastrointestinal stromal tumor of the stomach.

    PubMed

    Ionescu, Sever; Barbu, Emil; Ionescu, Călin; Costache, Adrian; Bălăşoiu, Maria

    2015-01-01

    Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal malignancies of the digestive tract. Gastric localization is the most frequent. The aim of this study is to evaluate the importance of immunohistochemical factors (CD117, CD34, α-SMA, vimentin, p53, Ki67) in diagnostic and size tumor and mitotic activity as prognostic factors for these tumors. We present the case of a 66-year-old male patient with a giant gastric GIST. Like in the vast majority, the symptomatology in this patient has long been faint, despite the large tumor size, and when it became manifest, it was nonspecific. Imagery wise, the computer tomography (CT) scan was the most efficient, showing the origin of the tumor from the greater curvature of the stomach, its dimensions, as well as the relations with the other abdominal viscera. Surgery in this patient was en-bloc, according to the principles of GIST. The histological aspect is characterized by a proliferation of spindle cells positive for CD117 and CD34. Despite complete microscopic resection, the size of the tumor (25×20×27 cm) and the mitotic activity (21÷5 mm2) remains important relapse factor.

  18. Giant multilocular prostatic cystadenoma: a distinctive lesion of the retroperitoneum in men. A report of two cases.

    PubMed

    Maluf, H M; King, M E; DeLuca, F R; Navarro, J; Talerman, A; Young, R H

    1991-02-01

    Two examples of large, multiloculated, cystic tumors that arose within the pelvis in men of 28 and 37 years of age are described. The tumors were composed of glands and cysts lined by prostatic-type epithelium lying in a hypocellular fibrous stroma. The prostatic nature of the lesions was confirmed by immunohistochemical staining of the epithelium for prostate-specific antigen and prostatic acid phosphatase. Two apparently similar lesions were found in the literature; one tumor was attached to the prostate by a pedicle, and the other arose in the retrovesical space. These tumors, for which we propose the designation "giant multilocular prostatic cystadenoma," appear to be benign, although they may recur if incompletely excised. They may pose considerable diagnostic difficulty if the prostatic nature of the epithelium is not appreciated, an error that is likely if a relationship to the prostate is not recognized. This lesion should be included in the differential diagnosis of retroperitoneal cystic tumors in men.

  19. Cystic fibrosis chronic rhinosinusitis: A comprehensive review

    PubMed Central

    Chaaban, Mohamad R.; Kejner, Alexandra; Rowe, Steven M.

    2013-01-01

    Background: Advances in the care of patients with cystic fibrosis (CF) have improved pulmonary outcomes and survival. In addition, rapid developments regarding the underlying genetic and molecular basis of the disease have led to numerous novel targets for treatment. However, clinical and basic scientific research focusing on therapeutic strategies for CF-associated chronic rhinosinusitis (CRS) lags behind the evidence-based approaches currently used for pulmonary disease. Methods: This review evaluates the available literature and provides an update concerning the pathophysiology, current treatment approaches, and future pharmaceutical tactics in the management of CRS in patients with CF. Results: Optimal medical and surgical strategies for CF CRS are lacking because of a dearth of well-performed clinical trials. Medical and surgical interventions are supported primarily by level 2 or 3 evidence and are aimed at improving clearance of mucus, infection, and inflammation. A number of novel therapeutics that target the basic defect in the cystic fibrosis transmembrane conductance regulator channel are currently under investigation. Ivacaftor, a corrector of the G551D mutation, was recently approved by the Food and Drug Administration. However, sinonasal outcomes using this and other novel drugs are pending. Conclusion: CRS is a lifelong disease in CF patients that can lead to substantial morbidity and decreased quality of life. A multidisciplinary approach will be necessary to develop consistent and evidence-based treatment paradigms. PMID:24119602

  20. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)

    PubMed Central

    Corradi, Valentina; Vergani, Paola; Tieleman, D. Peter

    2015-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) is a member of the ATP-binding cassette (ABC) transporter superfamily. CFTR controls the flow of anions through the apical membrane of epithelia. Dysfunctional CFTR causes the common lethal genetic disease cystic fibrosis. Transitions between open and closed states of CFTR are regulated by ATP binding and hydrolysis on the cytosolic nucleotide binding domains, which are coupled with the transmembrane (TM) domains forming the pathway for anion permeation. Lack of structural data hampers a global understanding of CFTR and thus the development of “rational” approaches directly targeting defective CFTR. In this work, we explored possible conformational states of the CFTR gating cycle by means of homology modeling. As templates, we used structures of homologous ABC transporters, namely TM(287–288), ABC-B10, McjD, and Sav1866. In the light of published experimental results, structural analysis of the transmembrane cavity suggests that the TM(287–288)-based CFTR model could correspond to a commonly occupied closed state, whereas the McjD-based model could represent an open state. The models capture the important role played by Phe-337 as a filter/gating residue and provide structural information on the conformational transition from closed to open channel. PMID:26229102

  1. Cystic echinococcosis is an occupational disease?

    PubMed

    Youssefi, Mohammad Reza; Mirshafiei, Sepide; Moshfegh, Zeinab; Soleymani, Norredin; Rahimi, Mohammad Taghi

    2016-09-01

    Cystic echinococcosis (CE) is considered as an occupational infection and among different careers greengroceries have a close contact with raw vegetables which can transmit eggs of Echinococcus granulosus. Therefore, the objective of this survey was to determine the seroprevalence of CE among greengroceries using ELISA test in Mazandaran and Gilan Provinces, northern Iran. Overall, 160 serum samples (60 male and 100 female) were collected from greengroceries in Mazandaran and Gilan Provinces, 2012. Sera were surveyed employing ELISA assay for diagnosis of CE. In addition, before sampling, a questionnaire was filled out for each subject. Seropositivity was 2.4 % (four cases) and females 1.8 % (three cases) were more positive compared to males 0.8 % (one case). Age group of 40-60 years encompassed the highest rate of positivity (1.8 %) and the least rate was in 20-40 years old age group (0 %). Besides, 1.8 % of seropositive subjects were dog owner, unaware of the disease and lives in rural areas. In conclusion, seroprevalence of cystic hydatid disease among greengroceries is relatively high in Mazandaran and Gilan Provinces. More continuous serological researches and preventive measures should be taken into consideration owing to the significance of the disease. PMID:27605752

  2. Targeting ion channels in cystic fibrosis.

    PubMed

    Mall, Marcus A; Galietta, Luis J V

    2015-09-01

    Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause a characteristic defect in epithelial ion transport that plays a central role in the pathogenesis of cystic fibrosis (CF). Hence, pharmacological correction of this ion transport defect by targeting of mutant CFTR, or alternative ion channels that may compensate for CFTR dysfunction, has long been considered as an attractive approach to a causal therapy of this life-limiting disease. The recent introduction of the CFTR potentiator ivacaftor into the therapy of a subgroup of patients with specific CFTR mutations was a major milestone and enormous stimulus for seeking effective ion transport modulators for all patients with CF. In this review, we discuss recent breakthroughs and setbacks with CFTR modulators designed to rescue mutant CFTR including the common mutation F508del. Further, we examine the alternative chloride channels TMEM16A and SLC26A9, as well as the epithelial sodium channel ENaC as alternative targets in CF lung disease, which remains the major cause of morbidity and mortality in patients with CF. Finally, we will focus on the hurdles that still need to be overcome to make effective ion transport modulation therapies available for all patients with CF irrespective of their CFTR genotype.

  3. CFTR protein repair therapy in cystic fibrosis.

    PubMed

    Quintana-Gallego, Esther; Delgado-Pecellín, Isabel; Calero Acuña, Carmen

    2014-04-01

    Cystic fibrosis is a single gene, autosomal recessive disorder, in which more than 1,900 mutations grouped into 6 classes have been described. It is an example a disease that could be well placed to benefit from personalised medicine. There are currently 2 very different approaches that aim to correct the basic defect: gene therapy, aimed at correcting the genetic alteration, and therapy aimed at correcting the defect in the CFTR protein. The latter is beginning to show promising results, with several molecules under development. Ataluren (PTC124) is a molecule designed to make the ribosomes become less sensitive to the premature stop codons responsible for class i mutations. Lumacaftor (VX-809) is a CFTR corrector directed at class ii mutations, among which Phe508del is the most frequent, with encouraging results. Ivacaftor (VX-770) is a potentiator, the only one marketed to date, which has shown good efficacy for the class iii mutation Gly551Asp in children over the age of 6 and adults. These drugs, or a combination of them, are currently undergoing various clinical trials for other less common genetic mutations. In the last 5 years, CFTR has been designated as a therapeutic target. Ivacaftor is the first drug to treat the basic defect in cystic fibrosis, but only provides a response in a small number of patients. New drugs capable of restoring the CFTR protein damaged by the most common mutations are required.

  4. [CYSTIC FIBROSIS: CARE OF THE LUNG DISEASE].

    PubMed

    Hubert, Dominique

    2015-10-01

    (Rh-DNase) and/or hydration (hypertonic saline) nebulisations, Moreover, treatment with inhaled antibiotics is indicated (tobramycin, colistine or aztreonam lysine) for chronic lung infection with Pseudomonas aeruginosa (PA). The treatment regimen also includes bronchodilators for bronchospasms and azithromycin. Regular physical activity is recommended. A treatment potentiating the CFTR protein, ivacaftor, is now indicated for patients with a class 3 mutation. Initial bronchial infection with PA must be treated as soon as possible in order to eradicate the pathogen. Pulmonary exacerbations require antibiotic courses, either orally or intravenously for PA infection. Complications require hospitalisation, with thoracic chest tube placement for a pneumothorax or bronchial artery embolisation for massive hemoptysis. Oxygen therapy and non-invasive ventilation with a nasal mask become necessary when respiratory insufficiency progresses, justifying the initiation of the lung transplant process. Lung disease affects the prognosis of cystic fibrosis, therefore its management in cystic fibrosis centres is of utmost importance. Maintenance treatment mainly relies on daily chest physiotherapy, which can be facilitated by mucolytic PMID:26749716

  5. Stereotaxic intracavitary irradiation for cystic craniopharyngiomas

    SciTech Connect

    Pollack, I.F.; Lunsford, L.D.; Slamovits, T.L.; Gumerman, L.W.; Levine, G.; Robinson, A.G.

    1988-02-01

    Stereotaxic intracavitary irradiation with instillation of phosphorus-32 (/sup 32/P) colloidal chromic phosphate was performed in nine patients with cystic craniopharyngiomas. Serial neurological, ophthalmological, neuroendocrinological, and radiological examinations were performed before and after treatment. Dosimetry was determined based on a computerized tomography (CT) estimation of tumor volume, and was calculated to provide a tumoricidal dose (200 to 300 Gy) to the cyst wall. The follow-up period ranged from 14 to 45 months (mean 27 months). After treatment, all nine patients showed improvement of symptoms and radiological evidence of cyst regression. Because of an expanding solid component producing recurrent symptoms, one patient required a craniotomy 14 months after isotope instillation. Three of five patients with impaired visual acuity before surgery had significant improvement in acuity after treatment. Preoperative visual field defects in eight patients improved in four after /sup 32/P therapy. Of seven patients with preoperative endocrine abnormalities, one individual showed almost complete normalization and another had improvement in endocrine function. Patients who exhibited residual neuroendocrine function before isotope instillation developed no significant deterioration in endocrine status during the follow-up period. The findings suggest that stereotaxic intracavitary irradiation is a safe and effective treatment which should be considered as the initial surgery for cystic craniopharyngiomas.

  6. CFTR protein repair therapy in cystic fibrosis.

    PubMed

    Quintana-Gallego, Esther; Delgado-Pecellín, Isabel; Calero Acuña, Carmen

    2014-04-01

    Cystic fibrosis is a single gene, autosomal recessive disorder, in which more than 1,900 mutations grouped into 6 classes have been described. It is an example a disease that could be well placed to benefit from personalised medicine. There are currently 2 very different approaches that aim to correct the basic defect: gene therapy, aimed at correcting the genetic alteration, and therapy aimed at correcting the defect in the CFTR protein. The latter is beginning to show promising results, with several molecules under development. Ataluren (PTC124) is a molecule designed to make the ribosomes become less sensitive to the premature stop codons responsible for class i mutations. Lumacaftor (VX-809) is a CFTR corrector directed at class ii mutations, among which Phe508del is the most frequent, with encouraging results. Ivacaftor (VX-770) is a potentiator, the only one marketed to date, which has shown good efficacy for the class iii mutation Gly551Asp in children over the age of 6 and adults. These drugs, or a combination of them, are currently undergoing various clinical trials for other less common genetic mutations. In the last 5 years, CFTR has been designated as a therapeutic target. Ivacaftor is the first drug to treat the basic defect in cystic fibrosis, but only provides a response in a small number of patients. New drugs capable of restoring the CFTR protein damaged by the most common mutations are required. PMID:24095197

  7. [Malignant Pleural Mesotheliomas].

    PubMed

    Biancosino, C; Redwan, B; Krüger, M; Eberlein, M; Bölükbas, S

    2016-09-01

    Malignant pleural mesotheliomas (MPM) are very aggressive tumors, which originate from the mesothelial cells of the pleural surface. The main risk factor associated with MPM is exposure to asbestos. The latency period between asbestos exposure and MPM can be 30-60 years. Clinical symptoms and signs are often nonspecifc. The diagnosis of MPM requires an adequate tissue specimen for pathological examination, and video assisted thoracoscopic surgey (VATS) is associated with the highest diagnostic yield. MPM are histologically classified into epitheloid, sacromatoid and biphasic (mixed) sub-types. Accurate staging with invasive tests, if needed, is an important step before an interdisciplinary team can decide on an optimal (multi-modal) treatment approach. A multi-modal treatment approach (surgery, radiation oncology and chemotherapy) is superior to all approaches relying only on a single modality, if the patient qualifies for it from an oncological and functional standpoint. The goal of the surgical therapy is to achieve macroscopic complete resection. There are two competing surgical approaches and philosophies: extrapleural pneumonectomy (EPP) and radical pleurectomy (RP). Over the last years a paradigm shift from EPP to RP occurred and RP is now often the preferred surgical option. PMID:27612329

  8. Liver transplantation for malignancies.

    PubMed

    Eghtesad, Bijan; Aucejo, Federico

    2014-09-01

    Liver transplantation (LT) has become an acceptable and effective treatment for selected patients with hepatocellular carcinoma with excellent outcomes. More recently, LT has been tried in different primary and secondary malignancies of the liver. The outcomes of LT for very selected group of patients with hilar cholangiocarcinoma (CCA) have been promising. Excellent results have been reported in LT for patients with unresectable hepatic epithelioid hemangioendothelioma (HEHE). In contrast to excellent results after LT for HEHE, results of LT for angiosarcoma have been disappointing with no long-term survivors. Hepatoblastoma (HB) is the most common primary liver cancer in pediatric age group. Long-term outcomes after LT in patients with unresectable tumor and good response to chemotherapy have been promising. Indication for LT for hepatic metastasis from neuroendocrine tumors (NETs) is mainly for patients with unresectable tumors and for palliation of medically uncontrollable symptoms. Posttransplant survival in those patients with low tumor activity index is excellent, despite recurrence of the tumor. More recent limited outcomes data on LT for unresectable hepatic metastases from colorectal cancer have claimed some survival benefit compared to the previous reports. However, due to the high rate of tumor recurrence in a very short time after LT, especially in the era of organ shortage, this indication has not been favored by the transplant community. PMID:24604263

  9. [Malignant mesothelioma and asbestos].

    PubMed

    Rüttner, J R

    1983-03-12

    Malignant mesothelioma is a rare neoplasm of rapidly lethal course arising primarily in the pleura and less often in the peritoneum. In the majority of cases the disease is closely related to occupational exposure to asbestos. The latency period, calculated from the first contact with asbestos to the appearance of mesothelioma, is generally in the order of 20 years or more irrespective of the duration of exposure. A causal relationship can be established with certainty only by a careful history and positive tissue analysis for the presence of asbestos. The author's own series of 48 pleural mesotheliomas comprises 39 cases involving occupational exposure to asbestos, 6 others with asbestos demonstrable in pulmonary tissue but no discernible source in the history, and 3 where no relation to asbestos could be established at all. Although a dose-response relation may be assumed for asbestos as for all other carcinogens, the lack of data on asbestos dust concentrations at former places of work rendered determination of the minimal noxious dose difficult or impossible. It also remains unclear whether the various asbestos types, such as chrysotile and amphiboles, differ in pathogenic effect. It is hoped that careful registration and continuing study of mesotheliomas will shed further light on their relationship to asbestos and on the possible hazards of the mineral to the general population.

  10. Formation of giant planets

    NASA Astrophysics Data System (ADS)

    Magni, G.; Coradini, A.

    2003-04-01

    In this presentation we address the problem of the formation of giant planets and their regular satellites. We study in particular the problem of formation of the Jupiter System comparing the results of the model with the present characteristics of the system, in order to identify what are those better represented by our approach. In fact here, using a 3-D hydro-dynamical code, we study the modalities of gas accretion onto a solid core, believed to be the seed from which Jupiter started. To do that we have modelled three main regions: the central planet, a turbulent accretion disk surrounding it and an extended region from which the gas is collected. In the extended region we treat the gas as a frictionless fluid. Our main goal is to identify what are the characteristics of the planet during its growth and the physical parameters affecting its growth at the expenses of the nebular gas present in the feeding zone. Moreover we want to understand what are the thermodynamical parameters characterizing the gas captured by the planet and swirling around it. Finally, we check if a disk can be formed in prograde rotation around the planet and if this disk can survive the final phases of the planet formation. Due to the interaction between the accreting planet and the disk it has been necessary to develop a complete model of the Jupiter’s structure. In fact the radiation emitted by the growing planet heats up the surrounding gas. In turn the planet’s thermodynamic structure depend on the mass accretion rate onto it. When the accretion is rapid, shock waves in the gas are formed close to the planet. This region cannot be safely treated by a numerical code; for this reason we have developed a semi-analytically model of a a turbulent accretion disk to be considered as transition between the planet and the surrounding disk.

  11. Giant Magellan Telescope: overview

    NASA Astrophysics Data System (ADS)

    Johns, Matt; McCarthy, Patrick; Raybould, Keith; Bouchez, Antonin; Farahani, Arash; Filgueira, Jose; Jacoby, George; Shectman, Steve; Sheehan, Michael

    2012-09-01

    The Giant Magellan Telescope (GMT) is a 25-meter optical/infrared extremely large telescope that is being built by an international consortium of universities and research institutions. It will be located at the Las Campanas Observatory, Chile. The GMT primary mirror consists of seven 8.4-m borosilicate honeycomb mirror segments made at the Steward Observatory Mirror Lab (SOML). Six identical off-axis segments and one on-axis segment are arranged on a single nearly-paraboloidal parent surface having an overall focal ratio of f/0.7. The fabrication, testing and verification procedures required to produce the closely-matched off-axis mirror segments were developed during the production of the first mirror. Production of the second and third off-axis segments is underway. GMT incorporates a seven-segment Gregorian adaptive secondary to implement three modes of adaptive-optics operation: natural-guide star AO, laser-tomography AO, and ground-layer AO. A wide-field corrector/ADC is available for use in seeing-limited mode over a 20-arcmin diameter field of view. Up to seven instruments can be mounted simultaneously on the telescope in a large Gregorian Instrument Rotator. Conceptual design studies were completed for six AO and seeing-limited instruments, plus a multi-object fiber feed, and a roadmap for phased deployment of the GMT instrument suite is being developed. The partner institutions have made firm commitments for approximately 45% of the funds required to build the telescope. Project Office efforts are currently focused on advancing the telescope and enclosure design in preparation for subsystem- and system-level preliminary design reviews which are scheduled to be completed in the first half of 2013.

  12. Rotation of Giant Stars

    NASA Astrophysics Data System (ADS)

    Kissin, Yevgeni; Thompson, Christopher

    2015-07-01

    The internal rotation of post-main sequence stars is investigated, in response to the convective pumping of angular momentum toward the stellar core, combined with a tight magnetic coupling between core and envelope. The spin evolution is calculated using model stars of initial mass 1, 1.5, and 5 {M}ȯ , taking into account mass loss on the giant branches. We also include the deposition of orbital angular momentum from a sub-stellar companion, as influenced by tidal drag along with the excitation of orbital eccentricity by a fluctuating gravitational quadrupole moment. A range of angular velocity profiles {{Ω }}(r) is considered in the envelope, extending from solid rotation to constant specific angular momentum. We focus on the backreaction of the Coriolis force, and the threshold for dynamo action in the inner envelope. Quantitative agreement with measurements of core rotation in subgiants and post-He core flash stars by Kepler is obtained with a two-layer angular velocity profile: uniform specific angular momentum where the Coriolis parameter {Co}\\equiv {{Ω }}{τ }{con}≲ 1 (here {τ }{con} is the convective time), and {{Ω }}(r)\\propto {r}-1 where {Co}≳ 1. The inner profile is interpreted in terms of a balance between the Coriolis force and angular pressure gradients driven by radially extended convective plumes. Inward angular momentum pumping reduces the surface rotation of subgiants, and the need for a rejuvenated magnetic wind torque. The co-evolution of internal magnetic fields and rotation is considered in Kissin & Thompson, along with the breaking of the rotational coupling between core and envelope due to heavy mass loss.

  13. Meibomian gland function and giant papillary conjunctivitis.

    PubMed

    Mathers, W D; Billborough, M

    1992-08-15

    We examined 42 contact lens-wearing patients for clinical evidence of giant papillary conjunctivitis and for meibomian gland dysfunction with gland dropout. Fifteen patients were free of clinical signs and symptoms of giant papillary conjunctivitis, whereas 27 had clinical symptoms and evidence of giant papillary conjunctivitis. Patients with giant papillary conjunctivitis had significantly more gland dropout with an average of 0.6 +/- 1.2 gland absent in both lower eyelids compared with 0.2 +/- 0.4 gland absent in patients without giant papillary conjunctivitis. Additionally, the viscosity of meibomian gland excreta was greater in the giant papillary conjunctivitis group. There was no difference in tear osmolarity or in the Schirmer test results between the two groups. These results indicated patients with giant papillary conjunctivitis were more likely to have meibomian gland dysfunction with gland dropout than patients without giant papillary conjunctivitis.

  14. Pancreatic pseudocyst or a cystic tumor of the pancreas?

    PubMed Central

    Rabie, Mohammad Ezzedien; El Hakeem, Ismail; Al Skaini, Mohammad Saad; El Hadad, Ahmad; Jamil, Salim; Tahir Shah, Mian; Obaid, Mahmoud

    2014-01-01

    Pancreatic pseudocysts are the most common cystic lesions of the pancreas and may complicate acute pancreatitis, chronic pancreatitis, or pancreatic trauma. While the majority of acute pseudocysts resolve spontaneously, few may require drainage. On the other hand, pancreatic cystic tumors, which usually require extirpation, may disguise as pseudocysts. Hence, the distinction between the two entities is crucial for a successful outcome. We conducted this study to highlight the fundamental differences between pancreatic pseudocysts and cystic tumors so that relevant management plans can be devised. We reviewed the data of patients with pancreatic cystic lesions that underwent intervention between June 2007 and December 2010 in our hospital. We identified 9 patients (5 males and 4 females) with a median age of 40 years (range, 30–70 years). Five patients had pseudocysts, 2 had cystic tumors, and 2 had diseases of undetermined pathology. Pancreatic pseudocysts were treated by pseudocystogastrostomy in 2 cases and percutaneous drainage in 3 cases. One case recurred after percutaneous drainage and required pseudocystogastrostomy. The true pancreatic cysts were serous cystadenoma, which was treated by distal pancreatectomy, and mucinous cystadenocarcinoma, which was initially treated by drainage, like a pseudocyst, and then by distal pancreatectomy when its true nature was revealed. We conclude that every effort should be exerted to distinguish between pancreatic pseudocysts and cystic tumors of the pancreas to avoid the serious misjudgement of draining rather than extirpating a pancreatic cystic tumor. Additionally, percutaneous drainage of a pancreatic pseudocyst is a useful adjunct that may substitute for surgical drainage. PMID:23958054

  15. Giant myoma and erythrocytosis syndrome.

    PubMed

    Ozsaran, A A; Itil, I M; Terek, C; Kazandi, M; Dikmen, Y

    1999-08-01

    The objective of this study is to discuss the myomatous erythrocytosis syndrome in a patient with a giant subserous uterine myoma. She presented with plethora and an abdominal mass. After venesection of 4 units of blood, the preoperative haematocrit value of 53.3% and haemoglobin value of 17.5 g/dL had decreased to 48.6% and 16.8 g/dL levels, respectively. After the operative extraction of the giant subserous myoma with attached uterus weighing 14.2 kg, the haematocrit and the haemoglobin values had regressed to 40.3% and 14.3 g/dL levels, respectively. The findings indicated that the giant subserous myoma was the cause of the myomatous erythrocytosis syndrome in this patient. PMID:10554963

  16. Structure of giant muscle proteins

    PubMed Central

    Meyer, Logan C.; Wright, Nathan T.

    2013-01-01

    Giant muscle proteins (e.g., titin, nebulin, and obscurin) play a seminal role in muscle elasticity, stretch response, and sarcomeric organization. Each giant protein consists of multiple tandem structural domains, usually arranged in a modular fashion spanning 500 kDa to 4 MDa. Although many of the domains are similar in structure, subtle differences create a unique function of each domain. Recent high and low resolution structural and dynamic studies now suggest more nuanced overall protein structures than previously realized. These findings show that atomic structure, interactions between tandem domains, and intrasarcomeric environment all influence the shape, motion, and therefore function of giant proteins. In this article we will review the current understanding of titin, obscurin, and nebulin structure, from the atomic level through the molecular level. PMID:24376425

  17. Drugs Approved for Malignant Mesothelioma

    Cancer.gov

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for malignant mesothelioma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  18. Defective Autophagy Initiates Malignant Transformation.

    PubMed

    Galluzzi, Lorenzo; Bravo-San Pedro, José Manuel; Kroemer, Guido

    2016-05-19

    In this issue of Molecular Cell, Park et al. (2016) elegantly demonstrate that a partial defect in autophagy supports malignant transformation as it favors the production of genotoxic reactive oxygen species by mitochondria.

  19. Malignant external otitis: CT evaluation

    SciTech Connect

    Curtin, H.D.; Wolfe, P.; May, M.

    1982-11-01

    Malignant external otitis is an aggressive infection caused by Pseudomonas aeruginosa that most often occurs in elderly diabetics. Malignant external otitis often spreads inferiorly from the external canal to involve the subtemporal area and progresses medially towards the petrous apex leading to multiple cranial nerve palsies. The computed tomographic (CT) findings in malignant external otitis include obliteration of the normal fat planes in the subtemporal area as well as patchy destruction of the bony cortex of the mastoid. The point of exit of the various cranial nerves can be identified on CT scans, and the extent of the inflammatory mass correlates well with the clinical findings. Four cases of malignant external otitis are presented. In each case CT provided a good demonstration of involvement of the soft tissues at the base of the skull.

  20. AMG 319 Lymphoid Malignancy FIH

    ClinicalTrials.gov

    2016-01-20

    Cancer; Chronic Lymphocytic Leukemia; Diffuse Large Cell Lymphoma; Hematologic Malignancies; Hematology; Leukemia; Low Grade Lymphoma; Lymphoma; Mantle Cell Lymphoma; Non-Hodgkin's Lymphoma; Oncology; Oncology Patients; T Cell Lymphoma; Tumors

  1. Malignant pleural mesothelioma.

    PubMed

    Ho, L; Sugarbaker, D J; Skarin, A T

    2001-01-01

    Malignant pleural mesothelioma remains a difficult tumor to treat, much less cure. Currently, the best chance for long-term survival lies with early diagnosis and aggressive surgical extirpation, but given the typically long delay between the onset of symptoms and diagnosis, this is only possible with a high index of suspicion and an aggressive diagnosis workup. Early referral to a tertiary center experienced in the treatment of MPM may be important for several reasons: (1) decreased risk of tumor spread along multiple thoracenesis/biopsy tracts, (2) the availability of specialized pathologic assays for definitive diagnosis, (3) the availability of critical staging modalities (aggressive mediastinoscopy +/- thoracoscopy, MRI scans performed according to specific mesothelioma protocols, and perhaps PET scans), (4) surgical experience with pleurectomy/decortication and/or extrapleural pneumonectomy, that may decrease morbidity and mortality, and (5) the availability of novel adjuvant protocols. Single-modality therapy is unlikely to result in long-term survival. Aggressive surgery is required for optimal debulking, and extrapleural pneumonectomy may offer better local control compared with pleurectomy/ecortication. Delivery of optimal radiation schedules, which may involve large fractions as well as large total doses, is limited by the presence of nearby dose-limiting structures. Current chemotherapy is severely lacking in producing objective responses and improved survival although gemcitabine and IL-2 may be active agents to be combined with radiation and/or other agents. Hyperthermia, photodynamic therapy, intracavitary therapy, and gene therapy are all relatively new techniques under active investigation that should be supported by enrollment in on-going protocols. Predictably, many of these techniques provide greater benefit when used in the setting of adjuvant protocols or minimal residual disease, emphasizing the importance of multimodality therapy.

  2. Cystic Fibrosis Transmembrane Conductance Regulator. Implications in Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.

    PubMed

    Cantin, André M

    2016-04-01

    Cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) have traditionally been viewed as two distinct entities of unrelated origins. However, molecular, cellular, and clinical studies have revealed that cystic fibrosis transmembrane conductance regulator (CFTR) protein dysfunction is common to both conditions, one (CF) being defined genetically and the other (COPD) as an acquired CFTR deficiency. Multiple molecular mechanisms of cigarette smoke-induced CFTR dysfunction have been reported. More importantly, considerable evidence of cigarette smoke-induced CFTR dysfunction in several respiratory and nonrespiratory tissues have been confirmed, making CFTR a target that cannot be overlooked in our quest to understand COPD and improve therapies for individuals affected by this disease. This review summarizes the molecular, cellular, and clinical evidence that CFTR dysfunction is induced by cigarette smoke exposure both in vitro and in vivo, and explores how this may contribute to the development of COPD. PMID:27115950

  3. Review of Giant cell arteritis

    PubMed Central

    Chacko, Joseph G.; Chacko, J. Anthony; Salter, Michael W.

    2014-01-01

    Giant-cell arteritis (GCA) is a systemic autoimmune disease affecting primarily the elderly. Giant cell arteritis can cause sudden and potentially bilateral sequential vision loss in the elderly. Therefore, it is considered a medical emergency in ophthalmology and a significant cause of morbidity in an increasingly aging population. Ophthalmologists need to be able to recognize the classic symptoms and signs of this disease, and then be able to work-up and treat these patients in an efficient manner. An in-depth review of GCA from the literature as well as personal clinical experience follows. PMID:25859139

  4. Chemical Abundances of Symbiotic Giants

    NASA Astrophysics Data System (ADS)

    Gałan, C.; Mikołajewska, J.; Hinkle, K. H.; Joyce, R. R.

    2015-12-01

    High resolution (R ˜ 50000), near-IR spectra were used to measure photospheric abundances of CNO and elements around the iron peak for 24 symbiotic giants. Spectrum synthesis was employed using local thermal equilibrium and hydrostatic model atmospheres. The metallicities are distributed in a wide range with maximum around [Fe/H] ˜-0.4 - - 0.3 dex. Enrichment in 14N indicates that all the sample giants have experienced the first dredge-up. The relative abundance of [Ti/Fe] is generally large in red symbiotic systems.

  5. Late presentation of congenital cystic adenomatoid malformation of the lung

    SciTech Connect

    Hulnick, D.H.; Naidich, D.P.; McCauley, D.I.; Feiner, H.D.; Avitabile, A.M.; Greco, M.A.; Genieser, N.B.

    1984-06-01

    Although most often recognized in neonates and young children, congenital cystic adenomatoid malformation of the lung (CCAM) occasionally appears in later years. Three patients, aged 35, 24, and 7 years, are reported. Chest radiographs in each case suggested a localized patchy density, a cystic mass, or a multicystic mass, but computed tomography (CT) best demonstrated the cystic and solid components while ruling out bronchiectasis or major bronchial obstruction. Bronchography contributed no further diagnostic information compared with CT. Each patient underwent lobectomy. Histologically, the characteristic overgrowth of bronchiolar elements replacing normal parenchymal architecture was accompanied by some superimposed inflammatory change.

  6. Remnant cystic duct adenocarcinoma presenting as gastric outlet obstruction

    PubMed Central

    Lo, Samuel Tsoon Wuan; Cheng, Yue; Cheung, Frances; Tang, Chung Ngai

    2016-01-01

    Only a few case reports of remnant cystic duct carcinoma exist. The presented case of remnant cystic duct carcinoma with invasion to pylorus and bulbus of duodenum leading to gastric outlet obstruction was the first of its kind. We reviewed all cases of remnant cystic duct carcinoma that we found in the literature and summarized its definition, presentation, extent of invasion and clinical outcome after operation. The diagnosis can be difficult due to the rarity of disease, locally advanced nature of disease and distorted postoperative anatomy. A high index of suspicion can increase the likelihood of a preoperative diagnosis. PMID:27154747

  7. Flow in Idealised Compliant Human Cystic Duct Models

    NASA Astrophysics Data System (ADS)

    Al-Atabi, M.; Chin, S. B.; Beck, S.; Luo, X. Y.

    The functions of the cystic duct and the role of its complicated geometry (in particular the valves of Heister), in the delivery of bile flow have always been a subject of speculation. This paper reports on an experimental investigation of the flow in idealised, compliant cystic duct models. The valves of Heister were idealised using eight semi-circular alternating baffles fitted inside the compliant tubes. These tubes were arranged in configurations representing the anatomical configurations of real cystic ducts. Models both with and without baffles were tested for comparison. The results show that the valves of Heister seem to play a role in facilitating both the filling and emptying of the gallbladder.

  8. Laparoscopic resection of a retroperitoneal cystic lymphangioma in an adult.

    PubMed

    Kasza, Jason; Brody, Fredrick J; Khambaty, Fatima; Vaziri, Khashayar; Wallace, Brian

    2010-06-01

    Cystic lymphangiomas are rare, benign anomalies of the lymphatic system. More than 95% of cystic lymphangiomas occur in the head, neck, and axilla with only 1% in the retroperitoneum. Most of these cases are diagnosed by the second year of life with only a handful of adult cases. Once a symptomatic cystic lesion of the abdomen or retroperitoneum is diagnosed, treatment usually consists of surgical excision. Traditionally, surgery requires a laparotomy. This paper describes a patient with a retroperitoneal cyst who underwent a successful laparoscopic resection. The etiology and management of adult retroperitoneal cysts are reviewed as well.

  9. Cystic change in primary paediatric optic nerve sheath meningioma.

    PubMed

    Narayan, Daniel; Rajak, Saul; Patel, Sandy; Selva, Dinesh

    2016-08-01

    Primary optic nerve sheath meningiomas (PONSM) are rare in children. Cystic meningiomas are an uncommon subgroup of meningiomas. We report a case of paediatric PONSM managed using observation alone that underwent cystic change and radiological regression. A 5-year-old girl presented with visual impairment and proptosis. Magnetic resonance (MR) imaging demonstrated a PONSM. The patient was left untreated and followed up with regular MR imaging. Repeat imaging at 16 years of age showed the tumour had started to develop cystic change. Repeat imaging at 21 years of age showed the tumour had decreased in size. PMID:27310300

  10. Malignant hypertension: a preventable emergency.

    PubMed

    van der Merwe, Walter; van der Merwe, Veronica

    2013-08-16

    The Waitemata Hypertension Clinic Database 2009-2012 (Auckland, New Zealand) was searched for patients meeting the definition of Malignant Hypertension. Eighteen of 565 patients met the criteria. All patients had essential hypertension which was either undiagnosed, untreated or undertreated. Most cases responded satisfactorily to standard drug therapy, but a number were left with significant chronic kidney disease. Malignant hypertension is a life-threatening disease which should be entirely preventable with regular blood pressure checks in primary care.

  11. Telomerase Activation in Hematological Malignancies

    PubMed Central

    Ropio, Joana; Merlio, Jean-Philippe; Soares, Paula; Chevret, Edith

    2016-01-01

    Telomerase expression and telomere maintenance are critical for cell proliferation and survival, and they play important roles in development and cancer, including hematological malignancies. Transcriptional regulation of the rate-limiting subunit of human telomerase reverse transcriptase gen (hTERT) is a complex process, and unveiling the mechanisms behind its reactivation is an important step for the development of diagnostic and therapeutic applications. Here, we review the main mechanisms of telomerase activation and the associated hematologic malignancies. PMID:27618103

  12. Telomerase Activation in Hematological Malignancies.

    PubMed

    Ropio, Joana; Merlio, Jean-Philippe; Soares, Paula; Chevret, Edith

    2016-01-01

    Telomerase expression and telomere maintenance are critical for cell proliferation and survival, and they play important roles in development and cancer, including hematological malignancies. Transcriptional regulation of the rate-limiting subunit of human telomerase reverse transcriptase gen (hTERT) is a complex process, and unveiling the mechanisms behind its reactivation is an important step for the development of diagnostic and therapeutic applications. Here, we review the main mechanisms of telomerase activation and the associated hematologic malignancies. PMID:27618103

  13. Craniofacial Resection for Cranial Base Malignancies Involving the Infratemporal Fossa

    PubMed Central

    Bilsky, Mark H.; Bentz, Brandon; Vitaz, Todd; Shah, Jatin; Kraus, Dennis

    2007-01-01

    OBJECTIVE Cranial base malignancies involving the infratemporal fossa have been considered unresectable. Advanced operative techniques have made tumor resection feasible in an en bloc fashion with negative histological margins, but there are limited data regarding outcome analysis in patients who have undergone resection of malignant tumors in this area. METHODS At Memorial Sloan-Kettering Cancer Center, 25 patients underwent anterolateral cranial base resections for tumors that involved the infratemporal fossa during a 7-year period. The most common tumors were sarcoma (n = 9), squamous cell carcinoma (n = 6), and adenoid cystic carcinoma (n = 3). The median size of the tumors was 6 cm, and 12 tumors involved the anterior cranial base and/or orbit. Tumor resections were divided into three types. Twelve patients underwent Type 1 dissection for tumors involving only the infratemporal fossa and maxillary sinus; 2 patients underwent Type 2 dissections involving the infratemporal fossa and anterior cranial base; and 11 patients underwent Type 3 dissection, which included the infratemporal fossa, anterior cranial base, and orbit. All patients required free flap reconstruction, 22 of which were rectus abdominis free flaps. RESULTS Complications occurred in seven patients, including a single mortality resulting from a myocardial infarction. The 2-, 3-, and 5-year survival rates were 69, 63, and 56%, respectively. The relapse-free survival rates were 47% at 2 and 3 years and 41% at 5 years. Recurrences were local in nine patients and distant in four patients. CONCLUSION Despite the extensive nature of many infratemporal fossa tumors, they can be resected with acceptable morbidity. Survival rates approach those of anterior cranial base malignancies without infratemporal fossa involvement. PMID:16234683

  14. Charting the Giants

    NASA Astrophysics Data System (ADS)

    2004-06-01

    zero expansion asymptotically after an infinite time and has a flat geometry). All three observational tests by means of supernovae (green), the cosmic microwave background (blue) and galaxy clusters converge at a Universe around Ωm ~ 0.3 and ΩΛ ~ 0.7. The dark red region for the galaxy cluster determination corresponds to 95% certainty (2-sigma statistical deviation) when assuming good knowledge of all other cosmological parameters, and the light red region assumes a minimum knowledge. For the supernovae and WMAP results, the inner and outer regions corespond to 68% (1-sigma) and 95% certainty, respectively. References: Schuecker et al. 2003, A&A, 398, 867 (REFLEX); Tonry et al. 2003, ApJ, 594, 1 (supernovae); Riess et al. 2004, ApJ, 607, 665 (supernovae) Galaxy clusters are far from being evenly distributed in the Universe. Instead, they tend to conglomerate into even larger structures, "super-clusters". Thus, from stars which gather in galaxies, galaxies which congregate in clusters and clusters tying together in super-clusters, the Universe shows structuring on all scales, from the smallest to the largest ones. This is a relict of the very early (formation) epoch of the Universe, the so-called "inflationary" period. At that time, only a minuscule fraction of one second after the Big Bang, the tiny density fluctuations were amplified and over the eons, they gave birth to the much larger structures. Because of the link between the first fluctuations and the giant structures now observed, the unique REFLEX catalogue - the largest of its kind - allows astronomers to put considerable constraints on the content of the Universe, and in particular on the amount of dark matter that is believed to pervade it. Rather interestingly, these constraints are totally independent from all other methods so far used to assert the existence of dark matter, such as the study of very distant supernovae (see e.g. ESO PR 21/98) or the analysis of the Cosmic Microwave background (e

  15. Syncytial giant cell hepatitis in a patient with chronic lymphocytic leukemia

    PubMed Central

    GUPTA, Neil; NJEI, Basile

    2016-01-01

    While it commonly occurs in the pediatric population, syncytial giant cell hepatitis is rare in adults, which is diagnosed histologically by the presence of multinucleated cells in the liver. The literature has recorded only approximately one hundred cases in adults during the past two decades in whom malignancy is rarely associated with giant cell hepatitis. Our patient is a 66-year-old woman who was diagnosed with chronic lymphocytic leukemia (CLL) and subsequently developed syncytial giant cell hepatitis. This disease is usually linked to virus infection, autoimmune diseases, and drug medications. The association between CLL and giant cell hepatitis is rare, with only three cases reported. In most cases viral particles on electron microscopy are reported and the patients usually have a history of chemotherapy and hypogammaglobulinemia. Unlike such cases, our patient developed giant cell hepatitis in the absence of such confounding variables. The treatment for our patient was a high-dose corticosteroid and rituxan, with improvement in liver enzymes. PMID:26147671

  16. Cytological patterns of primary malignant uterine fibrous histiocytoma. A case report.

    PubMed

    Torrisi, A; Giorgino, F; Onnis, G L; Minucci, D

    1990-01-01

    The Authors have reported the cytological patterns of malignant primitive uterine fibrous histiocytoma of a 57 year old woman. The endometrial cytological sampling was performed by an aspiration technique using a feeding-tube; a necrotic-haemorrhagic background filled with hymphocytes and polymorphonuclear leukocytes, and a mixture of highly anaplastic histiocytic and fibroblastic type cells, associated with undifferentiated small round cells, has been described. The result of the histological examination performed on the surgical specimen was the following: polymorphic malignant neoplasia characterized by spindle-like and giant plurinucleated cells, phagocytosis features, endoluminal polypoid growth, wide infiltration of the miometrium two thirds deep, vascular space invasion of the uterine wall and the hilus of the ovaries. The histological features and immunohistochemical analysis were consistent with malignant fibrous histiocytoma in the pleomorphic variety. PMID:1965802

  17. Cryptococcus neoformans infection in malignancy.

    PubMed

    Schmalzle, Sarah A; Buchwald, Ulrike K; Gilliam, Bruce L; Riedel, David J

    2016-09-01

    Cryptococcosis is an opportunistic invasive fungal infection that is well described and easily recognised when it occurs as meningitis in HIV-infected persons. Malignancy and its treatment may also confer a higher risk of infection with Cryptococcus neoformans, but this association has not been as well described. A case of cryptococcosis in a cancer patient is presented, and all cases of coincident C. neoformans infection and malignancy in adults published in the literature in English between 1970 and 2014 are reviewed. Data from these cases were aggregated in order to describe the demographics, type of malignancy, site of infection, clinical manifestations, treatment and outcomes of cryptococcosis in patients with cancer. Haematologic malignancies accounted for 82% of cases, with lymphomas over-represented compared to US population data (66% vs. 53% respectively). Cryptococcosis was reported rarely in patients with solid tumours. Haematologic malignancy patients were more likely to have central nervous system (P < 0.001) or disseminated disease (P < 0.001), receive Amphotericin B as part of initial therapy (P = 0.023), and had higher reported mortality rates than those with solid tumours (P = 0.222). Providers should have heightened awareness of the possibility of cryptococcosis in patients with haematologic malignancy presenting with infection. PMID:26932366

  18. Influenza vaccination in children with cystic fibrosis.

    PubMed

    Patria, Maria Francesca; Longhi, Benedetta; Esposito, Susanna

    2013-04-01

    Cystic fibrosis (CF) is an inherited autosomal recessive disease characterized by progressive pulmonary damage and respiratory failure. It is known that bacterial infections play a critical role in the development of significant lung damage, whereas the role of respiratory viruses in CF pulmonary exacerbations and the relationship between viral infections and the progression of lung damage are uncertain. Health authorities throughout the world recommend influenza vaccination for CF patients. The aim of this review is to analyze the impact of seasonal and pandemic influenza on CF patients and data concerning influenza vaccination in order to assess the current situation and identify areas for future study. As data are limited, further well-constructed clinical studies of the effectiveness of influenza vaccination on the main clinical outcome measures of pulmonary function and nutritional status in patients with CF are required.

  19. Control programme for cystic echinococcosis in Uruguay

    PubMed Central

    Irabedra, Pilar; Ferreira, Ciro; Sayes, Julio; Elola, Susana; Rodríguez, Miriam; Morel, Noelia; Segura, Sebastian; dos Santos, Estela; Guisantes, Jorge A

    2016-01-01

    Cystic echinococcosis is a highly endemic parasitic zoonosis that is present in the Southern Cone countries of America. For several decades, various prevention and control programmes have been implemented in different countries and regions, with varying results. In Uruguay, a new control programme was implemented in 2006 that employed new strategies for canine diagnosis and treatment, dog population control, diagnosis in humans, epidemiological surveillance, and health education, including community participation. The control programme in Uruguay addresses the control and surveillance of the disease from a holistic perspective based on Primary Health Care, which has strengthened the community’s participation in developing and coordinating activities in an interdisciplinary manner. Similarly, the control programme that is currently implemented is based on a risk-focused approach. The surveillance and control measures were focused on small villages and extremely poor urban areas. In this study, the strategies used and the results obtained from 2008-2013 are analysed and discussed. PMID:27223652

  20. Poly Cystic Ovarian Syndrome: An Updated Overview

    PubMed Central

    El Hayek, Samer; Bitar, Lynn; Hamdar, Layal H.; Mirza, Fadi G.; Daoud, Georges

    2016-01-01

    Poly Cystic Ovarian Syndrome (PCOS) is one of the most common metabolic and reproductive disorders among women of reproductive age. Women suffering from PCOS present with a constellation of symptoms associated with menstrual dysfunction and androgen excess, which significantly impacts their quality of life. They may be at increased risk of multiple morbidities, including obesity, insulin resistance, type II diabetes mellitus, cardiovascular disease (CVD), infertility, cancer, and psychological disorders. This review summarizes what the literature has so far provided from guidelines to diagnosis of PCOS. It will also present a general overview about the morbidities associated with this disease, specifically with its more severe classic form. Finally, the review will stress on the various aspects of treatment and screening recommendations currently used in the management of this condition. PMID:27092084

  1. Scoliosis in cystic fibrosis - an appraisal

    SciTech Connect

    Paling, M.R.; Spasovsky-Chernick, M.

    1982-03-01

    An unusually high prevalence (10%) of scoliosis is described in a series of 151 patients aged four years and older with cystic fibrosis. The scolioses were of the late onset (juvenile and adolescent) type, being typically thoracic with the curve convex to the right, although there was no significant preference for either sex. No direct relationship was found between the spinal curvature and the severity or distribution of the lung disease, although the worse scolioses tended to occur in patients with relatively severe pulmonary involvement. There was no evidence of metabolic bone disease as a predisposing cause. Some indication of a familial tendency towards scoliosis was apparent, and a genetic or constitutional basis is postulated with an unknown precipitating factor.

  2. Retroperitoneal bronchogenic cyst mimicking pancreatic cystic lesion.

    PubMed

    Wang, Shin-E; Tsai, Yi-Fang; Su, Cheng-Hsi; Shyr, Yi-Ming; Lee, Rheun-Chuan; Tsai, Wan-Chen; Li, Fen-Yau; Chen, Tien-Hua; Wu, Chew-Wun; Lui, Wing-Yiu

    2006-11-01

    Retroperitoneal bronchogenic cyst is detected extremely rarely and often masquerades as other diseases. Here, we report 2 cases of retroperitoneal bronchogenic cyst mimicking pancreatic mucinous tumor. Histologically, both cysts were composed of ciliated respiratory-like epithelium with abundant mucin content, smooth muscle bundles and mature cartilage, compatible with the diagnosis of retroperitoneal bronchogenic cyst. In addition to these 2 cases, another 42 retroperitoneal bronchogenic cysts reported in the English literature were collected for review and analysis. Twelve (28%) were located over the peripancreatic area. Just over half (51%) of them were asymptomatic. No accurate preoperative diagnosis could be made for any of the lesions. About a third (33.3%) of the peripancreatic retroperitoneal bronchogenic cysts masqueraded as pancreatic cystic lesions.

  3. Chronic Rhinosinusitis in Patients with Cystic Fibrosis.

    PubMed

    Hamilos, Daniel L

    2016-01-01

    Chronic rhinosinusitis (CRS) is highly prevalent in patients with cystic fibrosis (CF) and accounts for significant morbidity and contribution to CF lung disease. Mutations of the cystic fibrosis transmembrane regulator gene occur with increased prevalence in patients with CRS without CF, suggesting some contribution to CRS pathophysiology. Nasal polyps (NPs) occur with increased prevalence in patients with CF of all ages and have a more neutrophilic appearance with fewer eosinophils and increased submucosal glandular elements in comparison to NPs from patients without CF. Mainstays of medical treatment include isotonic saline irrigations and topical intranasal glucocorticoids, with some evidence that topical intranasal glucocorticoids reduce NP size. Although inhaled hypertonic saline (7%) has been widely studied as a mucolytic agent for CF lung disease, there are no reports of its use in CF CRS. Mucolytics have also not been studied as a treatment for CRS in CF, and most evidence does not support their use for CF lung disease. Nasally nebulized dornase alfa (recombinant human deoxyribonuclease) following sinus surgery shows promise for treatment. Other unproven therapies include addition of baby shampoo to isotonic saline to potentially thin mucus and help prevent biofilm formation. There are no data to support the use of low-dose oral macrolide antibiotics or the use of prophylactic oral antibiotics for CRS in patients with CF. However, there is some support for the use of topical antibiotics, including colistimethate sodium or tobramycin, administered as a sinus irrigation or antral lavage in patients following sinus surgery when susceptible bacteria are cultured. Key components of CF sinus surgical management include extensive surgery to ensure that the maxillary, frontal, sphenoid, and ethmoid sinuses are all widely opened with smoothing of bony overhangs to prevent mucus retention and bacterial recolonization, postoperative meticulous daily nasal irrigations

  4. Cystic fibrosis mutations in Costa Rica.

    PubMed

    Venegas, Patricia B; Novak, Jessica M; Oscar, Castro A; Sánchez, Félix L; Gutiérrez, Inés G; Rivera, Julio M; Salas, Jorge P; Montero, Jenny F; Grody, Wayne W

    2003-04-01

    Using polymerase chain reaction amplification of DNA in dried blood spots and a nonisotopic reverse dot blot hybridization method, we performed molecular genetic analysis for 6 and for 16 of the most common mutations of the cystic fibrosis transmembrane conductance regulator gene (CFTR) in 24 unrelated Costa Rican individuals with cystic fibrosis (CF). While many countries and ethnic groups have been surveyed for CF mutations since the cloning of CFTR, Costa Rica has not heretofore been studied. Moreover, Costa Rica represents an especially intriguing population because of its mixed European-African-Amerindian origins and the existence of a detailed historical record of the founding Spanish families. Thus, such a study may reveal not only the population frequencies of various mutant alleles in this country, but also something about their geographic migrations and ethnic founder effects. The most common CF mutation in Caucasians, deltaF508, was found in only 11 (23%) of the CF chromosomes studied, while the G542X mutation, relatively rare in the general population but more common in southern Europe, was observed in 12 (25%). None of the other mutations tested was found in any of the subjects. We failed to detect the second mutant allele in 17 subjects and could not detect either allele in 4 subjects. The high prevalence of the G542X mutation in our cohort, which exceeds that of both the general Caucasian population and the American Hispanic population, reflects the strong genetic influence of the original Spanish founding families of Costa Rica. These results highlight important differences in Costa Rican CF genotypes as compared both to other North American and European populations and to American Hispanics, raising important implications about isolated founder effects and strategies for population screening in that country.

  5. Basic and clinical aspects of malignant melanoma

    SciTech Connect

    Nathanson, L. )

    1987-01-01

    This book contains the following 10 chapters: The role of oncogenes in the pathogenesis of malignant melanoma; Laminin and fibronectin modulate the metastatic activity of melanoma cells; Structure, function and biosynthesis of ganglioside antigens associated with human tumors derived from the neuroectoderm; Epidemiology of ocular melanoma; Malignant melanoma: Prognostic factors; Endocrine influences on the natural history of human malignant melanoma; Psychosocial factors associated with prognostic indicators, progression, psychophysiology, and tumor-host response in cutaneous malignant melanoma; Central nervous system metastases in malignant melanoma; Interferon trials in the management of malignant melanoma and other neoplasms: an overview; and The treatment of malignant melanoma by fast neutrons.

  6. Controlling distant metastasis and surgical treatment are crucial for improving clinical outcome in uncommon head and neck malignancies, such as non-squamous cell carcinoma

    PubMed Central

    SHIIBA, MASASHI; UNOZAWA, MOTOHARU; HIGO, MORIHIRO; KOUZU, YUKINAO; KASAMATSU, ATSUSHI; SAKAMOTO, YOSUKE; OGAWARA, KATSUNORI; UZAWA, KATSUHIRO; TAKIGUCHI, YUICHI; TANZAWA, HIDEKI

    2014-01-01

    The objective of this study was to elucidate the clinical characteristics of uncommon head and neck malignancies, such as non-squamous cell carcinoma (SCC), in order to improve patient outcomes. A total of 463 head and neck malignancies were retrospectively analyzed, with 43 cases (9.3%) diagnosed as non-SCC. The overall survival rate of patients with adenoid cystic carcinoma was significantly worse compared to that of patients with SCC. The 5-year survival rates were <50% for patients with malignant melanoma, adenocarcinoma, small-cell carcinoma and sarcomas. Distant metastasis to the lung was frequently observed in cases with a poor outcome. Non-SCC malignancies treated without surgery were associated with a worse outcome. Some non-SCC patients had a poor prognosis and distant metastasis was associated with an unsatisfactory outcome. Timely treatment and control of distant metastasis are essential and surgical treatment should be prioritized in non-SCC cases to improve patient outcomes. PMID:24940505

  7. The giant panda gut microbiome.

    PubMed

    Wei, Fuwen; Wang, Xiao; Wu, Qi

    2015-08-01

    Giant pandas (Ailuropoda melanoleuca) are bamboo specialists that evolved from carnivores. Their gut microbiota probably aids in the digestion of cellulose and this is considered an example of gut microbiota adaptation to a bamboo diet. However, this issue remains unresolved and further functional and compositional studies are needed.

  8. Giant Serpentine Aneurysms: Multidisciplinary Management

    PubMed Central

    Anshun, W.; Feng, L.; Daming, W.

    2000-01-01

    Summary Sixty-five cases of intracranial giant serpentine aneurysms (GSΛs), including 61 cases reported in the literature and four additional cases presented in this study were reviewed. The clinical presentation, possible causes, natural history, and especially management of GSAs are discussed with emphasis on the need for aggressive intervention and multidisciplinary management. PMID:20667180

  9. The giant panda gut microbiome.

    PubMed

    Wei, Fuwen; Wang, Xiao; Wu, Qi

    2015-08-01

    Giant pandas (Ailuropoda melanoleuca) are bamboo specialists that evolved from carnivores. Their gut microbiota probably aids in the digestion of cellulose and this is considered an example of gut microbiota adaptation to a bamboo diet. However, this issue remains unresolved and further functional and compositional studies are needed. PMID:26143242

  10. [Mature cystic teratoma of the ovary with a small ganglioneuroma].

    PubMed

    Marucci, G; Collina, G

    2006-02-01

    A case of ganglioneuroma arising within a cystic mature teratoma of the ovary in a 34-year-old woman is reported. Patient underwent right adnexectomy. The ovary was completely replaced by a bilocular cystic lesion, measuring 8 cm in diameter and filled with adipose tissue and pilosebaceous material. Microscopically the cyst was composed by a mature cystic teratoma containing skin with dermal appendages, fatty tissue and bronchial epithelium. Furthermore a nodule (0.5 cm in size) composed of mature ganglion cells, axons and Schwann cells, was identified. Ganglion cells were positive for NSE and synaptophysin, while Schwann cells stained positively with S100 protein and GFAP. To the best of our knowledgment this is the first reported cases of ganglioneuroma arisen within a cystic mature teratoma of the ovary.

  11. Hydrator Therapies for Chronic Bronchitis. Lessons from Cystic Fibrosis.

    PubMed

    Bennett, William D; Henderson, Ashley G; Donaldson, Scott H

    2016-04-01

    Patients with the chronic bronchitis form of chronic obstructive pulmonary disease and cystic fibrosis share similar clinical features, including mucus obstruction of airways and the development of chronic/recurrent airways infections that often manifest as disease exacerbations. There is growing evidence that these diseases may have parallels in disease pathogenesis as well, including cystic fibrosis transmembrane conductance regulator dysfunction, mucus dehydration, and defective mucociliary clearance. As progress is made in the development of therapies that target the basic defects that lead to cystic fibrosis lung disease, it is possible that similar approaches could also benefit patients with chronic bronchitis. A deeper understanding of how tobacco smoke and other triggers of chronic bronchitis actually lead to disease, and exploration of the concept that therapies that restore cystic fibrosis transmembrane conductance regulator function, mucus hydration, and/or mucociliary clearance may benefit patients with chronic bronchitis, hold the prospect of significant progress in treating this prevalent disease. PMID:27115955

  12. [A method for the combined treatment of chronic cystic sinusitis].

    PubMed

    Svatko, L G; Krasnozhen, V N; Pokrovskaia, E M

    2008-01-01

    A pathogenetically substantiated method is proposed for the combined treatment of chronic cystic sinusitis that includes sparing surgical intervention and postoperative treatment with ximedone, a regenerator drug with immunotropic activity. PMID:19156109

  13. Florid Cystic Endosalpingiosis (Müllerianosis) in Pregnancy

    PubMed Central

    Montero-Balaguer, Beatriz; Desantes-Real, Domingo; Perales-Marín, Alfredo

    2016-01-01

    Cystic endosalpingiosis refers to the existence of heterotopic cystic müllerian tissue resembling structures of the fallopian tubes. We report a case of florid cystic endosalpingiosis discovered in a pregnant woman during a scheduled cesarean section and review the current knowledge of this disease. A 30-year-old woman with a twin pregnancy attended the hospital day unit at term. The first twin was in a breech presentation and a cesarean section was scheduled. During the procedure the uterine fundus and part of the body were seen completely seeded with multitude of cyst-like structures resembling hydatids of Morgagni. The immunohistochemistry analysis showed a positive expression for PAX8 (Box-8), CK7, and estrogen and progesterone receptors. The lesions did not disappear after pregnancy. Cystic endosalpingiosis should be always borne in mind, even in pregnancy, when it comes to making the differential diagnosis of a pelvic or systemic multicystic mass. PMID:27668111

  14. Florid Cystic Endosalpingiosis (Müllerianosis) in Pregnancy.

    PubMed

    Morales-Roselló, José; Pamplona-Bueno, Loida; Montero-Balaguer, Beatriz; Desantes-Real, Domingo; Perales-Marín, Alfredo

    2016-01-01

    Cystic endosalpingiosis refers to the existence of heterotopic cystic müllerian tissue resembling structures of the fallopian tubes. We report a case of florid cystic endosalpingiosis discovered in a pregnant woman during a scheduled cesarean section and review the current knowledge of this disease. A 30-year-old woman with a twin pregnancy attended the hospital day unit at term. The first twin was in a breech presentation and a cesarean section was scheduled. During the procedure the uterine fundus and part of the body were seen completely seeded with multitude of cyst-like structures resembling hydatids of Morgagni. The immunohistochemistry analysis showed a positive expression for PAX8 (Box-8), CK7, and estrogen and progesterone receptors. The lesions did not disappear after pregnancy. Cystic endosalpingiosis should be always borne in mind, even in pregnancy, when it comes to making the differential diagnosis of a pelvic or systemic multicystic mass. PMID:27668111

  15. Clinical Scenarios in Chronic Kidney Disease: Cystic Renal Diseases.

    PubMed

    Meola, Mario; Samoni, Sara; Petrucci, Ilaria

    2016-01-01

    Cysts are frequently found in chronic kidney disease (CKD) and they have a different prognostic significance depending on the clinical context. Simple solitary parenchymal cysts and peripelvic cysts are very common and they have no clinical significance. At US, simple cyst appears as a round anechoic pouch with regular and thin profiles. On the other hand, hereditary polycystic disease is a frequent cause of CKD in children and adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are the best known cystic hereditary diseases. ADPKD and ARPKD show a diffused cystic degeneration with cysts of different diameters derived from tubular epithelium. Medullary cystic disease may be associated with tubular defects, acidosis and lithiasis and can lead to CKD. Acquired cystic kidney disease, finally, is secondary to progressive structural end-stage kidney remodelling and may be associated with renal cell carcinoma. PMID:27169740

  16. Florid Cystic Endosalpingiosis (Müllerianosis) in Pregnancy

    PubMed Central

    Montero-Balaguer, Beatriz; Desantes-Real, Domingo; Perales-Marín, Alfredo

    2016-01-01

    Cystic endosalpingiosis refers to the existence of heterotopic cystic müllerian tissue resembling structures of the fallopian tubes. We report a case of florid cystic endosalpingiosis discovered in a pregnant woman during a scheduled cesarean section and review the current knowledge of this disease. A 30-year-old woman with a twin pregnancy attended the hospital day unit at term. The first twin was in a breech presentation and a cesarean section was scheduled. During the procedure the uterine fundus and part of the body were seen completely seeded with multitude of cyst-like structures resembling hydatids of Morgagni. The immunohistochemistry analysis showed a positive expression for PAX8 (Box-8), CK7, and estrogen and progesterone receptors. The lesions did not disappear after pregnancy. Cystic endosalpingiosis should be always borne in mind, even in pregnancy, when it comes to making the differential diagnosis of a pelvic or systemic multicystic mass.

  17. Florid Cystic Endosalpingiosis (Müllerianosis) in Pregnancy.

    PubMed

    Morales-Roselló, José; Pamplona-Bueno, Loida; Montero-Balaguer, Beatriz; Desantes-Real, Domingo; Perales-Marín, Alfredo

    2016-01-01

    Cystic endosalpingiosis refers to the existence of heterotopic cystic müllerian tissue resembling structures of the fallopian tubes. We report a case of florid cystic endosalpingiosis discovered in a pregnant woman during a scheduled cesarean section and review the current knowledge of this disease. A 30-year-old woman with a twin pregnancy attended the hospital day unit at term. The first twin was in a breech presentation and a cesarean section was scheduled. During the procedure the uterine fundus and part of the body were seen completely seeded with multitude of cyst-like structures resembling hydatids of Morgagni. The immunohistochemistry analysis showed a positive expression for PAX8 (Box-8), CK7, and estrogen and progesterone receptors. The lesions did not disappear after pregnancy. Cystic endosalpingiosis should be always borne in mind, even in pregnancy, when it comes to making the differential diagnosis of a pelvic or systemic multicystic mass.

  18. What Are the Signs and Symptoms of Cystic Fibrosis?

    MedlinePlus

    ... from the NHLBI on Twitter. What Are the Signs and Symptoms of Cystic Fibrosis? The signs and ... mucus that contains blood and bacteria. Respiratory System Signs and Symptoms People who have CF have thick, ...

  19. Benign Cystic Peritoneal Mesothelioma Revealed by Small Bowel Obstruction.

    PubMed

    Bray Madoué, Kaimba; Boniface, Moifo; Annick Laure, Edzimbi; Pierre, Herve

    2016-01-01

    Benign cystic peritoneal mesothelioma is a rare tumor which frequently occurs in women of reproductive age. Abdominal pain associated with pelvic or abdominal mass is the common clinical presentation. We report the case of a 22-year-old woman with a pathological proved benign cystic mesothelioma of the peritoneum revealed by a small bowel obstruction and a painful left-sided pelvic mass with signs of psoitis. Contrast enhanced abdominal CT-scan demonstrated a large pelvic cystic mass with mass effect on rectosigmoid and pelvic organs. The patient underwent surgical removal of the tumor. Pathological examination revealed the diagnosis of benign cystic mesothelioma of the peritoneum. The outcome was excellent with a 12-month recoil.

  20. Benign Cystic Peritoneal Mesothelioma Revealed by Small Bowel Obstruction

    PubMed Central

    Bray Madoué, Kaimba; Boniface, Moifo; Annick Laure, Edzimbi; Pierre, Herve

    2016-01-01

    Benign cystic peritoneal mesothelioma is a rare tumor which frequently occurs in women of reproductive age. Abdominal pain associated with pelvic or abdominal mass is the common clinical presentation. We report the case of a 22-year-old woman with a pathological proved benign cystic mesothelioma of the peritoneum revealed by a small bowel obstruction and a painful left-sided pelvic mass with signs of psoitis. Contrast enhanced abdominal CT-scan demonstrated a large pelvic cystic mass with mass effect on rectosigmoid and pelvic organs. The patient underwent surgical removal of the tumor. Pathological examination revealed the diagnosis of benign cystic mesothelioma of the peritoneum. The outcome was excellent with a 12-month recoil. PMID:27066288

  1. [Retroperitoneal cystic lymphangioma. Preoperative diagnosis. Apropos of a case].

    PubMed

    Guérin, E; Babin, C; Moulle, P; Barret, F

    1987-11-01

    Retroperitoneal cystic lymphangioma is an uncommon tumor. One case is reported, with surgical confirmation. Authors point out the diagnostic value of ultrasonography and computed tomography in the pre-operative diagnosis.

  2. CT findings in complications of acquired renal cystic disease.

    PubMed

    Soffer, O; Miller, L R; Lichtman, J B

    1987-01-01

    A 42-year-old man with end-stage renal disease developed acquired renal cystic disease. The left kidney underwent tumorous degeneration necessitating nephrectomy. Eight months later acute hemorrhagic renal cyst rupture culminated in right nephrectomy.

  3. Cystic fibrosis-related diabetes: a distinct condition.

    PubMed

    Cano Megías, Marta; González Albarrán, Olga

    2015-01-01

    Cystic fibrosis is the most common fatal inherited autosomal recessive disease in Caucasians, affecting approximately one out of every 2,000 births. Survival of patients with cystic fibrosis has significantly improved due to advances in respiratory and nutritional care, and their current average life expectancy is 30-40 years. Development of cystic fibrosis-related diabetes is a comorbidity that increases with age and may reach a prevalence up to 50% in adults. Its development is associated to impaired lung function and nutritional status, and early diagnosis and treatment are therefore essential to improve quality of life and performance status. Insulin therapy for diabetes and other early carbohydrate metabolism disorders may improve lung function and nutritional status of patients with cystic fibrosis. PMID:25151429

  4. Giant Endometrial Polyp in a Postmenopausal Woman without Hormone/Drug Use and Vaginal Bleeding

    PubMed Central

    Ünal, Betül; Doğan, Selen; Karaveli, Fatma Şeyda; Şimşek, Tayup; Erdoğan, Gülgün; Candaner, Işıl

    2014-01-01

    The objective of this study is to determine and discuss the causes of a giant endometrial polyp in a postmenopausal woman without hormone/drug use and to submit interesting clinical presentation. Here we report a seventy-year-old female patient who was admitted to our hospital with lower back pain. There were no other complaints from her. Physical examination was normal. For further examination, computed tomography was performed and a heterogeneous mass, with a diameter of 10 × 9 centimeters, was detected in the uterine cavity. Hysterectomy because of suspected endometrial cancer was performed. Histopathological examination showed us a giant endometrial polyp with edematous and focal fibrotic stroma, large thick walled blood vessels between normal sized and cystically dilated endometrial glands. To the best of our knowledge, this is the first report of a giant endometrial polyp which is unrelated to use of drugs such as tamoxifen and raloxifene; however, based on the history of the patient it may be associated with long-term consumption of thyme, which is a kind of phytoestrogen. PMID:25093134

  5. Detection of C-KIT (CD117) molecule in benign and malignant salivary gland tumours.

    PubMed

    Andreadis, Dimitrios; Epivatianos, Apostolos; Poulopoulos, Athanasios; Nomikos, Alexandros; Papazoglou, Georgios; Antoniades, Demetrios; Barbatis, Calypso

    2006-01-01

    C-KIT (CD117), a tyrosine kinase receptor, is involved in the growth and development of normal tissues and some types of neoplasms. In the present study we analysed the expression of this molecule in salivary gland tumours. Archival formalin-fixed, paraffin-embedded sections of 40 benign and 57 malignant salivary gland tumours were retrieved and retrospectively studied immunohistochemically using a polyclonal C-KIT antibody in an Envision/HRP technique. In addition five samples of chronic submandibular sialadenitis, five normal minor salivary glands and parotid or submandibular gland tissue adjacent to benign tumour were also studied. C-KIT expression was observed in cases of adenoid cystic, acinic cell polymorphous low grade, epithelial-myoepithelial, carcinosarcoma and basal cell adenocarcinomas, as in luminal cells of pleomorphic adenomas, in serous acinar and only in intercalated and a small number of striated ductal cells of inflammatory salivary gland tissue, whereas normal salivary lobules were generally negative except a weak positivity of intercalated cells. Contrary to other reports, this study suggests that, C-KIT protein does not appear to be an exclusively specific marker for benign or malignant salivary gland neoplasms, but may be useful in differential diagnosis of adenoid cystic carcinoma from polymorphous low grade adenocarcinoma. Furthermore its expression in serous acinar cells in sialadenitis and intercalated ductal cells in normal and inflammatory lesions may indicate a possible participation in pathogenesis of both neoplastic and non-neoplastic salivary gland diseases.

  6. beta2 adrenoceptor gene polymorphisms in cystic fibrosis lung disease.

    PubMed

    Büscher, Rainer; Eilmes, Katrin Jennifer; Grasemann, Hartmut; Torres, Brian; Knauer, Nicola; Sroka, Karin; Insel, Paul A; Ratjen, Felix

    2002-07-01

    The cystic fibrosis membrane conductance regulator can be activated through beta2-adrenoceptor (beta2AR) stimulation. We tested the hypothesis that coding sequence polymorphisms in the beta2AR gene contribute to the disease state in patients with cystic fibrosis. The Arg16Gly, Gln27Glu, and Thr164Ile beta2AR polymorphisms were studied by specific polymerase chain reaction and restriction fragment length polymorphism analysis in 126 cystic fibrosis patients. Forced expiratory volume in 1 s was significantly (P < 0.05) reduced in cystic fibrosis patients carrying the Gly16 allele in either homozygous or heterozygous form (Gly16Gly + Arg16Gly) compared to patients homozygous for the Arg16 allele (60.3 +/- 3.5% versus 75.7 +/- 4.9% predicted). Similarly, forced vital capacity and flows at lower lung volumes were significantly (P < 0.05 and P < 0.01) lower in cystic fibrosis patients carrying the Gly16 allele. In addition, the Gly16 allele was associated with a greater 5 year decline in pulmonary function (P < 0.01). Bronchodilator responses to albuterol were not significantly different between the groups. The Thr164Ile variant was found in four patients; these patients had markedly reduced pulmonary function. Isoproterenol-stimulated cyclic AMP formation was significantly blunted in cystic fibrosis patients carrying either the Gly16 allele or Thr164Ile genotype compared to cystic fibrosis patients homozygous for the respective Arg16 alleles. These data provide the first evidence suggesting that polymorphisms of the beta2AR gene contribute to clinical severity and disease progression in cystic fibrosis.

  7. Pulmonar collision tumor: metastatic adenoid cystic carcinoma and lung adenocarcinoma.

    PubMed

    Blanco, M; García-Fontán, E; Ríos, J; Rivo, J E; Fernández-Martín, R; Cañizares, M A

    2012-01-01

    We report an extraordinary case of collision tumor consisting of a lung adenocarcinoma and a metastatic adenoid cystic carcinoma in a 56 year-old man. He was diagnosed with a pulmonary nodule 11 years after treatment of an adenoid cystic carcinoma of the right maxillary sinus. A non-small cell carcinoma was observed when a transbronchial biopsy was performed. The other component of the nodule was only diagnosed with pathological examination of the resection specimen. PMID:21802893

  8. Laboratory confirmation of the diagnosis of cystic fibrosis.

    PubMed

    Tocci, P M; McKey, R M

    1976-11-01

    The recent commercial introduction of a method for detecting albumin in meconium makes screening for cystic fibrosis feasible for many hospitals. If the tests is adopted, confirmatory tests should be available. Quantitative analyses of sweat for sodium by flame photometry and for chloride by silver titration and ion-sleective electrodes are now used as confirmatory tests. We compare results of these confirmatory methods applied to presons with cystic fibrosis, respiratory disorders, or digestive disorders, and to control subjects. PMID:975543

  9. Pulmonar collision tumor: metastatic adenoid cystic carcinoma and lung adenocarcinoma.

    PubMed

    Blanco, M; García-Fontán, E; Ríos, J; Rivo, J E; Fernández-Martín, R; Cañizares, M A

    2012-01-01

    We report an extraordinary case of collision tumor consisting of a lung adenocarcinoma and a metastatic adenoid cystic carcinoma in a 56 year-old man. He was diagnosed with a pulmonary nodule 11 years after treatment of an adenoid cystic carcinoma of the right maxillary sinus. A non-small cell carcinoma was observed when a transbronchial biopsy was performed. The other component of the nodule was only diagnosed with pathological examination of the resection specimen.

  10. Cystic meningioma simulating arachnoid cyst: report of an unusual case.

    PubMed

    Docampo, Jorge; Jorge, Docampo; Gonzalez, Nadia; Nadia, Gonzalez; Vazquez, Claudio; Claudio, Vazquez; Morales, Carlos; Carlos, Morales; Gonzalez-Toledo, Eduardo; Eduardo, Gonzalez-Toledo

    2014-01-01

    The purpose of this paper is to show an unusual case of meningioma simulating arachnoid cyst on CT scan and MRI, diagnosed in a 63-year-old woman evaluated for headache and vision disorders. The meningioma shown is predominantly cystic with a small mural nodule enhancing after gadolinium and exhibiting diffusion restriction. Cystic portion of the tumor is hypodense on CT, and evidences fluid signal intensity on T1- and T2-weighted MR imaging.

  11. Chronic Low Back Pain due to Retroperitoneal Cystic Lymphangioma.

    PubMed

    Fattahi, Asieh Sadat; Maddah, Godratollah; Motamedolshariati, Mohammad; Ghiasi-Moghadam, Taghi

    2014-03-01

    Abdominal cystic lymphangioma is a rare benign neoplasm. Less than 1% of lymphangiomas is in the retroperitoneum. Lymphangioma is mostly asymptomatic. Chronic symptoms were reported in retroperitoneal type more than others. Acute symptoms due to complications like infection, cyst rupture or hemorrhage may occur. We report an 18-years-old girl with low back pain from 6 months ago with huge pelvic mass and diagnosis of retroperitoneal cystic lymphangioma.

  12. Gamma Knife Radiosurgery for the Treatment of Cystic Cerebral Metastases

    SciTech Connect

    Ebinu, Julius O.; Lwu, Shelly; Monsalves, Eric; Arayee, Mandana; Chung, Caroline; Laperriere, Normand J.; Kulkarni, Abhaya V.; Goetz, Pablo; Zadeh, Gelareh

    2013-03-01

    Purpose: To assess the role of Gamma Knife radiosurgery (GKRS) in the treatment of nonsurgical cystic brain metastasis, and to determine predictors of response to GKRS. Methods: We reviewed a prospectively maintained database of brain metastases patients treated at our institution between 2006 and 2010. All lesions with a cystic component were identified, and volumetric analysis was done to measure percentage of cystic volume on day of treatment and consecutive follow-up MRI scans. Clinical, radiologic, and dosimetry parameters were reviewed to establish the overall response of cystic metastases to GKRS as well as identify potential predictive factors of response. Results: A total of 111 lesions in 73 patients were analyzed; 57% of lesions received prior whole-brain radiation therapy (WBRT). Lung carcinoma was the primary cancer in 51% of patients, 10% breast, 10% colorectal, 4% melanoma, and 26% other. Fifty-seven percent of the patients were recursive partitioning analysis class 1, the remainder class 2. Mean target volume was 3.3 mL (range, 0.1-23 mL). Median prescription dose was 21 Gy (range, 15-24 Gy). Local control rates were 91%, 63%, and 37% at 6, 12, and 18 months, respectively. Local control was improved in lung primary and worse in patients with prior WBRT (univariate). Only lung primary predicted local control in multivariate analysis, whereas age and tumor volume did not. Lesions with a large cystic component did not show a poorer response compared with those with a small cystic component. Conclusions: This study supports the use of GKRS in the management of nonsurgical cystic metastases, despite a traditionally perceived poorer response. Our local control rates are comparable to a matched cohort of noncystic brain metastases, and therefore the presence of a large cystic component should not deter the use of GKRS. Predictors of response included tumor subtype. Prior WBRT decreased effectiveness of SRS for local control rates.

  13. Secondary aneurysmal bone cystic change of the chondroblastoma, mistaken for a primary aneurysmal bone cyst in the patella.

    PubMed

    Chung, Jin Wha; Lee, Hwa Sung

    2014-03-01

    A 29-year-old woman complained of a 3-month history of left knee pain without trauma history. X-ray showed a well-defined osteolytic lesion with a sclerotic margin in the patella and magnetic resonance imaging showed T1-low and T2-high signal intensity with different fluid level. Our impression was an aneurysmal bone cyst. At surgery, the lesion was a blood-filled cystic cavity, surrounded by a gray or brownish tissue. Hemorrhagic soft tissues with recognizable bone fragments were observed. Curettage and autogenous bone graft was done. Microscopically, sheets of tumor cells were intermingled with some areas of eosinophilic chondroid matrix. The tumor cells showed oval-shaped nuclei with moderate eosinophilic cytoplasm. Several multinucleated giant cells and blood filled cystic cavities were observed. The final diagnosis was a chondroblastoma with a secondary aneurysmal bone cyst. At the post-operative 1.5-year follow-up, grafted bones were well incorporated radiographically and there were no recurrent evidence or any other abnormal symptoms.

  14. Secondary aneurysmal bone cystic change of the chondroblastoma, mistaken for a primary aneurysmal bone cyst in the patella.

    PubMed

    Chung, Jin Wha; Lee, Hwa Sung

    2014-03-01

    A 29-year-old woman complained of a 3-month history of left knee pain without trauma history. X-ray showed a well-defined osteolytic lesion with a sclerotic margin in the patella and magnetic resonance imaging showed T1-low and T2-high signal intensity with different fluid level. Our impression was an aneurysmal bone cyst. At surgery, the lesion was a blood-filled cystic cavity, surrounded by a gray or brownish tissue. Hemorrhagic soft tissues with recognizable bone fragments were observed. Curettage and autogenous bone graft was done. Microscopically, sheets of tumor cells were intermingled with some areas of eosinophilic chondroid matrix. The tumor cells showed oval-shaped nuclei with moderate eosinophilic cytoplasm. Several multinucleated giant cells and blood filled cystic cavities were observed. The final diagnosis was a chondroblastoma with a secondary aneurysmal bone cyst. At the post-operative 1.5-year follow-up, grafted bones were well incorporated radiographically and there were no recurrent evidence or any other abnormal symptoms. PMID:24639947

  15. Potential role for inhibition of protein phosphatase 2A tumor suppressor in salivary gland malignancies.

    PubMed

    Routila, Johannes; Mäkelä, Juho-Antti; Luukkaa, Heikki; Leivo, Ilmo; Irjala, Heikki; Westermarck, Jukka; Mäkitie, Antti; Ventelä, Sami

    2016-01-01

    The aetiology and pathogenesis of salivary gland malignancies remain unknown. To reveal novel molecular factors behind the development of salivary gland cancer, we performed gene expression analyses from Smgb-Tag mouse salivary gland samples. The overall purpose was to apply these results for clinical use to find new approaches for both possible therapeutic targets and more accurate diagnostic tools. Smgb-Tag mouse strain, in which salivary neoplasms arise through a dysplastic phase in submandibular glands, was investigated using genome-wide microarray expression analysis, ingenuity pathway analysis, RT-PCR, and immunohistochemistry. Thirty-eight human salivary gland adenoid cystic carcinoma samples were investigated using immunohistochemistry for validation purposes. Our genome-wide study showed that Ppp2r1b, a PP2A subunit encoding tumor suppressor gene, is underexpressed in submandibular gland tumors of Smgb-Tag mice. mTOR signaling pathway was significantly enriched and mTOR linked PP2A subunit gene B55 gamma was significantly underexpressed in the analyses. Furthermore, parallel immunohistochemical analysis of three PP2A inhibitors demonstrated that two PP2A inhibitors, CIP2A and SET, are highly expressed in both dysplastic and adenocarcinomatous tumors of the Smgb-Tag mice. In addition, all 38 investigated human salivary adenoid cystic carcinoma samples stained positively for CIP2A and most for SET. Finally, p-S6 staining showed activation of mTOR pathway in human adenoid cystic carcinoma samples. Our results suggest that PP2A inhibition either via PP2A subunit underexpression or PP2A inhibitor overexpression play an important role in the formation of salivary gland malignancy, potentially due to mTOR signaling activation.

  16. Targeted therapies to improve CFTR function in cystic fibrosis.

    PubMed

    Brodlie, Malcolm; Haq, Iram J; Roberts, Katie; Elborn, J Stuart

    2015-01-01

    Cystic fibrosis is the most common genetically determined, life-limiting disorder in populations of European ancestry. The genetic basis of cystic fibrosis is well established to be mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for an apical membrane chloride channel principally expressed by epithelial cells. Conventional approaches to cystic fibrosis care involve a heavy daily burden of supportive treatments to combat lung infection, help clear airway secretions and maintain nutritional status. In 2012, a new era of precision medicine in cystic fibrosis therapeutics began with the licensing of a small molecule, ivacaftor, which successfully targets the underlying defect and improves CFTR function in a subgroup of patients in a genotype-specific manner. Here, we review the three main targeted approaches that have been adopted to improve CFTR function: potentiators, which recover the function of CFTR at the apical surface of epithelial cells that is disrupted in class III and IV genetic mutations; correctors, which improve intracellular processing of CFTR, increasing surface expression, in class II mutations; and production correctors or read-through agents, which promote transcription of CFTR in class I mutations. The further development of such approaches offers great promise for future therapeutic strategies in cystic fibrosis.

  17. Management of mucinous cystic neoplasms of the pancreas

    PubMed Central

    Testini, Mario; Gurrado, Angela; Lissidini, Germana; Venezia, Pietro; Greco, Luigi; Piccinni, Giuseppe

    2010-01-01

    The purpose of this study was to investigate the actual management of mucinous cystic neoplasm (MCN) of the pancreas. A systematic review was performed in December 2009 by consulting PubMed MEDLINE for publications and matching the key words “pancreatic mucinous cystic neoplasm”, “pancreatic mucinous cystic tumour”, “pancreatic mucinous cystic mass”, “pancreatic cyst”, and “pancreatic cystic neoplasm” to identify English language articles describing the diagnosis and treatment of the mucinous cystic neoplasm of the pancreas. In total, 16 322 references ranging from January 1969 to December 2009 were analysed and 77 articles were identified. No articles published before 1996 were selected because MCNs were not previously considered to be a completely autonomous disease. Definition, epidemiology, anatomopathological findings, clinical presentation, preoperative evaluation, treatment and prognosis were reviewed. MCNs are pancreatic mucin-producing cysts with a distinctive ovarian-type stroma localized in the body-tail of the gland and occurring in middle-aged females. The majority of MCNs are slow growing and asymptomatic. The prevalence of invasive carcinoma varies between 6% and 55%. Preoperative diagnosis depends on a combination of clinical features, tumor markers, computed tomography (CT), magnetic resonance imaging, endoscopic ultrasound with cyst fluid analysis, and positron emission tomography-CT. Surgery is indicated for all MCNs. PMID:21128317

  18. Increased oral bioavailability of ciprofloxacin in cystic fibrosis patients.

    PubMed Central

    Christensson, B A; Nilsson-Ehle, I; Ljungberg, B; Lindblad, A; Malmborg, A S; Hjelte, L; Strandvik, B

    1992-01-01

    The altered pharmacokinetic properties of, e.g., aminoglycosides in cystic fibrosis patients have to be considered when pulmonary exacerbations are treated. Since reported data on ciprofloxacin, a fluorinated quinolone, are conflicting, we compared intravenous and oral administration in cystic fibrosis patients when treating them for mild symptoms of pulmonary infection. All of the patients were colonized with Pseudomonas species. Ciprofloxacin was administered orally (15 mg/kg of body weight) or intravenously (6 mg/kg) twice a day for at least 10 days during separate treatment periods. Five healthy volunteers received single intravenous and oral doses. Pharmacokinetic evaluations were performed at first dose and at steady state. The results showed that cystic fibrosis patients have increased oral bioavailability of ciprofloxacin (80% in cystic fibrosis patients versus 57% in volunteers) and increased total clearance (688 ml/min in CF patients versus 528 ml/min in volunteers). Our data indicate that the pharmacokinetic properties of ciprofloxacin are altered in cystic fibrosis patients with mild symptoms of pulmonary exacerbations and that the changes most probably are due to cystic fibrosis per se or to the impact of chronic infection. PMID:1489195

  19. Cystic Odontoma in a Patient with Hodgkin's Lymphoma.

    PubMed

    Costa, Victor; Caris, Adriana Rocha; León, Jorge Esquiche; Ramos, Carolina Judica; Jardini, Vaneska; Kaminagakura, Estela

    2015-01-01

    Cystic odontoma is a rare entity, which is characterized by the association of a cyst with complex/compound odontoma. The aim of this study was to report the case of a 5-year-old male patient diagnosed previously with Hodgkin's lymphoma and treated successfully with chemotherapy and radiotherapy, who developed a mandibular odontogenic lesion. Physical examination revealed a swelling on the right side of the mandible. Radiographically, a well-defined radiolucent area surrounded by radiopaque material was observed. An incisional biopsy was performed and microscopic analysis showed a cystic lesion consisting of an atrophic epithelium comprising 2-3 cell layers and the absence of inflammation in the cystic capsule. The cyst was decompressed and the lesion was removed after 3 months of follow-up. Microscopic analysis of the surgical specimen showed a cystic hyperplastic epithelium surrounded by an intense chronic inflammatory cell infiltrate, which was in close contact with mineralized tissue resembling dentin and cementum. The final diagnosis was cystic odontoma. Since chemotherapy can affect the growth and development of infant teeth, a relationship between chemotherapy-associated adverse events and cystic odontoma is suggested in the present case.

  20. Cystic Odontoma in a Patient with Hodgkin's Lymphoma

    PubMed Central

    Costa, Victor; Caris, Adriana Rocha; León, Jorge Esquiche; Ramos, Carolina Judica; Jardini, Vaneska; Kaminagakura, Estela

    2015-01-01

    Cystic odontoma is a rare entity, which is characterized by the association of a cyst with complex/compound odontoma. The aim of this study was to report the case of a 5-year-old male patient diagnosed previously with Hodgkin's lymphoma and treated successfully with chemotherapy and radiotherapy, who developed a mandibular odontogenic lesion. Physical examination revealed a swelling on the right side of the mandible. Radiographically, a well-defined radiolucent area surrounded by radiopaque material was observed. An incisional biopsy was performed and microscopic analysis showed a cystic lesion consisting of an atrophic epithelium comprising 2-3 cell layers and the absence of inflammation in the cystic capsule. The cyst was decompressed and the lesion was removed after 3 months of follow-up. Microscopic analysis of the surgical specimen showed a cystic hyperplastic epithelium surrounded by an intense chronic inflammatory cell infiltrate, which was in close contact with mineralized tissue resembling dentin and cementum. The final diagnosis was cystic odontoma. Since chemotherapy can affect the growth and development of infant teeth, a relationship between chemotherapy-associated adverse events and cystic odontoma is suggested in the present case. PMID:26618008

  1. Molecular analyses of the candidate tumor suppressor gene, PLAGL1, in benign and malignant salivary gland tumors.

    PubMed

    Enlund, Fredrik; Persson, Fredrik; Stenman, Göran

    2004-12-01

    Deletions affecting the long arm of chromosome 6 are a characteristic feature of all major subtypes of malignant salivary gland tumors. Moreover, a subgroup of adenoid cystic carcinomas have t(6;9)(q23-25;p21-24) translocations with breakpoints located within the commonly deleted region. Here we have examined the possible involvement of the candidate tumor suppressor gene, PLAGL1, in these deletions and translocations. Northern blot and fluorescence in situ hybridization (FISH) analyses of a series of 27 salivary gland tumors revealed no significant changes in the gene expression or rearrangements of PLAGL1. FISH analysis also demonstrated that the 6q translocation breakpoint in adenoid cystic carcinomas with t(6;9) is proximal to the PLAGL1 locus. Collectively, these results indicate that PLAGL1 is not likely to be the major target gene of the 6q rearrangements in salivary gland tumors.

  2. Bioenergetic theory of prostate malignancy.

    PubMed

    Costello, L C; Franklin, R B

    1994-09-01

    Normal and benign prostate hyperplasia (BPH) prostate is characterized by the presence of extraordinarily high levels of citrate. Presumably, this results from the inability of the prostate epithelial cells to oxidize citrate due to a limiting mitochondrial (m-) aconitase. In contrast, prostate carcinoma (CA) is not characterized by high citrate levels. Malignant prostate epithelial cells apparently undergo a metabolic transformation from citrate-producing to citrate-oxidizing cells. A consequence of citrate production in normal and BPH cells is an inefficient and low level of ATP production. It is proposed that the process of malignancy necessitates an energy production that cannot be provided by citrate-producing cells. Consequently, the transformation of prostate epithelial cells to citrate-oxidizing cells which increases the energy production capability is essential to the process of malignancy and metastasis. The metabolic transformation likely occurs as a premalignant or early malignant stage. This bioenergetic theory of prostate malignancy, if correct, will provide new approaches to the diagnosis and treatment of CA. PMID:7520580

  3. [Thoracoscopy in malignant pleural effusions].

    PubMed

    Zhang, D; Chen, Y; Tu, C

    1996-03-01

    To assess the value of thoracoscopy in malignant pleural effusions, the procedure and results of thoracoscopy by using a fiberoptic bronchoscope and a rigid cold-light thoracoscope in 130 cases with malignant pleural effusion are reported. The overall diagnostic rate was 91.5% (119/130). The malignant pleural mesothelioma in 24 cases and metastatic cancers in 95 cases were histopathologically confirmed. Talcum powder, tetracycline and Corynebacterium parvum were separately sprayed through thoracoscope into pleural cavity in 69, 10 and 10 patients, and the success rates of complete and lasting pleurodesis were 87.0%, 5/10 and 8/10 respectively. Postoperative complications included transient fever and chest pain, local subcutaneous emphysema in 6 cases and tumor seeding at thoracoscopy site in 4 cases. It is concluded that thoracoscopy is simple, safe, reliable and of high practical value in the diagnosis of malignant pleural effusions and in assessment before exploratory thoracotomy, and that transendoscopical administration of drugs for pleurodesis is a very effective method for controlling malignant pleural effusions. The efficacy of the talc poudrage is better than tetracycline and Corynebacterium parvum. PMID:9206045

  4. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary

    PubMed Central

    Olivier, Kenneth N; Saiman, Lisa; Daley, Charles L; Herrmann, Jean-Louis; Nick, Jerry A; Noone, Peadar G; Bilton, Diana; Corris, Paul; Gibson, Ronald L; Hempstead, Sarah E; Koetz, Karsten; Sabadosa, Kathryn A; Sermet-Gaudelus, Isabelle; Smyth, Alan R; van Ingen, Jakko; Wallace, Richard J; Winthrop, Kevin L; Marshall, Bruce C; Haworth, Charles S

    2016-01-01

    Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease, such as cystic fibrosis (CF). Pulmonary disease (PD) caused by NTM has emerged as a major threat to the health of individuals with CF, but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened a panel of 19 experts to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM-PD in individuals with CF. PICO (population, intervention, comparison, outcome) methodology and systematic literature reviews were employed to inform draft recommendations, which were then modified to achieve consensus and subsequently circulated for public consultation within the USA and European CF communities. We have thus generated a series of pragmatic, evidence-based recommendations as an initial step in optimising management for this challenging condition. PMID:26678435

  5. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary.

    PubMed

    Floto, R Andres; Olivier, Kenneth N; Saiman, Lisa; Daley, Charles L; Herrmann, Jean-Louis; Nick, Jerry A; Noone, Peadar G; Bilton, Diana; Corris, Paul; Gibson, Ronald L; Hempstead, Sarah E; Koetz, Karsten; Sabadosa, Kathryn A; Sermet-Gaudelus, Isabelle; Smyth, Alan R; van Ingen, Jakko; Wallace, Richard J; Winthrop, Kevin L; Marshall, Bruce C; Haworth, Charles S

    2016-01-01

    Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease, such as cystic fibrosis (CF). Pulmonary disease (PD) caused by NTM has emerged as a major threat to the health of individuals with CF, but remains difficult to diagnose and problematic to treat. In response to this challenge, the US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) convened a panel of 19 experts to develop consensus recommendations for the screening, investigation, diagnosis and management of NTM-PD in individuals with CF. PICO (population, intervention, comparison, outcome) methodology and systematic literature reviews were employed to inform draft recommendations, which were then modified to achieve consensus and subsequently circulated for public consultation within the USA and European CF communities. We have thus generated a series of pragmatic, evidence-based recommendations as an initial step in optimising management for this challenging condition.

  6. Bone Windows for Distinguishing Malignant from Benign Primary Bone Tumors on FDG PET/CT

    PubMed Central

    Costelloe, Colleen M.; Chuang, Hubert H.; Chasen, Beth A.; Pan, Tinsu; Fox, Patricia S.; Bassett, Roland L.; Madewell, John E.

    2013-01-01

    Objective. The default window setting on PET/CT workstations is soft tissue. This study investigates whether bone windowing and hybrid FDG PET/CT can help differentiate between malignant and benign primary bone tumors. Materials and methods. A database review included 98 patients with malignant (n=64) or benign primary bone (n=34) tumors. The reference standard was biopsy for malignancies and biopsy or >1 year imaging follow-up of benign tumors. Three radiologists and/or nuclear medicine physicians blinded to diagnosis and other imaging viewed the lesions on CT with bone windows (CT-BW) without and then with PET (PET/CT-BW), and separate PET-only images for malignancy or benignity. Three weeks later the tumors were viewed on CT with soft tissue windows (CT-STW) without and then with PET (PET/CT-STW). Results. Mean sensitivity and specificity for identifying malignancies included: CT-BW: 96%, 90%; CT-STW: 90%, 90%; PET/CT-BW: 95%, 85%, PET/CT-STW: 95%, 86% and PET-only: 96%, 75%, respectively. CT-BW demonstrated higher specificity than PET-only and PET/CT-BW (p=0.0005 and p=0.0103, respectively) and trended toward higher sensitivity than CT-STW (p=0.0759). Malignant primary bone tumors were more avid than benign lesions overall (p<0.0001) but the avidity of benign aggressive lesions (giant cell tumors and Langerhans Cell Histiocytosis) trended higher than the malignancies (p=0.08). Conclusion. Bone windows provided high specificity for distinguishing between malignant and benign primary bone tumors and are recommended when viewing FDG PET/CT. PMID:23983816

  7. Gastrointestinal malignancy and the microbiome.

    PubMed

    Abreu, Maria T; Peek, Richard M

    2014-05-01

    Microbial species participate in the genesis of a substantial number of malignancies-in conservative estimates, at least 15% of all cancer cases are attributable to infectious agents. Little is known about the contribution of the gastrointestinal microbiome to the development of malignancies. Resident microbes can promote carcinogenesis by inducing inflammation, increasing cell proliferation, altering stem cell dynamics, and producing metabolites such as butyrate, which affect DNA integrity and immune regulation. Studies in human beings and rodent models of cancer have identified effector species and relationships among members of the microbial community in the stomach and colon that increase the risk for malignancy. Strategies to manipulate the microbiome, or the immune response to such bacteria, could be developed to prevent or treat certain gastrointestinal cancers. PMID:24406471

  8. Lenvatinib and Capecitabine in Patients With Advanced Malignancies

    ClinicalTrials.gov

    2016-09-23

    Advanced Cancer; Malignant Neoplasm of Breast; Malignant Neoplasms of Bone and Articular Cartilage; Malignant Neoplasms of Digestive Organs; Malignant Neoplasms of Eye Brain and Other Parts of Central Nervous System; Malignant Neoplasms of Female Genital Organs; Malignant Neoplasms of Ill-defined Secondary and Unspecified Sites; Malignant Neoplasms of Independent (Primary) Multiple Sites; Malignant Neoplasms of Lip Oral Cavity and Pharynx; Malignant Neoplasms of Male Genital Organs; Malignant Neoplasms of Mesothelial and Soft Tissue; Malignant Neoplasms of Respiratory and Intrathoracic Organs; Malignant Neoplasms of Thyroid and Other Endocrine Glands; Malignant Neoplasms of Urinary Tract

  9. Intensity-Modulated or Proton Radiation Therapy for Sinonasal Malignancy

    ClinicalTrials.gov

    2016-02-10

    Adenoid Cystic Carcinoma; Squamous Cell Carcinoma; Sinonasal Carcinoma; Sinonasal Undifferentiated Carcinoma; Mucoepidermoid Carcinoma; Schneiderian Carcinoma; Myoepithelial Carcinoma; Esthesioneuroblastoma; Melanoma

  10. A giant phyllodes tumor causing ulceration and severe breast disfigurement: case report and review of giant phyllodes

    PubMed Central

    Sbeih, Mohammed A.; Engdahl, Ryan; Landa, Marina; Ojutiku, Oreoluwa; Morrison, Norman; Depaz, Hector

    2015-01-01

    Phyllodes tumors are rare fibroepithelial tumors that account for <1% of the breast tumors in women. These tumors are often benign unilateral lesions of the female breast (70%). Less common are malignant phyllodes, which have the potential for hematogenous spread. Phyllodes tumors can be seen in all age groups, and the median age of presentation is 45 years. Surgery is the main form of treatment. Wide excisions with margins of 1cm are suggested. While smaller and moderate size phyllodes may typically be seen, gigantic ones are very rare. These may be seen in neglected tumors. By definition, a giant phyllodes tumor is one larger than 10 cm in diameter. We report a gigantic phyllodes tumor that grew over 7 years period causing significant ulceration and disfigurement and review features of these tumors and management. PMID:26703928

  11. A giant phyllodes tumor causing ulceration and severe breast disfigurement: case report and review of giant phyllodes.

    PubMed

    Sbeih, Mohammed A; Engdahl, Ryan; Landa, Marina; Ojutiku, Oreoluwa; Morrison, Norman; Depaz, Hector

    2015-01-01

    Phyllodes tumors are rare fibroepithelial tumors that account for <1% of the breast tumors in women. These tumors are often benign unilateral lesions of the female breast (70%). Less common are malignant phyllodes, which have the potential for hematogenous spread. Phyllodes tumors can be seen in all age groups, and the median age of presentation is 45 years. Surgery is the main form of treatment. Wide excisions with margins of 1cm are suggested. While smaller and moderate size phyllodes may typically be seen, gigantic ones are very rare. These may be seen in neglected tumors. By definition, a giant phyllodes tumor is one larger than 10 cm in diameter. We report a gigantic phyllodes tumor that grew over 7 years period causing significant ulceration and disfigurement and review features of these tumors and management. PMID:26703928

  12. Malignant hemangiopericytoma of pituitary fossa.

    PubMed

    Das, Prasenjit; Haresh, Kunhi P; Suri, Vaishali; Sharma, Mehar Chand; Sharma, Bhawani Shankar; Sarkar, Chitra

    2010-01-01

    Intracranial hemangiopericytomas are rare tumors with aggressive behavior. Other than the meninges, this lesion has rarely been reported in periventricular and sellar region. We report a case of malignant hemangiopericytoma in sellar region in a 47-year-old male who presented with history of sudden onset of bilateral visual disturbances. To best of our knowledge, this is the second case report of malignant hemangiopericytoma in this location. As this intracranial lesion shows aggressive behavior, in the form of recurrence or extracranial metastasis in comparison to its extracranial counterparts, diagnosis should be made cautiously. PMID:20090235

  13. Second Malignant Neoplasms Following Radiotherapy

    PubMed Central

    Kumar, Sanath

    2012-01-01

    More than half of all cancer patients receive radiotherapy as a part of their treatment. With the increasing number of long-term cancer survivors, there is a growing concern about the risk of radiation induced second malignant neoplasm [SMN]. This risk appears to be highest for survivors of childhood cancers. The exact mechanism and dose-response relationship for radiation induced malignancy is not well understood, however, there have been growing efforts to develop strategies for the prevention and mitigation of radiation induced cancers. This review article focuses on the incidence, etiology, and risk factors for SMN in various organs after radiotherapy. PMID:23249860

  14. Stenting in Malignant Biliary Obstruction.

    PubMed

    Almadi, Majid A; Barkun, Jeffrey S; Barkun, Alan N

    2015-10-01

    Decompression of the biliary system in patients with malignant biliary obstruction has been widely accepted and implemented as part of the care. Despite a wealth of literature, there remains a significant amount of uncertainty as to which approach would be most appropriate in different clinical settings. This review covers stenting of the biliary system in cases of resectable or palliative malignant biliary obstruction, potential candidates for biliary drainage, technical aspects of the procedure, as well as management of biliary stent dysfunction. Furthermore, periprocedural considerations including proper mapping of the location of obstruction and the use of antibiotics are addressed.

  15. Malignant Hypertension with Thrombotic Microangiopathy.

    PubMed

    Mitaka, Hayato; Yamada, Yuji; Hamada, Osamu; Kosaka, Shintaro; Fujiwara, Naoki; Miyakawa, Yoshitaka

    2016-01-01

    A 49-year-old man with malignant hypertension, acute kidney injury and mental deterioration was referred to our hospital. We initially observed microangiopathic hemolytic anemia, thrombocytopenia and kidney damage, indicating he had thrombotic microangiopathy (TMA). We considered TMA was caused by malignant hypertension and therefore did not start plasma therapy. The French TMA reference center reported that platelet counts and serum creatine levels have high values for predicting severe ADAMTS13 deficiency. The patient fully recovered from his illness after treatment with antihypertensive drugs and intermittent hemodialysis. This case might thus be useful to understand the proper differential diagnosis and treatment of TMA. PMID:27523008

  16. [Blood test for malignant lymphoma].

    PubMed

    Kobayashi, Tsutomu; Kuroda, Junya; Taniwaki, Masafumi

    2014-03-01

    Malignant lymphoma is a neoplastic disease that develops in the lymph system, which consists of various different subtypes. In addition, the differential diagnosis of malignant lymphoma includes infections, autoimmune diseases, allergic diseases, endocrine disorders, and so on. Therefore accurate diagnosis is very important to decide therapeutic strategy. Blood test is the most common examination in clinical practice and used extensively for evaluating etiology, pathology, disease state, efficacy of treatment and disease prognosis of lymphoma. We are required to understand the characteristics of blood examinations correctly and use them appropriately in daily medical practice. Here, we introduce some blood examinations used for treatment of lymphoma.

  17. Malignant tumours of the duodenum.

    PubMed

    Ryska, M; Hrabal, P

    2015-12-01

    No comprehensive knowledge of duodenal tumours exists in the current literature; individual types of malignant tumours may be described within malignancies of the small bowel, sets of case reports, or individual cases. Ampullary carcinomas are the exception and they are detailed in the current WHO histological classification of tumours of digestive system. Neither national nor international literature sources provide a comprehensive review of their therapy. The situation is similar when searching for surgical procedures. Resection procedures on the duodenum should thus be performed in specialized centres with sufficient experience with hepato-pancreato-biliary surgery. PMID:26767899

  18. Primary malignancy, secondary malignancy and semimalignancy of bone tumors.

    PubMed

    Uehlinger, E

    1976-01-01

    1. Bone tumors in contrast to tumors in soft tissue, show a wide variety of clinical behavior qualified by the expressions semimalignancy, low grade of malignancy, sarcomatous degeneration and primarily benign bone tumors and bone lesions. 2. The term semimalignancy is characterized by local invasive and destructive tumor growth with a tendency to recur locally but no hematogeneous spreading. Semimalignancy requires wide en-bloc resection of amputation. 3. The term low grade malignancy is used to describe a tumor of very slow growth and with very late metastasis. Low-grade malignancy requires resection with careful preservation of functional structures. 4. The term secondary malignancy means the sarcomatous degeneration of a primarily benign lesion or bone tumor. This transformation is enhanced by irradiation and probably by acceleration of the normal turnover of bone tissue. In Paget's disease sarcomatous degeneration is to be expected in 2 percent of cases and in fibrous dysplasia in 0.5 percent of cases. 5. Sarcomatous degeneration of bone infarcts is rare, but an increase is to be expected due to an increased frequency of bone infarcts caused by long-term treatment with cortisone. 6. Primary bone tumors and recurrences show the same structure and cytology. In a minority of cases the recurrences are less differentiated; in a very few cases the recurrences are more highly differentiated and have a better prognosis than the initial lesion. PMID:1070716

  19. Symplastic/pseudoanaplastic giant cell tumor of the bone

    PubMed Central

    Agaram, Narasimhan; Hwang, Sinchun; Lu, Chao; Wang, Lu; Healey, John; Hameed, Meera

    2016-01-01

    Objective Giant cell tumor of bone (GCTB) is a locally aggressive primary bone tumor. Its malignant counterpart is quite rare. Rarely, a conventional GCTB shows marked nuclear atypia, referred to as symplastic/pseudoanaplastic change, which can mimic sarcomatous transformation. Recently, somatic driver mutations of histone H3.3 exclusively in H3F3A have been described in GCTB. We report a series of 9 cases of GCTB with symplastic/pseudoanaplastic change, along with analysis of H3F3A variants. Materials and methods Nine cases of GCTB with symplastic change were identified. Clinico-radiological features, morphological features, and immunohistochemical stain for Ki-67 stain were reviewed. H3F3A variants were also analyzed using Sanger sequencing. Results Histologically, conventional giant cell tumor areas with scattered foci of markedly atypical cells were seen in all of the cases and all showed rare if any Ki-67 labeling. One patient had received denosumab treatment and another radiation therapy. Radiological features were characteristic of conventional GCTB. Mutation in H3F3A (p.Gly34Trp [G34W]) was found in 6 of the 7 cases. Clinical follow-up ranged from 6 to 208 months. Local recurrences were seen in 4 cases (44 %). Conclusions GCTB with symplastic/pseudoanaplastic change is an uncommon variant of conventional GCTB, which can mimic primary sarcoma or sarcomatous transformation. These tumors possess the same missense mutation in histone H3.3 as conventional GCTB. PMID:27020452

  20. Cystic Fibrosis Heterozygote Resistance to Cholera Toxin in the Cystic Fibrosis Mouse Model

    NASA Astrophysics Data System (ADS)

    Gabriel, Sherif E.; Brigman, Kristen N.; Koller, Beverly H.; Boucher, Richard C.; Stutts, M. Jackson

    1994-10-01

    The effect of the number of cystic fibrosis (CF) alleles on cholera toxin (CT)-induced intestinal secretion was examined in the CF mouse model. CF mice that expressed no CF transmembrane conductance regulator (CFTR) protein did not secrete fluid in response to CT. Heterozygotes expressed 50 percent of the normal amount of CFTR protein in the intestinal epithelium and secreted 50 percent of the normal fluid and chloride ion in response to CT. This correlation between CFTR protein and CT-induced chloride ion and fluid secretion suggests that CF heterozygotes might possess a selective advantage of resistance to cholera.