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Sample records for giant cystic malignant

  1. Giant Cystic Pheochromocytoma with Low Risk of Malignancy: A Case Report and Literature Review

    PubMed Central

    Maharaj, Ravi; Baijoo, Shanta; Greaves, Wesley; Harnanan, Dave

    2017-01-01

    Giant pheochromocytomas are rare silent entities that do not present with the classical symptoms commonly seen in catecholamine-secreting tumors. In many cases they are accidentally discovered. The algorithm to diagnose a pheochromocytoma consists of biochemical evaluation and imaging of a retroperitoneal mass. The female patient in this case report presented with a palpable abdominal mass and was cured with surgical resection. She suffered no recurrence or complications on follow-up. The left retroperitoneal mass measured 27 × 18 × 12 cm and weighed 3,315 grams. Biochemical, radiological, and pathological examinations confirmed the diagnosis of a pheochromocytoma. In this paper, we report on our experience treating this patient and provide a summary of all giant pheochromocytomas greater than 10 cm reported to date in English language medical journals. Our patient's giant cystic pheochromocytoma was the fourth heaviest and fifth largest maximal diameter identified using our literature search criteria. Additionally, this tumor had the largest maximal diameter of all histologically confirmed benign/low metastatic risk pheochromocytomas. Giant cystic pheochromocytomas are rare entities requiring clinical suspicion coupled with strategic diagnostic evaluation to confirm the diagnosis. PMID:28396811

  2. Giant malignant insulinoma

    PubMed Central

    Karavias, Dimitrios; Habeos, Ioannis; Maroulis, Ioannis; Kalogeropoulou, Christina; Tsamandas, Athanasios; Chaveles, Ioannis

    2015-01-01

    Insulinomas are the most common pancreatic neuroendocrine tumors. Most insulinomas are benign, small, intrapancreatic solid tumors and only large tumors have a tendency for malignancy. Most patients present with symptoms of hypoglycemia that are relieved with the administration of glucose. We herein present the case of a 75-year-old woman who presented with an acute hypoglycemic episode. Subsequent laboratory and radiological studies established the diagnosis of a 17-cm malignant insulinoma, with local invasion to the left kidney, lymph node metastasis, and hepatic metastases. Patient symptoms, diagnostic and imaging work-up and surgical management of both the primary and the metastatic disease are reviewed. PMID:25960993

  3. Mesenteric cystic lymphangioma mimicking malignancy.

    PubMed

    Hureibi, Khalid; Sunidar, Osama A

    2014-09-01

    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. 2014 BMJ Publishing Group Ltd.

  4. Giant ranula of the neck: differentiation from cystic hygroma.

    PubMed

    Macdonald, Andre J; Salzman, Karen L; Harnsberger, H Ric

    2003-04-01

    Occasionally, diving ranulas may attain large dimensions (giant ranula); gross involvement of the submandibular and parapharyngeal spaces makes differentiation from other cystic neck masses, particularly cystic hygroma, difficult. As diving ranula and cystic hygroma are managed with different surgical approaches, avoidance of this pitfall is critical. Our purpose was to review the imaging findings of giant ranula and compare them with those of cystic hygroma to define those features that are helpful in differentiating these different disease entities. We conducted a retrospective review of all cases of ranulas that had been surgically treated at our institution in a 15-year period. These were compared with cases of cystic hygroma that involved the same anatomic regions. Images were analyzed for anatomic location and morphology, with specific attention paid to those characteristics that might assist differentiation. Giant ranula was defined as any ranula that significantly involved the paraphyngeal space in addition to the submandibular space. Six patients with giant ranula and fifteen patients with cystic hygroma were reviewed. All giant ranulas retained tapered communication with the sublingual space and were homogeneous, thin-walled, anatomically defined, fluid-containing masses. One infected lesion enhanced, and another previously operated lesion demonstrated mild septation. Cystic hygroma commonly did not communicate with the sublingual space and had features of lobulation, septation and heterogeneity. Additional involvement of spaces not typically involved by ranula further assisted differentiation. Although giant ranulas may superficially resemble cystic hygroma, several imaging features allow confident differentiation of these two entities.

  5. A Case of Giant Uterine Lipoleiomyoma Simulating Malignancy.

    PubMed

    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.

  6. Giant Malignant Pheochromocytoma with Palpable Rib Metastases

    PubMed Central

    Gokce, Gokhan; Kilicli, Fatih; Elagoz, Sahande; Ayan, Semih; Gultekin, Emin Yener

    2014-01-01

    Pheochromocytoma is a rare and usually benign neuroendocrine neoplasm. Only 10% of all these tumors are malignant and there are no definitive histological or cytological criteria of malignancy. Single malignancy criteria are the presence of advanced locoregional disease or metastases. We report a case, with a giant retroperitoneal tumor having multiple metastases including palpable rib metastases, who was diagnosed as a malignant pheochromocytoma. The patient was treated with surgery. The literature was reviewed to evaluate tumor features and current diagnostic and therapeutic approaches for patients with metastatic or potentially malignant pheochromocytoma. PMID:25152826

  7. Giant cystic sacral schwannoma mimicking tarlov cyst: a case report.

    PubMed

    Attiah, Mark A; Syre, Peter P; Pierce, John; Belyaeva, Elizaveta; Welch, William C

    2016-05-01

    To present a rare case of a giant schwannoma of the sacrum mimicking a Tarlov cyst. A 58-year-old woman had a 1-year history of low back pain. MRI revealed a large cystic mass in the sacral canal with bony erosion. Radiological diagnosis of Tarlov cyst was made. The patient underwent surgical treatment for the lesion, which revealed a solid mass. Histopathological examination of the tumor confirmed the diagnosis of schwannoma. The postoperative course was uneventful and the patient has had significant improvement in her pain 1 month postoperatively. Giant cystic schwannoma of the sacrum is a very rare diagnosis overlooked by practitioners for more common cystic etiologies, but its treatment is significantly different. Care should be taken to include this diagnosis in a differential for a cystic sacral mass.

  8. [MALIGNANT TUMORS IN OVARIAN MATURE CYSTIC TERATOMAS INTRAOPERATIVE DIAGNOSTIC BASIS].

    PubMed

    Khachatryan, A

    2016-11-01

    Extremely rare ovarian primary tumors formed in a mature cystic teratomaare described in the literature. This research work studies the frequency of malignant mature cystic teratoma, as well as their clinical and morphological features and necessity of intraoperative histological examination of all teratomas. Cases histories of 56 patients, suffering from ovarian mature cystic teratomahave been studied in MC Shengavit in the period of 2003 - 2015. Among them 4 patients with the somatic malignancies were identified. Morphological methods, which are considered to be "gold standard" of tumor investigation, were used in staining the slides with hematoxylin - eosin. According to the literature the secondary malignant transformation rarely occurs and is typical in postmenopausal women, with a frequency of 0.17-3%. According to the results of our study, malignant tumors in mature cystic teratomas were observed in 4 (7,14%) from the total number of mature cystic teratomas (n=56). There was not revealed a correlation between the duration of the complaints, age of the patients, sizes of ovarian mature teratoma and malignization degree. Thus, the greatest difficulties of clinical diagnosis of malignant tumors in the ovarian mature cystic teratomas were in the early stage of the disease, because of a variety of clinical manifestations, not pathognomonic for malignization. All mentioned symptoms may be observed in the patients with usual mature cystic teratomas. Тhis cases confirm the necessity to take tissue samples from the other ovary for intraoperative histopathological evaluation in each case of mature cystic teratomas. It is necessary to examine a large number of tumor sites, to prevent errors in the assessment of the maturity degree of teratoma.

  9. A pulmonary mucinous cystic tumour of borderline malignancy.

    PubMed

    Bacha, D; Ayadi-Kaddour, A; Smati, B; Kilani, T; El Mezni, F

    2008-06-01

    We report a well-documented case of pulmonary mucinous cystic tumour of borderline malignancy involving the left lower lobe. The lesion was found incidentally by chest radiograph and CT scan with a provisional diagnosis of bronchioloalveolar carcinoma. The tumour was 4 cm in its greatest dimension, cystic and filled with gelatinous mucus. Microscopically, the neoplastic mucinous epithelium was composed of cuboidal cells with focally nuclear stratification and mild to moderate nuclear atypia. The patient has remained free from recurrence or metastases for 6 years. Pulmonary mucinous cystic tumour of borderline malignancy is a rare, recently described neoplasm, which spans a spectrum of tumours with malignant potential. The recent World Health Organization classification of lung tumours does not recognize this entity, which has a very good prognosis, and as such should be distinguished from classic pulmonary adenocarcinoma. Histological diagnosis can be difficult to distinguish from cystic bronchioloalveolar carcinoma or metastatic mucinous adenocarcinoma.

  10. 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.

  11. Malignant transformation of biliary adenofibroma: a rare biliary cystic tumor

    PubMed Central

    Zendejas-Mummert, Benjamin; Hartgers, Mindy L.; Venkatesh, Sudhakar K.; Smyrk, Thomas C.; Mahipal, Amit; Smoot, Rory L.

    2016-01-01

    Biliary adenofibromas (BAFs) are rare, benign biliary cystic tumors with potential for malignant transformation. Of the eleven prior cases of BAF reported in the literature, six showed evidence of malignant transformation. We describe the clinical, imaging and pathology features of two cases of malignant BAF and review the existing literature to raise awareness of this entity and provide additional tools for diagnosing this rare tumor Additionally, we identified a loss of function mutation in the cyclin-dependent kinase inhibitor 2A (CDKN2A) tumor suppressor gene in a malignant caudate lobe BAF, thereby providing potential insight into the molecular pathogenesis of BAF malignant transformation. Although additional cases and longer-term follow-up are needed, our cases suggest that recurrence or metastasis of malignant BAF is not common and that complete surgical resection can be curative. PMID:28078134

  12. Retroperitoneal Cystic Malignant Fibrous Histiocytoma Mimicking a Psoas Abscess

    PubMed Central

    Gupta, Madan Mohan; Bahri, Nandini; Watal, Pankaj; Rathod, Ketan; Thaker, Siddarth; Bhandari, Parthiv; Dhamecha, Ketul; Ajudia, Jasmin

    2015-01-01

    Primary neoplasms in the psoas muscle including schwannoma and soft tissue sarcoma with secondary cystic degeneration are rare entities. They are difficult to distinguish from psoas abscess purely based on radiological findings. Malignant fibrous histiocytoma (MFH) in the retroperitoneum is an uncommon entity in contrast to liposarcoma and leiomyosarcoma. Psoas abscess is a common infection in the retroperitoneum, especially in regions where tuberculosis is endemic. In the current case, the patient presented with gradually progressive lower abdominal pain and raised erythrocyte sedimentation rate (ESR), lymphocyte count and sputum positive for acid fast bacilli. There was a presence of previous history of skeletal tuberculosis. Imaging revealed well-defined multilocular cystic lesion involving the left psoas muscle which along with the clinical scenario suggested psoas abscess. However, post-operative biopsy showed the lesion to be a MFH with extensive cystic degeneration. To the best of our knowledge, cystic MFH mimicking an abscess has been previously reported only once in an oncology literature. PMID:26557275

  13. Giant cystic lymphangioma of the mesentery: varied clinical presentation of 3 cases.

    PubMed

    Rami, Mohamed; Mahmoudi, Abdelhalim; El Madi, Aziz; Khalid; Khattala; Afifi, Moulay Abderrahmane; Bouabdallah, Youssef

    2012-01-01

    Giant cystic lymphangioma is an uncommon mesenteric tumor which is usually reported in children. In this paper, we describe 3 cases, that was admitted in our department, two with abdominal distension, pain, and an abdominopelvic mass; the other present an acute abdomen. Preoperative studies including abdominal ultrasonography and computed tomography failed to determine the cause of the pain for the female patients. The laparotomy found a giant cystic tumor of the small bowel mesentery. The histological study showed a tumor that was diagnosed as a cystic lymphangioma. Based on those three cases a review of the literature is suggested.

  14. Removal of a malignant cystic brain tumor utilizing pyoktanin blue and fibrin glue: Technical note

    PubMed Central

    Hayashi, Nobuhide; Sasaki, Takahiro; Tomura, Nagatsuki; Okada, Hideo; Kuwata, Toshikazu

    2017-01-01

    Background: The leakage of cystic fluid during metastatic cystic brain tumor resection may cause tumor dissemination. When the cyst wall is thin, excision without removing the wall is often difficult. Methods: We were able to perform an en bloc resection of a cystic malignant brain tumor after aspirating the cystic fluid, injecting pyoktanin blue into the cyst to stain the cyst walls, and solidifying the empty cyst cavity by filling it with fibrin glue. Results: Pyoktanin blue readily stained the thin cystic walls and enabled visualization of mural damage. Solidification of the tumor made it easier to grasp and facilitated the dissection of tumor margins. Conclusions: This method has the potential to become a useful technique for the resection of malignant cystic brain tumors. PMID:28303204

  15. 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

  16. 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.

  17. Rare case of giant cystic adenomyoma mimicking a uterine malformation, diagnosed and treated by hysteroscopy.

    PubMed

    Pontrelli, Giovanni; Bounous, Valentina Elisabetta; Scarperi, Stefano; Minelli, Luca; Di Spiezio Sardo, Attilio; Florio, Pasquale

    2015-08-01

    We describe a case of giant cystic uterine adenomyoma that was diagnosed and treated by hysteroscopy. In a 27-year-old woman with menometrorrhagia, severe dysmenorrhea, and chronic pelvic pain, pelvic ultrasonography revealed an enlarged uterine cavity filled with homogeneous low echogenic fluid content. A large cornual hematometra of 8.0 cm in diameter in a bicornuate uterus was suspected, and this hypothesis was also supported by magnetic resonance imaging findings. On the contrary, hysteroscopy revealed a bilocular huge cystic lesion of the posterior uterine wall that was removed by means of monopolar loop resection. The operative finding and the histopathologic examination confirmed the diagnosis of cystic adenomyoma of the uterus. Hysteroscopy may represent a valid tool for diagnosis and minimally invasive treatment of cystic adenomyoma, including those of large volume. Its use is helpful in differential diagnosis between cystic adenomyoma and uterine malformations as a possible cause of pelvic pain.

  18. A case of mistaken identity: giant cystic pheochromocytoma.

    PubMed

    Antedomenico, Elena; Wascher, Robert A

    2005-01-01

    A 39-year-old Marshall Islands woman was referred for evaluation of an abdominal mass. Medical history was significant only for pulmonary tuberculosis and scrofula. The patient denied a personal or family history of pancreatic or endocrine disease. Physical examination revealed normal vital signs and a 12-cm mildly tender left upper quadrant mass. A computed tomography scan revealed an 11-cm cystic mass contiguous with the distal pancreas and closely associated with the hilum of the spleen and the left kidney. Based on these findings and the patient's history, a cystic neoplasm of the pancreas was suspected, and she was subsequently taken to the operating room for exploration. Intraoperatively, the patient became markedly hypertensive with manipulation of the mass, requiring intravenous nitroprusside. Histopathological evaluation confirmed the diagnosis of cystic pheochromocytoma (PCC). The patient's postoperative course was uncomplicated. Cystic PCCs may not present with the classic prodromal symptoms associated with solid PCCs. This case represents the complex and unsuspected presentation of an extremely rare functional cystic neoplasm. A high index of suspicion for cystic PCC is necessary when confronted with cystic lesions in the vicinity of the adrenal glands. Failure to recognize cystic PCC before resection may lead to uncontrollable hypertension in the operating room, with potentially serious consequences.

  19. Intracranial hypertension secondary to abdominal compartment syndrome in a girl with giant ovarian cystic mass.

    PubMed

    Zavras, Nick; Christianakis, E; Ereikat, K; Mpourikas, D; Velaoras, K; Alexandrou, J

    2012-04-01

    The abdominal compartment syndrome (ACS) is a potentially fatal entity that occurs as a result of an acute increase in intra-abdominal pressure (IAP). The authors report on a girl with a giant ovarian cystic mass, and clinical signs of ACS and intracranial hypertension (ΙΗ). The possible mechanism of IH secondary to ACS is discussed.

  20. Lymphoepithelial cyst of the pancreas mimicking malignant cystic tumor: report of a case.

    PubMed

    Ryu, Dong Hee; Sung, Ro Hyun; Kang, Min Ho; Choi, Jae Woon

    2015-08-01

    Lymphoepithelial cysts of the pancreas are a type of true cyst that can mimic pseudocysts and cystic neoplasms. They are very rare, non-malignant lesions that are unilocular or multilocular cystic lesions lined predominantly by mature squamous epithelium and surrounded by non-neoplastic lymphoid elements. We, herein, present a patient with a cystic pancreas tumor mimicking a malignant cystic neoplasm. The patient was admitted with upper abdominal discomfort. Computed tomography showed a 64×39 mm cystic mass in the pancreas tail. She underwent distal pancreatectomy and splenectomy. In the fluid analysis of the pancreas cystic mass, the CEA and CA19-9 were 618 ng/ml and 3.9 U/ml, respectively. The resected pancreas specimen showed a 6.5 cm-sized cyst the pancreas tail. The cyst was well circumscribed and multilocular. The final pathology report of the resected pancreas specimen noted that the cyst was multilocular, and the cyst lining was showing stratified squamous epithelium covering the lymphoid tissue (containing lymphoid follicles), which was consistent with a lymphoepithelial cyst. The patient recovered uneventfully from surgery and has been doing well for the past 3 months. A differential diagnosis of cystic pancreatic lesions is important. We suggest that lymphoepithelial cysts, although very rare, may be included in the differential diagnosis of cystic pancreatic tumors.

  1. Giant solid-cystic hypothalamic hamartoma. Case report.

    PubMed

    Dorfer, Christian; Kasprian, Gregor; Mühlebner, Angelika; Czech, Thomas

    2011-02-01

    Hypothalamic hamartomas are rare lesions for which different classification schemes have been proposed. The authors report on an exceptionally large solid-cystic hamartoma that led to hydrocephalus, precocious puberty, and intractable gelastic seizures. They discuss potential mechanisms of the development of hypothalamic hamartomas.

  2. 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.

  3. A Squamous Cell Carcinoma and a Malignant Fibrous Histiocytoma Developing in a Patient with a Mature Cystic Teratoma

    PubMed Central

    Tahaoglu, Ali Emre; Ege, Serhat; Bakir, Mehmet Sait; Saruhan, Gülbin; Aksin, Serif; Gul, Talip

    2017-01-01

    We present a very rare case of malignant transformation of a benign mature cystic teratoma. The pathology report revealed malignant transformation of both the epithelial and sarcomatous elements of a benign dermoid cyst. To the best of our knowledge, this appears to be the third case of a malignant fibrous histiocytoma and a squamous cell carcinoma developing from a mature cystic teratoma. Malignant transformation of a dermoid cyst is usually diagnosed postmenopausally, but our patient was premenopausal. The etiology and prognosis of malignant transformation of this benign condition remain unknown. PMID:28203171

  4. 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.

  5. 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

  6. 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.

  7. A case report: Giant cystic parathyroid adenoma presenting with parathyroid crisis after Vitamin D replacement

    PubMed Central

    2012-01-01

    Background Parathyroid adenoma with cystic degeneration is a rare cause of primary hyperparathyroidism. The clinical and biochemical presentation may mimic parathyroid carcinoma. Case presentation We report the case of a 55 year old lady, who had longstanding history of depression and acid peptic disease. Serum calcium eight months prior to presentation was slightly high, but she was never worked up. She was found to be Vitamin D deficient while being investigated for generalized body aches. A month after she was replaced with Vitamin D, she presented to us with parathyroid crisis. Her corrected serum calcium was 23.0 mg/dL. She had severe gastrointestinal symptoms and acute kidney injury. She had unexplained consistent hypokalemia until surgery. Neck ultrasound and CT scan revealed giant parathyroid cyst extending into the mediastinum. After initial medical management for parathyroid crisis, parathyroid cystic adenoma was surgically excised. Her serum calcium, intact parathyroid hormone, creatinine and potassium levels normalized after surgery. Conclusion This case of parathyroid crisis, with very high serum calcium and parathyroid hormone levels, is a rare presentation of parathyroid adenoma with cystic degeneration. This case also highlights that Vitamin D replacement may unmask subclinical hyperparathyroidism. Consistent hypokalemia until surgery merits research into its association with hypercalcemia. PMID:22840059

  8. Giant cystic Virchow-Robin spaces with adjacent white matter signal alteration.

    PubMed

    Wani, Nisar A; Mir, Farooq; Bhat, Irshad M; Gojwari, Tariq; Bhat, Salma

    2011-01-01

    Perivascular spaces surround the small arteries and veins as they enter into the brain parenchyma from the subarachnoid spaces. Also called as Virchow-Robin spaces, these are prominent in the basal ganglia and high convexity white matter of the elderly. Occasionally VR spaces may get massively enlarged and may mimic a cystic mass lesion. The typical CSF-like signal intensity of the cysts and location on MRI, in the absence of a neurological abnormality help in the diagnosis of the giant VR spaces and thus biopsy is avoided. Typically there is no significant adjacent brain abnormality; however FLAIR images may sometimes reveal perilesional white matter hyperintensity, which may be an indication of gliosis due to the mass effect of the lesion. Such a signal alteration should not deter one from making a diagnosis of giant Virchow-Robin spaces when the rest of the imaging findings are typical. We describe a case of a 50-year-old female with incidental giant Virchow-Robin spaces in the right hemispheric subcortical white matter with adjacent white matter hyperintense signal intensity on T2-weighted and FLAIR images.

  9. A novel distinguishing system for the diagnosis of malignant pancreatic cystic neoplasm.

    PubMed

    Shen, Xiaoyong; Lu, Di; Xu, Xiao; Wang, Jianguo; Wu, Jian; Yan, Sheng; Zheng, Shu-sen

    2013-11-01

    To explore a simple and reliable non-invasive distinguishing system for the pre-operative evaluation of malignancy in pancreatic cystic neoplasm (PCN). This study first enrolled an observation cohort of 102 consecutive PCN patients. Demographic information, results of laboratory examinations, and computed tomography (CT) presentations were recorded and analyzed to achieve a distinguishing model/system for malignancy. A group of 21 patients was then included to validate the model/system prospectively. Based on the 11 malignancy-related features identified by univariate analysis, a distinguishing model for malignancy in PCN was established by multivariate analysis: PCN malignant score=2.967 × elevated fasting blood glucose (FBG) (≥6.16 mmol/L) ± 4.496 × asymmetrically thickened wall (or mural nodules ≥ 4 mm) ± 1.679 × septum thickening (≥2 mm)-5.134. With the optimal cut-off value selected as -2.8 in reference to the Youden index, the proposed system for malignant PCN was established: septum thickening (>2 mm), asymmetrically thickened wall (or mural nodules>4 mm), or elevated FBG (>6.16 mmol/L, accompanying commonly known malignant signs), the presence of at least one of these 3 features indicated malignancy in PCN. The accuracy, sensitivity and specificity of this system were 81.4%, 95.8% and 76.9%, respectively. MRI was performed on 32 patients, making correct prediction of malignancy explicitly in only 68.8% (22/32). The subsequent prospective validation study showed that the proposed distinguishing system had a predictive accuracy of 85.7% (18/21). Moreover, a higher model score, or aggregation of the features in the proposed system, indicated a higher grade of malignancy (carcinoma) in PCN. Elevated FBG (>6.16 mmol/L), asymmetrically thickened wall (or mural nodules>4 mm) and septum thickening (>2 mm) are of great value in differentiating the malignancy in PCN. The developed distinguishing system is reliable in the diagnosis of malignant PCN

  10. 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

  11. Secondary malignant giant cell tumor of bone due to malignant transformation 40 years after surgery without radiation therapy, presenting as fever of unknown origin: a case report.

    PubMed

    Takesako, Hisataka; Osaka, Eiji; Yoshida, Yukihiro; Sugitani, Masahiko; Tokuhashi, Yasuaki

    2016-03-08

    Malignant transformation of giant cell tumors of bones, that is, secondary malignant giant cell tumor of bone, is rare. The most common symptoms are local pain and swelling. There are no prior reports of giant cell tumor of bone with fever of unknown origin at the onset. Here we present a case of a secondary malignant giant cell tumor of bone due to malignant transformation 40 years after surgery without radiation therapy, presenting as fever of unknown origin. A 75-year-old Asian man presented with a 3-week history of continuous pyrexia and left knee pain and swelling. He had been diagnosed at age 35 years with a giant cell tumor of bone of his left distal femur and underwent bone curettage and avascular fibula grafting at that time. Postoperative radiation therapy was not performed. He remained recurrence-free for 40 years after surgery. At age 75, histopathological findings suggested a secondary malignant giant cell tumor of bone. The tumor specimen expressed tumor necrosis factor-α. Neoplastic fever was suspected, and a naproxen test was conducted. His pyrexia showed immediate resolution. Surgery was performed under a diagnosis of a secondary malignant giant cell tumor of bone with neoplastic fever. His pyrexia and inflammatory activities diminished postoperatively. This is the first reported case, to the best of our knowledge, of the detection of a secondary malignant giant cell tumor of bone based on fever of unknown origin after long-term (40 years) follow-up. After curettage and bone grafting, giant cell tumor of bone may transform to malignancies within a few years or even decades after surgery. Therefore, meticulous follow-up is essential. The fever might be attributable to the tumor releasing inflammatory cytokines. Not only pain and swelling but also continuous pyrexia may suggest the diagnosis of a secondary malignant giant cell tumor of bone.

  12. Malignant transformation rate and p53, and p16 expression in teratomatous skin of ovarian mature cystic teratoma.

    PubMed

    Zhu, Hai-Li; Zou, Zhen-Ning; Lin, Pei-Xin; Li, Wen-Xia; Huang, Ye-En; Shi, Xiao-Xin; Shen, Hong

    2015-01-01

    To investigate the incidence of malignant transformation and P53 and P16 expression in teratomatous skin of ovarian mature cystic teratoma. Data on ovarian teratoma specimens in nearly 10 years were reviewed. P53 and P16 expression were detected by immunohistochemistry in 25 cases of teratomatous skin of ovarian mature cystic teratoma, 20 cases of squamous cell carcinoma and 2 cases of squamous cell carcinoma originated from teratomatous skin. Of 1913 cases of ovarian mature cystic teratoma in nearly 10 years, only two cases of squamous cell carcinoma were found in teratomatous skin, with malignant transformation rate of 0.1045%. P53 expression was detected in 2 cases squamous cell carcinoma originated from teratomatous skin and P16 overexpression in one. There were no expressions of P53 and P16 in 25 cases of teratomatous skin of ovarian mature cystic teratoma. Of 20 cases of squamous cell carcinoma P53 overexpression (positive rate of 55%) was detected in 11 cases, P16 overexpression (positive rate of 35%) in 7 cases. The positive rates of P53 and P16 expression in squamous cell carcinomas were significantly higher than that in the teratomatous skins (p< 0.001, p= 0.002). There was low risk of malignant transformation in teratomatous skin of ovarian mature cystic teratoma which can be explained by lower P53 and P16 expressionin teratomas than that in squamous cell carcinoma.

  13. [Giant cervical angioma caused by "malignant" arteriovenous fistulae].

    PubMed

    Palou, J; Mir y Mir, L

    1992-01-01

    A case of angioma by malignant AV fistula (described by F. Martorell in 1970) is reported. Such kind of fistulas have usually a lethal course. The case reported underwent a surgical correction, and after a following of 10 years, no major complications have been reported.

  14. 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.

  15. Denosumab-treated Giant Cell Tumor of Bone Exhibits Morphologic Overlap With Malignant Giant Cell Tumor of Bone.

    PubMed

    Wojcik, John; Rosenberg, Andrew E; Bredella, Miriam A; Choy, Edwin; Hornicek, Francis J; Nielsen, G Petur; Deshpande, Vikram

    2016-01-01

    Giant cell tumor (GCT) of bone is a locally aggressive benign neoplasm characterized by an abundance of osteoclastic giant cells that are induced by the neoplastic mononuclear cells; the latter express high levels of receptor activator of nuclear factor κ-B ligand (RANKL). Denosumab, a RANKL inhibitor, which is clinically used to treat GCT, leads to a marked alteration in the histologic appearance of the tumor with giant cell depletion and new bone deposition, leading to substantial histologic overlap with other primary tumors of bone. Most significantly, denosumab-treated GCT (tGCT) with abundant bone deposition may mimic de novo osteosarcoma, or GCT that has undergone malignant transformation. To histologically characterize tGCT, we identified 9 cases of GCT biopsied or resected after denosumab treatment. tGCT cases included 16 specimens from 9 patients including 6 female and 3 male individuals aged 16 to 47 (median 32) years. Duration of treatment varied from 2 to 55 months. We compared these tumors with malignant neoplasms arising in GCTs (n=9). The histology of tGCT was variable but appeared to relate to the length of therapy. All tGCTs showed marked giant cell depletion. Early lesions were highly cellular, and the combination of cellularity, atypia, and haphazard bone deposition caused the lesion to resemble high-grade osteosarcoma. Unlike de novo high-grade osteosarcoma or malignancies arising in GCT, however, tGCT showed less severe atypia, reduced mitotic activity, and lack of infiltrative growth pattern. Tumor in patients on prolonged therapy showed decreased cellularity and abundant new bone, deposited as broad, rounded cords or long, curvilinear arrays. The latter morphology was reminiscent of low-grade central osteosarcoma, but, unlike low-grade central osteosarcoma, tGCT was negative for MDM2 and again lacked an infiltrative growth pattern. Overall, tGCT may have a wide range of morphologic appearances. Because the treated tumors bear little

  16. Lymphoepithelial cysts of the pancreas. Can preoperative imaging distinguish this benign lesion from malignant or pre-malignant cystic pancreatic lesions?

    PubMed

    Kavuturu, Srinivas; Sarwani, Nabeel E; Ruggeiro, Fransesca M; Deshaies, Isabelle; Kimchi, Eric T; Kaifi, Jussuf T; Staveley-O'Carroll, Kevin F; Gusani, Niraj J

    2013-05-10

    Lymphoepithelial cysts of the pancreas are rare true benign cystic tumors of the pancreas of uncertain etiology. Cystic neoplasms of the pancreas present a significant diagnostic dilemma in differentiating benign from premalignant or malignant variants. Since the first description of lymphoepithelial cysts in 1985, 109 cases have been reported in the literature. We describe 6 cases of this rare tumor, the preoperative imaging results, and a review the literature. Five males and one female ranging in age from 47 to 76 years underwent resection for lymphoepithelial cysts. Five patients presented with abdominal pain related to the lesion and in one patient the lesion was discovered incidentally. Four patients had elevated serum CA 19-9 levels. Pre-operative imaging with a CT scan and MRI of the abdomen typically revealed a well defined hypodense mass with Hounsfield units (HU) in the range of 15 to 20. One patient had papillary projections into the lesion. The mean size was 3.3 cm (ranging from 1.8 cm to 4 cm). All lesions were exophytic off the pancreatic parenchyma (1 cyst was located in the head of the pancreas, 2 were in the body, and 3 were in the tail region). Pre-operative EUS-guided/CT-guided needle aspiration, when performed, was not diagnostic. All patients underwent resection (one pancreaticoduodenectomy, five left pancreatectomies) to remove these cystic neoplasms. Pathology revealed a cyst lined by non-dysplastic squamous cells surrounded by sheets of benign lymphocytes. No evidence of malignancy was found. Lymphoepithelial cysts of the pancreas are rare and are characteristically seen in men. While a hypodense mass (less than 20 HU) with papillary projections should be considered suspicious for lymphoepithelial cyst, a definitive diagnosis cannot be made solely based on preoperative imaging. EUS-guided biopsy coupled with biochemical/tumor marker studies are increasingly being used as a diagnostic tool to help differentiate between the various types of

  17. Malignant Melanoma in Association With a Thymic Nevus in a Patient With a Giant Congenital Nevus.

    PubMed

    Shvartser-Beryozkin, Yulia; Yakobson, Alexander; Benharroch, Daniel; Saute, Milton; Feinmesser, Meora

    2017-07-01

    Nevi and melanocytic proliferations are known to appear in multiple extracutaneous sites, including lymph nodes and meninges. We report a case of an anterior mediastinal mass in a patient with a giant congenital nevus and neurofibromatosis type I. Histologically, the tumor was found to be a malignant melanoma in the thymus arising in association with a nevus that involved most of the thymic tissue. There was no sign of cutaneous melanoma on skin examination. We suggest that the tumor originated from the benign nevus in the thymus, a rare extracutaneous location for nevi and malignant melanoma.

  18. Primary giant cell malignant fibrous histiocytoma-associated with renal calculus

    PubMed Central

    Altunkol, Adem; Savas, Murat; Ciftci, Halil; Gulum, Mehmet; Yagmur, Ismail; Bitiren, Muharrem

    2014-01-01

    Malignant fibrous histiocytomas (MFH) are the most commonly seen soft tissue sarcomas in adults. It is rarely seen in some visceral organs. Kidneys are the parenchymal organs in which MFHs are most frequently seen. More than 50 cases of primary renal MFH have been reported. Among these cases, only 1 was reported as primary giant cell subtype in association with urolithiasis. This case report is the second such case with the these characteristics. PMID:24678364

  19. Soft Tissue Giant Cell Tumour of Low Malignant Potential: A Rare Tumour at a Rare Site

    PubMed Central

    Bhat, Amoolya; V., Geethamani; C., Vijaya

    2013-01-01

    “Soft tissue giant cell tumour of low malignant potential” is considered as the soft tissue counterpart of osteoclastoma of the bone. It is a primary soft tissue tumour which is classified under the category of fibrohistiocytic tumours of intermediate malignancy.Seventy percent of the tumours involve the extremities and only about seven percent of them arise in head and neck region. They are composed of nodules of histiocytes in a vascular stroma, with multinucleated osteoclast-like giant cells positive for vimentin, smooth muscle actin (SMA), CD68 and Tarterate Resistant Acid Phosphatase (TRAP). We are presenting a case of a 75-year-old man who had a nodule on the ala of the nose. Histopathology showed a histiocytic lesion. Benign fibrous histiocytoma, plexiform fibrohistiocytic tumour, solitary reticulohistiocytoma and histioid leprosy were ruled out by using special stains and immunostains. Expression of smooth muscle actin and CD68 confirmed the diagnosis of a soft tissue giant cell tumour with a low malignant potential. PMID:24551690

  20. Giant intrapelvic malignant peripheral nerve sheath tumor mimicking disc herniation: A case report

    PubMed Central

    Wang, Peng; Chen, Cong; Xin, Xiaotang; Liu, Bo; Li, Wei; Yin, Dezhen; Mu, Weidong

    2016-01-01

    Giant intrapelvic malignant peripheral nerve sheath tumors arising in the sciatic nerve in the pelvic cavity are a rare occurrence and their symptomatology is usually misdiagnosed as intervertebral disc herniation. We herein report the case of a 46-year old woman presenting with pain, hypesthesia and weakness of the left lower extremity due to a giant intrapelvic malignant peripheral nerve sheath tumor of the sciatic nerve. Prior to being referred to our institution, the patient was misdiagnosed as a case of sciatica due to a lumbar disc herniation and underwent an operation unsuccessfully, as there was little symptomatic improvement 2 months after the surgery. A magnetic resonance imaging examination of the pelvic cavity revealed a tumor of the sciatic nerve. The mass was resected via the posterior approach and histopathological examination confirmed the diagnosis of malignant peripheral nerve sheath tumor. Intrapelvic malignant peripheral nerve sheath tumors are an uncommon cause of sciatica and are commonly misdiagnosed as lumbar intervertebral disc herniation. Accurate diagnosis and complete surgical excision prior to metastasis are crucial for effective management of this condition. PMID:27900106

  1. Giant cystic umbilical cord associated with patent urachus and intrauterine fetal demise

    PubMed Central

    Guileyardo, Joseph; Le, Don; Joykutty, Jesy

    2017-01-01

    True cystic structures within the umbilical cord are rare, and when they persist into the second and third trimester, they are often associated with an abnormal karyotype or other developmental abnormalities. Clinically significant pseudocysts resulting from massive edema of the umbilical cord have been associated with a congenitally patent urachus. We present a case of intrauterine fetal demise at 28 weeks' gestation in which cystic dilatation of the umbilical cord was diagnosed prenatally by ultrasound imaging. At autopsy, a congenitally patent urachus was contiguous with the massively edematous umbilical cord. PMID:28405085

  2. 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.

  3. The human Bosniak model applied to a cat with renal cystadenoma. A classification to differentiate benign and malignant cystic renal masses via computed tomography and ultrasound.

    PubMed

    Baloi, P; Del Chicca, F; Ruetten, M; Gerber, B

    2015-01-01

    A 13-year-old domestic shorthair cat was presented with weight loss and azotemia. Abdominal ultrasound revealed a large cystic space- occupying lesion with multiple septae in the left kidney. A core needle biopsy yielded a renal cystadenoma originating from the epithelial cells. This report describes the clinical, ultrasonographic and computed tomographic features and the growth progression of a renal cystadenoma. We describe the first attempt to apply the human Bosniak classification to a cat with renal cystic neoplasia to differentiate between benign and malignant lesions. Cystadenoma should be a differential diagnosis in cases of renal cystic space-occupying lesions. Other differentials, imaging features to differentiate benign and malignant lesions and the risk of malignant transformation will be discussed.

  4. Diffuse-type giant cell tumor/pigmented villonodular synovitis arising in the sacrum: malignant form.

    PubMed

    Oda, Yoshinao; Takahira, Tomonari; Yokoyama, Ryohei; Tsuneyoshi, Masazumi

    2007-09-01

    Diffuse-type giant cell tumor (GCT)/pigmented villonodular synovitis (PVNS) in the axial skeleton or spine is rare. Herein is reported a case of diffuse-type GCT/PVNS involving the sacrum and the fifth lumbar vertebra, in which the patient developed regional lymph node swelling after recurrence. The recurrent tumor was found to have atypical histological features such as spindle cell morphology, cytological atypia and high mitotic rate, which are compatible with the diagnostic criteria of secondary malignant diffuse-type GCT/PVNS. Although the nodal lesions were not sampled histologically, the clinical and histological features indicate that the current case is an example of malignant diffuse-type GCT/PVNS. This case is considered to be the first case of malignant diffuse-type GCT/PVNS in the spine, because no such lesions have been previously reported in the axial skeleton or spine. Careful surveillance should be required for diffuse-type GCT/PVNS arising at unusual site.

  5. 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

  6. Giant Sporadic Low Grade Malignant Peripheral Nerve Sheath (MPNST) of Left Thigh.

    PubMed

    Nikumbh, Dhiraj B; Suryawanshi, K H; Dravid, N V; Patil, T B; Rokade, C M

    2013-06-01

    Malignant Peripheral Nerve Sheath Tumours (MPNST) are rare spindle- cell sarcomas which are derived from the schwann cells or the pleuripotent cells of the neural crest. MPNSTs are usually located in the peripheral nerve trunk roots, extremities and in the head and neck region. These tumours may arise as sporadic variants or in patients with neurofibromatosis1 (NF1). The estimated incidence of MPNSTs in the patients with NF1is 2-5% as compared to a 0.001% incidence in the general population. Herein, we are reporting a case of a giant sporadic (NF1 independent) low grade MPNST of the left thigh in 65 year female patient, which had a good prognosis.

  7. 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.

  8. Serum Carbohydrate Antigen 19-9 in Differential Diagnosis of Benign and Malignant Pancreatic Cystic Neoplasms: A Meta-Analysis

    PubMed Central

    Cao, Shaobo; Hu, Ya; Gao, Xiang; Liao, Quan; Zhao, Yupei

    2016-01-01

    Background Using serum carbohydrate antigen 19–9 (CA 19–9) in discriminating between benign and malignant pancreatic disease remains controversial. We aim to evaluate the diagnostic value of serum CA 19–9 in predicting malignant pancreatic cystic lesions. Methods Eligible studies were identified through searching MEDLINE and EMBASE prior to March 2016. Studies were assessed for quality using the Quality Assessment for Studies of Diagnostic Accuracy, 2nd version (QUADAS-2). Pooled sensitivity and specificity with 95% confidence interval (CI) were calculated using random-effects models. Summary receiver operator characteristic (SROC) curves and the area under curve (AUC) were performed. Results A total of thirteen studies including 1437 patients were enrolled in this meta-analysis. The pooled sensitivity and specificity were 0.47(95% CI: 0.35–0.59), and 0.88(95% CI: 0.86–0.91), respectively, and the AUC was 0.87(95% CI, 0.84–0.90). Meta-regression analysis showed that sample size, region and reference standards were not the main sources of heterogeneity. Conclusions Serum CA 19–9 has satisfying pooled specificity while poor pooled sensitivity for discriminating benign from malignant PCNs. It deserves to be widely used as complementary to other clinical diagnostic methods. PMID:27835676

  9. Surgical treatment of rare giant malignant tumors of the scalp: A report of 3 cases with different tumor types

    PubMed Central

    Liu, Xiaoliang; Li, Wenzhong; Yuan, Hepei; Gu, Weihong; Chen, Dawei

    2016-01-01

    The scalp is the most frequent site of occurrence of malignant tumors. As an area that is generally neglected by the patient and not closely monitored during physical examinations, scalp tumors can go unnoticed until they become malignant. The present study reports 3 cases of rare giant malignant tumors of the scalp, namely a peripheral nerve sheath tumor, a fibrous tumor and a malignant proliferating trichilemmal tumor, that were treated at The First Bethune Hospital of Jilin University (Changchun, China). Vascularized free anterolateral thigh flap surgery was performed in 2 of the 3 cases. A local flap repair was applied to the third case. The implanted skin grafts remained viable post-operatively and wound repair was uneventful. No signs of malignancy were detected on the edge of the pathological section upon closer pathological examination. In the follow-up period, no recurrence was detected in any of the cases. PMID:27900013

  10. Giant malignant peripheral nerve sheath tumor of thigh in an adolescent with neurofibromatosis type 1: a case report

    PubMed Central

    Tosun, Hacı Bayram; Serbest, Sancar; Turk, Bilge Aydın; Gumustas, Seyit Ali; Uludag, Abuzer

    2015-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of children and adolescents, and they are aggressive tumors with a high rate of local recurrence. We present a 15-year-old boy with neurofibromatosis type 1 (NF1), who had a giant MPNST on the right thigh taking into account the available literature. Diagnosis of MPNST may be delayed in NF1 patients due to confusion with a neurofibroma and/or a plexiform neurofibroma. Malignancy should be considered, especially in cases with big masses, with heterogeneous involvement, or in the presence of cysts or necrotic nodules. The aim of surgical treatment is complete surgical excision. PMID:26604833

  11. Giant mesenteric cyst

    PubMed Central

    Guraya, Salman Yousuf; Salman, Shaista; Almaramhy, Hamdi H.

    2011-01-01

    Mesenteric cysts are uncommon benign abdominal lesions with no classical clinical features. The preoperative diagnosis requires the common imaging modalities but the final diagnosis is established only during surgery or histological analysis. The treatment of choice is complete surgical excision. We report an 18-year-old female with a non-specific abdominal pain and discomfort since 3 weeks. Her CT scan showed a huge cystic swelling, which necessitated surgical exploration. Preoperatively, a giant cyst was encountered with displacement of bowel loops. The cyst was completely removed and histology report confirmed mesenteric cyst without evidence of malignancy. PMID:24765349

  12. Giant mesenteric cyst.

    PubMed

    Guraya, Salman Yousuf; Salman, Shaista; Almaramhy, Hamdi H

    2011-09-28

    Mesenteric cysts are uncommon benign abdominal lesions with no classical clinical features. The preoperative diagnosis requires the common imaging modalities but the final diagnosis is established only during surgery or histological analysis. The treatment of choice is complete surgical excision. We report an 18-year-old female with a non-specific abdominal pain and discomfort since 3 weeks. Her CT scan showed a huge cystic swelling, which necessitated surgical exploration. Preoperatively, a giant cyst was encountered with displacement of bowel loops. The cyst was completely removed and histology report confirmed mesenteric cyst without evidence of malignancy.

  13. Phase II trial of dasatinib for recurrent or metastatic c-KIT expressing adenoid cystic carcinoma and for nonadenoid cystic malignant salivary tumors

    PubMed Central

    Wong, S. J.; Karrison, T.; Hayes, D. N.; Kies, M. S.; Cullen, K. J.; Tanvetyanon, T.; Argiris, A.; Takebe, N.; Lim, D.; Saba, N. F.; Worden, F. P.; Gilbert, J.; Lenz, H. J.; Razak, A. R. A.; Roberts, J. D.; Vokes, E. E.; Cohen, E. E. W.

    2016-01-01

    Background Adenoid cystic carcinoma (ACC) is a subtype of malignant salivary gland tumors (MSGT), in which 90% of cases express cKIT. Dasatinib is a potent and selective inhibitor of five oncogenic protein tyrosine kinases (PTKs)/kinase families including cKIT. We conducted a phase II study to determine the antitumor activity of dasatinib in ACC and non-ACC MSGT. Patients and methods In a two-stage design, patients with progressive, recurrent/metastatic ACC (+cKIT) and non-ACC MSGT (separate cohort) were treated with dasatinib 70 mg p.o. b.i.d. Response was assessed every 8 weeks using RECIST. Results Of 54 patients: 40 ACC, 14 non-ACC (1, ineligible excluded); M:F = 28 : 26, median age 56 years (range 20–82 years), ECOG performance status 0 : 1 : 2 = 24 : 28 : 2, prior radiation: 44, prior chemotherapy: 21. The most frequent adverse events (AEs) (as % of patients, worst grade 2 or higher) were: fatigue (28%), nausea (19%), headache (15%), lymphopenia (7%), dyspnea (11%), alanine aminotransferase increased (7%), anorexia (7%), vomiting (7%), alkaline phosphatase increased (6%), diarrhea (6%), neutropenia (6%), and noncardiac chest pain (6%). No grade 4 AE occurred, 15 patients experienced a grade 3 AE, primarily dyspnea (5) and fatigue (4), and cardiac toxicity (1 prolonged QTc). Among ACC patients, best response to dasatinib: 1 patient (2.5%) had partial response, 20 patients (50%) had stable disease (SD) (3–14 months), 12 patients (30%) had PD, 2 withdrew, 3 discontinued therapy due to AE, and 2 died before cycle 2. Median progression-free survival was 4.8 months. Median overall survival was 14.5 months. For 14 assessable non-ACC patients, none had objective response, triggering early stopping rule. Seven had SD (range 1–7 months), 4 PD, 2 discontinued therapy due to AE, and 1 died before cycle 2. Conclusion Although there was only one objective response, dasatinib is well tolerated, with tumor stabilization achieved by 50% of ACC patients. Dasatinib

  14. Phase II trial of dasatinib for recurrent or metastatic c-KIT expressing adenoid cystic carcinoma and for nonadenoid cystic malignant salivary tumors.

    PubMed

    Wong, S J; Karrison, T; Hayes, D N; Kies, M S; Cullen, K J; Tanvetyanon, T; Argiris, A; Takebe, N; Lim, D; Saba, N F; Worden, F P; Gilbert, J; Lenz, H J; Razak, A R A; Roberts, J D; Vokes, E E; Cohen, E E W

    2016-02-01

    Adenoid cystic carcinoma (ACC) is a subtype of malignant salivary gland tumors (MSGT), in which 90% of cases express cKIT. Dasatinib is a potent and selective inhibitor of five oncogenic protein tyrosine kinases (PTKs)/kinase families including cKIT. We conducted a phase II study to determine the antitumor activity of dasatinib in ACC and non-ACC MSGT. In a two-stage design, patients with progressive, recurrent/metastatic ACC (+cKIT) and non-ACC MSGT (separate cohort) were treated with dasatinib 70 mg p.o. b.i.d. Response was assessed every 8 weeks using RECIST. Of 54 patients: 40 ACC, 14 non-ACC (1, ineligible excluded); M:F = 28 : 26, median age 56 years (range 20-82 years), ECOG performance status 0 : 1 : 2 = 24 : 28 : 2, prior radiation: 44, prior chemotherapy: 21. The most frequent adverse events (AEs) (as % of patients, worst grade 2 or higher) were: fatigue (28%), nausea (19%), headache (15%), lymphopenia (7%), dyspnea (11%), alanine aminotransferase increased (7%), anorexia (7%), vomiting (7%), alkaline phosphatase increased (6%), diarrhea (6%), neutropenia (6%), and noncardiac chest pain (6%). No grade 4 AE occurred, 15 patients experienced a grade 3 AE, primarily dyspnea (5) and fatigue (4), and cardiac toxicity (1 prolonged QTc). Among ACC patients, best response to dasatinib: 1 patient (2.5%) had partial response, 20 patients (50%) had stable disease (SD) (3-14 months), 12 patients (30%) had PD, 2 withdrew, 3 discontinued therapy due to AE, and 2 died before cycle 2. Median progression-free survival was 4.8 months. Median overall survival was 14.5 months. For 14 assessable non-ACC patients, none had objective response, triggering early stopping rule. Seven had SD (range 1-7 months), 4 PD, 2 discontinued therapy due to AE, and 1 died before cycle 2. Although there was only one objective response, dasatinib is well tolerated, with tumor stabilization achieved by 50% of ACC patients. Dasatinib demonstrated no activity in non-ACC MSGT. © The Author 2015

  15. Malignancy rates and diagnostic performance of the Bosniak classification for the diagnosis of cystic renal lesions in computed tomography - a systematic review and meta-analysis.

    PubMed

    Sevcenco, Sabina; Spick, Claudio; Helbich, Thomas H; Heinz, Gertraud; Shariat, Shahrokh F; Klingler, Hans C; Rauchenwald, Michael; Baltzer, Pascal A

    2017-06-01

    To systematically review the literature on the Bosniak classification system in CT to determine its diagnostic performance to diagnose malignant cystic lesions and the prevalence of malignancy in Bosniak categories. A predefined database search was performed from 1 January 1986 to 18 January 2016. Two independent reviewers extracted data on malignancy rates in Bosniak categories and several covariates using predefined criteria. Study quality was assessed using QUADAS-2. Meta-analysis included data pooling, subgroup analyses, meta-regression and investigation of publication bias. A total of 35 studies, which included 2,578 lesions, were investigated. Data on observer experience, inter-observer variation and technical CT standards were insufficiently reported. The pooled rate of malignancy increased from Bosniak I (3.2 %, 95 % CI 0-6.8, I(2) = 5 %) to Bosniak II (6 %, 95 % CI 2.7-9.3, I(2) = 32 %), IIF (6.7 %, 95 % CI 5-8.4, I(2) = 0 %), III (55.1 %, 95 % CI 45.7-64.5, I(2) = 89 %) and IV (91 %, 95 % CI 87.7-94.2, I(2) = 36). Several study design-related influences on malignancy rates and subsequent diagnostic performance indices were identified. The Bosniak classification is an accurate tool with which to stratify the risk of malignancy in renal cystic lesions. • The Bosniak classification can accurately rule out malignancy. • Specificity remains moderate at 74 % (95 % CI 64-82). • Follow-up examinations should be considered in Bosniak IIF and Bosniak II cysts. • Data on the influence of reader experience and inter-reader variability are insufficient. • Technical CT standards and publication year did not influence diagnostic performance.

  16. 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

  17. 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

  18. Giant intra-abdominal mature cystic teratoma (dermoid cyst) in an adult man, with male genitourinary tissue including prostatic and penile elements.

    PubMed

    Thway, Khin; Berney, Dan; Hayes, Andrew J; Fisher, Cyril

    2016-08-01

    We describe a case of a giant intra-abdominal mature cystic teratoma in a 36-year-old man, which comprised typical features of differentiated teratoma/dermoid cyst but which contained a macroscopic rudimentary penis, with vasoformative erectile tissue-like structures consistent with corpora cavernosa, as well as scrotal-type skin and prostatic tissue. The genitourinary structures were well formed both grossly and microscopically and sharply demarcated from the rest of the neoplasm, which comprised typical differentiated teratoma, without any other macroscopic foci of organoid differentiation or of other histologic differentiation. The plasticity of the cells of differentiated teratoma, which enables it to undergo multidirectional differentiation, is well recognized, but the factors determining this distinct path of differentiation remain to be established. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. A male case of an undifferentiated carcinoma with osteoclast-like giant cells originating in an indeterminate mucin-producing cystic neoplasm of the pancreas. A case report and review of the literature.

    PubMed

    Wada, Takeyuki; Itano, Osamu; Oshima, Go; Chiba, Naokazu; Ishikawa, Hideki; Koyama, Yasumasa; Du, Wenlin; Kitagawa, Yuko

    2011-09-08

    We report a rare male case of an undifferentiated carcinoma with osteoclast-like giant cells originating in an indeterminate mucin-producing cystic neoplasm of the pancreas. A 59-year-old Japanese man with diabetes visited our hospital, complaining of fullness in the upper abdomen. A laboratory analysis revealed anemia (Hemoglobin; 9.7 g/dl) and elevated C-reactive protein (3.01 mg/dl). Carbohydrate antigen 19-9 was 274 U/ml and Carcinoembryonic antigen was 29.6 ng/ml. A computed tomography scan of the abdomen revealed a 14-cm cystic mass in the upper left quadrant of the abdomen that appeared to originate from the pancreatic tail. The patient underwent distal pancreatectomy/splenectomy/total gastrectomy/cholecystectomy. The mass consisted of a multilocular cystic lesion. Microscopically, the cyst was lined by cuboidal or columnar epithelium, including mucinous epithelium. Sarcomatous mononuclear cells and multinucleated osteoclast-like giant cells were found in the stroma. Ovarian-type stroma was not seen. We made a diagnosis of osteoclast-like giant cell tumor originating in an indeterminate mucin-producing cystic neoplasm of the pancreas. All surgical margins were negative, however, two peripancreatic lymph nodes were positive. The patient recovered uneventfully. Two months after the operation, multiple metastases occurred in the liver. He died 4 months after the operation.

  20. Unique Presentation of Giant Metastatic Microcystic Serous Adenocarcinoma of the Pancreas

    PubMed Central

    Kalal, Chetan Ramesh; Bihari, Chhagan; Sahney, Amrish; Kumar, KN Chandan; Rastogi, Archana

    2014-01-01

    Tumors of the pancreas that contain substantial cystic components include mainly mucinous cystic neoplasm, intraductal papillary mucinous neoplasm, solid pseudopapillary tumor, and cystadenomas (which encompass microcystic, macrocystic/oligocystic, and rare solid serous adenomas). Microcystic adenoma of the pancreas is a tumor that is benign in nature. Malignant transformation in the tumor with metastases is rare and only about 26 cases have been reported so far. Here we present a giant microcystic adenoma of the pancreas, possibly the largest ever malignant type in this group ever reported in the literature with extensive metastases to the liver and causing extensive compression and encasement on surrounding structures. PMID:24782930

  1. 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

  2. Polymyalgia rheumatica and giant cell arteritis—three challenges—consequences of the vasculitis process, osteoporosis, and malignancy

    PubMed Central

    Emamifar, Amir; Hess, Søren; Gerke, Oke; Hermann, Anne Pernille; Laustrup, Helle; Hansen, Per Syrak; Thye-Rønn, Peter; Marcussen, Niels; Svendstrup, Frank; Gildberg-Mortensen, Rannveig; Bang, Jacob Christian; Farahani, Ziba Ahangarani; Chrysidis, Stavros; Toftegaard, Pia; Andreasen, Rikke Asmussen; le Greves, Sebastian; Andersen, Hanne Randi; Olsen, Rudolf Nezlo; Hansen, Inger Marie Jensen

    2017-01-01

    Abstract Introduction: Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are common inflammatory conditions. The diagnosis of PMR/GCA poses many challenges since there are no specific diagnostic tests. Recent literature emphasizes the ability of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) to assess global disease activity in inflammatory diseases. 18F-FDG PET/CT may lead to the diagnosis at an earlier stage than conventional imaging and may also assess response to therapy. With respect to the management of PMR/GCA, there are 3 significant areas of concern as follows: vasculitis process/vascular stiffness, malignancy, and osteoporosis. Methods and analysis: All patients with suspected PMR/GCR referred to the Rheumatology section of Medicine Department at Svendborg Hospital, Denmark. The 4 separate studies in the current protocol focus on: the association of clinical picture of PMR/GCA with PET findings; the validity of 18F-FDG PET/CT scan for diagnosis of PMR/GCA compared with temporal artery biopsy; the prevalence of newly diagnosed malignancies in patients with PMR/GCA, or PMR-like syndrome, with the focus on diagnostic accuracy of 18F-FDG PET/CT scan compared with conventional workup (ie, chest X-ray/abdominal ultrasound); and the impact of disease process, and also steroid treatment on bone mineral density, body composition, and vasculitis/vascular stiffness in PMR/GCA patients. Ethics and dissemination: The study has been approved by the Regional Ethics Committee of the Region of Southern Denmark (identification number: S-20160098) and Danish Data Protection Agency (J.nr 16/40522). Results of the study will be disseminated via publications in peer-reviewed journals, and presentation at national and international conferences. PMID:28658131

  3. A giant and insidious subphrenic biloma formation due to gallbladder perforation mimicking biliary cystic tumor: A case report

    PubMed Central

    Ji, Guwei; Zhu, Feipeng; Wang, Ke; Jiao, Chenyu; Shao, Zicheng; Li, Xiangcheng

    2017-01-01

    Gallbladder perforation (GBP) represents a rare, but potentially life-threatening, complication of acute cholecystitis. GBP is subdivided into three categories whereas the development of biloma is extremely rare. The present case study reports on a 40-year-old man with a 10-year history of calculus cholecystitis, who was referred to The First Affiliated Hospital of Nanjing Medical University (Nanjing, China) for the surgical treatment of an emerging massive hepatic entity with insidious symptoms and normal laboratory tests. A preoperative imaging study demonstrated the collection with internal septations and mural nodules, but no visible communication with the biliary system. Given the suspected biliary cystic tumor, a laparotomy was performed and the lumen was scattered with papillae. An intraoperative frozen section examination illustrated a simple hepatic cyst. Biochemical analysis of the collection and histopathology of the gallbladder and capsule substantiated the diagnosis of biloma formation due to GBP. The purpose of the present case report was to demonstrate how a pinhole-sized perforation with extravasation of unconcentrated bile from the gallbladder may result in insidious clinical presentation and an undetected leak site. According to the clinicopathological characteristics and composition, formation of biloma should be classified as type IV GBP. To differentiate bilomas with intracystic septations and mural nodules from BCTs is difficult via a preoperative examination, and the definitive diagnosis should be based on a histological examination. Laparotomy with frozen section examination may be the optimal approach in such a case. PMID:28123732

  4. Diagnostic pitfalls in the preoperative 18F-FDG PET/CT evaluation of a case of giant malignant solitary fibrous tumor of the pleura.

    PubMed

    Lococo, F; Rapicetta, C; Ricchetti, T; Cavazza, A; Filice, A; Treglia, G; Tenconi, S; Paci, M; Sgarbi, G

    2014-01-01

    Solitary fibrous tumor of the pleura (SFTP) is an uncommon entity, generally with an indolent behavior. Nevertheless, some malignant forms have been rarely reported. These, often have an aggressive biological behavior with pathological findings of invasiveness. The preoperative diagnosis and evaluation of the grade of malignancy are extremely challenging. Herein we report a case of a 64-year-old man who presented with a left giant intra-thoracic mass imaged with fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG/PET-CT) and sampled via fine-needle aspiration biopsy (FNAB). Imaging and FNAB findings showed suspicion of a benign form of SFTP. Surgical radical resection of the giant mass was performed. The definitive histological diagnosis showed a malignant SFTP. Based on this report, we take the opportunity to briefly discuss the insidious pitfalls concerning the radiological and (18)F-FDG/PET-CT features as well as cyto/histological findings in the pre-operative diagnostic work-up examination of this rare entity. Copyright © 2013 Elsevier España, S.L. and SEMNIM. All rights reserved.

  5. Giant cell tumor of soft tissue arising in breast.

    PubMed

    May, Steve A; Deavers, Michael T; Resetkova, Erika; Johnson, Deborah; Albarracin, Constance T

    2007-10-01

    Primary giant cell tumor of soft tissue (GCT-ST) arising in breast is exceedingly rare. We report a case of a 60-year-old woman with a primary breast giant cell tumor that appeared histologically identical to giant cell tumor of bone and had a clinically malignant course. The patient presented with a cystic mass of the breast, suspected on imaging to be an organizing hematoma, possibly related to previous injury. Histopathological evaluation revealed a neoplasm composed of mononuclear cells admixed with osteoclast-like giant cells resembling giant cell tumor of bone. Immunohistochemical staining was positive for CD68, smooth muscle actin, and vimentin, but was negative for a panel of epithelial and additional muscle markers. These features were most consistent with GCT-ST, an uncommon neoplasm of low malignant potential. Despite aggressive surgical treatment achieving clear surgical margins, the patient expired with pulmonary metastases within a year of her initial presentation. This case demonstrates the difficulty of predicting clinical behavior of GCT-ST of breast on the basis of histological features and depth of tumor alone. To our knowledge, this is the first case report of a GCT-ST arising in the breast associated with a fatal outcome. The distinction of this entity from other more common primary breast tumors with giant cell morphology is also emphasized.

  6. Cystic tumors of the pancreas

    PubMed Central

    Morana, Giovanni; Guarise, Alessandro

    2006-01-01

    Cystic tumors of the pancreas are less frequent than solid lesions and are often detected incidentally, as many of these lesions are small and asymptomatic. However, they may be associated with pancreatitis or have malignant potential. With advancements in diagnostic imaging, cystic lesions of the pancreas are being detected with increasing frequency. Many lesions can cause a pancreatic cyst, most being non-neoplastic while approximately 10% are cystic tumors, ranging from benign to highly malignant tumors. With increasing experience it is becoming clear that the prevalence of pseudocyst among cystic lesions of the pancreas is lower than usually presumed. A presumptive diagnosis of pseudocyst based on imaging appearance alone can cause a diagnostic error, and neoplastic cysts of the pancreas are particularly susceptible to this misdiagnosis, which can result in inappropriate treatment. Cystic tumors of the pancreas are formed by serous or mucinous structures showing all stages of cellular differentiation. According to the WHO classification, they can be subdivided on the basis of their histological type and biological behavior into benign tumors, borderline tumors, and malignant tumors. Cystic pancreatic tumors can be subdivided into peripheral (serous cystadenomas, mucinous cystic tumors, solid and papillary epithelial neoplasms, cystic islet cell tumors), which do not communicate with the main pancreatic duct, and ductal tumors (mucinous tumor), according to their site of origin. On the basis of imaging criteria alone, it can be very difficult to differentiate non-tumoral cystic lesions from neoplastic ones. The management of these patients is complex, and it is important to correlate imaging findings with knowledge of the patient’s symptoms and of the natural history and predictors of malignancy in pancreatic cysts. PMID:16861136

  7. Giant malignant fibrous histiocytoma of the testis/spermatic cord: psychologic and possible etiologic complications of unethical Nazi medical experimentation.

    PubMed

    Staiman, V R; O'Toole, K M; Rubin, M A; Lowe, F C

    1996-12-01

    This case of malignant fibrous histiocytoma of the testis/spermatic cord was found in a Holocaust survivor who was injected with an unknown substance in the left testicle while in Auschwitz concentration camp in 1943. Because malignant fibrous histiocytoma is a neoplasm rarely found in this location, with only 26 previously reported cases, a review of this entity was performed. A review of Nazi medical practices in the literature and through the Holocaust Museum research department was undertaken in an attempt to ascertain identification of the unknown substance injected into this patient; however, exact identification of the Auschwitz experiment or experimenter could not be determined. A left radical orchiectomy was performed, and subsequent histolopathologic review revealed a well-encapsulated 27 x 22 x 17-cm malignant fibrous histiocytoma. The left testis was not clearly identified due to necrosis of the tumor. This is the largest malignant fibrous histiocytoma of the spermatic cord/testis recorded in the literature to date. Based on the unusual location and size, the intratesticular injection probably contributed to the tumor development and certainly caused the patient's delay in seeking medical treatment.

  8. Sonographic-pathologic correlation of complex cystic breast lesions

    PubMed Central

    Pongrattanaman, Saravech; Prueksadee, Jenjeera

    2013-01-01

    Objective To understand the pathologic basis for sonographic features of complex cystic lesions. Methods From 2 646 female patients underwent breast sonography at King Chulalongkorn Memorial Hospital from January 2005 through December 2010, 103 cystic lesions were included. Pathologic confirmation was performed by fine-needle aspiration (n=42), core needle biopsy (n=6), excision (n=54) and mastectomy (n=1). Complex cystic breast masses were classified into 3 types as followings; thick outer wall and/or thick internal septa (type I); thick septation and thick wall were defined as equal or more than 0.5 cm, masses containing mixed cystic and solid components (at least 50% of cystic component) (type II), predominantly solid with eccentric cystic foci (at least 50% of solid component) (type III). Results In 103 complex cystic masses, there are 27 lesions (26%) classified as type I cystic breast masses, 37 lesions (36%) as type II cystic breast masses and 39 lesions (38%) type III cystic breast masses, 26 lesions (25.2%) are proved to be malignant. All of type I cystic breast masses in our study are benign, and 14 (38%) of type II cystic breast masses and 12 lesions (31%) of type III cystic breast lesions are proved to be malignant. Conclusions Type II and III lesions should suggest possibility of malignancy and biopsy should be performed in all lesions. All type I lesion in this study are benign. None of other parameters we included in this study (size or margin) can effectively differentiate between benign or malignant cystic breast lesions. Also, grading of the malignant lesions by using type of cystic breast mass cannot be applied.

  9. Cystic tumors of the pancreas: imaging and management.

    PubMed

    Dewhurst, Catherine E; Mortele, Koenraad J

    2012-05-01

    Cystic tumors of the pancreas are a subset of rare pancreatic tumors that vary from benign to malignant. Many have specific imaging findings that allow them to be differentiated from each other. This article (1) reviews the imaging features of the common cystic pancreatic lesions, including serous microcystic adenoma, mucinous cystic tumor, intraductal papillary mucinous tumor, and solid pseudopapillary tumor, and including the less common lesions such as cystic endocrine tumors, cystic metastases, cystic teratomas, and lymphangiomas; and (2) provides comprehensive algorithms on how to manage the individual lesions, with recommendations on when to reimage patients. Copyright © 2012 Elsevier Inc. All rights reserved.

  10. Characteristics, treatment, and survival of invasive malignant melanoma (MM) in giant pigmented nevi (GPN) in adults: 976 cases from the National Cancer Data Base (NCDB).

    PubMed

    Turkeltaub, Ashley E; Pezzi, Todd A; Pezzi, Christopher M; Dao, Harry

    2016-06-01

    Malignant melanoma (MM) arising in a giant pigmented nevus (GPN) is a rare disease in adults with no large series published to our knowledge. We sought to describe the characteristics, treatment, and survival of MM in GPN for adults. Adults with invasive MM in GPN (n = 976) reported to the National Cancer Data Base from 1998 to 2012 were evaluated for patient and tumor characteristics, treatment, and survival. For comparison, data from adults with invasive superficial spreading melanoma (SSM) (n = 111,870) and nodular melanoma (n = 35,962) were used. Compared with patients with SSM, patients with MM in GPN had a thicker Breslow depth, more positive lymph nodes, and distant metastasis more frequently. Multivariate analysis identified age older than 65 years, Breslow thickness greater than 2 mm, presence of ulceration, presence of distant metastasis, and positive margins as independent predictors of survival in patients with MM in GPN. At all stages, having MM in GPN has similar overall survival compared with SSM. The study is retrospective and registry-based. Invasive MM in GPN occurs in adults, with overall survival similar to SSM. Clinicians should be aware of the continued risk of MM in adults with GPN with low threshold for biopsy. Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  11. [Surgical treatment of giant serous cystadenoma of pancreas: report of two cases].

    PubMed

    Targarona, Javier; Garatea, Rafael; Romero, Cesar; Rosamedina, José Luís; Lora, Alfonso; Beltrán, Jorge; Rotta, César; Tapia, Pedro; Montoya, Eduardo

    2007-01-01

    The cystic tumor of the pancreas is a relatively uncommon entity. There are different types of pancreatic cystic tumors and they all exhibit different degrees of malignancy. These tumors represent 1% of all primary pancreatic tumors and only 15% of the cystic lesions. The serous cystadenomas (SCA) are mostly benign lesions with an average size of 4 cm; nevertheless, in some rare cases these are giant lesions, generally larger than 15 cm. Sometimes these tumors produce a symptomatology caused by the compression of neighboring structures, therefore they are generally operable. During the period from June 2004 to June 2005, the 3A II unit of the Edgardo Rebagliati Martins Hospital operated on two cases of giant serous cystadenomas of the pancreas, one located in the tail of the pancreas and the other in the head of the pancreas, with an average size of 16 cm. The giant SCAs of the pancreas are rarely seen lesions that, according to different authors, are usually larger than 10 to 15 cm. in diameter. These lesions do not represent a diagnosis problem and are generally operable since they produce a symptomatology by compression. The surgical resection can be complicated due to their large size and to the considerable neovascularization.

  12. Giant melanotic neuroectodermal tumor of infancy (melanotic progonoma) of the head and neck: report of a malignant case.

    PubMed

    Nicosia, Giancarlo; Spennato, Pietro; Aliberti, Ferdinando; Cascone, Daniele; Quaglietta, Lucia; Errico, Maria Elena; Muto, Mario; Ionna, Franco; Cinalli, Giuseppe

    2017-05-01

    Melanotic neuroectodermal tumor of infancy is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, growing rapidly and developing during the 1st year of life. It most commonly arises from the maxilla, cranial vault, and mandible. Occasionally, it exhibits malignant behavior with local lymph nodes involvement. Cases misdiagnosed and left untreated for a long time can present challenges due to the tumor mass and infiltration. In these cases, adjuvant chemotherapy can be extremely helpful before radical excision. Authors of this report describe a 4-year-old boy from a developing country who was referred to their hospital with an ulcerated bulging lesion in the midline/right parietooccipital region, extending to the right laterocervical and parotid regions, resulting in significant craniofacial deformation. Magnetic resonance imaging of the brain revealed a highly enhancing tumor with intracranial and extracranial development extending mainly at the level of the right parietooccipital region, with lytic and hypertrophic alterations of the skull. The patient was managed with neoadjuvant and adjuvant chemotherapy and radically resective surgery on metastatic lymph nodes and the primary tumor of the skull. Scheduled radiotherapy was not performed, according to the parents' wishes. The patient returned to his native country where the lesion recurred, and he ultimately died approximately 10 months after the end of the treatment. The literature indicates that tumor removal alone has been the treatment of choice in most isolated cases, but in cases of highly advanced tumor with involvement of the skull and cervical lymph nodes, it is preferable to proceed with preoperative chemotherapy with the aim of reducing the tumor volume, allowing better technical conditions for complete surgical removal, and decreasing the risk of local recurrence or metastasis.

  13. 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. ...

  14. Cystic hygroma

    MedlinePlus

    ... white blood cells. This material is called embryonic lymphatic tissue. After birth, a cystic hygroma most often ... 198. Tower RL II, Camitta BM. Abnormalities of lymphatic vessels. In: Kliegman RM, Stanton BF, St Geme ...

  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 fibrosis.

    PubMed

    Ratjen, Felix; Bell, Scott C; Rowe, Steven M; Goss, Christopher H; Quittner, Alexandra L; Bush, Andrew

    2015-05-14

    Cystic fibrosis is an autosomal recessive, monogenetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The gene defect was first described 25 years ago and much progress has been made since then in our understanding of how CFTR mutations cause disease and how this can be addressed therapeutically. CFTR is a transmembrane protein that transports ions across the surface of epithelial cells. CFTR dysfunction affects many organs; however, lung disease is responsible for the vast majority of morbidity and mortality in patients with cystic fibrosis. Prenatal diagnostics, newborn screening and new treatment algorithms are changing the incidence and the prevalence of the disease. Until recently, the standard of care in cystic fibrosis treatment focused on preventing and treating complications of the disease; now, novel treatment strategies directly targeting the ion channel abnormality are becoming available and it will be important to evaluate how these treatments affect disease progression and the quality of life of patients. In this Primer, we summarize the current knowledge, and provide an outlook on how cystic fibrosis clinical care and research will be affected by new knowledge and therapeutic options in the near future. For an illustrated summary of this Primer, visit: http://go.nature.com/4VrefN.

  18. Giant Earlobe Epidermoid Cyst

    PubMed Central

    Pérez-Guisado, Joaquín; Scilletta, Alessandra; Cabrera-Sánchez, Emilio; Rioja, Luis F; Perrotta, Rosario

    2012-01-01

    Epidermoid cysts represent the most common cutaneous cysts. They are usually small and benign; however, sometimes they can grow to giant epidermoid cists, and occasionally malignancies develop. Giant epidermoid cysts at the earlobe have never been described but in other locations. We describe a case of a giant epidermoid cyst at the earlobe, a location where such a large cyst has never been reported before. The mass was completely resected and the wound of the pedunculated base was sutured with four stitches of nylon 5/0. Histopathology confirmed the presumptive diagnosis of an epidermoid cyst. Six months after the resection, the patient did not have any relapse of the epidermoid cyst. The earlobe is a potential location for giant epidermoid cysts. Although the clinical diagnosis could be enough, due to the possibility of malignancy and to ensure appropriate diagnosis, we consider that all cysts should be sent to the anatomic pathology laboratory for histological evaluation. PMID:22557855

  19. Cystic fibrosis

    MedlinePlus

    ... cannot be prevented. Screening those with a family history of the disease may detect the CF gene in many carriers. Alternative ... FJ. Cystic fibrosis. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap ...

  20. An Innocent Giant

    PubMed Central

    Solanki, Lakhan Singh; Dhingra, Mandeep; Raghubanshi, Gunjan; Thami, Gurvinder Pal

    2014-01-01

    A cutaneous horn (cornu cutaneum) is a protrusion from the skin composed of a cornified material. It may be associated with a benign, premalignant, or malignant lesion at the base, masking numerous dermatoses. In a 24-year-old female, a giant cutaneous horn arising from a seborrheic keratosis located on the leg is presented. This case has been reported to emphasize that a giant cutaneous horn may also occur in young patients, even in photoprotected areas, and are not always associated with malignancy. PMID:25484426

  1. 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.

  2. What Causes Cystic Fibrosis?

    MedlinePlus

    ... page from the NHLBI on Twitter. What Causes Cystic Fibrosis? A defect in the CFTR gene causes cystic ... in the severity of the disease. How Is Cystic Fibrosis Inherited? Every person inherits two CFTR genes—one ...

  3. Giant neoplastic omental cyst masquerading as ascites: a case report

    PubMed Central

    Abdulkadir, Adekunle Y; Olatoke, Samuel A; Uwaezuoke, Stanley; Yusuf, Ibrahim F; Braimoh, Kolawole T

    2009-01-01

    Introduction Cystic lesion of the omentum and mesentery are rare. The incidence of both cyst types has been variously reported to vary from 1/27,000-100,000 hospital admission. Omental cysts occur three to ten times less frequently than mesenteric cyst. Preoperative diagnosis is infrequently made because of lack of characteristic symptoms and signs. Case presentation We present our diagnostic and management challenges in a 43-year-old man with an unusually giant omental cyst confirmed as fibrosarcoma at histology. The cyst gave the abdomen an anteroposterior diameter of about 74 cm that could not be penetrated sufficiently by X-ray photons to produce diagnostic image even at maximum attainable output. Patient benefited from surgical excision. The removed cyst contained about 35 litres of fluid. Conclusion Neglected omental cysts as in this case may grow to enormous size, undergo malignant transformation and poses serious diagnostic and surgical challenges. PMID:19918526

  4. 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

  5. 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.

  6. 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.

  7. Giant congenital melanocytic nevus*

    PubMed Central

    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. PMID:24474093

  8. Cystic Fibrosis

    PubMed Central

    Asay, Lyal D.

    1965-01-01

    Cystic fibrosis, a disease thought to be transmitted as a recessive genetic trait, is found as a disease in about one in 1,000 to one in 10,000 births. It involves all of the exocrine glands with presenting symptoms dependent upon the extent of involvement of any group of glands. Many aspects of the disease can be corrected by substitution therapy. This applies particularly to the use of animal pancreas for the steatorrhea and salt for prevention of heat prostration. Unfortunately, the obstructive pulmonary disease with secondary bronchial infections can only be treated symptomatically by the use of mucus thinning agents, postural drainage, and antibiotics. Nevertheless, longevity can be increased and a great deal of hope offered to the families of these unfortunate children by careful supervision of their medical care. ImagesFigure 1.Figure 2.Figure 3.Figure 4.Figure 5.Figure 6.Figure 7.Figure 8.Figure 9.Figure 10.Figure 11. PMID:14288148

  9. 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

  10. [Malignized dermoid cyst of the ovary].

    PubMed

    Koivisto-Korander, Riitta; Bützow, Ralf

    2016-01-01

    Mature germ cell derived cystic teratoma of the ovary, i.e. dermoid cyst, is a common ovarian neoplasm, seldom progressing to malignancy. Malignized dermoid cyst is most commonly diagnosed in a woman of approx. 50 years of age, either during or after an operation. Owing to the rarity of the disease, the lines of treatment are based on retrospective series of cases.

  11. Cystic hypersecretory ductal carcinoma of the breast: a rare cause of cystic breast mass.

    PubMed

    Song, Sun Wha; Whang, In Yong; Chang, Eun Deok

    2011-11-01

    We present the case of a surgically confirmed, invasive, cystic hypersecretory ductal carcinoma (CHDC) of the breast in a 43-year-old woman. The initial sonography showed a complex cyst, which required a core biopsy; however, the diagnosis was delayed as the patient refused to undergo the biopsy and the cyst decreased in size, as seen on follow-up sonography. Excision biopsy was performed, and invasive CHDC was diagnosed after regrowth of the cystic lesion. Meticulous sonographic evaluation of a cystic breast mass is always important, and pathology confirmation must be considered if the lesion shows features suspicious for malignancy, as a CHDC could be the cause of a cystic breast mass.

  12. Cystic fibrosis - nutritional considerations

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/002437.htm Cystic fibrosis - nutrition To use the sharing features on this page, please enable JavaScript. Cystic fibrosis (CF) is a life-threatening disease that causes ...

  13. 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

  14. Cytologic characteristics of adenoid cystic carcinoma of the cervix uteri--case report.

    PubMed

    Barisić, Ana; Mahovlić, Vesna; Ovanin-Rakić, Ana; Skopljanac-Macina, Lada; Rajhvajn, Sanda; Juric, Danijela; Babić, Damir

    2010-03-01

    Adenoid cystic carcinoma is a rare malignancy, usually found in the salivary glands, although this unusual tumor can be found at other sites of the body. In particular, regarding adenoid cystic carcinoma of the cervix (ACCC) most frequently reports are given for postmenopausal women. In this respect, our work is one among very few in the literature that considers a cytologic picture of this uncommon cervix carcinoma. The case of 74 year old patient with postmenopausal bleeding is described. Both Pap smear and air dried smear of the uterine cervix were analyzed. The cytologic findings revealed very few small clusters of abnormal glandular cells, as well as some amorphous eosinophilic globule-like material, with granulomatous and necrotic background. The latter includes a lot of histiocytes, multinucleated giant cells, large aggregates of epitheloid cells and lymphocytes. Histology revealed the diagnosis of ACCC. We emphasize the importance of careful screening of Pap smear that might be crucial in the case of suspicious clinical findings in postmenopausal women, when the possibility of ACCC has to be considered.

  15. 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.

  16. Endoscopic ultrasound and computer tomography are inaccurate methods of classifying cystic pancreatic lesions.

    PubMed

    Gerke, H; Jaffe, T A; Mitchell, R M; Byrne, M F; Stiffler, H L; Branch, M S; Baillie, J; Jowell, P S

    2006-01-01

    Despite advances in imaging modalities, preoperative diagnosis of pancreatic cystic lesions remains difficult. To assess the accuracy of endoscopic ultrasound and computer tomography to preoperatively distinguish benign from potentially malignant and malignant pancreatic cystic lesions. Photograph series obtained from endoscopic ultrasound examinations of 66 patients with cystic pancreatic lesions were blindly reviewed by three endoscopic ultrasonographers. Forty-one of those 66 patients also underwent a computer tomography scan at our institution, which was blindly reviewed by a single radiologist. Computer tomography and endoscopic ultrasound classification into benign and malignant and potentially malignant pancreatic cystic lesions was correlated with the final diagnosis, which was established by surgical pathology (n = 43), diagnostic fine needle aspiration (n = 13) or follow-up imaging (n = 10). Interobserver agreement was measured using kappa statistics. Endoscopic ultrasound classification by the three examiners into benign versus malignant or potentially malignant cystic lesions was correct in 65-67%. Interobserver agreement was 50%. Kappa values for pairs of endoscopic ultrasound examiners were 0.16, 0.43 and 0.53. Computer tomography classification was correct in 71% and in agreement with the endoscopic ultrasound classification in 56-61% (kappa 0.12 to 0.27). Endoscopic ultrasound and computer tomography cannot accurately distinguish between benign pancreatic cystic lesions and malignant or potentially malignant ones. There is poor-to-modest interobserver agreement in classifying these lesions.

  17. Giant serous microcystic pancreas adenoma.

    PubMed

    Dikmen, Kursat; Bostanci, Hasan; Yildirim, Ali Cihat; Sakrak, Omer; Kerem, Mustafa

    2012-10-10

    Serous cystadenomas are rare tumors comprising 1-2% of exocrine pancreas tumors. They are mostly known as benign conditions but malign transformation as serous cystadenocarcinoma is also reported. It is usually seen in females. Non-specific symptoms, such as abdominal pain or symptoms due to mass affect, are usually seen. A 64-year old female patient was investigated for abdominal pain. Physical and laboratory findings were normal. Abdomen ultrasonography confirmed an 11×9.5 cm solid cystic lesion and abdomen computed tomography scan confirmed a 12×11 cm lobulated cystic solid lesion which had central cystic necrotic areas extending from liver hilus inferiorly. Fine needle biopsy confirmed benign cytology and trucut biopsy of the pancreatic mass reported chronic inflamation. Nevertheless, this mass could have malignant contents and transformation potential. A laparatomy was decided due to patient's symptoms and mass effect. Due to vascular invasion of the tumor, Whipple procedure was performed. The pathology report confirmed serous microcystic adenoma. These rare tumors are usually benign but pre-operative malignity criterias are not identified. There are few differential diagnostic tools for excluding malignity. We suggest surgical resection as best treatment approach for selected cases.

  18. Mesenteric lymphangioma mimicking a cystic ovarian mass on imaging.

    PubMed

    Hitzerd, Emilie; van Hamont, Dennis; Pijnenborg, Johanna M A

    2016-02-01

    Pelvic cystic masses are frequently observed in women. Most lesions are benign and of ovarian origin. However, non-ovarian lesions can be easily confused with cystic ovarian masses on imaging, which hampers diagnostic and therapeutic management. In this report, a rare case of mesenteric lymphangioma mimicking an ovarian cystic mass, discovered as an incidental finding on orthopaedic MRI in an adult female, is presented. The report highlights the sometimes difficult diagnostic process of pelvic cystic masses, due to an extensive differential diagnosis and the fact that imaging is often inconclusive. Even though most cystic masses are of ovarian origin, non-ovarian causes can mimic ovarian masses and should be considered as differential diagnoses. Surgical exploration may be necessary to exclude malignant causes. 2016 BMJ Publishing Group Ltd.

  19. Bilateral Cystic Adrenal Neuroblastoma with Cystic Liver metastasis

    PubMed Central

    Aslan, Mine; Kalyoncu, Ayse Ucar; Habibi, Hatice Arioz; Ozdemir, Gul Nihal; Koc, Basak; Adaletli, Ibrahim

    2017-01-01

    Bilateral congenital cystic adrenal neuroblastoma (NB) with cystic liver metastasis is a very rare condition and only few cases have been reported in the literature. Herein we report a case of a congenital bilateral cystic adrenal NB with cystic liver metastasis and briefly discuss characteristic imaging features of cystic NB. PMID:28163998

  20. Cystic precursors to invasive pancreatic cancer

    PubMed Central

    Matthaei, Hanno; Schulick, Richard D.; Hruban, Ralph H.; Maitra, Anirban

    2011-01-01

    Improvements in the sensitivity and quality of cross-sectional imaging have led to increasing numbers of patients being diagnosed with cystic lesions of the pancreas. In parallel, clinical, radiological, pathological and molecular studies have improved the systems for classifying these cysts. Patients with asymptomatic serous cystic neoplasms can be managed conservatively with regular monitoring; however, the clinical management of patients with intraductal papillary mucinous neoplasms and mucinous cystic neoplasms is far more challenging, as it is difficult to determine whether these lesions will progress to malignancy. Fortunately, prospective studies have helped to establish that proposed clinical and radiological criteria (the Sendai guidelines) can be used to guide the care of patients with cystic lesions of the pancreas. Despite this progress in imaging and clinical guidelines, sensitive and specific tests have not yet been developed that can reliably predict the histology and biological properties of a cystic lesion. Such biomarkers are urgently needed, as noninvasive precursors of pancreatic cancer are curable, while the vast majority of invasive pancreatic adenocarcinomas are not. PMID:21383670

  1. CA9 as a molecular marker for differential diagnosis of cystic renal tumors.

    PubMed

    Li, Guorong; Bilal, Issam; Gentil-Perret, Anne; Feng, Gang; Zhao, An; Peoc'h, Michel; Genin, Christian; Tostain, Jacques; Gigante, Marc

    2012-01-01

    CA9 is proven to be a powerful marker for clear cell renal cell carcinoma. The studies on CA9 have been limited to solid renal cell carcinomas (RCC). We have conducted a study of CA9 expression in renal cystic tumors. The purpose of the present study was to extend the utility of CA9 for cystic renal tumors. Immunohistochemistry and enzyme-linked immunosorbent assay (ELISA) were used to detect CA9 expression in cystic renal tumors. Forty-three cystic renal tumors (22 benign and 21 malignant) were included for the immunohistochemical staining. Thirty-six patients with a cystic renal mass (20 malignant and 16 benign cystic tumors) were studied to measure CA9 level in the fluid by ELISA. Sixteen cysts (9 malignant and 7 benign cysts) were subjected both to immunohistochemistry and CA9 measurement in the fluid. Using immunohistochemical staining, all the benign cystic renal tumors including the 18 simple cyst and 4 benign multilocular cystic nephromas did not express CA9. All 13 cystic clear cell RCC were scored as strong staining for CA9. For 8 multilocular clear cell RCC, 7 were scored as strong staining for CA9 and the other one was negative. There was a significant difference in positive percentage (P < 0.001) between the 2 groups of malignant and benign cysts. For the 16 benign cysts, the mean concentration of CA9 in the fluid of cyst was 162 ± 133 pg/ml (median: 0 pg/ml; range: 0-2140 pg/ml). For the 20 malignant renal cystic tumors, the mean concentration of CA9 in the fluid of cyst was 2043 ± 62 pg/ml (median: 2,140 pg/ml; range: 1,112-2,140 pg/ml). There was a significant difference in mean concentration of CA9 between the two groups of malignant and benign cysts (P < 0.001). The presence or absence of CA9 expression measured by immunohistochemistry and ELISA test was concordant in 14 out of 16 cases (88%). Malignant cystic renal tumors expressed strongly CA9 while the benign renal cysts did not express CA9. CA9 can be detected in the fluid of malignant

  2. Rare cystic liver lesions: A diagnostic and managing challenge

    PubMed Central

    Bakoyiannis, Andreas; Delis, Spiros; Triantopoulou, Charina; Dervenis, Christos

    2013-01-01

    Cystic formations within the liver are a frequent finding among populations. Besides the common cystic lesions, like simple liver cysts, rare cystic liver lesions like cystadenocarcinoma should also be considered in the differential diagnosis. Thorough knowledge of each entity’s nature and course are key elements to successful treatment. Detailed search in PubMed, Cochrane Database, and international published literature regarding rare cystic liver lesions was carried out. In our research are included not only primary rare lesions like cystadenoma, hydatid cyst, and polycystic liver disease, but also secondary ones like metastasis from gastrointestinal stromal tumors lesions. Up-to date knowledge regarding diagnosis and management of rare cystic liver lesions is provided. A diagnostic and therapeutic algorithm is also proposed. The need for a multidisciplinary approach by a team including radiologists and surgeons familiar with liver cystic entities, diagnostic tools, and treatment modalities is stressed. Patients with cystic liver lesions must be carefully evaluated by a multidisciplinary team, in order to receive the most appropriate treatment, since many cystic liver lesions have a malignant potential and evolution. PMID:24282350

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

    PubMed

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

    2009-05-19

    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. 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. 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. 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.

  4. Adenoid Cystic Carcinoma of the Uterine Cervix: A Report of 2 Cases

    PubMed Central

    Kharmoum, Jinane; Ech-Charif, Soumaya; El Khannoussi, Basma

    2017-01-01

    Adenoid cystic carcinoma is malignant tumor that exceptionally occurs in the uterine cervix. It is mostly seen in postmenopausal women and has an aggressive clinical course. We report two cases of an adenoid cystic carcinoma associated with a high grade squamous intraepithelial lesion and invasive squamous cell carcinoma of the uterine cervix and discuss briefly its clinical and pathological characteristics. PMID:28348909

  5. How Is Cystic Fibrosis Treated?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Is Cystic Fibrosis Treated? Cystic fibrosis (CF) has no cure. However, ... help oral pancreatic enzymes work better. Treatments for Cystic Fibrosis Complications A common complication of CF is diabetes . ...

  6. Cystic Fibrosis: Diet and Nutrition

    MedlinePlus

    ... Right Sport for You Healthy School Lunch Planner Cystic Fibrosis: Diet and Nutrition KidsHealth > For Teens > Cystic Fibrosis: ... Food Enzyme Supplements Beating the Frustration What Is Cystic Fibrosis? At lunch, Lindsay often gets bored with having ...

  7. Diffusion-weighted magnetic resonance imaging in cystic renal masses

    PubMed Central

    Balyemez, Fikret; Aslan, Ahmet; Inan, Ibrahim; Ayaz, Ercan; Karagöz, Vildan; Özkanli, Sıdıka Şeyma; Acar, Murat

    2017-01-01

    Introduction: We aimed to introduce the diagnostic value of diffusion-weighted (DWI) magnetic resonance imaging (MRI) for distinguishing benign and malignant renal cystic masses. Methods: Abdominal DWI-MRIs of patients with Bosniak categories 2F, 3, and 4 cystic renal masses were evaluated retrospectively. Cystic masses were assigned as benign or malignant according to histopathological or followup MRI findings and compared with apparent diffusion coefficient (ADC) values. Results: There were 30 patients (18 males and 12 females, mean age was 59.23 ± 12.08 years [range 38–83 years]) with cystic renal masses (eight Bosniak category 2F, 12 Bosniak category 3, 10 Bosniak category 4). Among them, 14 cysts were diagnosed as benign and 16 as malignant by followup imaging or histopathological findings. For the malignant lesions, the mean ADC values were lower than for benign lesions (p=0.001). An ADC value of ≤2.28 ×10−6 mm2/s or less had a sensitivity of 75% and a specificity of 92.86% for detecting malignancy. Conclusions: ADC can improve the diagnostic performance of MRI in the evaluation of complex renal cysts when used together with conventional MRI sequences. PMID:28163806

  8. Testicular Mass in Late Adolescence: Not Always Malignant.

    PubMed

    Delto, Joan C; Mittal, Angela G; Castellan, Miguel

    2016-08-01

    We present a rare case of cystic dysplasia of the testes in an adolescent boy who presented with testicular pain and found to have a palpable intratesticular mass. Ultrasound revealed an avascular cystic dilation of the testicle. Usually, a palpable intratesticular mass is malignant unless proven otherwise. However, on computed tomography scan, he was found to have agenesis of the ipsilateral kidney and dilation of the ipsilateral seminal vesicle. These findings were consistent with a congenital abnormality, suggesting that the testicular finding was likely cystic dysplasia of the testes, with low malignant potential. Thus, the patient did not undergo radical orchiectomy.

  9. Advanced endoscopic ultrasound management techniques for preneoplastic pancreatic cystic lesions

    PubMed Central

    Arshad, Hafiz Muhammad Sharjeel; Bharmal, Sheila; Duman, Deniz Guney; Liangpunsakul, Suthat; Turner, Brian G

    2017-01-01

    Pancreatic cystic lesions can be benign, premalignant or malignant. The recent increase in detection and tremendous clinical variability of pancreatic cysts has presented a significant therapeutic challenge to physicians. Mucinous cystic neoplasms are of particular interest given their known malignant potential. This review article provides a brief but comprehensive review of premalignant pancreatic cystic lesions with advanced endoscopic ultrasound (EUS) management approaches. A comprehensive literature search was performed using PubMed, Cochrane, OVID and EMBASE databases. Preneoplastic pancreatic cystic lesions include mucinous cystadenoma and intraductal papillary mucinous neoplasm. The 2012 International Sendai Guidelines guide physicians in their management of pancreatic cystic lesions. Some of the advanced EUS management techniques include ethanol ablation, chemotherapeutic (paclitaxel) ablation, radiofrequency ablation and cryotherapy. In future, EUS-guided injections of drug-eluting beads and neodymium:yttrium aluminum agent laser ablation is predicted to be an integral part of EUS-guided management techniques. In summary, International Sendai Consensus Guidelines should be used to make a decision regarding management of pancreatic cystic lesions. Advanced EUS techniques are proving extremely beneficial in management, especially in those patients who are at high surgical risk. PMID:27574295

  10. Living with Cystic Fibrosis

    MedlinePlus

    ... page 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 can ... with your doctors to learn how to manage CF. Ongoing Care Having ongoing medical care by a ...

  11. Chromomycosis: Subcutaneous cystic type.

    PubMed

    Agrawal, S N; Bhise, P R; Sony, P R

    2000-01-01

    A 38-year -old male farmer presented with a solitary, asymptomatic, cystic lesion on the palm since last four years. He underwent excision of this cyst two times during this period but the lesion recurred near the same site. The histopathology and the microbiological examination led to the diagnosis of the rare subcutaneous cystic type of chromomycosis.

  12. The role of laparoscopy and laparoscopic ultrasound in the diagnosis of cystic lesions of the pancreas.

    PubMed

    Schachter, Pinhas P; Shimonov, Mordechai; Czerniak, Abraham

    2002-10-01

    With the widespread use of advanced imaging techniques, cystic lesions of the pancreas are now diagnosed relatively frequently. The nature of these lesions vary from benign cysts (serous cvstadenoma) or an inflammatory process (pseudocyst), to premalignant (mucinous cystadenoma) or frankly malignant lesions (cystadenocarcinoma). Differentiation of various types of pancreatic cysts presents a diagnostic and therapeutic challenge, as clinical presentation may be vague. Laparoscopic ultrasonography (LAPUS), the biopsy of the cystic wall, and analysis of the cystic aspirate, although expensive and rather invasive procedures, significantly contribute to the differential diagnosis of pancreatic cystic lesions.

  13. 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

  14. Multicystic benign cystic mesothelioma presenting as a pelvic mass.

    PubMed

    Momeni, Mazdak; Pereira, Elena; Grigoryan, Gennadiy; Zakashansky, Konstantin

    2014-01-01

    Background. Benign cystic mesothelioma (BCM) is a rare tumor that arises from the abdominal peritoneum with a predilection to the pelvic peritoneum. For this reason, it can often mimic gynecologic malignancies. Case. A 47-year-old perimenopausal female presented reporting several weeks of abdominal distention associated with abdominal tenderness and constipation. Computed tomography revealed a 24 cm multiloculated pelvic mass, and tumor markers were notable for an elevated CA-125. The patient was taken to the operating room for an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingoophorectomy, and removal of pelvic mass. Final pathologic evaluation revealed a benign cystic mesothelioma. Conclusion. Classically these tumors present as large multicystic masses with thin-walled septations and on preoperative evaluation BCM can mimic many different disease entities including ovarian malignancies and cystic lymphangioma. Often diagnosis can only be made at time of surgery.

  15. Multicystic Benign Cystic Mesothelioma Presenting as a Pelvic Mass

    PubMed Central

    Pereira, Elena; Zakashansky, Konstantin

    2014-01-01

    Background. Benign cystic mesothelioma (BCM) is a rare tumor that arises from the abdominal peritoneum with a predilection to the pelvic peritoneum. For this reason, it can often mimic gynecologic malignancies. Case. A 47-year-old perimenopausal female presented reporting several weeks of abdominal distention associated with abdominal tenderness and constipation. Computed tomography revealed a 24 cm multiloculated pelvic mass, and tumor markers were notable for an elevated CA-125. The patient was taken to the operating room for an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingoophorectomy, and removal of pelvic mass. Final pathologic evaluation revealed a benign cystic mesothelioma. Conclusion. Classically these tumors present as large multicystic masses with thin-walled septations and on preoperative evaluation BCM can mimic many different disease entities including ovarian malignancies and cystic lymphangioma. Often diagnosis can only be made at time of surgery. PMID:24716035

  16. Differential tumor microenvironment in human ovarian cystic tumors.

    PubMed

    Tavares Murta, Beatriz Martins; Cunha, Fernando de Queiróz; Miranda, Rodrigo; Adad, Sheila Jorge; Murta, Eddie Fernando Candido

    2004-01-01

    Cells and soluble mediators obtained from tumor effusions are useful in evaluating the tumor microenvironment. Our aim was to examine cytologically and to quantify the leukocyte infiltrate, nitric oxide, cytokines and tumor markers in the intracystic fluid from patients with a cystic adnexal mass, for a possible differentiation between benign and malignant findings. Sixty-six women who had their cystic fluids collected were prospectively divided into benign tumor (22, 33.3%), malignant tumor (10, 15.2%) or other gynecological alterations (34, 51.5%). Cytology, total and differential leukocyte counts were determined by light microscopy. Tumor markers, cytokines and nitric oxide were assayed in the supernatants using the Immulite system, ELISA and Griess reaction, respectively. The sensitivity and specificity of the cytological analysis was 66.7% and 97.7%, respectively. The levels of CA 19.9, CA 15.3, alpha-fetoprotein, carcinoembryonic antigen, progesterone and beta-HCG were significantly higher in the benign and/or malignant group than in the other gynecological alterations. Also, the local concentrations of CA 15.3 and beta-HCG were significantly higher in malignant than in benign tumors. In malignant tumors, increased leukocyte counts and higher concentrations of IL-6, IL-10 and nitric oxide were detected than in benign tumors or other gynecological alterations. In malignant tumors, the microenvironment could be differentiated from benign tumors or other gynecological alterations by cystic fluid analysis.

  17. Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells: a rare case report and review of the literature.

    PubMed

    Sah, Shambhu K; Li, Ying; Li, Yongmei

    2015-01-01

    Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (UCPOGC) is an extremely rare non-endocrine pancreatic tumor. To date, some cases have been reported, however, histogenesis and biologic behavior of UCPOGC remain controversial. We report a case of an UCPOGC in a 54-year-old female, who presented with a three-month history of recurrent abdominal pain without any incentive. Abdominal computed tomography (CT) revealed a large cystic mass of 10.5 × 9.3 cm in the body and tail of the pancreas compressing the adjacent bowel loop and stomach. The preliminary diagnosis was considered as a malignant tumor of body and tail of the pancreas. The patient had open distal pancreatic mass resection with splenectomy and according to the results of histopathological and immunohistochemical studies, the diagnosis of an UCPOGC was established.

  18. Ex utero intrapartum treatment (EXIT) for congenital giant ranula.

    PubMed

    Chan, Dorothy F Y; Lee, Cheuk H; Fung, Tak Y; Chan, Daniel L W; Abdullah, Victor; Ng, Pak C

    2006-10-01

    Congenital giant ranula causing life-threatening upper airway obstruction is a rare condition. A well-planned ex utero intrapartum treatment (EXIT) procedure for aspiration and decompression of the cystic lesion is simple, efficacious and effective in securing a patent airway before the fetus is completely delivered, and may prevent lifelong neurodevelopmental complications associated with perinatal asphyxia in such cases. EXIT could be a life-saving procedure for congenital giant ranula.

  19. [Giant simple hepatic cysts as dyspnea symptom in a 93-year-old patient].

    PubMed

    Macho Pérez, O; Gómez Pavón, J; Núñez González, A; Narvaiza Grau, L; Albéniz Aguiriano, L

    2007-03-01

    Giant simple hepatic cysts is generally asymptomatic in the 3% of cases of adult patients. We present a woman case of 93 years old who was diagnoses of giant simple hepatic cyst presented as dysnea. The management of this patient was with percutaneous aspiration and fenol alcohol. It made a review of cystic lesions of the liver and of simple hepatic cysts management.

  20. Learning about Cystic Fibrosis

    MedlinePlus

    ... Cystic Fibrosis: Your Genes, Your Health [yourgenesyourhealth.org] Multimedia educational site including screening and treatment information. Hosted by the Dolan DNA Learning Center at Cold Spring Harbor Laboratory. What is ...

  1. Epidural Cystic Spinal Meningioma

    PubMed Central

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

    2016-01-01

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

  2. 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

  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. Copyright © 2013. Published by Elsevier Ltd.

  4. Malignancy and chronic renal failure.

    PubMed

    Peces, Ramon

    2003-01-01

    Increased incidence of cancer at various sites is observed in patients with end-stage renal disease (ESRD). Certain malignant diseases, such as lymphomas and carcinomas of the kidney, prostate, liver and uterus, show an enhanced prevalence compared with the general population. In particular, renal cell carcinoma (RCC) shows an excess incidence in ESRD patients. A multitude of factors, directly or indirectly associated with the renal disease and the treatment regimens, may contribute to the increased tumor formation in these patients. Patients undergoing renal replacement therapy (RRT) are prone to develop acquired cystic kidney disease (ACKD), which may subsequently lead to the development of RCC. In pre-dialysis patients with coexistent renal disease, as in dialysis and transplant patients, the presence of ACKD may predispose to RCC. Previous use of cytotoxic drugs (eg, cyclophosphamide) or a history of analgesic abuse, are additional risk factors for malignancy. Malignancy following renal transplantation is an important medical problem during the follow-up. The most common malignancies are lymphoproliferative disorders (early after transplantation) and skin carcinomas (late after transplantation). Another important confounder for risk of malignancy after renal transplantation is the type of immunosuppression. The type of malignancy is different in various countries and dependent on genetic and environmental factors. Finally, previous cancer treatment in a uremic patient on the transplant waiting list is of great importance in relation to waiting time and post-malignancy screening.

  5. 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 ...

  6. 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 ...

  7. What's it Like to Have Cystic Fibrosis?

    MedlinePlus

    ... CPR: A Real Lifesaver Kids Talk About: Coaches Cystic Fibrosis KidsHealth > For Kids > Cystic Fibrosis Print A A ... strength to deal with cystic fibrosis. What Is CF? Cystic fibrosis (CF) is a disease that causes ...

  8. 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

  9. Giant Cell Arteritis

    MedlinePlus

    ... Patient / Caregiver Diseases & Conditions Giant Cell Arteritis Giant Cell Arteritis Fast Facts Giant cell arteritis (GCA) is ... polymyalgia rheumatica (also called PMR). What is giant cell arteritis? GCA is a type of vasculitis or ...

  10. SYNOVIAL GIANT CELL TUMOR OF THE KNEE.

    PubMed

    Abdalla, Rene Jorge; Cohen, Moisés; Nóbrega, Jezimar; Forgas, Andrea

    2009-01-01

    Synovial giant cell tumor is a benign neoplasm, rarely reported in the form of malignant metastasis. Synovial giant cell tumor most frequently occurs on the hand, and, most uncommon, on the ankle and knee. In the present study, the authors describe a rare case of synovial giant cell tumor on the knee as well as the treatment approach. Arthroscopy has been shown, in this case, to be the optimal method for treating this kind of lesion, once it allowed a less aggressive approach, while providing good visualization of all compartments of knee joint and full tumor resection.

  11. SYNOVIAL GIANT CELL TUMOR OF THE KNEE

    PubMed Central

    Abdalla, Rene Jorge; Cohen, Moisés; Nóbrega, Jezimar; Forgas, Andrea

    2015-01-01

    Synovial giant cell tumor is a benign neoplasm, rarely reported in the form of malignant metastasis. Synovial giant cell tumor most frequently occurs on the hand, and, most uncommon, on the ankle and knee. In the present study, the authors describe a rare case of synovial giant cell tumor on the knee as well as the treatment approach. Arthroscopy has been shown, in this case, to be the optimal method for treating this kind of lesion, once it allowed a less aggressive approach, while providing good visualization of all compartments of knee joint and full tumor resection. PMID:27004193

  12. Accuracy of endoscopic ultrasound in the evaluation of cystic pancreatic neoplasms: a community hospital experience.

    PubMed

    Wright, Gerald Paul; Morrow, Jay Bradley; Shaheen, Michael; Goslin, Brent J; Baatenburg, Lorel; Chung, Mathew H

    2014-04-01

    Reports on the use of endoscopic ultrasound (EUS) in differentiating benign, premalignant, and malignant pancreatic lesions have been widely variable, particularly with cystic neoplasms. We evaluated the use of EUS for cystic pancreatic lesions in a community hospital setting. All patients who underwent EUS for cystic pancreatic neoplasms from 2007 to 2010 were reviewed. A final EUS diagnosis was determined based on the examiner's impression and fine-needle aspiration results if available. Lesions were stratified as benign, premalignant, or malignant. Patients underwent surgical resection, serial imaging studies, or medical oncology/palliative care consultation as indicated. One hundred eighteen patients with cystic pancreatic lesions underwent EUS during the study period. Endoscopic ultrasound diagnoses included 75 benign (63.6%), 35 premalignant (29.7%), and 8 malignant (4.2%) lesions. Thirty-eight patients (32.2%) underwent surgery, 77 (65.3%) were monitored with imaging, and 3 (2.5%) had unresectable malignancies. Elevated carcinoembryonic antigen levels showed a trend toward predicting mucinous cysts (P = 0.062). Accuracy, sensitivity, specificity, positive predictive value, and negative predictive value for cystic lesions were 87.3%, 86.8%, 87.5%, 76.7%, and 93.3%, respectively. Endoscopic ultrasound is a valuable diagnostic modality in the evaluation of cystic pancreatic neoplasms in a community hospital setting.

  13. MicroRNA from pancreatic duct aspirate differentiates cystic lesions of the pancreas.

    PubMed

    Henry, Jon C; Bassi, Claudio; Giovinazzo, Francesco; Bloomston, Mark

    2013-12-01

    Prognostication for cystic neoplasms of the pancreas continues to evolve. Beyond simple size and cystic fluid CEA determination, microRNA (miRNA) detection holds great promise as molecular diagnostics for cancer risk. In this study, we sought to identify miRNAs that could predict malignant potential of pancreatic cystic lesions. RNA was harvested from the pancreatic duct aspirate of 72 cystic neoplasms of the pancreas. Samples with adequate RNA concentration (≥ 3 ng/μL) were selected for qRTPCR profiling using assays to 379 of the most common miRNAs. miRNA profiles were correlated with histopathology from resected specimens and grouped by benign (serous cystadenomas), premalignant (intraductal papillary mucinous neoplasms and mucinous cystadenomas), or malignant lesions (adenocarcinoma). Adequate RNA for analysis was obtained from 42 (58.3 %) of the samples. Malignant lesions were more likely to have adequate RNA (n = 17, 81 %) than either benign (n = 6, 33 %) or premalignant lesions (n = 19, 59 %; p = 0.011). Nine miRNA were identified as differentially expressed between benign and premalignant/malignant lesions (p < 0.05). A significant correlation was found between the number of differentially expressed miRNA and the likelihood of a premalignant/malignant lesion. All premalignant or malignant lesions expressed at least one miRNA surpassing the threshold of mean miRNA expression, whereas no benign lesions had more than one miRNA surpassing the threshold. The presence of RNA in the duct aspirate from patients with pancreatic cystic neoplasms may be a predictor of premalignancy or malignancy. miRNA may be utilized to further differentiate between benign, premalignant, and malignant cystic lesions of the pancreas.

  14. Adenoid Cystic Carcinoma of the Trachea Resulting in Fatal Asphyxia.

    PubMed

    Huston, Butch; Froloff, Victor; Mills, Kelly; McGee, Michael

    2017-01-01

    Primary malignant tumors of the trachea are uncommon. The authors report a case of a 72-year-old female who died from asphyxia due to an undiagnosed obstructing adenoid cystic carcinoma of the trachea. The decedent became unresponsive while eating cereal and was pronounced upon arrival at the local hospital. The autopsy revealed a near occlusive tumor mass just superior to the bifurcation of the distal trachea. There was no evidence of aspiration. The death was the result of asphyxia due to obstruction of the trachea by an adenoid cystic carcinoma. This case demonstrates that an obstructive tumor mass may remain undiagnosed until an obstructive episode results in a sudden death.

  15. Rare liver tumor: symptomatic giant von Meyenburg complex

    PubMed Central

    Singh, Yardesh; Cawich, Shamir O.; Ramjit, Chunilal; Naraynsingh, Vijay

    2016-01-01

    von Meyenburg complexes are hamartomas that arise from intra-hepatic bile ducts. Symptomatic lesions are uncommon and giant lesions are exceedingly rare. When encountered, they should be excised because there are reports of malignant change in large, symptomatic lesions. We report a case of a symptomatic giant von Meyenburg complex. PMID:28068648

  16. Cystic and ductal tumors of the pancreas: diagnosis and management.

    PubMed

    Scoazec, J Y; Vullierme, M P; Barthet, M; Gonzalez, J M; Sauvanet, A

    2013-04-01

    Incidentally discovered cystic tumors of the pancreas (CTP) are an increasingly frequent entity. It is essential to differentiate lesions whose malignant potential is either nil or negligible (pseudocyst, serous cystadenoma, simple cysts) from lesions with intermediate malignant potential (intraductal papillary mucinous tumor of the pancreas [IPMN] involving the secondary ducts, cystic endocrine tumor) or those with high malignant potential (mucinous cystadenoma, solid pseudopapillary tumors and IPMN involving the main pancreatic duct). The approach to defining malignant potential is based on diagnostic CT scan, magnetic resonance imaging (MRI), and endoscopic ultrasound (EUS), often complemented by EUS-guided cyst puncture for biochemical and cytological analysis of cyst fluid. Surgery for diagnostic purposes should be avoided because of its significant morbidity. For pseudocysts, simple cysts and serous cystadenomas, abstention is the general rule. Resection, preserving as much pancreatic parenchyma as possible, is the rule for IPMN involving the main pancreatic duct, mucinous cystadenomas, solid and pseudopapillary tumors, and cystic endocrine tumors. Resection is rarely indicated at the outset for IPMN involving secondary pancreatic ducts; morphologic observation is the general rule and preventive excision may be indicated secondarily. Good collaboration between surgeons, radiologists and endosonographists is necessary for optimal management of CTP.

  17. [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.

  18. Ovarian mature cystic teratoma with fistula formation into the rectum: a case report.

    PubMed

    Kizaki, Yuichiro; Nagai, Tomonori; Ohara, Ken; Gomi, Yosuke; Akahori, Taichi; Ono, Yoshihisa; Matsunaga, Shigetaka; Takai, Yasushi; Saito, Masahiro; Baba, Kazunori; Seki, Hiroyuki

    2016-01-01

    While ovarian mature cystic teratomas are benign ovarian germ-cell tumors and the most common type of all ovarian tumors, the formation of fistulas into surrounding organs such as the bladder and the intestinal tract is extremely rare. This report documents a case of ovarian mature cystic teratoma with a rectal fistula, thought to be caused by local inflammation. A pelvic mass was diagnosed as an ovarian mature cystic teratoma of approximately 10 cm in diameter on transvaginal ultrasound and magnetic resonance examinations. Endoscopic examination of the lower gastrointestinal tract to investigate diarrhea revealed an ulcerative lesion with hair in the rectal wall adjacent to the ovarian cyst, and formation of a fistula from the ovarian teratoma into the rectum was suspected. Laparotomy revealed extensive inflammatory adhesions between a left ovarian tumor and the rectum. Left salpingo-oophorectomy and upper anterior resection of the rectum were performed. The final pathological diagnosis was ovarian mature cystic teratoma with no malignant findings, together with severe rectal inflammation and fistula formation with no structural disorders such as diverticulitis of the colon or malignant signs. The formation of fistulas and invasion into the neighboring organs are extremely rare complications for ovarian mature cystic teratomas. The invasion of malignant cells into neighboring organs due to malignant transformation of the tumor is reported as the cause of fistula formation into the neighboring organs. A review of 17 cases including the present case revealed that fistula formation due to malignant transformation comprised only 4 cases (23.5 %), with inflammation as the actual cause in the majority of cases (13 cases, 76.5 %). Although malignancy is the first consideration when fistula formation is observed between ovarian tumors and surrounding organs, in mature cystic teratoma, local inflammation is more likely than malignant transformation.

  19. Cystic carcinoma of the breast: a trap for the unwary.

    PubMed

    Ravichandran, D; Carty, N J; al-Talib, R K; Rubin, C; Royle, G T; Taylor, I

    1995-03-01

    Cystic breast masses are a common presentation to breast clinics. While the majority of cysts can be managed by simple aspiration, a small proportion are malignant. Histology records for a 10-year period have been examined to identify patients with cystic breast carcinomas. In all, 31 patients were identified. Of these, 18 had cystic degeneration of high-grade tumours, while 13 had intracystic papillary carcinoma. Both of these tumour types were diagnosed by a combination of cyst fluid cytology and breast imaging. The prognosis of high-grade tumours was poor, while that of intracystic papillary carcinomas was excellent. After cyst aspiration, bloodstained fluid should be sent for cytology and breast imaging arranged in all patients. Patients in whom a cyst refills within 2 week of aspiration require a careful re-evaluation. Cysts in postmenopausal women should be viewed with suspicion. Excision should be performed in patients with positive cytology or imaging.

  20. [Giant esophageal fibrovascular polyp].

    PubMed

    Palacios, Fernando; Contardo, Carlos; Guevara, Jorge; Vera, Augusto; Aguilar, Luis; Huamán, Manuel; Palomino, Américo; Yabar, Alejandro

    2003-01-01

    Fibrovascular polyps are extremely rare benign neoplasias of the esophagus, which usually originate in the lower cricoid area. They do not produce any discomfort in the patient for a long time, however it may make itself evident by the patient's regurgitation of the polyp, producing asphyxia or, more frequently, dysphagia. The case of a 58 year old male patient is presented herein, with a 9 month record of dysphagia, weight loss and intermittent melena. The barium x-ray showed a distended esophagus, with a tumor running from the upper esophageal sphincter to the cardia. The endoscopy confirmed the presence of a pediculated tumor, implanted in the cervical esophagus. Surgeons suspected the potential malignancy of the tumor and performed a transhiatal esophagectomy. The final pathologic diagnosis was giant fibrovascular esophageal polyp.

  1. Mesothelioma - malignant

    MedlinePlus

    ... Names Mesothelioma - malignant; Malignant pleura mesothelioma (MPM) Images Respiratory system References Broaddus VC, Robinson BWS. Pleural tumors. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine . 6th ed. Philadelphia, PA: Elsevier Saunders; 2016: ...

  2. Pleural malignancies.

    PubMed

    Friedberg, Joseph S; Cengel, Keith A

    2010-07-01

    Pleural malignancies, primary or metastatic, portend a grim prognosis. In addition to the serious oncologic implications of a pleural malignancy, these tumors can be highly symptomatic. A malignant pleural effusion can cause dyspnea, secondary to lung compression, or even tension physiology from a hydrothorax under pressure. The need to palliate these effusions is a seemingly straightforward clinical scenario, but with nuances that can result in disastrous complications for the patient if not attended to appropriately. Solid pleural malignancies can cause great pain from chest wall invasion or can cause a myriad of morbid symptoms because of the invasion of thoracic structures, such as the heart, lungs, or esophagus. This article reviews pleural malignancies, the purely palliative treatments, and the treatments that are performed with definitive (curative) intent. Copyright 2010 Elsevier Inc. All rights reserved.

  3. Cystic lesions in the pancreas: when to watch, when to resect.

    PubMed

    Balcom IV, J H; Fernandez-Del Castillo, C; Warshaw, A L

    2000-04-01

    The diagnosis of cystic lesions in the pancreas is becoming more common, largely due to the increases in diagnostic imaging done for other reasons. This review considers pseudocysts, mucinous cystic neoplasms, intraductal papillary mucinous tumors, and serous cystadenomas in some detail. The emphasis is on the fact that, through a careful history, physical examination, radiologic studies, and, often, cyst fluid analysis, a diagnosis can be reached expeditiously. This pursuit is important because two thirds of pancreatic cystic neoplasms are malignant or premalignant and should be resected, whereas pseudocysts and serous cystadenomas are benign, and, depending on the case, may be treated through observation, resection, or, for pseudocysts, by internal drainage.

  4. Overview of the investigation and management of cystic neoplasms of the pancreas.

    PubMed

    Spence, Robert A J; Dasari, Bobby; Love, Mark; Kelly, Barry; Taylor, Mark

    2011-01-01

    Cystic neoplasms of the pancreas contribute to 10-20% of pancreatic tumours. Malignant cystic tumours of the pancreas behave similar to adenocarcinomas and thus warrant aggressive management. However, certain benign cystic neoplasms do not require operative intervention. It is, therefore, important to differentiate benign lesions from malignant lesions and from those with malignant potential. To provide an overview of the role of radiological investigations in the management of cystic neoplasms of the pancreas, with emphasis on the characteristic features of aggressive tumours. The role of different imaging modalities is discussed, and an investigative algorithm suggested. A literature review was carried out on Medline, Cochrane library, and PubMed using the MeSH terms 'pancreas' and 'cysts' to source relevant papers. Search criteria were limited to English literature, meta-analyses, systematic reviews, prospective and retrospective case series, published during or after 1998. Each pancreatic cystic lesion has characteristic radiological findings. However, the diagnostic accuracy of individual imaging techniques is still limited. A combination of imaging modalities is essential for preoperative diagnosis. CT complemented by endoscopic ultrasound and cyst fluid analysis appears to be the most promising investigation in diagnosing cystic neoplasms. Follow-up with serial imaging is useful for lesions of uncertain aetiology. Copyright © 2011 S. Karger AG, Basel.

  5. Cystic fibrosis: a clinical view.

    PubMed

    Castellani, Carlo; Assael, Baroukh M

    2017-01-01

    Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male genital system, intestine, liver, bone, and kidney are involved. The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. Previously considered lethal in infancy and childhood, CF has now attained median survivals of 50 years of age, mainly thanks to the early diagnosis through neonatal screening, recognition of mild forms, and an aggressive therapeutic attitude. Classical treatment includes pancreatic enzyme replacement, respiratory physiotherapy, mucolitics, and aggressive antibiotic therapy. A significant proportion of patients with severe symptoms still requires lung or, less frequently, liver transplantation. The great number of mutations and their diverse effects on the CFTR protein account only partially for CF clinical variability, and modifier genes have a role in modulating the clinical expression of the disease. Despite the increasing understanding of CFTR functioning, several aspects of CF need still to be clarified, e.g., the worse outcome in females, the risk of malignancies, the pathophysiology, and best treatment of comorbidities, such as CF-related diabetes or CF-related bone disorder. Research is focusing on new drugs restoring CFTR function, some already available and with good clinical impact, others showing promising preliminary results that need to be confirmed in phase III clinical trials.

  6. Primary retroperitoneal mature cystic teratoma with focal enteric type adenocarcinoma in a post-partum woman: report of a case with literature review.

    PubMed

    Hong, Wei; Dumoff, Kimberly L; Torigian, Drew A; Bing, Zhanyong

    2013-02-11

    Teratomas are characterized by containing tissue from all three germinal cell layers. Occasionally, somatic type malignancies develop within a mature cystic teratoma. We reported here a rare case of enteric type adenocarcinoma, with associated dysplastic epithelial precursor lesion, arising within a mature cystic teratoma in the retroperitoneum of a 30-year-old woman status post vaginal delivery 11 weeks earlier. The mass is 17.5 cm and cystic. A polypoid mass component measuring 4.7×4.2×2.5 cm was located inside the cystic component. Microscopically, the majority of the specimen was a mature cystic teratoma with all three germinal cell layers. The polypoid mass component was an adenocarcinoma with an adjacent dysplastic epithelial precursor lesion. The adenocarcinoma was diffusely positive for CK20 and CDX-2, and focally positive for CD7, indicating enteric differentiation. A brief review of retroperitoneal mature cystic teratomas with associated somatic type malignancy was performed.

  7. Pathologic Review of Cystic and Cavitary Lung Diseases

    PubMed Central

    Kim, Na Rae

    2012-01-01

    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

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

    PubMed

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

    2016-05-01

    The accessory parotid gland is salivary gland tissue separated from the main gland at a variable distance. This gland is histologically similar to the main gland, but has a higher incidence of malignant neoplasms than the main gland. Regarding the various malignant neoplasms, studies have shown higher incidences of mucoepidermoid carcinoma, with less than 2% being adenoid cystic carcinoma. We present a case of swelling in the midcheek region that, after clinical examination, was diagnosed as a case of neoplasm of the accessory parotid gland. On the basis of auxiliary investigations including intraoperative frozen section, it was concluded that it was adenoid cystic carcinoma, grade I, and after wide surgical resection, the tumor was removed without undergoing superficial parotidectomy. The patient received postoperative radiotherapy (RT) and was followed for 14 months without any recurrence or substantial facial asymmetry. Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  9. Infantile Cystic Hygroma: An Unusual Perioperative Course

    PubMed Central

    Saini, Suman; Dayal, Madhu; Gupta, Amita

    2017-01-01

    Airway management of an infant with a giant cervical lump may be a difficult task. The anesthesiologist must be prepared to face associated challenges during securing the airway in such patients. We report our experience with One year old infant who presented with huge cystic hygroma in the cervical region leading to recurrent episodes of respiratory tract infection and distress. Surgical removal was needed as sclerotherapy proved ineffective in reducing its size. Proseal laryngeal mask airway was used as a conduit after inhalational induction since airway could not be maintained with bag and mask. The child was tracheostomized postoperatively and also had a prolonged Intensive Care Unit stay. Difficulties encountered in intubation and postoperative management of this child are discussed in this report.

  10. Sebaceous carcinoma arising in mature cystic teratoma of ovary.

    PubMed

    An, Hyo Jeong; Jung, Yong Han; Yoon, Hye Kyoung; Jung, Soo Jin

    2013-08-01

    Roughly 1% of mature cystic teratomas undergo malignant transformation. In particular, cutaneous-type adnexal neoplasms may occur in mature cystic teratomas. Sebaceous carcinomas, which arise from mature cystic teratomas, have rarely been observed, with only seven cases previously reported. Here, we present a case of a 69-year-old female who had pelvic pain for two weeks and who subsequently underwent bilateral salpingo-oophorectomy and hysterectomy. Her left ovary showed a unilocular cyst, measuring 22.0 cm in diameter, filled with sebaceous material and a few hairs. A luminally-protruding solid mass measuring 4.0 cm in diameter was also noted. Microscopic findings revealed lobular or diffusely arranged basophilic, atypical sebaceous cells connected to a typical mature cystic teratoma. Tumor cells demonstrated positive immunoreactivity for high molecular weight cytokeratin, cytokeratin 7, cytokeratin 19, epithelial membrane antigen, and carcinoembryonic antigen. Here, we present a case of sebaceous carcinoma arising from a mature cystic teratoma along with a review of previously published reports.

  11. 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

  12. Computed tomography of primary intrahepatic biliary malignancy

    SciTech Connect

    Itai, Y.; Araki, T.; Furui, S.; Yashiro, N.; Ohtomo, K.; Iio, M.

    1983-05-01

    Fifteen patients with primary intrahepatic biliary malignancy (cholangiocarcinoma in 13, biliary cystadenocarcinoma in two) were examined by computed tomography (CT). The CT features were classified into three types: (A) a well-defined round cystic mass with internal papillary projections, (B) a localized intrahepatic biliary dilatation without a definite mass lesion, and (C) miscellaneous low-density masses. Intraphepatic biliary dilatation was noted in all cases of Types A and B and half of those of Type C; dilatation of extrahepatic bile ducts occurred in 4/4, 1/3, and 0/8, respectively. CT patterns, such as a well-defined round cystic mass with papillary projections or dilatation of intra- and extrahepatic ducts, give important clues leading to a correct diagnosis of primary intrahepatic biliary malignancy.

  13. A Challenging Giant Dermatofibrosarcoma Protuberans on the Face

    PubMed Central

    Pérez, Gimena Castro; Arias, Cintia; Luna, Paula; Sorín, Irene

    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

  14. Analysis of clinical characteristics and treatment of pancreatic cystic tumors

    PubMed Central

    You, Lei; Xiao, Jianchun; Cao, Zhe; Zhang, Wanying; Liao, Quan; Dai, Menghua; Zhang, Taiping; Zhao, Yupei

    2016-01-01

    Objective To summarize experience in the diagnosis and treatment of pancreatic cystic neoplasms. Methods This is a retrospective study of 207 patients who were diagnosed with pancreatic cystic tumors at Peking Union Medical College Hospital between Jan 2009 and Mar 2014. Clinical data, such as clinical manifestations, radiological and pathological images and surgical recordings, were collected. Results Of the 207 included patients, females accounted for 76.81%, and the mean patient age was 52.04 years. Malignancy was more common in older patients who presented with marasmus and jaundice. Other risk factors included solid components in the tumor, a large tumor size, and elevated levels of tumor markers. Surgical treatment was required when a malignant tumor was suspected. The operation approach was selected based on the location, size and characteristics of the tumor. The position of the tumor relative to the pancreatic duct also played a significant role. Conclusions No specific symptoms were observed for the patients with pancreatic cystic tumors. Imaging played an important role in making a differential diagnosis. Furthermore, surgical treatment should be proposed for patients with significant symptoms and potentially malignant tumors. The tumor resection rate is high, suggestive of good prognosis. PMID:27877011

  15. Adenocarcinoma arising from the gastrointestinal epithelium in benign cystic teratoma of the ovary.

    PubMed

    Fishman, A; Edelstein, E; Altaras, M; Beyth, Y; Bernheim, J

    1998-09-01

    Malignant transformation of benign cystic teratoma of the ovary is rare, with an incidence of 1.8%. The commonest malignant neoplasm to develop is squamous carcinoma (80%). Adenocarcinoma occurs with less frequency, and only one of which has ever been cited to be of gastrointestinal origin. A 38-year-old female underwent TAH-BSO due to a large right ovarian tumor. Microscopically and immunohistochemically, the tumor was defined as mucinous adenocarcinoma originating from gastrointestinal epithelium in benign cystic teratoma. Copyright 1998 Academic Press.

  16. Multimodality imaging of ovarian cystic lesions: Review with an imaging based algorithmic approach

    PubMed Central

    Wasnik, Ashish P; Menias, Christine O; Platt, Joel F; Lalchandani, Usha R; Bedi, Deepak G; Elsayes, Khaled M

    2013-01-01

    Ovarian cystic masses include a spectrum of benign, borderline and high grade malignant neoplasms. Imaging plays a crucial role in characterization and pretreatment planning of incidentally detected or suspected adnexal masses, as diagnosis of ovarian malignancy at an early stage is correlated with a better prognosis. Knowledge of differential diagnosis, imaging features, management trends and an algorithmic approach of such lesions is important for optimal clinical management. This article illustrates a multi-modality approach in the diagnosis of a spectrum of ovarian cystic masses and also proposes an algorithmic approach for the diagnosis of these lesions. PMID:23671748

  17. Malignant hyperthermia

    MedlinePlus

    ... counseling is recommended for anyone with a family history of myopathy, muscular dystrophy, or malignant ... et al, eds. Harrison's Principles of Internal Medicine . 17th ed. [online version]. New York, NY: McGraw ...

  18. Cystic Fibrosis: Prenatal Screening and Diagnosis

    MedlinePlus

    ... Management Education & Events Advocacy For Patients About ACOG Cystic Fibrosis: Prenatal Screening and Diagnosis Home For Patients Search ... Screening and Diagnosis FAQ171, June 2017 PDF Format Cystic Fibrosis: Prenatal Screening and Diagnosis Pregnancy What is cystic ...

  19. The Cystic Fibrosis Intestine

    PubMed Central

    De Lisle, Robert C.; Borowitz, Drucy

    2013-01-01

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

  20. Abdominal cystic lymphangioma mimicking appendicitis.

    PubMed

    Wake, Sarah; Abhyankar, Aruna; Hutton, Kim

    2013-06-01

    A cystic lymphangioma arising within the abdomen is a rare entity in children. It may present with an abdominal mass and symptoms of abdominal pain, vomiting, and anorexia. These nonspecific clinical symptoms are often attributed to more common acute pediatric conditions. In this report, we describe two pediatric cases of intra-abdominal cystic lymphangioma that were initially diagnosed and treated as appendicitis. True diagnosis was only achieved on surgical excision and pathological investigation of cystic material.

  1. Benign cystic peritoneal mesothelioma.

    PubMed Central

    Bhandarkar, D S; Smith, V J; Evans, D A; Taylor, T V

    1993-01-01

    The well defined but rare entity of benign cystic mesothelioma is reported. The aetiology of this neoplasm remains obscure. The presenting features make a precise preoperative diagnosis difficult; information provided by computed tomography and cytology may help. A firm diagnosis can only come from electron microscopic or immunohistochemical examination of the tumour. Diagnostic accuracy and diligent follow up are essential because, although the tumour is considered benign, it does tend towards local recurrence. Images PMID:8227441

  2. 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

  3. [News in cystic fibrosis].

    PubMed

    Delaisi, B

    2013-08-01

    The improvement over the last two decades in the treatment of cystic fibrosis led to an increase in life expectancy approaching 40 years at birth. Logically, the population of adult patients has been increasing and is currently 50% of patients followed in France. These therapeutic advances have justified the establishment in 2003 of a generalized neonatal screening for cystic fibrosis. The latest data of this screening show an incidence of CF of 1/5359 live births, far below the incidence of 1/2500 which was widely accepted twenty years ago. The performance of this screening is currently based on the dosage of trypsin immuno reactive, followed in case of exceeding the threshold of a search of the 30 most common mutations, can detect around 96% of 150 to 200 CF cases every year. Therefore, the possibility of a false negative of the screening cannot be excluded and evocative symptoms of cystic fibrosis, even for children born after 2003, will lead to prescribe a sweat test. While treatments available so far goal consequences of cystic fibrosis, a new therapeutic class to correct the functional defect of the mutated protein, called CFTR modulators, is emerging. Ivacaftor, leader of this new class, belonging to the category of "CFTR potentiator" got its access on the market in September 2012 for patients carrying the G551D mutation. New other molecules, named "CFTR correctors" which can have synergistic effect with ivacaftor and concern patients carrying the most common mutation--DF 508--are under development. Copyright © 2013. Published by Elsevier Masson SAS.

  4. Primary carcinoma of the cystic duct: a case report and review of classifications.

    PubMed

    Bains, Lovenish; Kaur, Daljit; Kakar, Arun Kumar; Batish, Aman; Rao, Seema

    2017-01-19

    The incidence of extrahepatic bile duct malignancies is about 2-3.6% of all gastrointestinal malignancies. Primary carcinoma of cystic duct is a rare condition comprising a fraction of all extrahepatic bile duct malignancies with less than 70 cases reported worldwide. Majority of these cases were reported from East Asia. There is paucity in such case being reported from Indian subcontinent. We present a case of primary carcinoma of the cystic duct encountered during laparoscopic cholecystectomy. A 65-year-old lady presented to us with symptomatic gall stone disease. Investigations revealed a distended gall bladder with multiple stones. Patient was taken up for laparoscopic cholecystectomy, during surgery a stony hard structure was found at cystic duct-common bile duct junction which was not amenable for clear dissection. Procedure was converted to open, and the patient underwent cholecystectomy with resection of common bile duct with Roux-en-Y hepaticojejunostomy and regional lymphadenectomy. Histopathological findings revealed it to be moderately differentiated adenocarcinoma of the cystic duct. Primary carcinoma of cystic duct is a rare condition where early diagnosis can be difficult and if accidentally detected may add to surgeon's dilemma. Proper surgery with en-bloc resection of gallbladder, cystic duct, common bile duct, and regional lymphadenectomy is the mainstay of treatment. The prognosis of carcinoma of cystic duct is better than extrahepatic bile duct malignancies. The old classification system has outlived its time and is more rigid in definition which is not practical in advanced cases; the new classification systems of this century offer better insight into understanding the tumor characteristics and prognosis.

  5. Malignant transformation in an anal condyloma acuminatum.

    PubMed

    Ejeckam, G C; Idikio, H A; Nayak, V; Gardiner, J P

    1983-03-01

    A 61-year-old man had malignant transformation of an anal condyloma acuminatum, demonstrated by light and electron microscopic studies. Intranuclear virus-like particles were seen in the benign condylomatous koilocytotic cells but these were absent in the malignant cells. Multinucleation, syncytial giant cells and nuclear atypia in a condyloma acuminatum are considered features of in-situ carcinomatous change. Anal condyloma acuminatum requires wide excision and thorough examination of anorectal canal in order to exclude hidden disease, which will predispose to recurrence. Homosexuality is considered a predisposing factor. The authors stress the importance of histopathologic examination of all anorectal warts to exclude malignant change.

  6. Cystic fibrosis and coeliac disease

    PubMed Central

    Goodchild, Mary C.; Nelson, R.; Anderson, Charlotte M.

    1973-01-01

    Two children, unrelated, in whom cystic fibrosis had been diagnosed at the ages of 14 weeks and 10 months, respectively, were found subsequently to suffer from coeliac disease as well. Both children are responding well to dietary gluten withdrawal and to conventional treatment for cystic fibrosis. It is suggested that cystic fibrosis may predispose to the development of coeliac disease. Therefore, jejunal biopsy may be a useful investigation in the occasional child with cystic fibrosis, who presents with unusual features, and who fails to thrive as well as expected. ImagesFIG. 2FIG. 3FIG. 5FIG. 6 PMID:4517654

  7. Neonatal cystic fibrosis screening test

    MedlinePlus

    Cystic fibrosis screening - neonatal; Immunoreactive trypsinogen; IRT test; CF - screening ... better nutrition, growth, and lung function. This screening test helps doctors identify children with CF before they ...

  8. Are the Sendai and Fukuoka consensus guidelines for cystic mucinous neoplasms of the pancreas useful in the initial triage of all suspected pancreatic cystic neoplasms? A single-institution experience with 317 surgically-treated patients.

    PubMed

    Goh, Brian K P; Tan, Damien M Y; Thng, Choon-Hua; Lee, Ser-Yee; Low, Albert S C; Chan, Chung-Yip; Wong, Jen-San; Lee, Victor T W; Cheow, Peng-Chung; Chow, Pierce K H; Chung, Alexander Y F; Wong, Wai-Keong; Ooi, London L P J

    2014-06-01

    The Sendai Consensus Guidelines (SCG) were formulated in 2006 and updated in Fukuoka in 2012 (FCG) to guide management of cystic mucinous neoplasms of the pancreas. This study aims to evaluate the clinical utility of the SCG and FCG in the initial triage of all suspected pancreatic cystic neoplasms. Overall, 317 surgically-treated patients with a suspected pancreatic cystic neoplasm were classified according to the SCG as high risk (HR(SCG)) and low risk (LR(SCG)), and according to the FCG as high risk (HR(FCG)), worrisome (W(FCG)), and low risk (LR(FCG)). Cystic lesions of the pancreas (CLP) were classified as potentially malignant/malignant or benign according to the final pathology. The presence of symptoms, proximal lesions with obstructive jaundice, elevated serum carcinoembryonic antigen/carbohydrate antigen 19-9 (CEA/CA 19-9), size ≥3 cm, presence of solid component, main pancreatic duct dilatation, thickened enhancing walls, and change in ductal caliber with distal atrophy were predictive of a potentially malignant/malignant CLP on univariate analyses. The positive predictive value (PPV) and negative predictive value (NPV) of HR(SCG) and HR(ICG2012) for a potentially malignant/malignant lesion was 67 and 88 %, and 88 and 92.5 %, respectively. There were no malignant lesions in both LR groups but some potentially malignant lesions such as cystic pancreatic neuroendocrine neoplasms with uncertain behavior were classified as LR. The updated FCG was superior to the SCG for the initial triage of all suspected pancreatic cystic neoplasms. CLP in the LR(FCG) group can be safely managed conservatively, and those in the HR(FCG) group should undergo resection.

  9. Multilocular Cystic Renal Cell Carcinoma or Cystic Nephroma?

    PubMed Central

    Cortez-Betancourt, Roberto; Alías-Melgar, Alejandro; Botello-Gómez, Pedro Jair; Ramírez-Garduño, Emilio; Trujillo-Vázquez, Eric Iván; Torres-Santos, Yosimart; Mata-Martínez, José Antonio; Carreño- de la Rosa, Fernando

    2016-01-01

    The incidence of Multilocular cystic renal cell carcinoma (MCRCC) in literature is very low and confounding MCRCC with cystic nephroma (CN) is even more unusual. The aim of this report is to present a case of MCRCC and emphasize the importance of the preoperative radiologic evaluation and immunohistochemical staining confirmation to obtain an accurate diagnosis. A 73-year-old woman presented with a history of 4-month right flank pain. CT showed a Bosniak type III renal mass. After laparoscopic partial nephrectomy the initial report was cystic nephroma. Immunohistochemical staining was performed being positive for Epithelial Membrane Antigen thus changing the diagnosis to MCRCC. Multilocular cystic renal cell carcinoma cannot reliably be distinguished from cystic nephroma neither by physical examination nor by radiologic evaluation; immunohistochemical staining assay is useful to differentiate between these conditions allowing an accurate diagnosis and proper follow-up. PMID:28074169

  10. Malignant mesothelioma

    PubMed Central

    Moore, Alastair J; Parker, Robert J; Wiggins, John

    2008-01-01

    Malignant mesothelioma is a fatal asbestos-associated malignancy originating from the lining cells (mesothelium) of the pleural and peritoneal cavities, as well as the pericardium and the tunica vaginalis. The exact prevalence is unknown but it is estimated that mesotheliomas represent less than 1% of all cancers. Its incidence is increasing, with an expected peak in the next 10–20 years. Pleural malignant mesothelioma is the most common form of mesothelioma. Typical presenting features are those of chest pain and dyspnoea. Breathlessness due to a pleural effusion without chest pain is reported in about 30% of patients. A chest wall mass, weight loss, sweating, abdominal pain and ascites (due to peritoneal involvement) are less common presentations. Mesothelioma is directly attributable to occupational asbestos exposure with a history of exposure in over 90% of cases. There is also evidence that mesothelioma may result from both para-occupational exposure and non-occupational "environmental" exposure. Idiopathic or spontaneous mesothelioma can also occur in the absence of any exposure to asbestos, with a spontaneous rate in humans of around one per million. A combination of accurate exposure history, along with examination radiology and pathology are essential to make the diagnosis. Distinguishing malignant from benign pleural disease can be challenging. The most helpful CT findings suggesting malignant pleural disease are 1) a circumferential pleural rind, 2) nodular pleural thickening, 3) pleural thickening of > 1 cm and 4) mediastinal pleural involvement. Involvement of a multidisciplinary team is recommended to ensure prompt and appropriate management, using a framework of radiotherapy, chemotherapy, surgery and symptom palliation with end of life care. Compensation issues must also be considered. Life expectancy in malignant mesothelioma is poor, with a median survival of about one year following diagnosis. PMID:19099560

  11. A Giant Lymphatic Cyst of the Transverse Colon Mesentery

    PubMed Central

    Batool, Tayyaba; Ahmed, Soofia

    2010-01-01

    Mesenteric cysts are not uncommon in pediatric age group but giant lymphatic cysts of mesentery are reported infrequently. This is a report of six years old female who had vague abdominal pain with distension for two years. Investigations revealed a large cystic mass in abdomen. On exploration a giant lymphatic cyst in the mesentery of transverse colon found. More than 1500 ml of milky fluid was drained. The cyst was unilocular and appeared to be the collection of lymph (chyle) between two leaves of the mesentery of transverse colon. It is postulated that trauma to or malformation of lymphatics at the root of mesentery might have lead to this pathology. PMID:22953250

  12. A giant lymphatic cyst of the transverse colon mesentery.

    PubMed

    Batool, Tayyaba; Ahmed, Soofia; Akhtar, Jamshed

    2010-01-01

    Mesenteric cysts are not uncommon in pediatric age group but giant lymphatic cysts of mesentery are reported infrequently. This is a report of six years old female who had vague abdominal pain with distension for two years. Investigations revealed a large cystic mass in abdomen. On exploration a giant lymphatic cyst in the mesentery of transverse colon found. More than 1500 ml of milky fluid was drained. The cyst was unilocular and appeared to be the collection of lymph (chyle) between two leaves of the mesentery of transverse colon. It is postulated that trauma to or malformation of lymphatics at the root of mesentery might have lead to this pathology.

  13. Cystic Fibrosis: Diet and Nutrition

    MedlinePlus

    ... Getting an X-ray Cystic Fibrosis: Diet and Nutrition KidsHealth > For Kids > Cystic Fibrosis: Diet and Nutrition Print A A A What's in this article? ... is someone who knows all about food and nutrition. Each kid is different, but most kids with ...

  14. Cystic Fibrosis: Diet and Nutrition

    MedlinePlus

    ... in the Operating Room? Cystic Fibrosis: Diet and Nutrition KidsHealth > For Kids > Cystic Fibrosis: Diet and Nutrition A A A What's in this article? CF ... is someone who knows all about food and nutrition. Each kid is different, but most kids with ...

  15. Cystic peritoneal mesothelioma: report of a case.

    PubMed

    Cavallaro, Andrea; Berretta, Massimiliano; Lo Menzo, Emanuele; Cavallaro, Vincenzo; Zanghì, Antonio; Di Vita, Maria; Cappellani, Alessandro

    2011-01-01

    Benign multicystic peritoneal mesothelioma (BMPM) is a rare disease with good short-term prognosis and rare malignant transformation. However, its biological significance remains unexplained. A neoplastic origin is considered by many authors to require a surgical excision, based on the high recurrence and progressive growth rate of the tumors. However, alternative or integrative treatment options have also been proposed. A 45-year-old woman presented to our unit with a history of occasional discomfort and pain in the left hip. On physical examination, we noticed a tough-elastic, fixed mass located in the iliac fossa. Computed tomography scan detected a mass with multiseptated cystic-like areas. Due to the similarity of these findings to a primitive sarcomatous tumor of the retroperitoneum, an arteriographic study was also performed. The patient underwent en bloc resection of the mass, including a segment of the sigmoid colon. The final pathologic diagnosis was cystic mesothelioma. Further studies are needed to better understand the etiology and pathogenesis of this rare disease, and to define a more tailored treatment plan.

  16. [Adenoid cystic carcinoma of the lacrimal gland].

    PubMed

    Holz, F G; Tetz, M; Born, I A; Völcker, H E

    1992-07-01

    A 38-year-old female presented with a left-sided peripheral N. VI-paresis and negative neuroradiologic work-up. After one year she developed an incomplete N. III paresis, numbness of her face correlating to N. V1 and V2, reduced lacrimal secretion as well as a palpable mass at the lateral orbital rim. Magnetic resonance imaging revealed an orbital tumor extending into the cavernous sinus. Biopsy disclosed an adenoid cystic carcinoma of the lacrimal gland with basaloid, cribriform and tubular pattern. Therefore, an exenteration with postoperative radiation therapy was performed. Immunohistochemical findings included positive reactions for keratin (KL-1 and AE 1 + 3), Vimentin and Egp 34. By determination of Ki 67 the proliferative activity of the tumor was found to be 15%. Our case report demonstrates that even in absence of an exophthalmus or pain a malignant orbital tumor may be assumed as the underlying cause for a combined monolateral oculomotor and trigeminal paresis. Immunohistochemical results support the notion, that the adenoid cystic carcinoma of the lacrimal gland may arise from precursor cells of the terminal duct system.

  17. 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.

  18. [Cystic fibrosis in 2008].

    PubMed

    Durieu, I; Josserand, R Nove

    2008-11-01

    To describe the epidemiological, physiopathological, clinical and therapeutic knowledge concerning cystic fibrosis (CF). Important modifications in the health organization of the care concerning this orphan disease have been implemented in France. The life expectancy has dramatically increased, as well as the knowledge concerning the pathological structure and function of the CFTR gene and protein. This will lead to the development of emerging drug treatments for this lethal disease. The life expectancy is predicted to exceed 40 years for children born in the 2000s. As a result, there has been a tremendous growth of the adult population that reached 40% of the overall approximately 5000 patients included in the CF French registry (Observatoire National de la Mucoviscidose). Lung disease remains the primary cause of morbidity and mortality. The characteristic phenotypic presentation associates bronchial and rhinosinusal symptoms, pancreatic insufficiency and liver disease. Bronchial damage leads to progressive chronic respiratory insufficiency. Diabetes mellitus and osteoporosis frequently appears in adulthood. Neonatal screening has been implemented in France since 2002. It will prevent delayed diagnosis and its deleterious consequences. Some atypical cases of CF presenting only with one or two organ system involvement can be diagnosed in adulthood. Isolated chronic rhinosinusitis, bronchiectasis, congenital bilateral absence of vas deferens, recurrent pancreatitis, allergic bronchopulmonary aspergillosis, and some case of cholangitis may so revealed late form of cystic fibrosis. The health care is organized in cystic fibrosis centres. Despite gene discovery, treatment still remains symptomatic, based on intensive pulmonary and nutritional treatments. Challenges for new treatments are to correct the basic defect, either by gene therapy or by pharmacological modulation of the abnormal physiological processes.

  19. 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.

  20. Screening for cystic fibrosis.

    PubMed Central

    Dodge, J A; Ryley, H C

    1982-01-01

    Practicable methods are now available for whole population screening of neonates for cystic fibrosis. Although diagnosis and treatment of the disease from birth has not yet been unequivocally shown to improve prognosis, existing evidence suggests that this is likely. Further ethical reasons are proposed in support of neonatal diagnosis and early treatment. The development of tests for prenatal diagnosis and carrier detection is under active investigation, and raises ethical problems for heterozygotes and their medical advisers. The heavy financial and emotional burden this disease imposes on the patient and the family should not be underestimated when policy decisions are made. PMID:7138066

  1. Cystic fibrosis and sleep.

    PubMed

    Katz, Eliot S

    2014-09-01

    Sleep disturbances are frequently observed in cystic fibrosis (CF). The resultant sleep fragmentation, short sleep duration, and gas-exchange abnormalities are postulated to contribute to the neurocognitive, cardiovascular, and metabolic abnormalities associated with CF. There are no outcomes data to establish the optimal procedure for screening and treating CF patients for sleep-related respiratory abnormalities. Therapy with supplemental oxygen and bilevel ventilation are widely considered to be effective in the short term, but there are few evidence-based data to support long-term improvements in morbidity and mortality. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. [Giant paraovarian cyst in childhood - Case report].

    PubMed

    Torres, Janina P; Íñiguez, Rodrigo D

    2015-01-01

    Paraovarian cysts are very uncommon in children To present a case of giant paraovarian cyst case in a child and its management using a modified laparoscopic-assisted technique A 13-year-old patient with a 15 day-history of intermittent abdominal pain, located in the left hemiabdomen and associated with progressive increase in abdominal volume. Diagnostic imaging was inconclusive, describing a giant cystic formation that filled up the abdomen, but without specifying its origin. Laboratory tests and tumor markers were within normal range. Video-assisted transumbilical cystectomy, a modified laparoscopic procedure with diagnostic and therapeutic intent, was performed with a successful outcome. The histological study reported giant paraovarian cyst. Cytology results were negative for tumor cells. The patient remained asymptomatic during the postoperative follow-up. The video-assisted transumbilical cystectomy is a safe procedure and an excellent diagnostic and therapeutic alternative for the treatment of giant paraovarian cysts. Copyright © 2015. Publicado por Elsevier España, S.L.U.

  3. Ongoing challenges in the treatment of adenoid cystic carcinoma of the head and neck.

    PubMed

    Subramaniam, T; Lennon, P; O'Neill, J P

    2015-09-01

    Adenoid cystic carcinoma is a malignant tumour of major and minor salivary glands. Distant metastasis and poor survival are persistent in the literature, with recent publications aimed at understanding molecular pathogenesis and development of pharmaceutical therapeutic options. Provide an update of recent studies in the management of adenoid cystic carcinoma of the head and neck. Literature search using Medline, Scopus, Google Scholar, the Cochrane Database of Systematic Reviews and the Cochrane central register of controlled trials for articles on adenoid cystic carcinoma from January 2005 to January 2015. Adenoid cystic carcinoma is characterized by a slow growing mass, with distant metastasis independent of local or regional control. Primary tumour resection remains the preferred option with radiotherapy having an adjuvant role. Recent advances have been made with novel targeted therapies however, limited to clinical trials and advanced disease.

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

    PubMed Central

    Farrell, James J.

    2015-01-01

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

  5. 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

  6. Histiocytoid giant cellulitis-like Sweet's syndrome: case report and review of the literature.

    PubMed

    So, Jessica Kim; Carlos, Casey A; Frucht, Corey S; Cohen, Philip R

    2015-01-25

    Histiocytoid Sweet syndrome is an uncommon variant in which the dermal infiltrate is composed of mononuclear cells with a histiocytic appearance that represent immature myeloid cells. Giant cellulitis-like Sweet syndrome is a recently described variant characterized by relapsing widespread giant lesions. We report a unique patient with histiocytoid giant cellulitis-like Sweet syndrome and review the current literature on histiocytoid Sweet syndrome and giant cellulitis-like Sweet syndrome. We reviewed PubMed for the following terms and have reviewed the literature: histiocytoid, giant cellulitis-like, and Sweet syndrome. Six individuals, including our patient, have been reported with giant cellulitis-like Sweet syndrome; four had obesity, two had a hematologic malignancy, and one had breast cancer. Histiocytoid Sweet syndrome has been reported in association with autoimmune diseases, infection or inflammation, inflammatory bowel disease, malignancies, medications, and other conditions. Histiocytoid Sweet syndrome is a rare variant of Sweet syndrome, often associated with malignancy. Giant cellulitis-like Sweet syndrome has been reported in six individuals; four of the patients were obese and three of the patients had an associated cancer. Our patient had histiocytoid giant cellulitis-like Sweet syndrome-associated myelodysplastic syndrome/myeloproliferative disorder. The diagnosis of histiocytoid Sweet syndrome or giant cellulitis-like Sweet syndrome should prompt the clinician to consider additional evaluation for a Sweet syndrome-associated malignancy.

  7. Cystic fibrosis since 1938.

    PubMed

    Davis, Pamela B

    2006-03-01

    Cystic fibrosis (CF) was distinguished from celiac disease in 1938. Then, it was a pathologic diagnosis, life expectancy was approximately 6 months, and the autosomal recessive disease was believed to arise from abnormal mucus plugging exocrine ducts. Death often occurred from lung infection. Discovery of the sweat electrolyte defect in 1953 and standardization of the sweat test in 1959 allowed identification of milder cases, and CF was no longer considered only a disorder of mucus. In 1955, establishment of centers with programs of aggressive, comprehensive care initiated striking improvement in longevity. The pillars of care established then (attention to nutrition, airway clearance, treatment of lung infection) remain today. In 1983, chloride transport was identified as the basic physiologic CF defect, accompanied by increased sodium reabsorption. In 1980, we learned that inflammation contributes independently to lung disease and constitutes an independent therapeutic target. In 1989, the discovery of the CF gene demonstrated the basic defect to be in a cAMP-regulated chloride channel. This afforded new diagnostic tests, opportunities for research, and prospects for using the gene as therapy. Since then, substantial advances in basic and clinical research catalyzed therapeutic improvements: median survival age now exceeds 30 years. The Cystic Fibrosis Foundation center network provides not only opportunity to conduct clinical trials but also means to disseminate new therapies. In the future, treatments directed at the basic defect can be expected, with concomitant improvements in morbidity and mortality.

  8. Cystic fibrosis in Uruguay.

    PubMed

    Luzardo, Gerardo; Aznarez, Isabel; Crispino, Beatriz; Mimbacas, Adriana; Martínez, Liria; Poggio, Rossana; Zielenski, Julian; Tsui, Lap-Chee; Cardoso, Horacio

    2002-03-31

    We conducted clinical and genetic analyses of 52 cystic fibrosis (CF) patients in Uruguay, which is about half of the known affected individuals in the country. A relatively high proportion had a mild presentation, characterized by pancreatic sufficiency (28%), a strong pulmonary component (97%), and borderline sweat electrolyte measurements (25%). Mutational analysis of CF chromosomes demonstrated a relatively low incidence of the DeltaF508 allele (40%) and a large number of other cystic fibrosis conductance regulator mutations, with an overall detection rate of about 71%. Fifteen different mutations were detected in our patients: DeltaF508, G542X, R1162X, G85E, N1303K, R334W, R75Q, R74W, D1270N, W1282X, DeltaI507, 2789+5G-->A, R1066C, -816C/T, R553X, as well as RNA splicing variant IVS8-5T. This group of Uruguayan CF patients has some characteristics in common with other populations of similar origin (Hispanics), as well as some unique characteristics.

  9. Cystic fibrosis related diabetes.

    PubMed

    O'Shea, Donal; O'Connell, Jean

    2014-08-01

    Improved life expectancy in cystic fibrosis (CF) has led to an expanding population of adults with CF, now representing almost 50 % of the total CF population. This creates new challenges from long-term complications such as diabetes mellitus (DM), a condition that is present in 40 %-50 % of adults with CF. Cystic fibrosis-related diabetes (CFRD) results from a primary defect of insulin deficiency and although sharing features with type 1 (DM1) and type 2 diabetes (DM2), it is a clinically distinct condition. Progression to diabetes is associated with poorer CF clinical outcomes and increased mortality. CFRD is not associated with an increased risk of cardiovascular disease and the prevalence of microvascular complications is lower than DM1 or DM2. Rather, the primary goal of insulin therapy is the preservation of lung function and optimization of nutritional status. There is increasing evidence that appropriate screening and early intervention with insulin can reverse weight loss and improve pulmonary function. This approach may include targeting postprandial hyperglycemia not detected by standard diagnostic tests such as the oral glucose tolerance test. Further clinical research is required to guide when and how much to intervene in patients who are already dealing with the burden of one chronic illness.

  10. 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.

  11. Giant rectal gastrointestinal stromal tumours: a diagnostic and therapeutic challenge

    PubMed Central

    Alder, L.S.; Elver, G.; Foo, F.J.; Dobson, M.

    2013-01-01

    Gastrointestinal stromal tumour (GIST are the most common mesenchymal tumours; however, rectal GISTs account for <5%. In the pelvis they represent a diagnostic challenge with giant GISTs likely to be malignant. They may present with urological, gynaecological or rectal symptoms. Sphincter-preserving surgery can be aided by neoadjuvant therapy. We present an uncommon case of giant rectal GIST masquerading as acute urinary retention. PMID:24968434

  12. 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.

  13. Gastrointestinal Manifestations of Cystic Fibrosis

    PubMed Central

    2016-01-01

    Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis. PMID:27330503

  14. 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.

  15. A bizarre abdominal cystic lesion.

    PubMed

    Zucchini, Giorgia; Pezzilli, Raffaele; Ricci, Claudio; Casadei, Riccardo; Santini, Donatella; Calculli, Lucia; Corinaldesi, Roberto

    2010-09-06

    In spite of careful intraoperative precautions and gauze counts, mistakes can still occur during surgery. In the case reported, a retained gauze leaved during a surgical approach for removing a solid-cystic papillary tumor localized in the pancreatic tail, caused both persistent abdominal discomfort and the presence of an abdominal cystic lesion at imaging techniques. When a previous operative history is present, a foreign body should be taken into account in the differential diagnosis of a patient with an intra-abdominal cystic mass. Finally, radio-opaque marker should be routinely used by surgeons in order to reach a correct diagnosis in operated patients having retained gauze.

  16. Recurrent renal giant leiomyosarcoma

    PubMed Central

    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

  17. A Case of Giant Cowper's Gland Syringocele in an Adult Male Patient

    PubMed Central

    Surana, Santosh; Elshazly, Mohamed; Allam, Adel; Jayappa, Sateesh; AlRefai, Deena

    2015-01-01

    Cowper's gland syringocele is an uncommon, underdiagnosed cystic dilatation of Cowper's gland ducts showing various radiological patterns. Herein we report a rare case of giant Cowper's gland syringocele in an adult male patient, with description of MRI findings and management outcome. PMID:26413368

  18. Giant Hidrocystoma of the Orbit Presenting with Inversion and Ptosis of the Upper Eyelid

    PubMed Central

    Palamar, Melis; Yaman, Banu; Akalın, Taner; Yağcı, Ayşe

    2017-01-01

    A case of giant hidrocystoma of the orbit in a 57-year-old female causing pain, epiphora and ptosis is reported. The cystic mass was totally excised as a whole. Histopathologic examination revealed eccrine hidrocystoma of the orbit. Hidrocystoma must be considered in the differential diagnosis of patients presenting with periocular masses causing pain and ptosis. PMID:28405487

  19. Primary Cystic Lesions of the Retrorectal Space: MRI Evaluation and Clinical Assessment.

    PubMed

    Dwarkasing, Roy S; Verschuuren, Sylvia I; van Leenders, Geert J L H; Braun, Loes M M; Krestin, Gabriel P; Schouten, W Rudolph

    2017-10-01

    The purpose of this study was to assess the a priori chance that primary cystic lesions of the retrorectal space are malignant and to investigate MRI characteristics that indicate malignancy. Patients referred to a center for colorectal surgery were recruited from 2000 to 2014. Lesions were proven by clinical assessment and histopathology. MRI was performed at 1.5 T with examinations evaluated by two radiologists. Interobserver agreement was assessed (Cohen kappa) and differences between malignant and benign lesions calculated (Fisher exact test). Twenty-eight patients (22 women, six men; age range, 18-70 years) with 31 lesions were included. Lesions were categorized as tailgut cysts (n = 16, 52%), teratomas (n = 9, 29%), lesions of colorectal origin (n = 4, 13%), or neurogenic lesions (n = 2, 6%). Five patients (18%) had malignant lesions. Colorectal lesions had the highest percentage of malignancy (3/4, 75%). A solid tissue component was found in all five (100%) malignant lesions and two (8%) of the benign lesions, which were both teratomas (p < 0.05). Sensitivity and specificity for malignancy according to the presence of a solid tissue component was 100% (5/5) and 92% (24/26). For unilocularity, multilocularity, debris, septa, and wall thickening, differences were not significant. Interobserver agreement was excellent (κ = 1) for all characteristics except debris (κ = 0.795). The majority of retrorectal cystic lesions are benign. The presence of a solid tissue component should raise suspicion for malignancy.

  20. A rare case of giant multicystic solitary fibrous tumor of the orbit.

    PubMed

    Alam, Shahid; Backiavathy, Varsha; Mukherjee, Bipasha; Subramanian, Krishnakumar

    2017-08-17

    Solitary fibrous tumor (SFT) is a rare spindle cell tumor of the orbit of mesenchymal origin. Though these tumors are mostly solid, partial or complete cystic changes can rarely occur. Only six such previous cases of cystic fibrous tumor of the orbit have been mentioned in the literature. We report a case of an elderly male who presented with a huge left sided medial orbital mass. Magnetic resonance imaging showed a predominant cystic orbital mass separated by septae and suggested a diagnosis of Hydatid cyst. The patient underwent complete excision of the mass and histopathology and immunohistochemistry were suggestive of cystic SFT. Cystic degeneration in SFT is extremely rare and can be a harbinger of malignancy, and pose risk of recurrence. Close follow up and monitoring is required for all such cases.

  1. 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

  2. [Tracheal adenoid cystic carcinoma presenting as a thyroid tumor].

    PubMed

    Bahadir, Osman; Imamoğlu, Mehmet; Cobanoğlu, Umit; Korkmaz, Omer

    2006-01-01

    Primary tumors of the trachea are rare. Adenoid cystic carcinoma is the second most common tracheal malignancy and its symptoms are nonspecific. A 66-year-old woman presented with complaints of progressive dyspnea and cough of a two-week duration. She had received asthmatic treatment many times for the past four years. Physical examination revealed a large, firm, nontender, multilobular mass in the thyroid gland. Indirect laryngoscopic examination showed subglottic stenosis. On a plain cervical radiograph, there was narrowing of the tracheal lumen. Emergent tracheotomy was performed and a biopsy was taken from the mass. Histopathologic examination revealed adenoid cystic carcinoma, suggesting a thyroid mass with a tracheal origin. Despite medical treatment, the patient died in the fourth month.

  3. 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 ...

  4. 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

  5. Metastatic giant basal cell carcinoma: a case report

    PubMed Central

    Bellahammou, Khadija; Lakhdissi, Asmaa; Akkar, Othman; Rais, Fadoua; Naoual, Benhmidou; Elghissassi, Ibrahim; M’rabti, Hind; Errihani, Hassan

    2016-01-01

    Basal cell carcinoma is the most common skin cancer, characterised by a slow growing behavior, metastasis are extremely rare, and it occurs in less than 0, 1% of all cases. Giant basal cell carcinoma is a rare form of basal cell carcinoma, more aggressive and defined as a tumor measuring more than 5 cm at its largest diameter. Only 1% of all basal cell carcinoma develops to a giant basal cell carcinoma, resulting of patient's negligence. Giant basal cell carcinoma is associated with higher potential of metastasis and even death, compared to ordinary basal cell carcinoma. We report a case of giant basal cell carcinoma metastaticin lung occurring in a 79 years old male patient, with a fatal evolution after one course of systemic chemotherapy. Giant basal cell carcinoma is a very rare entity, early detection of these tumors could prevent metastasis occurrence and improve the prognosis of this malignancy. PMID:27795755

  6. Metastatic giant basal cell carcinoma: a case report.

    PubMed

    Bellahammou, Khadija; Lakhdissi, Asmaa; Akkar, Othman; Rais, Fadoua; Naoual, Benhmidou; Elghissassi, Ibrahim; M'rabti, Hind; Errihani, Hassan

    2016-01-01

    Basal cell carcinoma is the most common skin cancer, characterised by a slow growing behavior, metastasis are extremely rare, and it occurs in less than 0, 1% of all cases. Giant basal cell carcinoma is a rare form of basal cell carcinoma, more aggressive and defined as a tumor measuring more than 5 cm at its largest diameter. Only 1% of all basal cell carcinoma develops to a giant basal cell carcinoma, resulting of patient's negligence. Giant basal cell carcinoma is associated with higher potential of metastasis and even death, compared to ordinary basal cell carcinoma. We report a case of giant basal cell carcinoma metastaticin lung occurring in a 79 years old male patient, with a fatal evolution after one course of systemic chemotherapy. Giant basal cell carcinoma is a very rare entity, early detection of these tumors could prevent metastasis occurrence and improve the prognosis of this malignancy.

  7. Cystic fibrosis in pregnancy.

    PubMed Central

    Kent, N E; Farquharson, D F

    1993-01-01

    OBJECTIVE: To review the outcomes of pregnancies in women with cystic fibrosis (CF) and to address issues pertinent to the obstetric care of such women. DATA SOURCES: English-language case reports and case series published from 1960 to 1991 identified through a search of MEDLINE and Index Medicus. The terms of reference were "cystic fibrosis" and "pregnancy". Not all the reports reviewed addressed all the outcomes under consideration. STUDY SELECTION: A total of 20 reports citing cases of pregnancy in women with CF. DATA EXTRACTION: Outcomes included the number of spontaneous abortions, pregnancies continued beyond 20 weeks, preterm deliveries, maternal deaths at 6 months and 2 years after delivery and perinatal deaths. Breast-feeding was addressed. Measures to assess the severity of maternal disease included the mean age at diagnosis of CF, weight gain during pregnancy, pulmonary function studies if available and the need for pancreatic enzyme replacement therapy. DATA SYNTHESIS: Of 217 pregnancies in 162 women spontaneous abortion occurred in 10 (4.6%). Pregnancy progressed beyond 20 weeks in 81.6% of cases; 24.3% of the deliveries were preterm. The maternal death rate did not exceed that among age-related women with CF who were not pregnant. The rate of perinatal death was 7.9%. Breast milk was not hypernatremic. Poor outcomes were associated with a weight gain of less than 4.5 kg and a forced vital capacity of less than 50% of the predicted value. CONCLUSIONS: Premature labour and delivery remain a significant risk for pregnant women with CF, contributing to a high rate of perinatal death. Maternal illness and death result from deteriorating pulmonary function. Breast-feeding is not contraindicated. Attention to energy intake and pulmonary function is important. PMID:8374843

  8. 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. Copyright © 2016 John Wiley & Sons, Inc.

  9. Haemophilus infection in cystic fibrosis.

    PubMed Central

    Rayner, R J; Hiller, E J; Ispahani, P; Baker, M

    1990-01-01

    Twenty seven patients with cystic fibrosis under the age of 12 years and 27 matched patients with asthma were followed up in a prospective study for one year. The isolation rate of non-capsulated strains of Haemophilus influenzae from cough swabs and sputum specimens taken at routine clinic visits every two months was significantly greater in cystic fibrosis than in asthma. Haemophilus para-influenzae was equally common in both groups. During exacerbations the isolation rate of H influenzae in cystic fibrosis was significantly greater than at other times, whereas in asthma there was no significant difference. The distribution of biotypes of H influenzae and H parainfluenzae was similar in the two groups. In cystic fibrosis, biotype I was associated with exacerbations. Biotype V was more common than in previous studies, but was not associated with exacerbations. PMID:2185699

  10. [Lung physiotherapy in cystic fibrosis].

    PubMed

    Gursli, S; Haanaes, O C

    1991-02-28

    This article is intended as a brief practical guide for physicians and physiotherapists concerned with the treatment of cystic fibrosis. Physiotherapeutic techniques for the treatment of chest diseases have been developed and modified as advances have taken place in the medical management of cystic fibrosis. The article describes forced expiratory technique, positive expiratory pressure, postural drainage, autogenic drainage and other techniques. Patients with cystic fibrosis live longer and have a better quality of life than ever before, but progressive deterioration of lung function will always be their most serious problem. Physical activity and chest physiotherapy are essential parts of all treatment regimens for cystic fibrosis. It is important to realize that the physiotherapist is a very important member of the team which includes nurses, physicians-and the patient.

  11. Newborn screening for cystic fibrosis.

    PubMed

    Castellani, Carlo; Massie, John; Sontag, Marci; Southern, Kevin W

    2016-08-01

    Since the late 1970s when the potential of the immunoreactive trypsinogen assay for early identification of infants with cystic fibrosis was first recognised, the performance of newborn blood spot screening (NBS) has been continually assessed and its use has gradually expanded. NBS for cystic fibrosis is a cost-effective strategy and, if standards of care are fully implemented and robust management pathways are in place, has a positive effect on clinical outcomes. In the past decade, NBS has undergone rapid expansion and an unprecedented number of infants with cystic fibrosis have access to early diagnosis and care. Cystic fibrosis NBS has now moved on from the development phase and is entering an era of consolidation. In the future, research should focus on the rationalisation and optimisation of existing programmes, with particular attention to bioethical implications such as unwanted detection of carriers and inconclusive diagnoses. Copyright © 2016 Elsevier Ltd. All rights reserved.

  12. 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.

  13. 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.

  14. Giant impacts on giant planets

    NASA Astrophysics Data System (ADS)

    de Pater, Imke

    2012-10-01

    The 2009 impact 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 impact flash detections and lightcurve measurements}.We propose a Target of Opportunity 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.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.

  15. Physical training for cystic fibrosis.

    PubMed

    Bradley, J; Moran, F

    2008-01-23

    Physical training may form an important part of the care package for people with cystic fibrosis. To determine whether a prescribed regimen of physical training produces improvement or prevents deterioration in physiological and clinical outcomes in cystic fibrosis compared to no training. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Date of the most recent search: September 2007. All randomised and quasi-randomised controlled clinical trials in which a prescribed regimen of physical training is compared to no physical training in people with cystic fibrosis. Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. Of the 26 studies identified, seven studies which included 231 participants, met the inclusion criteria. This review does provide some limited evidence from both short- and long-term studies that aerobic or anaerobic physical training has a positive effect on primary outcomes (exercise capacity, strength and lung function) but improvements are not consistent between studies. Conclusions about the efficacy of physical training in cystic fibrosis are limited by the small size, short duration and incomplete reporting of most of the studies included in this review. Physical training is already part of the care package offered to most people with cystic fibrosis and there is a lack of evidence to actively discourage this. The benefits obtained from including physical training in a package of care may be influenced by the type of training programme. Further research is needed to assess comprehensively the benefits of exercise programmes in people with cystic fibrosis and the relative benefits of the addition of aerobic versus anaerobic versus a combination of both types of physical training to the care

  16. Pneumococcal vaccines for cystic fibrosis.

    PubMed

    Burgess, Laura; Southern, Kevin W

    2014-08-05

    Invasive pneumococcal disease is associated with significant mortality and many countries have introduced routine pneumococcal vaccination into their childhood immunisation programmes. Whilst pneumococcal disease in cystic fibrosis is uncommon, pneumococcal immunisation may offer some protection against pulmonary exacerbations caused by this pathogen. In the USA and UK pneumococcal vaccination is currently recommended for all children and adults with cystic fibrosis. To assess the efficacy of pneumococcal vaccines in reducing morbidity in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Cystic Fibrosis Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. In addition, the pharmaceutical manufacturers of the polysaccharide and conjugate pneumococcal vaccines were approached.Date of the most recent search: 15 May 2014. Randomised and quasi-randomised controlled trials comparing pneumococcal vaccination (with either a polysaccharide or conjugate pneumococcal vaccine) with non-vaccination or placebo in children or adults with cystic fibrosis were eligible for inclusion. No relevant trials were identified. There are no trials included in this review. As no trials were identified we cannot draw conclusions on the efficacy of routine pneumococcal immunisation in people with cystic fibrosis in reducing their morbidity or mortality. As many countries now include pneumococcal immunisation in their routine childhood vaccination schedule it is unlikely that future randomised controlled trials will be initiated. Rigorously conducted epidemiological studies may offer the opportunity to evaluate the efficacy of pneumococcal vaccination in reducing morbidity and mortality in people with cystic fibrosis.

  17. Pneumococcal vaccines for cystic fibrosis.

    PubMed

    Burgess, Laura; Southern, Kevin W

    2012-09-12

    Invasive pneumococcal disease is associated with significant mortality and many countries have introduced routine pneumococcal vaccination into their childhood immunisation programmes. Whilst pneumococcal disease in cystic fibrosis is uncommon, pneumococcal immunisation may offer some protection against pulmonary exacerbations caused by this pathogen. In the USA and UK pneumococcal vaccination is currently recommended for all children and adults with cystic fibrosis. To assess the efficacy of pneumococcal vaccines in reducing morbidity in people with cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Cystic Fibrosis Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. In addition, the pharmaceutical manufacturers of the polysaccharide and conjugate pneumococcal vaccines were approached.Date of the most recent search: 10 July 2012. Randomised and quasi-randomised controlled trials comparing pneumococcal vaccination (with either a polysaccharide or conjugate pneumococcal vaccine) with non-vaccination or placebo in children or adults with cystic fibrosis were eligible for inclusion. No relevant trials were identified. There are no trials included in this review. As no trials were identified we cannot draw conclusions on the efficacy of routine pneumococcal immunisation in people with cystic fibrosis in reducing their morbidity or mortality. As many countries now include pneumococcal immunisation in their routine childhood vaccination schedule it is unlikely that future randomised controlled trials will be initiated. Rigorously conducted epidemiological studies may offer the opportunity to evaluate the efficacy of pneumococcal vaccination in reducing morbidity and mortality in people with cystic fibrosis.

  18. 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.

  19. What Are the Signs and Symptoms of Cystic Fibrosis?

    MedlinePlus

    ... Twitter. What Are the Signs and Symptoms of Cystic Fibrosis? The signs and symptoms of cystic fibrosis (CF) ... respiratory, digestive, or reproductive systems of the body. Cystic Fibrosis Figure A shows the organs that cystic fibrosis ...

  20. Giant congenital melanocytic nevus with developmental dysplasia of bilateral hip: a rare association.

    PubMed

    Imchen, Sutsungkokla; Ghosh, Sangita; Dayal, Surabhi; Marwah, Nisha; Jindal, Nidhi; Sangal, Shikha

    2013-11-01

    Giant congenital melanocytic nevi are rare congenital disfiguring benign neoplasms with a risk of transformation to malignant melanoma. They often present with various extra-cutaneous features. Here, we describe a case of giant melanocytic nevus with developmental dysplasia of bilateral hip, a novel association.

  1. 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

  2. [Neonatal curettage of giant congenital nevi].

    PubMed

    Michel, J L; Laborde-Milaa Roux, V; Chavrier, Y; Roux, V; Metafiot, H; Chalencon, F; Cambazard, F

    2000-01-01

    All agree upon the need for early treatment of giant congenital nevi, basically because of the risk of melanoma degeneration, estimated at about 5 p. 100. Another reason is the cosmetic, psychological and social impact of such nevi. The aim of this study was to assess neonatal curettage of giant congenital nevi as an alternative to classical surgery. Between 1996 and 1999, the curettage technique was used in 14 newborns with giant congenital nevi. Three nevi were located on the scalp, 4 on lower limbs and 7 on the trunk with a jacket configuration in 1 case and a cape configuration in 4. Curettage achieved 70-95 p. 100 clearing of the giant nevi in 10 of the 14 children. Four of the children developed hypertrophic scar tissue which resolved with time. Secondary hair growth was observed in 5 cases. Outcome was better when the curettage was performed very early (before 2 weeks of life). Curettage is a surface technique proposed when surgical excision cannot be performed because the surface is too large or the localization is incompatible with surgery. Curettage is a simple low-cost technique which provides particularly satisfactory cosmetic results for very extensive giant congenital nevi. The risk of malignant transformation is greatly reduced although not totally. Regular clinical surveillance under conditions greatly improved by the clearing should help reduce the risk.

  3. Contrast enhanced transabdominal ultrasound in the characterisation of pancreatic lesions with cystic appearance.

    PubMed

    Beyer-Enke, Stefan A; Hocke, Michael; Ignee, Andre; Braden, Barbara; Dietrich, Christoph F

    2010-09-06

    Contrast enhanced ultrasound (CEUS) has been established for detection and characterisation of liver tumours and differential diagnosis of solid pancreatic lesions. The role of transabdominal CEUS in cystic pancreatic disease is less obvious. We prospectively evaluated CEUS for characterization of undetermined cystic pancreatic lesions with respect to the differential diagnosis of pseudocysts and cystic neoplasia and differentiation between benign and malignant disease (gold standard: histology or cytology). One-hundred and fourteen patients (63 males, 51 females; median age: 62 years, range: 33-87 years) were prospectively examined. Conventional B-mode and transabdominal CEUS. Conventional B-mode (criteria: solid nodules, septae), and contrast enhancing features of cystic pancreatic lesions (microperfusion of solid nodules) were analysed. Final diagnoses were made by surgery (47 patients) or histology/cytology and follow-up of at least one year (67 patients). Fifty patients proved to have neoplastic lesions (37 malignant, 13 of benign origin). Sixty-four patients had pseudocysts caused by acute (27 patients) or chronic pancreatitis (37 patients). Conventional B-mode had a sensitivity of 94% and a low specificity of 44% in the differentiation of pseudocysts versus neoplasia. CEUS had a higher specificity of 77% with the same sensitivity of conventional B-mode ultrasound. The combination of conventional ultrasound and CEUS improved the specificity even more to 97% with an unchanged sensitivity. CEUS was not reliable in the differentiation of benign and malignant neoplasia. CEUS improves the differentiation between pseudocysts and pancreatic neoplasia in comparison to the conventional B-mode imaging. The microvascularisation visualised using CEUS even in small nodules (with or without septae) associated with cystic lesions is an indicator for cystic pancreatic neoplasia.

  4. Numerous intracystic floating balls as a sonographic feature of benign cystic teratoma: report of 5 cases.

    PubMed

    Tongsong, Theera; Wanapirak, Chanane; Khunamornpong, Surapan; Sukpan, Kornkanok

    2006-12-01

    The purpose of this series was to describe the unusual but unique sonographic pattern of mature cystic teratoma. Five patients of reproductive age with clinical manifestations of a pelvic mass were evaluated with sonography for treatment planning. All 5 cases showed the similar sonographic pattern of a large cystic tumor filled with multiple echogenic spherical structures floating in the cystic background. Of the 5 patients, 3 had rather large balls varying in size between 1 and 4 cm in the same tumor masses, whereas the other 2 had numerous smaller balls of about 0.5 cm in diameter. The numerous crowded very small echogenic balls in the last 2 cases mimicked solid nodules representing malignancy. However, there was no vascularization in the balls, which suggested a benign nature. The postoperative pathologic diagnosis was mature cystic teratoma without any malignant component in all cases. The sonographic feature of intracystic floating echogenic balls is probably pathognomonic for mature teratoma and is easily detected in most cases. Color Doppler sonography is helpful in differentiating these benign nodules (small balls) from malignant tumors.

  5. Adenoid cystic carcinoma - Clinical presentation and cytological diagnosis.

    PubMed

    Dutta, N N; Baruah, R; Das, L

    2002-01-01

    Adenoid cystic carcinoma is a tumor arising from the minor salivary glands, the palate being the commonest site. It accounts for about 1% of all head and neck malignancies. We report a case with the typical presentation of a palatal growth with extensive intra-cranial invasion. The diagnosis of this case and a brief review of literature is discussed. Final diagnosis of this case was made from cytological reports. The dry smears stained with MGG were found to be definitely superior to the alcohol fixed slides stained with papanicolaou. The aim here is to highlight the importance of cytology in the diagnosis of such tumors.

  6. Percutaneous Placement of Permanent Metallic Stents in the Cystic Duct to Treat Obstructive Cholecystitis.

    PubMed

    Brown, Nicholas I; Jhamb, Ashu; Brooks, Duncan M; Little, Andrew F

    2015-12-01

    This report presents a series of five patients unsuitable for surgery who had nonretrievable self-expanding metallic stents deployed along the cystic duct as treatment for benign and malignant causes of gallbladder obstruction. Techniques are described for draining cholecystitis, removing gallstones, bypassing gallbladder obstructions, and inserting metallic stents across the cystic duct to restore permanent antegrade gallbladder drainage in acute and chronic cholecystitis. Symptoms resolved in all cases, and stents remained patent for as long as 22 months. This procedure may be an effective alternative to cholecystectomy or long-term gallbladder drainage for patients in inoperable condition.

  7. Adenoid Cystic Carcinoma- A rare Differential Diagnosis for a mass in the External Auditory Canal.

    PubMed

    Prasad, Vishnu; Shenoy, Vijendra S; Rao, Raghavendra A; Kamath, Panduranga M; Shihab, Haseena

    2015-01-01

    Primary external auditory canal malignancies are very rare; in which, adenoid cystic carcinoma is extremely rare tumour accounting for approximately 5%. Majority of the patients presents with unilateral severe or dull aching constant ear pain of prolonged duration, reduced hearing and mass in the External Ear. These tumours are treated with aggressive surgical excision and adjuvant radiotherapy. Despite this, the overall prognosis is poor due to recurrences and distant metastasis. We report a rare case of adenoid cystic carcinoma in a 36-year-old female, who presented with right ear pain for the last one year. She was treated with wide local excision of the mass followed by adjuvant radiotherapy.

  8. Adenoid Cystic Carcinoma– A rare Differential Diagnosis for a mass in the External Auditory Canal

    PubMed Central

    Shenoy, Vijendra S; Rao, Raghavendra A; Kamath, Panduranga M; Shihab, Haseena

    2015-01-01

    Primary external auditory canal malignancies are very rare; in which, adenoid cystic carcinoma is extremely rare tumour accounting for approximately 5%. Majority of the patients presents with unilateral severe or dull aching constant ear pain of prolonged duration, reduced hearing and mass in the External Ear. These tumours are treated with aggressive surgical excision and adjuvant radiotherapy. Despite this, the overall prognosis is poor due to recurrences and distant metastasis. We report a rare case of adenoid cystic carcinoma in a 36-year-old female, who presented with right ear pain for the last one year. She was treated with wide local excision of the mass followed by adjuvant radiotherapy. PMID:25738012

  9. Malignant hyperthermia.

    PubMed

    Bandschapp, Oliver; Girard, Thierry

    2012-07-31

    Malignant hyperthermia (MH) is a subclinical myopathy, usually triggered by volatile anaesthetics and depolarising muscle relaxants. Clinical symptoms are variable, and the condition is sometimes difficult to identify. Nevertheless, rapid recognition and specific as well as symptomatic treatment are crucial to avoid a lethal outcome. Molecular genetic investigations have confirmed the skeletal muscle type ryanodine receptor to be the major MH locus with more than 70% of MH families carrying a mutation in this gene. There is no screening method to test for MH, as current tests are invasive (open muscle biopsy) or restricted to MH families with known MH-associated mutations (molecular genetic testing). The prevalence of the MH trait is unknown, because the clinical penetrance after contact with triggering agents is very variable. More recently, MH mutations have been associated with rhabdomyolysis following statin therapy or with non-pharmacological triggering, such as exertional heat stroke.

  10. 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

  11. [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).

  12. The Electric Giant Resonances

    NASA Astrophysics Data System (ADS)

    van der Woude, A.

    The following sections are included: * Introduction * Experimental Methods to Study Giant Resonances * Introduction * The Tools * Introduction * Tools for Isoscalar Scattering * INELASTIC α-SCATTERING * INELASTIC PROTON SCATTERING * Tools for Isovector Excitations * γ-ABSORPTION AND PARTICLE CAPTURE REACTIONS * CHARGE EXCHANGE REACTIONS - THE (π+, π0) REACTION * Tools For Isoscalar And Isovector Excitations * INELASTIC ELECTRON SCATTERING * GIANT RESONANCE EXCITATION BY FAST HEAVY IONS * From Multipole Cross Section To Multipole Strength * The Electric Isoscalar Resonances * The Isoscalar Giant Monopole Resonance * Systematics on the GMR * Compressibility and the Giant Monopole Resonance * Introduction * The Compressibility of nuclear matter from the GMR energies * Discussion * The Isoscalar Giant Quadrupole Resonance * General Trends In Medium-Heavy and Heavy Nuclei * The GQR In Light Nuclei * The Isoscalar 3- Strength, LEOR and HEOR * Isoscalar 4+ Strength * Miscellaneous; Isoscalar 1- and L > 4-Strength * The Electric Isovector Giant Resonances * The Isovector Giant Dipole Resonance: GDR * The Isovector Giant Monopole Resonances: IVGMR * The Isovector Quadrupole Resonance: IVGQR * The Effect of Ground State Deformation on the Shape of Giant Resonance: Microscopic Picture * Giant Resonances Built on Excited States * Introduction * Capture Reactions on Light Nuclei * Statistical decay of GDR γ Emission in Heavy Compound Systems * Introduction * Theoretical Predictions * Some Experimental Results * Summary and Outlook * Acknowledgements * General References * References

  13. Sensory ecology: giant eyes for giant predators?

    PubMed

    Partridge, Julian C

    2012-04-24

    Mathematical models suggest the enormous eyes of giant and colossal squid evolved to see the bioluminescence induced by the approach of predatory whales. Copyright © 2012 Elsevier Ltd. All rights reserved.

  14. [A cystic mesenteric lymphangioma presented at the colon sigmoid. Case report].

    PubMed

    Ramírez-Ortega, Miguel Angel; Villegas-Romero, Javier; Márquez-Díaz, Adrián; Gómez-Díaz, Antonio

    2010-01-01

    Cystic lymphangioma of the mesentery is an uncommon tumor; its incidence is 1:160,000. Our objective was to present the case of a patient with cystic lymphangioma of mesentery located at the colon. Female 25 years, attending consultation with a clinical picture suggestive of right ovarian cyst and increased abdominal perimeter. On physical examination abdominal painful tumor was identified, with deep palpation and mobilization. Abdominal ultrasound findings suggested giant right ovarian cyst. Laparotomy showed a cystic mass (18 x 11 cm size) depending mesenterium and involving sigmoid colon, surgical intervention was done after two days for bowel preparation. Resection of the cyst, and colon section involving sigmoid colon with termino-terminal anastomosis, was performed. The pathological report was: cystic lymphangioma of sigmoid mesentery. There was no recurrence at six month follow up. Cystic lymphangioma of the mesentery is a benign abdominal tumor, which occurs frequently in children but in adults is rare. We found 49 cases of mesenteric cysts reported in Mexico, of which 21 were lymphangiomas with a range of 1 to 73 years of age.

  15. Cystic Neoplasms of the Pancreas

    PubMed Central

    Tran Cao, Hop S.; Kellogg, Benjamin; Lowy, Andrew M.; Bouvet, Michael

    2015-01-01

    Whereas pancreatic duct adenocarcinoma (PDA) is a well-studied (but still poorly understood) disease with a dismal prognosis, cystic neoplasms of the pancreas form a more recently recognized group of pancreatic tumors. They are diverse and variable in their pathologic characteristics, clinical course, and outcomes,1–3 although all portend a better overall prognosis than PDA. In recent years, with the improved sensitivity and increasing use of cross-sectional imaging in clinical practice, these lesions are more commonly identified,4 with many being discovered incidentally. Indeed, large radiological series using computed tomography (CT) or magnetic resonance imaging (MRI) have reported detection rates of pancreatic cystic lesions between 1.2% and almost 20%,5,6 approaching the 24.3% prevalence rate in an autopsy series by Kimura and colleagues.7 Although most of these lesions are pseudocysts, a significant portion consist of cystic neoplasms, which are estimated to represent 10% to 15% of all primary pancreatic cystic lesions.8 Given the growing clinical relevance of these tumors, a keen understanding of their natural history and pathophysiology is needed. This article reviews pancreatic cystic neoplasms, with a focus on the challenges encountered in their diagnosis and treatment. PMID:20159515

  16. 851 resected cystic tumors of the pancreas: A 33-year experience at the Massachusetts General Hospital

    PubMed Central

    Valsangkar, Nakul P.; Morales-Oyarvide, Vicente; Thayer, Sarah P.; Ferrone, Cristina R.; Wargo, Jennifer A.; Warshaw, Andrew L.; Castillo, Carlos Fernández-del

    2013-01-01

    Background The objective of this study was to identify trends in the diagnosis and treatment of cystic neoplasms of the pancreas using a retrospective review of patients from a surgical database at an academic referral center during a 33-year period. Methods Patient characteristics, including demographics, pathology, and survival, were analyzed over 5 time periods between 1978 and 2011. Results A total of 851 consecutive patients underwent resection for a cystic neoplasm of the pancreas during a 33-year period. Sixty-five percent of patients were female, and mean age was 60 years. The most common pathologic diagnoses were intraductal papillary mucinous neoplasm (38%), mucinous cystic neoplasm (23%), serous cystadenoma (16%), and cystic neuroendocrine neoplasm (7%). There was a stepwise increase in the number of resections across time periods (67 between 1978 and 1989; 376 between 2005 and 2011), with a parallel increase in the proportion of incidentally discovered lesions (22% to 50%). Diagnosis of intraductal papillary mucinous neoplasm was very uncommon in the first 2 time periods (before the first recognition of intraductal papillary mucinous neoplasm as a distinct entity) but predominated in the last 2 (41% and 49%), and cystic neuroendocrine neoplasms, which constituted 3% of the cystic neoplasms in the first time-period, now comprise more than 8% of pancreatic cystic neoplasms. The proportion of malignant neoplasms decreased over time (41% between 1978 and 1989; 12% between 2005 and 2011), reflecting probably the earlier diagnosis and treatment of premalignant neoplasms. Although operative mortality was minimal (4/849, 0.5%), the postoperative complication rate was 38%. Overall 5-year survival for all mucinous lesions was 87%. Conclusion Cystic neoplasms of the pancreas are being diagnosed and treated with increasing frequency. At present, most are incidentally discovered intraductal papillary mucinous neoplasms. (Surgery 2012;152:S4–12.) PMID:22770958

  17. Role of contrast harmonic-endoscopic ultrasound in pancreatic cystic lesions

    PubMed Central

    Serrani, Marta; Lisotti, Andrea; Caletti, Giancarlo; Fusaroli, Pietro

    2017-01-01

    Incidental pancreatic cysts (PCs) are frequently encountered in the general population often in asymptomatic patients who undergo imaging tests to investigate unrelated conditions. The detection of a PC poses a significant clinical dilemma, as the differential diagnosis is quite broad ranging from benign to malignant conditions. Endoscopic ultrasound (EUS) with fine-needle aspiration (FNA) has been reported to be an accurate tool in the differential diagnosis; however, its sensitivity is suboptimal and false negative results do occur. Contrast harmonic EUS (CH-EUS) was demonstrated to be a useful tool to investigate pancreatic solid lesions to differentiate between benign and malignant ones. In the setting of PCs, CH-EUS could help identify areas of malignant growth inside the cystic cavities. Several studies have reported promising results showing malignant areas in PCs as hyperenhanced lesions. Confirmation of malignancy can then be obtained by FNA, which should be precisely targeted according to the findings of the contrast harmonic study. PMID:28218197

  18. [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.

  19. Cystic Fibrosis (CF): Chloride Sweat Test

    MedlinePlus

    ... to 2-Year-Old Cystic Fibrosis (CF) Chloride Sweat Test KidsHealth > For Parents > Cystic Fibrosis (CF) Chloride Sweat Test Print A A A What's in this ... en el sudor What It Is A chloride sweat test helps diagnose cystic fibrosis (CF) , an inherited ...

  20. Cystic Fibrosis (CF): Chloride Sweat Test

    MedlinePlus

    ... 1- to 2-Year-Old Cystic Fibrosis (CF) Chloride Sweat Test KidsHealth > For Parents > Cystic Fibrosis (CF) Chloride Sweat Test A A A What's in this ... cloruro en el sudor What It Is A chloride sweat test helps diagnose cystic fibrosis (CF) , an ...

  1. What's it Like to Have Cystic Fibrosis?

    MedlinePlus

    ... Room? What Happens in the Operating Room? Cystic Fibrosis KidsHealth > For Kids > Cystic Fibrosis A A A What's in this article? What ... a condition she's known all her life — cystic fibrosis (say: SIS-tik fi-BRO-sus). Her parents ...

  2. Pancreatic endometrial cyst mimics mucinous cystic neoplasm of the pancreas.

    PubMed

    Mederos, Michael A; Villafañe, Nicole; Dhingra, Sadhna; Farinas, Carlos; McElhany, Amy; Fisher, William E; Van Buren Ii, George

    2017-02-14

    Pancreatic cysts include a variety of benign, premalignant, and malignant lesions. Endometrial cysts in the pancreas are exceedingly rare lesions that are difficult to diagnose pre-operatively. This report describes the findings in a 43-year-old patient with a recent episode of acute pancreatitis who presented with a large cyst in the tail of the pancreas. Imaging demonstrated a loculated pancreatic cyst, and cyst fluid aspiration revealed an elevated amylase and carcinoembryonic antigen. The patient experienced an interval worsening of abdominal pain, fatigue, diarrhea, and a 15-pound weight loss 3 mo after the initial episode of pancreatitis. With concern for a possible pre-malignant lesion, the patient underwent a laparoscopic distal pancreatectomy with splenectomy, which revealed a 16 cm × 12 cm × 4 cm lesion. Final histopathology was consistent with an intra-pancreatic endometrial cyst. Here we discuss the overlapping imaging and laboratory features of pancreatic endometrial cysts and mucinous cystic neoplasms of the pancreas.

  3. Radioisotope therapy of cystic craniopharyngeomas

    SciTech Connect

    Strauss, L.; Sturm, V.; Georgi, P.; Schlegel, W.; Ostertag, H.; Clorius, J.H.; Van Kaick, G.

    1982-09-01

    Eighteen patients suffering from cystic craniopharyngeoma were treated with intracavitary irradiation. The beta-emitting radioisotope /sup 90/y (2.25 MeV) was instilled into the cyst following stereotactic puncture of the space-occupying lesion. The surgical approach was planned using angiograms and reconstructed transmission computer tomography (TCT) coronal and saggital sections. Therapy was devised to deliver 20,000 rad to the cyst's wall. Eleven patients received follow-up TCT examinations after four months. Eight of 11 patients had a significant volume decrease in the craniopharyngeoma cyst. In two patients, the cystic volume remained unchanged; one had progression of disease. It is concluded that the intracavitary treatment of cystic craniopharyngeoma will result in a reduction of the size of the space-occupying lesion.

  4. [Intestinal cystic duplication. Case report].

    PubMed

    Herranz Barbero, Ana; Prat Ortells, Jordi; Muñoz Fernández, M Elena; Castañón García-Alix, Montserrat; Figueras Aloy, Josep

    2017-08-01

    Intestinal cystic duplications are rare congenital anomalies, with an estimated incidence of approximately 1:4500 autopsies. The etiopathogenesis is uncertain. These duplications are cystic, tubular or diverticular structures lined with gastrointestinal mucosa. They share a common smooth muscle wall with the gastrointestinal tract but usually their lumens do not communicate with each other. Gastric duplication cysts represent 7-9% of the gastrointestinal tract duplication. They can be diagnosed prenatally by fetal ultrasound; magnetic resonance imaging characterizes the cyst and excludes other malformations. Postnatal ultrasound shows a characteristic double walled cyst. Newborns are usually asymptomatic, although nonspecific gastrointestinal symptoms, intestinal obstruction due to mass effect, volvulus or infection are described. In asymptomatic patients, clinical follow-up and periodic image controls are recommended. Elective surgical resection is the treatment of choice, using minimally invasive technique whenever possible. A case of prenatally suspected intestinal cystic duplication is presented. Sociedad Argentina de Pediatría.

  5. [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.

  6. Nutritional management of cystic fibrosis.

    PubMed Central

    MacDonald, A

    1996-01-01

    Nutritional support is an integral part of the management of cystic fibrosis patients. It is arguably best provided by a qualified dietitian and nutritional care sister working in conjunction with the rest of the cystic fibrosis team. The patient's nutritional needs should be assessed, regularly reviewed, and nutritional treatment tailored to meet the changing clinical and psychosocial needs of the patient. Nutritional intervention is not without complications, and in particular attention to normal feeding behaviour and vigilance when instituting supplementary nutrition may prevent many feeding difficulties. PMID:8660059

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

    PubMed Central

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

    2003-01-01

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

  8. Congenital cystic adenomatoid malformation of the lung: hazards of delayed diagnosis.

    PubMed

    Collins, Anne M; Ridgway, Paul F; Killeen, Ronan P; Dodd, Jonathan D; Tolan, Michael

    2009-09-01

    Congenital cystic adenomatoid malformation is a rare pulmonary developmental anomaly, which typically manifests in neonates and infants. Presentation in adulthood is uncommon, with <60 cases reported in the literature. The majority of cases involve one lobe only. We report a case of type 1 congenital cystic adenomatoid malformation in an adult presenting with a respiratory tract infection and haemoptysis. At thoracotomy, complex cystic masses were noted in the right upper and lower lobes. Lung-sparing surgery, in the form of two segmentectomies and a non-anatomical resection, was performed in order to avoid pneumonectomy. Such presentations may be problematic as potentially incomplete resections may increase the risk of complications and malignant transformation. This suggests the importance of appropriate clinical and radiological follow up.

  9. Giant mesenteric cyst: a rare cause of abdominal distension diagnosed with CT and managed with ultrasound-guided drainage.

    PubMed

    Ma, Alexander; Ayre, Karyn; Wijeyekoon, Sanjaya

    2012-09-03

    This rare cystic lesion has an approximate incidence of 1 in 200,000. This patient complained of abdominal distension and was diagnosed with a giant mesenteric cyst measuring 26 cm in height using CT imaging. She underwent a successful ultrasound-guided drainage, which is not previously reported in the literature. This represents utilisation of an established procedure in a novel situation.

  10. An atypical growth of a giant fibroadenoma after trauma.

    PubMed

    Izadpanah, Ali; Karunanayake, Mihiran; Izadpanah, Arash; Sinno, Hani; Gilardino, Mirko

    2012-10-01

    Fibroadenomas are the most common benign breast lesion in female adolescents. However, it is important to recognize that a small percentage have been shown to progress to giant fibroadenomas. Giant fibroadenomas can spontaneously infarct leading to significant morbidity and are also difficult to distinguish from the more aggressive phyllodes tumors. We describe the first case, to the best of our knowledge, of a 12-year-old girl who presented with a giant fibroadenoma complicated by a central infarct and an intra-lesional hemorrhage from a trauma to the breast. The complicated giant fibroadenoma with an intra-lesional hemorrhage has characteristics of both benign and malignant lesions, and is difficult to distinguish by history and physical alone. Ultrasonography is a valuable tool yet the core needle biopsy remains the gold standard to confirm the diagnosis. Copyright © 2012 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  11. Undiagnosed Pheochromocytoma Simulating Malignant Hyperthermia.

    PubMed

    Ramani, Nisha S; Stoppacher, Robert; Morani, Ajaykumar C; Catanese, Charles

    2017-09-01

    Pheochromocytomas are rare catecholamine-producing neuroendocrine tumors. They are surgically curable but can be lethal if remain undiagnosed. We report a patient earlier diagnosed with malignant hyperthermia but later found to have pheochromocytoma on autopsy. After a preprocedural pain block for elective right shoulder arthroscopy, a 53-year-old hypertensive white man developed chest pain. In the operating room, he had increased blood pressure. Postoperatively, his blood pressures dropped from 220/100 to 80/30 mm Hg. He later developed high fever with core temperature reaching a peak of 42.2°C, rapid breathing, and died after unsuccessful attempts to stabilize him. Autopsy revealed a tumor in his right adrenal gland, measuring 10 cm in greatest dimension and weighing 530 g. It was red brown with a hemorrhagic and cystic cut surface. A thin rim of yellow-orange adrenal cortex was visible at the margin of the tumor, indicating that it originated from the underlying adrenal medulla. The left adrenal gland was unremarkable.Sections showed hypercellular tumor with zellballen architecture. The tumor cells were round to oval with finely granular basophilic cytoplasm and mild pleomorphism. A 24-hour urine sample collected before his death showed greater than 22727 μg/g Ratio to Creatinine metanephrines and normetanephrine, indicating that the tumor was active and secreted high levels of catecholamine. The cause of death was established as the complications of pheochromocytoma in the settings of general anesthesia for shoulder arthroscopy. The manner of death was natural. Pheochromocytoma can mimic malignant hyperthermia, and it should always be considered and managed appropriately in such scenarios to avoid untoward consequences. Pathologists must also be aware of this when conducting an autopsy in cases with a previous clinical diagnosis of malignant hyperthermia.

  12. Giant cell reparative granuloma of the sphenoid bone.

    PubMed

    Aralasmak, A; Aygun, N; Westra, W H; Yousem, D M

    2006-09-01

    We present 2 patients with giant cell reparative granuloma (GCRG) of the sphenoid bone. The first patient is an 8-year-old boy with involvement of the greater wing, and the second is a 53- year-old man with a lateral pterygoid plate mass. Both patients presented with rapid expansion of lytic bone lesions, which had solid and cystic components and lacked matrix calcification. Biopsies were indeterminate for definitive diagnoses. The radiologic appearance, location, and incidence of the lesions, and the patient's age and medical history are helpful aids in narrowing the differential diagnosis of sphenoid bone lesions. However, the imaging and, occasionally, even the histologic findings may not suggest the specific diagnosis of GCRG, which must be added into the differential diagnosis of rapidly enlarging cystic bone lesions of the sphenoid bone.

  13. Survival rates in cystic fibrosis.

    PubMed Central

    Wilmott, R W; Tyson, S L; Dinwiddie, R; Matthew, D J

    1983-01-01

    Life tables were calculated for 273 British children with cystic fibrosis for the period 1974-9. There was a marked improvement in survival rates in the meconium ileus group compared with the 1969-73 data, but there was little improvement in patients presenting later with other symptoms. PMID:6639137

  14. Nutritional management of cystic fibrosis.

    PubMed

    Goodchild, M C

    1987-01-01

    Cystic fibrosis patients have an increased requirement for calories and probably for all the major nutrients. The newer, enteric-coated granular preparations of pancreatic enzyme are more effective than preceding preparations and should permit a normal fat intake. Recent work has emphasized the interdependence of respiratory disease and nutrition.

  15. Cystic lumphangioma of the colon

    SciTech Connect

    Agha, F.P.; Francis, I.R.; Simms, S.M.

    1983-10-01

    Cystic lymphangioma is a rare benign lesion of the gastrointestinal tract, in which the colon is the least frequntly involved site. A case is reported displaying the characteristic radiographic features of an extramucosal intramural mass lesion in a patient with concurrent cystadenocarcinoma of the pancreas, in whom the possibility of a metastatic lesion to the colon could not be excluded except by surgical resection.

  16. Malignant hyperthermia

    PubMed Central

    Rosenberg, Henry; Davis, Mark; James, Danielle; Pollock, Neil; Stowell, Kathryn

    2007-01-01

    Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle that presents as a hypermetabolic response to potent volatile anesthetic gases such as halothane, sevoflurane, desflurane and the depolarizing muscle relaxant succinylcholine, and rarely, in humans, to stresses such as vigorous exercise and heat. The incidence of MH reactions ranges from 1:5,000 to 1:50,000–100,000 anesthesias. However, the prevalence of the genetic abnormalities may be as great as one in 3,000 individuals. MH affects humans, certain pig breeds, dogs, horses, and probably other animals. The classic signs of MH include hyperthermia to marked degree, tachycardia, tachypnea, increased carbon dioxide production, increased oxygen consumption, acidosis, muscle rigidity, and rhabdomyolysis, all related to a hypermetabolic response. The syndrome is likely to be fatal if untreated. Early recognition of the signs of MH, specifically elevation of end-expired carbon dioxide, provides the clinical diagnostic clues. In humans the syndrome is inherited in autosomal dominant pattern, while in pigs in autosomal recessive. The pathophysiologic changes of MH are due to uncontrolled rise of myoplasmic calcium, which activates biochemical processes related to muscle activation. Due to ATP depletion, the muscle membrane integrity is compromised leading to hyperkalemia and rhabdomyolysis. In most cases, the syndrome is caused by a defect in the ryanodine receptor. Over 90 mutations have been identified in the RYR-1 gene located on chromosome 19q13.1, and at least 25 are causal for MH. Diagnostic testing relies on assessing the in vitro contracture response of biopsied muscle to halothane, caffeine, and other drugs. Elucidation of the genetic changes has led to the introduction, on a limited basis so far, of genetic testing for susceptibility to MH. As the sensitivity of genetic testing increases, molecular genetics will be used for identifying those at risk with greater frequency. Dantrolene

  17. Whole exome sequencing of adenoid cystic carcinoma

    PubMed Central

    Stephens, Philip J.; Davies, Helen R.; Mitani, Yoshitsugu; Van Loo, Peter; Shlien, Adam; Tarpey, Patrick S.; Papaemmanuil, Elli; Cheverton, Angela; Bignell, Graham R.; Butler, Adam P.; Gamble, John; Gamble, Stephen; Hardy, Claire; Hinton, Jonathan; Jia, Mingming; Jayakumar, Alagu; Jones, David; Latimer, Calli; McLaren, Stuart; McBride, David J.; Menzies, Andrew; Mudie, Laura; Maddison, Mark; Raine, Keiran; Nik-Zainal, Serena; O’Meara, Sarah; Teague, Jon W.; Varela, Ignacio; Wedge, David C.; Whitmore, Ian; Lippman, Scott M.; McDermott, Ultan; Stratton, Michael R.; Campbell, Peter J.; El-Naggar, Adel K.; Futreal, P. Andrew

    2013-01-01

    Adenoid cystic carcinoma (ACC) is a rare malignancy that can occur in multiple organ sites and is primarily found in the salivary gland. While the identification of recurrent fusions of the MYB-NFIB genes have begun to shed light on the molecular underpinnings, little else is known about the molecular genetics of this frequently fatal cancer. We have undertaken exome sequencing in a series of 24 ACC to further delineate the genetics of the disease. We identified multiple mutated genes that, combined, implicate chromatin deregulation in half of cases. Further, mutations were identified in known cancer genes, including PIK3CA, ATM, CDKN2A, SF3B1, SUFU, TSC1, and CYLD. Mutations in NOTCH1/2 were identified in 3 cases, and we identify the negative NOTCH signaling regulator, SPEN, as a new cancer gene in ACC with mutations in 5 cases. Finally, the identification of 3 likely activating mutations in the tyrosine kinase receptor FGFR2, analogous to those reported in ovarian and endometrial carcinoma, point to potential therapeutic avenues for a subset of cases. PMID:23778141

  18. Cystic lesions of the adrenal gland: our experience over the last 20 years.

    PubMed

    Sebastiano, Christopher; Zhao, Xiangrong; Deng, Fang-Ming; Das, Kasturi

    2013-09-01

    Cystic lesions of the adrenal gland are uncommon, often presenting with nonspecific clinical and radiologic findings, and are thus underrecognized. They are occasionally associated with malignant neoplasms, which can greatly mimic benign lesions and carry detrimental clinical consequences if misdiagnosed. Here we present our 20-year experience (1992-2012) with these lesions at an academic medical center. Among more than 4500 adrenal gland specimens, 31 cases of adrenal lesions with a predominant cystic component were identified in 30 patients with an age range of 34 to 86 years (median, 55.5 years) and a male/female ratio of 13:17. Macroscopic descriptions, available histologic and immunostain slides, and available radiologic records were reviewed for all included cases. Radiologic studies and gross examination correlated well, and hemorrhage (26 cases; 84%) and encapsulation (25 cases; 81%) appeared to be nonspecific radiologic/gross features shared across histologic subtypes. Microscopic review identified 12 cases (39%) of pseudocysts, 2 cases (6%) of endothelium-derived cysts, and 17 cases (55%) of epithelium-derived cysts. Among these 31 cystic adrenal lesions, 2 cases (6%) were malignant neoplasms (1 epithelioid angiosarcoma, 1 adrenocortical carcinoma). Radiologic impression and histopathologic diagnosis were concordant in 11 (73%) of the 15 cases for which radiologic records were available. This study represents the second largest case series to date on cystic adrenal lesions and presents a comprehensive review on their demographic, clinical, radiologic, and gross and microscopic pathologic features, as well as their differential diagnoses.

  19. [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.

  20. Metastatic pancreatic carcinoma masquerading as cystic lung disease: a rare presentation.

    PubMed

    Stern, Emily; Huseini, Taha; Kuok, YiJin; Lake, Fiona

    2017-09-01

    This 52-year-old male ex-smoker presented with a six-month history of progressive breathlessness and weight loss. He deteriorated acutely, and was admitted with severe type 1 respiratory failure. Apart from diffuse coarse crackles on chest auscultation, physical examination was unremarkable. High-resolution computed tomography (HRCT) showed diffuse cystic changes throughout the lungs. A diagnosis of pulmonary Langerhans cell histiocytosis (PLCH) was considered. Further workup identified a coincidental pancreatic lesion of uncertain significance, which remained indeterminate on magnetic resonance imaging (MRI) and on positron emission tomography (PET). Transbronchial biopsy revealed enteric differentiated adenocarcinoma exhibiting lepidic spread, and autopsy later confirmed primary pancreatic malignancy. This case demonstrates that metastatic pancreatic malignancy can present with severe respiratory failure and masquerade as cystic lung disease.

  1. Adenoid Cystic Carcinoma of the Oral Cavity: Radiology-Pathology Correlation.

    PubMed

    Uraizee, Imran; Cipriani, Nicole A; Ginat, Daniel T

    2017-09-06

    Adenoid cystic carcinoma in the oral cavity is an uncommon salivary gland malignancy that has a propensity for perineural spread. A high-grade variant is evidenced by an abundance of pleomorphic cells, loss of the classic biphasic epithelial-myoepithelial growth pattern, and comedonecrosis, as well as elevated Ki-67. CT and MRI can both be useful for demonstrating the extent of invasion in oral cavity-associated adenoid cystic carcinoma, which can attain the inferior alveolar nerve for perineural spread by direct invasion through the mandible. Reflecting the aggressive nature of this high-grade malignancy, (18)FDG-PET can demonstrate hypermetabolism and can be useful for staging. These features are exemplified in this sine qua non radiology-pathology correlation article.

  2. Interventions for preventing and managing advanced liver disease in cystic fibrosis.

    PubMed

    Palaniappan, Senthil K; Than, Nan Nitra; Thein, Aung Win; Moe, Soe; van Mourik, Indra

    2017-08-29

    Cystic fibrosis is an autosomal recessive inherited defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene resulting in abnormal regulation of salt and water movement across the membranes. In the liver this leads to focal biliary fibrosis resulting in progressive portal hypertension and end-stage liver disease in some individuals. This can be asymptomatic, but may lead to splenomegaly and hypersplenism, development of varices and variceal bleeding, and ascites; it has negative impact on overall nutritional status and respiratory function in this population. Prognosis is poor once significant portal hypertension is established. The role and outcome of various interventions for managing advanced liver disease (non-malignant end stage disease) in people with cystic fibrosis is currently unidentified. To review and assess the efficacy of currently available treatment options for preventing and managing advanced liver disease in children and adults with cystic fibrosis. We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books.Date of last search: 06 April 2017.We also searched the reference lists of relevant articles and reviews and online trials registries. Date of last search: 04 January 2017. Any published and unpublished randomised controlled trials and quasi-randomised controlled trials of advanced liver disease in cystic fibrosis with cirrhosis or liver failure, portal hypertension or variceal bleeding (or both). Authors independently examined titles and abstracts to identify potentially relevant trials, but none were eligible for inclusion in this review. A comprehensive search of the literature did not identify any published eligible randomised controlled trials. In order to develop the best source of evidence, there is a need to undertake randomised controlled trials of interventions for preventing and managing advanced liver disease in

  3. Malignant pigmented villonodular synovitis in the knee - report of a case with rapid clinical progression.

    PubMed

    Imakiire, Naoaki; Fujino, Takashi; Morii, Takeshi; Honya, Keita; Mochizuki, Kazuo; Satomi, Kazuhiko; Fujioka, Yasunori

    2011-01-07

    Malignant pigmented villonodular synovitis (PVNS) (or malignant giant cell tumor of tendon sheath (GCTTS) is an extremely rare condition defined as a malignant lesion occurring with concomitant or previously documented PVNS at the same site. To date, only less than 20 cases have been reported in English literatures. We report a case of malignant PVNS in the knee in a 56-year-old woman with unpredictable rapid progression. This case raised a caution that when atypical components in specimens of recurrent benign PVNS are detected, even if low-grade or tiny, both pathologists and surgeons should consider the risk of malignant PVNS, which could display aggressive clinical progression.

  4. 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. Crown Copyright © 2012. Published by Elsevier Inc. All rights reserved.

  5. Adamantinoma of the tibia mimicking a benign cystic lesion: a case report.

    PubMed

    Mavrogenis, Andreas F; Galanakos, Spyridon; Savvidou, Olga D; Papagelopoulos, Panayiotis J

    2010-01-01

    Adamantinoma of the long bones is a rare primary malignant bone tumor accounting for less than 1% of all primary malignant bone tumors. Most typical imaging findings include heterogeneous osteolytic cortical lesions showing multiple sharply circumscribed lucent zones of various sizes with sclerotic bone surroundings, interspersing between, and extending above and below the lucent zones by the thinning and bulging cortex. Advanced or recurrent lesions may be associated with destruction of the cortex and soft tissue extension. In this article, the authors present a case report of a patient with a distal tibia adamantinoma mimicking a benign cystic lesion.

  6. Giant Baker's Cyst Associated with Rheumatoid Arthritis

    PubMed Central

    Bılgın, Emre; Ketencı, İsmail Emre; Ugurlar, Meriç

    2017-01-01

    We report a rare case of a “giant Baker's cyst-related rheumatoid arthritis (RA)” with 95 × 26 mm dimensions originating from the semimembranosus tendon. The patient presented with chronic pain and a palpable mass behind his left calf located between the posteriosuperior aspect of the popliteal fossa and the distal third of the calf. In MRI cystic lesion which was located in soft tissue at the posterior of gastrocnemius, extensive synovial pannus inside and degeneration of medial meniscus posterior horn were observed. Arthroscopic joint debridement and partial excision of the cyst via biomechanical valve excision were performed. The patient continued his follow-up visits at Rheumatology Department and there was no recurrence of cyst-related symptoms in 1-year follow-up. Similar cases were reported in the literature previously. However, as far as we know, a giant Baker's cyst-related RA, which was treated as described, has not yet been presented. PMID:28116197

  7. Giant prostatic calculi

    PubMed Central

    Najoui, Mohammed; Qarro, Abdelmounaim; Ammani, Abdelghani; Alami, Mohammed

    2013-01-01

    Prostatic parenchymal calculi are common, usually incidental, findings on morphological examinations. They are typically asymptomatic and may be present in association with normal glands, benign prostatic hyperplasia, and prostate cancer. However giant prostatic calculi are rare. Less than 20 cases have been reported in the literature. We present the case of a 35-year-old man with two giant prostatic calculi that replaced the entire gland. He underwent an open cystolithotomy, two giant stones were removed from the prostate, and we used a lithotripsy in situ for extraction of stone fragments. PMID:23565316

  8. 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.

  9. Giant Gartner duct cyst and elevated CA-125.

    PubMed

    Vlahovic, A; Stankovic, Z B; Djuricic, S; Savic, D

    2014-12-01

    Gartner duct cysts represent vestigial remnants of the caudal end of the mesonephric (Wolffian) ducts. They are often associated with ureteral and renal abnormalities. In most cases they are solitary, less than 2 cm in diameter. Giant Gartner duct cysts are extremely rare. We present a girl with a giant Gartner duct cyst, without notable associated abnormalities or pathology, successfully treated by surgical excision. Preoperative aspiration revealed elevated level of CA-125 in the cyst fluid, with normal serum level of the same marker. Analyzing the aspirated fluid for CA-125 may be a useful tool for evaluation of cystic lesions in this region. Crown Copyright © 2014. Published by Elsevier Inc. All rights reserved.

  10. [Giant retroperitoneal liposarcoma--case report].

    PubMed

    Bánky, Balázs; Bányász, Zsolt; Mayer, Arpád; Almási, Kálmán; Szucs, Iván

    2005-06-01

    Retroperitoneal liposarcomas are the most frequent soft tissue sarcomas and the second most frequent retroperitoneal tumours. They represent less then 0.1% of all human malignancies. Hereby we describe the treatment of our patient where of a 15-kg giant retroperitoneal liposarcoma was successfully removed and a local recurrence two years later was operated on as well. This was the 3rd largest retroperitoneal malignant tumor that was successfully removed according to the available literature of the last five decades. These typically symptom-free tumors usually grow extreme size before diagnosed. The "gold-standard" of treatment remains surgical total excision, but the high local recurrence rate (50-60%) hopefully can be reduced by adjuvant radio- and chemotherapy. Multidisciplinary treatment and long-time follow-up can provide as high as 40% 5-year survival rate. We summarize the recent clinical, diagnostic and therapeutic methods of this rare condition.

  11. Laparoscopic management of enlarged cystic duct.

    PubMed

    Nowzaradan, Y; Meador, J; Westmoreland, J

    1992-12-01

    After laparoscopic exploration of the common bile duct, or when a patient has acute cholecystitis, the cystic duct is sometimes edematous and too large to be ligated safely with an Endoclip. In such cases, ligation of the cystic duct with an Endoloop offers a solution to the problem. The standard technique for application of an Endoloop consists of dividing the cystic duct and then applying the Endoloop. This becomes more difficult if, after the cystic duct is divided, loss of traction on the common bile duct results in retraction of the divided cystic stump outside of the laparoscopic field of view. To avoid this difficulty, the authors apply an Endoloop with the grasping forceps on the cystic duct before the duct is divided so that it cannot retract from operative view and for this task developed an instrument that allows simultaneous introduction of both grasping forceps and the Endoloop through a single port.

  12. 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.

  13. Congenital Giant Hydroureteric Cistern in a Duplex System of an Infant

    PubMed Central

    Awolaran, O. T.; Abdur-Rahman, L. O.; Bamigbola, K. T.; Adesiyun, O. M.; Nasir, A. A.

    2013-01-01

    Duplex collecting system is a congenital genitourinary anomaly commonly found incidentally. Our experience with a duplex system associated with giant hydroureter presenting as mobile abdominal swelling that was noticed from birth, constipation, and failure to thrive is described. Ultrasound and IVU did not assist in making the diagnosis, while a barium enema suggested a colonic duplication. Congenital giant hydroureter should be considered as a differential diagnosis in infants with cystic abdominal swelling. A preserved renal moiety attributed to a dilated ureteric cistern was a unique theory in this case. PMID:24171132

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

    ClinicalTrials.gov

    2016-11-01

    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

  15. Cystic Fibrosis Research | NIH MedlinePlus the Magazine

    MedlinePlus

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

  16. [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. Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.

  17. 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.

  18. Pseudomembranous colitis in cystic fibrosis.

    PubMed

    Nagakumar, Prasad

    2013-05-01

    Cystic fibrosis (CF) patients may require frequent courses of antibiotics and repeated hospital admissions. Although children with CF have high carriage rate for C.difficile, they rarely develop colitis. Pseudomembranous colitis is more common in adult post lung transplant CF patients. Although rare, paseudomembranous colitis should be considered in CF patients presenting with abdominal symptoms even in the absence of diarrhoea. Copyright © 2013 Elsevier Ltd. All rights reserved.

  19. Etanercept-induced cystic acne.

    PubMed

    Kashat, Maria; Caretti, Katherine; Kado, Jessica

    2014-07-01

    Tumor necrosis factor α antagonists are potent biologics used to treat a variety of autoimmune disorders such as rheumatoid arthritis, ankylosing spondylitis, Crohn disease, psoriasis, and psoriatic arthritis. These medications are known to have many side effects (eg, infusion reactions, cytopenia, risk for infection, heart failure); however, only a few cases of acne vulgaris have been associated with the use of these biologics, particularly infliximab and adalimumab. We report a rare case of etanercept-induced cystic acne.

  20. Tracks of a Giant

    NASA Image and Video Library

    2010-08-25

    The giant, 70-meter-wide antenna at NASA Deep Space Network complex in Goldstone, Calif., tracks a spacecraft on Nov. 17, 2009. This antenna, officially known as Deep Space Station 14, is also nicknamed the Mars antenna.

  1. The Next Giant Step

    NASA Image and Video Library

    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 ...

  2. Silvics of Giant Sequoia

    Treesearch

    C. Phillip Weatherspoon

    1986-01-01

    Ecological relationships-including habitat and life history---of giant sequoia (Sequoiadendron giganteum [Lindl.] Buchholz) in natural stands are summarized. Such silvical information provides an important foundation for sound management of the species.

  3. 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)

  4. 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)

  5. Giant Cell Arteritis and Polymyalgia Rheumatica

    MedlinePlus

    ... Controlfamilydoctor.org editorial staff Home Diseases and Conditions Giant Cell Arteritis and Polymyalgia Rheumatica Condition Giant Cell Arteritis and Polymyalgia Rheumatica Share Print Giant ...

  6. Capella: Separating the Giants

    NASA Astrophysics Data System (ADS)

    Young, P. R.; Dupree, A. K.

    2002-01-01

    Images from the Faint Object Camera (FOC) on the Hubble Space Telescope (HST) are used to spatially separate the two giants of Capella (α Aurigae; HD 34029) for the first time at ultraviolet wavelengths. The images were obtained with broadband filters that isolate the wavelength regions 2500-3000 Å and 1300-1500 Å. The cool G8 giant is found to be weaker than the hot G1 giant by factors of around 4 and 17, respectively, in these bands. The latter factor is largely due to the much stronger G1 continuum at short wavelengths. No evidence is found for material lying between the two stars in the images. In addition, the objective prisms of the FOC were used to obtain low-resolution spectra from 1200 to 3000 Å, allowing individual emission lines from each star to be spatially separated. Cool-to-hot star ratios for the emission lines H I Lyα, O I λ1305, Si II λ1816, C II λ1335, He II λ1640, and Si IV λ1393 are presented, showing that the cool giant is weaker than the hot giant by factors of 5-10 in these lines. The O I emission is only a factor of 2.5 weaker in the cool giant, most probably resulting from fluorescence in the extended atmosphere of the cool giant. The line ratios are compared with values derived from International Ultraviolet Explorer and HST/Goddard High Resolution Spectrograph spectra, which could separate the stars spectrally but not spatially. Reasonable agreement is found although the FOC ratios generally imply lower contributions from the cool giant. Based on observations with the NASA/ESA Hubble Space Telescope obtained at the Space Telescope Science Institute, which is operated by AURA, Inc., under NASA contract NAS 5-26555.

  7. Nasopharyngeal Carcinoma with Cystic Cervical Metastasis Masquerading as Branchial Cleft Cyst: A Potential Pitfall in Diagnosis and Management

    PubMed Central

    Sai-Guan, Lum; Min-Han, Kong; Kah-Wai, Ngan; Mohamad-Yunus, Mohd-Razif

    2017-01-01

    Introduction: Most metastatic lymph nodes from head and neck malignancy are solid. Cystic nodes are found in 33% - 61% of carcinomas arise from Waldeyer’s ring, of which only 1.8% - 8% originate are from the nasopharynx. Some cystic cervical metastases were initially presumed to be branchial cleft cyst. This case report aims to highlight the unusual presentation of cystic cervical metastasis secondary to nasopharyngeal carcinoma in a young adult. The histopathology, radiological features and management strategy were discussed. Case Report: A 36-year-old man presented with a solitary cystic cervical swelling, initially diagnosed as branchial cleft cyst. Fine needle aspiration yielded 18 ml of straw-coloured fluid. During cytological examination no atypical cells were observed. Computed tomography of the neck showed a heterogeneous mass with multiseptation medial to the sternocleidomastoid muscle. Histopathological examination of the mass, post excision, revealed a metastatic lymph node. A suspicious mucosal lesion at the nasopharynx was detected after repeated thorough head and neck examinations and the biopsy result confirmed undifferentiated nasopharyngeal carcinoma. Conclusion: Cystic cervical metastasis may occur in young patients under 40 years. The primary tumour may not be obvious during initial presentation because it mimicks benign branchial cleft cyst clinically. Retrospective review of the computed tomography images revealed features that were not characteristic of simple branchial cleft cyst. The inadequacy of assessment and interpretation had lead to the error in diagnosis and subsequent management. Metastatic head and neck lesion must be considered in a young adult with a cystic neck mass. PMID:28393061

  8. [Malignant soft tissue tumors].

    PubMed

    Schauer, A; Altmannsberger, M

    1984-01-01

    This article is a survey of actual aspects. With regard to frequency, the malignant fibrous histocytoma comes first, followed by lipo- and fibrosarcoma, synovial sarcoma, malignant schwannoma, malignant tumours proceeding from arteries and veins and the unstriated musculature. Staging and grading of these tumours are difficult. Until now their overall TNM-classification was not possible due to insufficient hard criteria.

  9. 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

  10. Cystic adrenal lesions: focus on pediatric population (a review)

    PubMed Central

    CARSOTE, MARA; GHEMIGIAN, ADINA; TERZEA, DANA; GHEORGHISAN-GALATEANU, ANCUTA AUGUSTINA; VALEA, ANA

    2017-01-01

    Background and aim The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. General data Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression. Adrenal hemorrhage represents a particular condition associating precipitating factors such as: coagulation defects as Factor IX or X deficiency, von Willebrand disease, thrombocytopenia; antiphospholipid syndrome; previous therapy with clopidogrel or corticosteroids; the rupture of a prior tumour. At birth, the most suggestive features are abdominal palpable mass, anemia, and persistent jaundice. Adrenal insufficiency may be found especially in premature delivery. The hemorrhage is mostly self-limiting. Antenatal ultrasound diagnosis of a cyst does not always predict the exact pathology result. The most important differential diagnosis of adrenal hemorrhage/hemorrhagic cyst is cystic neuroblastoma which is highly suggestive in the presence of distant metastases and abnormal catecholamine profile. The major clue to differentiate the two conditions is the fact that the tumor is stable or increases over time while the adrenal hemorrhage is expected to remit within one to two weeks. Conclusion Pediatric adrenal cysts vary from simple cysts with a benign behavior to neoplasia- related lesions displaying severe prognosis as seen in cystic neuroblastoma. A multidisciplinary team is required for their management which is conservative as close follow-up or it makes necessary different surgical procedures in cases with large masses or if a malignancy suspicion is presented

  11. Cystic adrenal lesions: focus on pediatric population (a review).

    PubMed

    Carsote, Mara; Ghemigian, Adina; Terzea, Dana; Gheorghisan-Galateanu, Ancuta Augustina; Valea, Ana

    2017-01-01

    The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression. Adrenal hemorrhage represents a particular condition associating precipitating factors such as: coagulation defects as Factor IX or X deficiency, von Willebrand disease, thrombocytopenia; antiphospholipid syndrome; previous therapy with clopidogrel or corticosteroids; the rupture of a prior tumour. At birth, the most suggestive features are abdominal palpable mass, anemia, and persistent jaundice. Adrenal insufficiency may be found especially in premature delivery. The hemorrhage is mostly self-limiting. Antenatal ultrasound diagnosis of a cyst does not always predict the exact pathology result. The most important differential diagnosis of adrenal hemorrhage/hemorrhagic cyst is cystic neuroblastoma which is highly suggestive in the presence of distant metastases and abnormal catecholamine profile. The major clue to differentiate the two conditions is the fact that the tumor is stable or increases over time while the adrenal hemorrhage is expected to remit within one to two weeks. Pediatric adrenal cysts vary from simple cysts with a benign behavior to neoplasia- related lesions displaying severe prognosis as seen in cystic neuroblastoma. A multidisciplinary team is required for their management which is conservative as close follow-up or it makes necessary different surgical procedures in cases with large masses or if a malignancy suspicion is presented. Recently, laparoscopic approach is regarded as

  12. Negative sweat tests and cystic fibrosis.

    PubMed Central

    Sarsfield, J K; Davies, J M

    1975-01-01

    Two brothers are described with chronic suppurative pulmonary disease. One has classical cystic fibrosis with complete pancreatic involvement and abnormal sweat test. The other had incomplete pancreatic disease with repeatedly normal sweat tests. The implications of a negative sweat test in patients with cystic fibrosis are discussed. Images FIG. PMID:1147688

  13. Precursors to Lymphoproliferative Malignancies

    PubMed Central

    Goldin, Lynn R.; McMaster, Mary L.; Caporaso, Neil E.

    2013-01-01

    We review monoclonal B-cell lymphocytosis (MBL) as a precursor to chronic lymphocytic leukemia and monoclonal gammopathy of undetermined significance (MGUS) as a precursor to plasma cell disorders. These conditions are present in the general population and increase with age. These precursors aggregate with lymphoproliferative malignancies in families suggesting shared inheritance. MBL and MGUS may share some of the same risk factors as their related malignancies but data are limited. While these conditions are characterized by enhanced risk for the associated malignancy, the majority of individuals with these conditions do not progress to malignancy. A key focus for current work is to identify markers that predict progression to malignancy. PMID:23549397

  14. Primary Giant Splenic Echinococcal Cyst Treated by Laparoscopy

    PubMed Central

    Arce, Maira A.; Limaylla, Himerón; Valcarcel, Maria; Garcia, Hector H.; Santivañez, Saul J.

    2016-01-01

    Cystic echinococcosis (CE) is a zoonosis caused by the larval stage of the dog tapeworm Echinococcus granulosus. Liver and lungs are the most commonly affected organs whereas splenic infection is rarer and its primary involvement occurs in less than 2% of abdominal CE. We report a case of primary giant splenic hydatid cyst in a 75-year-old Peruvian woman that was laparoscopically removed without any complications, perioperative prophylactic chemotherapy with albendazole 400 mg twice a day 5 days before, and 7 days after the surgical procedure was administered, postoperative recovery was uneventful, and; at her 3-month follow-up the patient remains asymptomatic and an abdominal computed tomography scan demonstrated a cystic cavity of 15 cm diameter with no daughter vesicles, neither other abdominal organ involvement. This case is in line with the existing literature on laparoscopical treatment of splenic cystic hydatid disease, suggesting that laparoscopical treatment is a safe and effective approach for large splenic hydatid cysts to be preferred to open surgical techniques. PMID:26556833

  15. Primary Giant Splenic Echinococcal Cyst Treated by Laparoscopy.

    PubMed

    Arce, Maira A; Limaylla, Himerón; Valcarcel, Maria; Garcia, Hector H; Santivañez, Saul J

    2016-01-01

    Cystic echinococcosis (CE) is a zoonosis caused by the larval stage of the dog tapeworm Echinococcus granulosus. Liver and lungs are the most commonly affected organs whereas splenic infection is rarer and its primary involvement occurs in less than 2% of abdominal CE. We report a case of primary giant splenic hydatid cyst in a 75-year-old Peruvian woman that was laparoscopically removed without any complications, perioperative prophylactic chemotherapy with albendazole 400 mg twice a day 5 days before, and 7 days after the surgical procedure was administered, postoperative recovery was uneventful, and; at her 3-month follow-up the patient remains asymptomatic and an abdominal computed tomography scan demonstrated a cystic cavity of 15 cm diameter with no daughter vesicles, neither other abdominal organ involvement. This case is in line with the existing literature on laparoscopical treatment of splenic cystic hydatid disease, suggesting that laparoscopical treatment is a safe and effective approach for large splenic hydatid cysts to be preferred to open surgical techniques.

  16. 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

  17. Cystic meningioma: unusual imaging appearance of a common intracranial tumor

    PubMed Central

    Layton, Kennith F.; Finn, S. Sam; Snipes, George J.; Opatowsky, Michael J.

    2010-01-01

    Meningiomas are common tumors of the central nervous system that account for approximately 15% of all intracranial tumors and are the most common extra-axial neoplasm. Most meningiomas are benign, although atypical and malignant meningiomas also exist. Typical imaging characteristics include a well-circumscribed, homogeneously enhancing, extra-axial mass on both computed tomography and magnetic resonance imaging. The presence of an associated cyst is an uncommon imaging feature that may make it difficult to distinguish the tumor from a primary intra-axial glial neoplasm. The presence of peritumoral edema can also be a misleading finding. We present a case of a woman who presented with a history of multiple recent falls, decreased energy, and increased somnolence and was found to have a “cystic meningioma.” Typical imaging characteristics, histologic subtypes, treatment, and prognosis are also discussed. PMID:21240328

  18. 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

  19. Malignant Clear Cell Hidradenoma of the Breast

    PubMed Central

    Rahal, Ahmad K.; Reddy, Pavan S; Kallail, K. James

    2017-01-01

    A 58-year-old female had a mass in the right breast palpable beneath the areola. A mammogram revealed a 1.5-centimeter soft tissue density that was confirmed with a subsequent ultrasound. The patient underwent a core needle biopsy which was initially reported as a moderately differentiated invasive ductal carcinoma. Immunohistochemical analysis revealed negative staining for estrogen receptor (ER), progesterone receptor (PR), human epidermal growth factor receptor (HER2), mammaglobin, and gross cystic disease fluid protein 15 (GCDFP-15). A wide local excision of the mass was performed. The pathology report stated the tumor had an infiltrative growth pattern with a desmoplastic stromal response with enhanced epithelial atypia consistent with malignant transformation of a nodular clear cell hidradenoma. Clear cell hidradenoma is a very rare tumor originating from the sweat gland and has a propensity for the face and extremities. The malignant variant of this tumor is extremely rare and has been reported to originate from the breast in few cases. This case represents the difficulty in diagnosing this tumor along with the radiographic and histologic features that can distinguish this malignancy from other entities. PMID:28409065

  20. Cystic Lung Diseases: Algorithmic Approach.

    PubMed

    Raoof, Suhail; Bondalapati, Praveen; Vydyula, Ravikanth; Ryu, Jay H; Gupta, Nishant; Raoof, Sabiha; Galvin, Jeff; Rosen, Mark J; Lynch, David; Travis, William; Mehta, Sanjeev; Lazzaro, Richard; Naidich, David

    2016-10-01

    Cysts are commonly seen on CT scans of the lungs, and diagnosis can be challenging. Clinical and radiographic features combined with a multidisciplinary approach may help differentiate among various disease entities, allowing correct diagnosis. It is important to distinguish cysts from cavities because they each have distinct etiologies and associated clinical disorders. Conditions such as emphysema, and cystic bronchiectasis may also mimic cystic disease. A simplified classification of cysts is proposed. Cysts can occur in greater profusion in the subpleural areas, when they typically represent paraseptal emphysema, bullae, or honeycombing. Cysts that are present in the lung parenchyma but away from subpleural areas may be present without any other abnormalities on high-resolution CT scans. These are further categorized into solitary or multifocal/diffuse cysts. Solitary cysts may be incidentally discovered and may be an age related phenomenon or may be a remnant of prior trauma or infection. Multifocal/diffuse cysts can occur with lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, tracheobronchial papillomatosis, or primary and metastatic cancers. Multifocal/diffuse cysts may be associated with nodules (lymphoid interstitial pneumonia, light-chain deposition disease, amyloidosis, and Langerhans cell histiocytosis) or with ground-glass opacities (Pneumocystis jirovecii pneumonia and desquamative interstitial pneumonia). Using the results of the high-resolution CT scans as a starting point, and incorporating the patient's clinical history, physical examination, and laboratory findings, is likely to narrow the differential diagnosis of cystic lesions considerably. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  1. Giant hydronephrosis: still a reality!

    PubMed

    Kaura, Kawaljit Singh; Kumar, Manoj; Sokhal, Ashok Kr; Gupta, Ashok Kr; Purkait, Bimalesh; Saini, Durgesh; Sankhwar, Satyanarayan

    2017-09-01

    Giant hydronephrosis (GH) is a rare entity in both developed and developing countries with less than 500 cases reported in the literature. Delayed diagnosis and management of GH, can result in long-term complications like hypertension, rupture of the kidney, renal failure and malignant change. We aim to highlight the importance of this often neglected entity and build a consensus for its early diagnosis and management. Patients with GH were thoroughly worked up, managed and followed up between June 2013 and December 2015 and epidemiologic, radiological, perioperative and follow-up data was recorded. A total of 35 patients (adults and children) were reported. Flank pain in adults and abdominal lump in children were the most common clinical presentation. Percutaneous nephrostomy tube was placed in all patients and detailed work up was done to reach final diagnosis. Pelvi-ureteric junction obstruction (PUJO) was the final diagnosis in 32 patients (91.4%). Kidneys were non-functioning in 13 cases (37.1%) so nephrectomies were performed. Reduction pyeloplasty with nephropexy was done in 21 patients (60%) with 81% success and 23.1% complication rates. GH requires early diagnosis and management to prevent higher nephrectomy rate along with poor success rate of conservative surgery like pyeloplasty.

  2. Incidence Trends in the Diagnosis of Giant Cell Tumor of Bone in Sweden Since 1958.

    PubMed

    Rockberg, Julia; Bach, Bruce A; Amelio, Justyna; Hernandez, Rohini K; Sobocki, Patrik; Engellau, Jacob; Bauer, Henrik C F; Liede, Alexander

    2015-11-04

    The Swedish Cancer Registry (founded in 1958) constitutes a unique resource for epidemiological studies of giant cell tumor of bone with potential for use for population-based studies of incidence over time. The aim of this study was to provide what we believe is the first modern population-based assessment of the incidence trends of giant cell tumor, a unique osteoclastogenic lytic stromal tumor with both benign and malignant histological forms, and to compare the findings with data from the same registry on osteosarcoma, a tumor that may display similar histological characteristics. Cases were identified with use of codes for pathological bone tumor (International Classification of Diseases [ICD]-7 196). Specific morphological coding distinguishes benign (PAD 741) from malignant giant cell tumor (PAD 746) and osteosarcoma (PAD 766). During the period of 1958 to 2011, 4625 bone tumors were reported, including 505 giant cell tumors (383 benign and 122 malignant) and 1152 osteosarcomas. From 1958 to 1982 the ratio of malignant to benign giant cell tumors was 1.3, whereas from 1983 to 2011 the ratio inverted to 0.09, suggesting a change in the reporting or diagnosis of malignant or benign cases. Cases of giant cell tumor diagnosed from 1983 to 2011 displayed an age and sex distribution (median age at diagnosis, 34.0 years; 54% female) that were consistent with those in large published case series but differed from those in 1958 to 1982 (median age at diagnosis, 31.5 years; 48% female). The most current data (1983 to 2011) showed the giant cell tumor incidence in Sweden to be 1.3 per million per year, while the osteosarcoma incidence was 2.3 per million per year. Early Swedish Cancer Registry data (1958 to 1982) revealed a higher proportion of malignant giant cell tumors than seen in large sequential case series and a distinct age and sex profile compared with more recent data (1983 to 2011). This likely represents changes in the diagnostic workup and introduction of

  3. [Bronchopulmonary infection in cystic fibrosis].

    PubMed

    Munck, Anne; Bingen, Edouard

    2003-01-15

    Bronchopulmonary infection determines the vital prognosis of the patients with cystic fibrosis. Following Staphylococcus aureus infection, patients are colonized or cocolonized by Pseudomonas aeruginosa, greatly involved in the pulmonary deterioration; intensive antibiotic treatment of primocolonisation helps to prevent or delay chronic colonisation. Chronic colonization needs a rational long term antibiotic strategy to prevent the occurrence of multiresistant germs; antibiotic cures are performed every 3 or 4 months before pulmonary exacerbation symptoms. Antibiotherapy, physiotherapy and nutritional management helps to increase the survival and quality of life.

  4. 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. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. 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.

  6. Cystic schwannoma of the pelvis.

    PubMed

    Jindal, T; Mukherjee, S; Kamal, M R; Sharma, R K; Ghosh, N; Mandal, S N; Das, A K; Karmakar, D

    2013-01-01

    Schwannomas are benign tumours that arise from the Schwann cells of nerve fibres. They commonly occur in the head and neck, mediastinum and extremities. They are extremely rare in the pelvis. These are usually slow-growing tumours and are often detected incidentally. Preoperative diagnosis is extremely difficult as there are no definitive signs on imaging. Aspiration biopsy is often inconclusive or misleading. Surgical excision is both diagnostic and therapeutic. As these tumours are often large in size, open excision is most commonly performed. We describe a case of a large, cystic schwannoma of the pelvis causing bladder outlet obstruction and bilateral hydroureteronephrosis. Complete surgical excision was performed laparoscopically.

  7. A giant ancient schwannoma mimicking an adnexal mass

    PubMed Central

    Karaköse, Oktay; Pülat, Hüseyin; Oğuz, Serhat; Zihni, İsmail; Özçelik, Kazım Çağlar; Yalta, Tülin Deniz; Eken, Hüseyin

    2016-01-01

    Abstract Introduction: Ancient schwannoma is a rare tumor of the peripheral nerve sheath. As degenerative properties are defined histologically, it can be wrongly interpreted as malignant. Case presentation: The case presented here is of a giant ancient schwannoma with a pelvic retroperitoneal location, which was mimicking an adnexal mass. Conclusion: In the rarely seen cases in the retroperitoneum, it may reach very large dimensions. PMID:27472696

  8. A giant radiopaque mass in the masticatory space.

    PubMed

    Cho, Young-Ah; Yoon, Hye-Jung; Hong, Seong-Doo; Lee, Jae-Il; Hong, Sam-Pyo

    2013-05-01

    A 2-year-old boy presented with a giant mass in the masticatory space. The mass exhibited a lobulating ossification, with no attachment to the adjacent normal bone. An enucleation was performed under the tentative diagnosis of extra-articular synovial chondromatosis, benign ossifying neoplasm, non-neoplastic heterotopic ossification, or low-grade malignancy. Upon microscopic examination, the excised mass was composed of multiple osteocartilaginous areas. We hereby present detailed clinicopathological findings. Copyright © 2013 Elsevier Inc. All rights reserved.

  9. Giant star seismology

    NASA Astrophysics Data System (ADS)

    Hekker, S.; Christensen-Dalsgaard, J.

    2017-06-01

    The internal properties of stars in the red-giant phase undergo significant changes on relatively short timescales. Long near-uninterrupted high-precision photometric timeseries observations from dedicated space missions such as CoRoT and Kepler have provided seismic inferences of the global and internal properties of a large number of evolved stars, including red giants. These inferences are confronted with predictions from theoretical models to improve our understanding of stellar structure and evolution. Our knowledge and understanding of red giants have indeed increased tremendously using these seismic inferences, and we anticipate that more information is still hidden in the data. Unraveling this will further improve our understanding of stellar evolution. This will also have significant impact on our knowledge of the Milky Way Galaxy as well as on exo-planet host stars. The latter is important for our understanding of the formation and structure of planetary systems.

  10. EGFR mutation of adenocarcinoma in congenital cystic adenomatoid malformation/congenital pulmonary airway malformation: a case report.

    PubMed

    Hasegawa, Mizue; Sakai, Fumikazu; Arimura, Ken; Katsura, Hideki; Koh, Eitetsu; Sekine, Yasuo; Hiroshima, Kenzo

    2014-03-01

    An 80-year-old man underwent right upper lobectomy for the resection of multiple cysts accompanied by a nodule. The pathological diagnosis was adenocarcinoma with surrounding atypical epithelial cell proliferation in a Type 1 congenital cystic adenomatoid malformation/congenital pulmonary airway malformation. There was epidermal growth factor receptor mutation in the adenocarcinoma and surrounding atypical epithelial cells that had proliferated. Malignant transformation of congenital cystic adenomatoid malformation/congenital pulmonary airway malformation may be related to the epidermal growth factor receptor pathway in this case, with atypical epithelial cell proliferation as a precursor. We emphasize the importance of complete resection of congenital cystic adenomatoid malformation/congenital pulmonary airway malformation and the possibility of treatment with epidermal growth factor receptor tyrosine kinase inhibitors in epidermal growth factor receptor-mutated cases.

  11. 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

  12. Androgen excess in cystic acne.

    PubMed

    Marynick, S P; Chakmakjian, Z H; McCaffree, D L; Herndon, J H

    1983-04-28

    We measured hormone levels in 59 women and 32 men with longstanding cystic acne resistant to conventional therapy. Affected women had higher serum levels of dehydroepiandrosterone sulfate, testosterone, and luteinizing hormone and lower levels of sex-hormone-binding globulin than controls. Affected men had higher levels of serum dehydroepiandrosterone sulfate and 17-hydroxyprogesterone and lower levels of sex-hormone-binding globulin than controls. To lower dehydroepiandrosterone sulfate, dexamethasone was given to men, and dexamethasone or an oral contraceptive pill, Demulen (or both), was given to women. Of the patients treated for six months, 97 per cent of the women and 81 per cent of the men had resolution or marked improvement in their acne. The dose of dexamethasone required to reduce dehydroepiandrosterone sulfate levels was low, rarely exceeding the equivalent of 20 mg of hydrocortisone per day. We conclude that most patients with therapeutically resistant cystic acne have androgen excess and that lowering elevated dehydroepiandrosterone sulfate results in improvement or remission of acne in most instances.

  13. Aspergillus infections in cystic fibrosis.

    PubMed

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

    2016-07-05

    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. Copyright © 2016. Published by Elsevier Ltd.

  14. Internal irradiation for cystic craniopharyngioma

    SciTech Connect

    Kobayashi, T.; Kageyama, N.; Ohara, K.

    1981-12-01

    The authors report the results of internal irradiation with labeled chromic phosphate (32P) and gold-198 (198Au) colloid in eight cases of cystic craniopharyngiomas. They used a newly developed dosimetric formula, by which the radiation dose at the cyst wall and at any point far from the radioactive source can be calculated. Ten courses of irradiation in eight patients were carried out by injection of either 32P or 198Au colloid into the cyst through an Ommaya drainage system that had been placed at craniotomy. Follow-up studies ranging from 13 to 156 months revealed that all cysts were effectively treated, with elimination of fluid or collapse of the cyst. This was confirmed by Conray cystography and/or computerized tomography. Not only the dose delivered to the wall but also the thickness of the cyst wall and the location of the cyst are important factors in planning internal irradiation. A safe and adequate dose to the cyst wall could range between 9000 to 30,000 rads for craniopharyngioma. This treatment is suitable for large cysts that are thought to be difficult to remove radically, recurrent cysts resistant to previous treatment, or multiple cysts. Internal irradiation may also be applicable in other cystic intracranial tumors if dosimetry is calculated accurately.

  15. Endosonographic features predictive of benign and malignant gastrointestinal stromal cell tumours

    PubMed Central

    Palazzo, L; Landi, B; Cellier, C; Cuillerier, E; Roseau, G; Barbier, J

    2000-01-01

    BACKGROUND/AIM—Some endoscopic ultrasonographic (EUS) features have been reported to be suggestive of malignancy in gastrointestinal stromal cell tumours (SCTs). The aim of this study was to assess the predictive value of these features for malignancy.
METHODS—A total of 56 histologically proven cases of SCT studied by EUS between 1989 and 1996 were reviewed. There were 42 gastric tumours, 12 oesophageal tumours, and two rectal tumours. The tumours were divided into two groups: (a) benign SCT, comprising benign leiomyoma (n = 34); (b) malignant or borderline SCT (n = 22), comprising leiomyosarcoma (n = 9), leiomyoblastoma (n = 9), and leiomyoma of uncertain malignant potential (n = 4). The main EUS features recorded were tumour size, ulceration, echo pattern, cystic spaces, extraluminal margins, and lymph nodes with a malignant pattern. The two groups were compared by univariate and multivariate analysis.
RESULTS—Irregular extraluminal margins, cystic spaces, and lymph nodes with a malignant pattern were most predictive of malignant or borderline SCT. Pairwise combinations of the three features had a specificity and positive predictive value of 100% for malignant or borderline SCT, but a sensitivity of only 23%. The presence of at least one of these three criteria had 91% sensitivity, 88% specificity, and 83% predictive positive value. In multivariate analysis, cystic spaces and irregular margins were the only two features independently predictive of malignant potential. The features most predictive of benign SCTs were regular margins, tumour size ⩽30 mm, and a homogeneous echo pattern. When the three features were combined, histology confirmed a benign SCT in all cases.
CONCLUSIONS—The combined presence of two out of three EUS features (irregular extraluminal margins, cystic spaces, and lymph nodes with a malignant pattern) had a positive predictive value of 100% for malignant or borderline gastrointestinal SCT. Tumours less than 30

  16. 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.

  17. Added value of intravenous contrast-enhanced ultrasound for characterization of cystic pancreatic masses: a prospective study on 37 patients.

    PubMed

    Vasile, Tudor Andrei; Socaciu, Mihai; Stan Iuga, Roxana; Seicean, Andrada; Iancu, Cornel; al Hajjar, Nadim; Zaharie, Toader; Badea, Radu

    2012-06-01

    The aim of this study was to evaluate the added value of contrast-enhanced ultrasound (CEUS) in the pancreatic cystic mass (PCM) diagnosis by using a qualitative and quantitative analysis in order to make a relevant characterization. Between December 2008 and November 2011, 37 patients with PCM discovered at ultrasound examination were prospectively followed. A qualitative and quantitative CEUS analysis was performed in order to differentiate etiologies of the PCM. In the quantitative analysis several parameters were followed: Peak Intensity (PI), Time to Peak (TTP), maximum ascending gradient (GRAD), Time to maximum gradient (TTG) and Area Under the Curve (AUC). Normalized ratios were also calculated. In all patients a definite cytological or histological diagnosis was obtained. Thirty-seven patients were studied: 12 with pancreatitis-associated pseudocyst and 25 with cystic tumors (10 serous cystic adenoma, 5 mucinous cystic adenoma, 6 cystadenocarcinomas, 2 solid pseudopapillary tumors and 2 intraductal papillary mucinous neoplasms). There was a significant difference of the nAUC and nTTP between pseudocyst and cystic tumors, p=0.03 and p=0.01, respectively. A normalized TTP value above 7 sec was suggestive for the diagnosis of pseudocysts with 79.16 % accuracy. There was a significant difference of nTTP and nTTG between the benign and malignant lesions. nTTP < 9 sec and nTTG < 8.5 sec rules out malignant cysts in almost 90% of cases. The CEUS is useful in the diagnosis of PCM. The quantitative analysis of the enhancement of the cystic wall may discriminate the different types of the PCM.

  18. Vasculitis associated with malignancy.

    PubMed

    Mertz, L E; Conn, D L

    1992-02-01

    A large variety of vasculopathic syndromes are uncommonly associated with malignancies. Vasculitis is usually manifested by skin lesions and is generally associated with hematologic malignancies rather than solid tumors. Evidence of autoantibodies, immune complexes, and complement consumption is typically absent. Myelodysplastic syndromes can be confidently linked to vasculitis on the basis of recent literature. The temporal relationship of malignancy to vasculitis development is variable except that vasculitis generally follows the discovery of hairy cell leukemia and splenectomy. Vasculitis may occasionally be a complication of chemotherapy, radiation therapy, and bone marrow transplantation. Occasionally, malignant disorders may mimic vasculitic syndromes. The etiopathogenesis of vasculitis in patients with malignant disorders is unknown. The recent literature on vasculitis and malignancy addresses predominantly case reports and small patient cohorts and identifies clinical characteristics rather than pathogenic mechanisms.

  19. Voice Disorder in Cystic Fibrosis Patients

    PubMed Central

    Lourenço, Bruna Mendes; Costa, Kauê Machado; da Silva Filho, Manoel

    2014-01-01

    Cystic fibrosis is a common autosomal recessive disorder with drastic respiratory symptoms, including shortness of breath and chronic cough. While most of cystic fibrosis treatment is dedicated to mitigating the effects of respiratory dysfunction, the potential effects of this disease on vocal parameters have not been systematically studied. We hypothesized that cystic fibrosis patients, given their characteristic respiratory disorders, would also present dysphonic symptoms. Given that voice disorders can severely impair quality of life, the identification of a potential cystic fibrosis-related dysphonia could be of great value for the clinical evaluation and treatment of this disease. We tested our hypothesis by measuring vocal parameters, using both objective physical measures and the GRBAS subjective evaluation method, in male and female cystic fibrosis patients undergoing conventional treatment and compared them to age and sex matched controls. We found that cystic fibrosis patients had a significantly lower vocal intensity and harmonic to noise ratio, as well as increased levels of jitter and shimmer. In addition, cystic fibrosis patients also showed higher scores of roughness, breathiness and asthenia, as well as a significantly altered general grade of dysphonia. When we segregated the results according to sex, we observed that, as a group, only female cystic fibrosis patients had significantly lower values of harmonic to noise ratio and an abnormal general grade of dysphonia in relation to matched controls, suggesting that cystic fibrosis exerts a more pronounced effect on vocal parameters of women in relation to men. Overall, the dysphonic characteristics of CF patients can be explained by dysfunctions in vocal fold movement and partial upper airway obstruction, potentially caused by the accumulation of mucus and chronic cough characteristic of CF symptomatology. Our results show that CF patients exhibit significant dysphonia and suggest they may

  20. Voice disorder in cystic fibrosis patients.

    PubMed

    Lourenço, Bruna Mendes; Costa, Kauê Machado; da Silva Filho, Manoel

    2014-01-01

    Cystic fibrosis is a common autosomal recessive disorder with drastic respiratory symptoms, including shortness of breath and chronic cough. While most of cystic fibrosis treatment is dedicated to mitigating the effects of respiratory dysfunction, the potential effects of this disease on vocal parameters have not been systematically studied. We hypothesized that cystic fibrosis patients, given their characteristic respiratory disorders, would also present dysphonic symptoms. Given that voice disorders can severely impair quality of life, the identification of a potential cystic fibrosis-related dysphonia could be of great value for the clinical evaluation and treatment of this disease. We tested our hypothesis by measuring vocal parameters, using both objective physical measures and the GRBAS subjective evaluation method, in male and female cystic fibrosis patients undergoing conventional treatment and compared them to age and sex matched controls. We found that cystic fibrosis patients had a significantly lower vocal intensity and harmonic to noise ratio, as well as increased levels of jitter and shimmer. In addition, cystic fibrosis patients also showed higher scores of roughness, breathiness and asthenia, as well as a significantly altered general grade of dysphonia. When we segregated the results according to sex, we observed that, as a group, only female cystic fibrosis patients had significantly lower values of harmonic to noise ratio and an abnormal general grade of dysphonia in relation to matched controls, suggesting that cystic fibrosis exerts a more pronounced effect on vocal parameters of women in relation to men. Overall, the dysphonic characteristics of CF patients can be explained by dysfunctions in vocal fold movement and partial upper airway obstruction, potentially caused by the accumulation of mucus and chronic cough characteristic of CF symptomatology. Our results show that CF patients exhibit significant dysphonia and suggest they may

  1. 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. Copyright © 2016 Elsevier Inc. All rights reserved.

  2. Fungi in cystic fibrosis and non-cystic fibrosis bronchiectasis.

    PubMed

    Moss, Richard B

    2015-04-01

    Bronchiectasis is a pathologic bronchial dilatation with loss of function that can result from multiple inflammatory and infectious injuries to the conducting airways of the lung. Molds, particularly the filamentous fungus Aspergillus fumigatus, have been implicated as a common cause of both cystic fibrosis (CF) and non-CF bronchiectasis, the latter primarily in patients with severe asthma. The pathogenesis of mold-associated bronchiectasis is usually due to atopic sensitization to mold allergens in the presence of active chronic endobronchial fungal infection with host innate and adaptive immune deviation to a Th2-dominated inflammation, a condition known as allergic bronchopulmonary aspergillosis (ABPA) (or allergic bronchopulmonary mycosis if a non-Aspergillus mold is implicated). Diagnostic criteria of ABPA continue to evolve, while treatment relies upon downregulation of the allergic inflammatory response with immunomodulatory agents and antifungal pharmacotherapy.

  3. Shadows on a Giant

    NASA Image and Video Library

    2012-07-02

    Saturn rings cast wide shadows on the planet, and the shadow of a moon also graces the gas giant in this scene from NASA Cassini spacecraft. The moon Enceladus is not shown in this view, but it does cast a small, elongated shadow.

  4. Giant scrotal elephantiasis.

    PubMed

    Kuepper, Daniel

    2005-02-01

    How much can a man carry? Penoscrotal elephantiasis is a debilitating syndrome. This is a case report of a patient with giant genital elephantiasis secondary to long-standing lymphogranuloma venereum infection in Ethiopia. Complete surgical resection of the pathologic tissue and penile reconstruction was undertaken with good cosmetic and functional results.

  5. [Giant retroperitoneal liposarcoma].

    PubMed

    Mezzour, Mohamed Hicham; El Messaoudi, Yasser Arafat; Fekak, Hamid; Rabii, Redouane; Marnissi, Farida; Karkouri, Mehdi; Salam, Siham; Iraki, Moulay Ahmed; Joual, Abdenbi; Meziane, Fathi

    2006-02-01

    The authors report a case of giant retroperitoneal liposarcoma. The diagnosis was suspected after scanography and magnetic resonance imaging and confirmed by the histological analysis of the extracted piece after surgical treatment. Postoperative evolution was favourable after one year without recurrence or distant metastasis. The authors discuss the pathologic and therapeutic aspects and the prognosis of retroperitoneal liposarcoma.

  6. Electroluminescence of Giant Stretchability.

    PubMed

    Yang, Can Hui; Chen, Baohong; Zhou, Jinxiong; Chen, Yong Mei; Suo, Zhigang

    2016-06-01

    A new type of electroluminescent device achieves giant stretchability by integrating electronic and ionic components. The device uses phosphor powders as electroluminescent materials, and hydrogels as stretchable and transparent ionic conductors. Subject to cyclic voltage, the phosphor powders luminesce, but the ionic conductors do not electrolyze. The device produces constant luminance when stretched up to an area strain of 1500%.

  7. 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

  8. Malignant Vagal Paraganglioma.

    PubMed

    Hamersley, Erin R S; Barrows, Amy; Perez, Angel; Schroeder, Ashley; Castle, James T

    2016-06-01

    Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have malignant potential. They arise from the carotid body, jugular bulb or vagus nerves. There is limited literature discussing the management of malignant vagal paragangliomas. We present a case of a 25 year old female with a left malignant vagal paraganglioma. The following case presentation will describe the presentation, classic radiologic findings, and management of a malignant vagal paraganglioma along with a review of the literature.

  9. Cystic avascular necrosis of the triquetrum.

    PubMed

    Albtoush, Omar M; Esmadi, Mohammad; Al-Omari, Mamoon H

    2013-01-01

    Carpal bones are rarely affected by avascular necrosis (AVN) in the absence of fractures. The lunate is the most frequently affected carpal bone, followed by the scaphoid and the capitate. The triquetrum is rarely affected by AVN. We report a case of multiple cystic changes in the triquetrum in a patient with a history of trauma. He was treated by below elbow Colles plaster cast for 3 months, with no improvement. Cystic changes resulted from irreversible AVN of the triquetrum. This is the first case to be reported in the literature with cystic AVN changes in the triquetrum.

  10. Cystic Pheochromocytoma Presenting as Adrenal Cyst

    PubMed Central

    Abdulsalam, Mohammed Shafi; Satish, Priyanka; Janakiraman, Raghunath Keddy; Singh, Shivshankar

    2016-01-01

    Pheochromocytomas are usually solid tumours. But it can present as cystic lesions in the adrenal gland. Cystic lesions in adrenal gland with hypertension needs attention to rule out pheochromocytoma. If ignored, it may lead to hypertensive emergency, multisystem crisis and death. Early diagnosis with biochemistry, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) of abdomen, proper functional imaging like Meta Iodo Benzyl Guanidine (MIBG) scan is essential. Proper preoperative preparation is important to prevent hypertensive crisis during and after surgery. We are reporting a case of cystic pheochromocytoma in a young male. PMID:28050427

  11. Cystic Pheochromocytoma Presenting as Adrenal Cyst.

    PubMed

    Abdulsalam, Mohammed Shafi; Ganapathy, Vijaya; Satish, Priyanka; Janakiraman, Raghunath Keddy; Singh, Shivshankar

    2016-11-01

    Pheochromocytomas are usually solid tumours. But it can present as cystic lesions in the adrenal gland. Cystic lesions in adrenal gland with hypertension needs attention to rule out pheochromocytoma. If ignored, it may lead to hypertensive emergency, multisystem crisis and death. Early diagnosis with biochemistry, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) of abdomen, proper functional imaging like Meta Iodo Benzyl Guanidine (MIBG) scan is essential. Proper preoperative preparation is important to prevent hypertensive crisis during and after surgery. We are reporting a case of cystic pheochromocytoma in a young male.

  12. 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

  13. Biliary mucinous cystic neoplasm: a case report and review of the literature

    PubMed Central

    Safari, Mohammad Taghi; Shahrokh, Shabnam; Miri, Mohammad Bagher; Foroughi, Forough; Sadeghi, Amir

    2016-01-01

    Hepatobiliary cystadenomas (HBC) is a rare neoplasm which comprising less than one percent of liver cystic neoplasms. Although it’s known as a benign tumor, but they have a potential for neoplastic transformation. Making a proper diagnosis and ruling out of other differential diagnosis is important because of different treatment. In the present study, we described a case of HBC manifested as idiopathic dominant biliary stricture in common hepatic duct (CHD), on the basis of spiral CT scan and MRI, and elevated CA19-9. With a probable diagnosis of malignant biliary stricture, she underwent ERCP and cholangioscopy that were non-diagnostic and final diagnosis was made surgically. HBCs usually found incicentally as a cystic lesion and biliary stricture without visible cyst in imaging like that seen in cholangiocarcinoma is very unlikely. In truth, this patient is an unusual manifestation of one rare disease. PMID:28224034

  14. 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.

  15. 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

  16. Two Different Cell Populations Is an Important Clue for Diagnosis of Primary Cutaneous Adenoid Cystic Carcinoma: Immunohistochemical Study

    PubMed Central

    Alkan, Banu Ince; Karadeniz, Müjde; Bozdoğan, Nazan

    2017-01-01

    Primary cutaneous adenoid cystic carcinoma (PCACC) is a very rare malignancy. The differential diagnosis of PCACCs in pathology practice can be difficult and a group of primary and metastatic lesions, including adenoid basal cell carcinoma of the skin, should be considered in the differential diagnosis. Besides histomorphological clues, immunohistochemistry studies are very helpful in the differential diagnosis of PCACC. We report herein a case of PCACC with extensive immunohistochemical studies and review the literature from an immunohistochemistry perspective. PMID:28243477

  17. Cystic mesothelioma of the peritoneum.

    PubMed

    Datta, R V; Paty, P B

    1997-10-01

    A 48-year-old man presented with a 3-month history of weight loss and progressive right lower quadrant abdominal pain. His medical history was notable for appendectomy at age 17. Ultrasonography and computed tomography of the abdomen revealed a 12 cm multicystic mass in the right paracolic space. At laparotomy a large serous cyst was found arising from the lateral wall of the cecum, and four additional small cysts were found on the small bowel mesentery, greater omentum, liver capsule, and right hemi-diaphragm. Complete removal of the tumor was accomplished by right colectomy with extraperitoneal dissection of the large cyst and simple excision of the four smaller cysts. Final pathology with immunohistochemical staining confirmed cystic mesothelioma of the peritoneum. In this report we discuss the diagnostic workup and treatment of this rare disease.

  18. Laboratory diagnosis of cystic fibrosis.

    PubMed

    Webster, H L

    1983-01-01

    The demonstration of abnormally high concentrations of electrolytes in eccrine sweat is still the only practical laboratory procedure available for diagnosis of cystic fibrosis. Properly performed, the sweat test is very reliable, but there are many published reports that all of the methods in current use frequently generate incorrect diagnoses. Analysis of potential for error in sweat test methods shows that of the three essential phases involved, stimulation, collection, and analysis, the major cause of intrinsic inaccuracy occurs in the collection process. In this case the problem is due to condensate formation, which leads to the subsequent analysis of nonrepresentative sweat. Human error is also an important cause of false results and is a direct function of the number of critical manual operations involved in the technic. This review provides a critical examination of sweat test methods, identifying problem areas and suggesting ways to improve procedures in the interests of clinically reliable laboratory data in support of diagnosis.

  19. Cystic cerebellar astrocytomas in childhood.

    PubMed

    Griffin, T W; Beaufait, D; Blasko, J C

    1979-07-01

    Thirty-nine patients with low grade cystic cerebellar astrocytomas were treated at the University of Washington and Children's Orthopedic Hospital in Seattle, Washington, between 1955 and 1977; 29 were treated with partial or complete resection alone, and 10 received radiation therapy after various types of surgical procedures. With a mean follow-up time of 7 years, the survival rate for patients who had complete resections of their primary disease was 100%. The relapse-free survival rate was 82%. The relapse-free survival rate for patients treated primarily with partial resection alone was 36%. Postoperative irradiation after partial resection for both primary and recurrent disease resulted in a relapse-free survival rate of 83%. If complete tumor excision is not possible, postoperative radiation therapy is recommended following partial resection.

  20. 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

  1. 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.

  2. [New tools in cystic fibrosis].

    PubMed

    Dournes, G; De Boeck, K; Bui, S; Vermeulen, F; Ramalho, A; Chateil, J-F; Laurent, F; Fayon, M

    2016-12-01

    The use of 3 novel tools available for the diagnosis and treatment in cystic fibrosis are described here. 1) The lung clearance index is a sensitive method which can detect functional impairment in the first months after birth. 2) Detailed morphological analyses of the lung can be performed with the new MRI sequences, without any contrast medium or risk of radiation. The analysis of functional MRI data (perfusion, diffusion, ventilation, inflammation) will be possible, and these data will be correlated to morphological data. The exploration of other organs such as the sinuses, liver and abdomen during the same examination represents another definite advantage. 3) Organoïds are a good example of personalized medicine. This tool explores CFTR function and treatment response in each of the 2000 or so known CFTR mutations. These tests are limited to specialized centers, mostly within a research context. However, their generalization after standardization is expected in the near future.

  3. Asymptomatic peritoneal carcinomatosis originating from benign cystic peritoneal mesothelioma.

    PubMed

    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.

  4. 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

  5. Pancreatic endometrial cyst mimics mucinous cystic neoplasm of the pancreas

    PubMed Central

    Mederos, Michael A; Villafañe, Nicole; Dhingra, Sadhna; Farinas, Carlos; McElhany, Amy; Fisher, William E; Van Buren II, George

    2017-01-01

    Pancreatic cysts include a variety of benign, premalignant, and malignant lesions. Endometrial cysts in the pancreas are exceedingly rare lesions that are difficult to diagnose pre-operatively. This report describes the findings in a 43-year-old patient with a recent episode of acute pancreatitis who presented with a large cyst in the tail of the pancreas. Imaging demonstrated a loculated pancreatic cyst, and cyst fluid aspiration revealed an elevated amylase and carcinoembryonic antigen. The patient experienced an interval worsening of abdominal pain, fatigue, diarrhea, and a 15-pound weight loss 3 mo after the initial episode of pancreatitis. With concern for a possible pre-malignant lesion, the patient underwent a laparoscopic distal pancreatectomy with splenectomy, which revealed a 16 cm × 12 cm × 4 cm lesion. Final histopathology was consistent with an intra-pancreatic endometrial cyst. Here we discuss the overlapping imaging and laboratory features of pancreatic endometrial cysts and mucinous cystic neoplasms of the pancreas. PMID:28246486

  6. Alcaligenes infection in cystic fibrosis.

    PubMed

    Tan, Kenneth; Conway, Steven P; Brownlee, Keith G; Etherington, Christine; Peckham, Daniel G

    2002-08-01

    The aim of this study was to investigate the effect of chronic Alcaligenes species infection of the respiratory tract on the clinical status of patients with cystic fibrosis. We conducted a retrospective case-controlled study. The microbiological records of all patients attending the Leeds Regional Pediatric and Adult Cystic Fibrosis Units from 1992-1999 were examined. Chronic Alcaligenes infection was defined as a positive sputum culture on at least three occasions over a 6-month period. These patients were compared with controls matched for age, gender, respiratory function, and Pseudomonas aeruginosa infection status. Respiratory function tests, anthropometric data, Shwachman-Kulczycki score, Northern chest x-ray score, intravenous and nebulized antibiotic treatment, and corticosteroid treatment were compared from 2 years before to 2 years after Alcaligenes infection. From a clinic population of 557, 13 (2.3%) fulfilled the criteria for chronic infection. The median age at acquisition of infection was 17.2 years (range, 6.5-33.6). There was no significant difference in the changes of percentage predicted values for FEV(1), FVC, FEF(25-75), or Shwachman-Kulczycki and Northern chest x-ray scores, or in weight, height, and body mass index z-scores between Alcaligenes-infected cases and controls. There was also no significant difference in the use of antibiotics (intravenous and nebulized) or corticosteroids (inhaled and oral). We conclude that in our clinic, chronic infection with Alcaligenes species was uncommon. Chronically infected patients showed no excess deterioration in clinical or pulmonary function status from 2 years before to 2 years after primary acquisition.

  7. Epidural Cystic Spinal Meningioma: A Case Report.

    PubMed

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

    2016-03-01

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

  8. [Cystic fibrosis in a woman aged seventy].

    PubMed

    Ras, Janneke E; van Velzen, Edwin; van Berkhout, Ferdinand Teding; van den Brand, Joop J G

    2010-01-01

    A seventy-year-old woman was admitted to hospital with a Staphylococcus aureus respiratory tract infection. She had a history of extensive bronchiectasis and allergic bronchopulmonary aspergillosis (ABPA). Cystic fibrosis (CF) was suspected and cystic fibrosis transmembrane conductance regulator (CFTR) gene analysis showed F508del and R117H-7T mutations. In these mutations there is residual activity in the chloride channel in the cell membrane coded by the CFTR gene. This results in a much milder disease pattern varying from no disease at all to isolated organ disease. This type of disease is known as non-classical cystic fibrosis. In our patient the diagnosis of cystic fibrosis was made exceptionally late in life.

  9. Bronchial malignant melanoma.

    PubMed

    Weshler, Z; Sulkes, A; Kopolovitch, J; Leviatan, A; Shifrin, E

    1980-01-01

    We describe a case of malignant melanoma presenting initially as an endobronchial lesion located in the left main bronchus causing total atelectasis. This resolved with radiation therapy. Widespread metastases developed shortly thereafter. The differential diagnosis of primary and metastatic bronchial malignant melanoma is discussed. Other isolated case reports are reviewed.

  10. Malignant tumors —

    Cancer.gov

    Main criteria for malignancy include size over 5 mm in diameter, invasion of airways, blood or lymphatic vessels, regional and distant metastasis, and ability to grow upon transplantation. Nuclear and cellular atypia, and loss of architecture should be considered as ancillary criteria for defining malignancy.

  11. 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

  12. Antibiotic-associated colitis and cystic fibrosis.

    PubMed

    Pokorny, C S; Bye, P T; MacLeod, C; Selby, W S

    1992-09-01

    The use of antibiotics in patients with cystic fibrosis is widespread, and fecal carriage of Clostridium difficile occurs in up to 50% of these patients; however, antibiotic-associated colitis appears to be a rare occurrence. The reasons why this is so remain unknown. A case of antibiotic-associated colitis occurring in a patient with cystic fibrosis is described. Possible mechanisms for the rarity of antibiotic-associated colitis are reviewed and implications for prompt diagnosis and therapy are discussed.

  13. Cervical solitary long segment cystic Schwannoma.

    PubMed

    Vikram, Muthusubramanian; Pande, Anil; Vasudevan, Madhabushi Chakravarthy; Ravi, Ramamurthi

    2010-04-01

    Cystic neurofibromas are very rare and are of separate entity; occurrence in the cervical spine extending over a long segment intra-durally is very rare. There is a paucity of literature available on cervical cystic Schwannoma and its MRI characteristics. As the preoperative diagnosis of these tumors helps in planning the surgery for this rare tumor the imaging findings reported herein may aid in the preoperative diagnosis and management.

  14. [Retrocervical cystic hygroma: about 35 cases].

    PubMed

    Masmoudi, Aida; Neji, Kaled; Kacem, Sémia; Boudhraa, Kaled; Jabnoun, Samy; Chelli, Hella; Reziga, Hédi; Zouari, Faouzia; Khrouf, Naima; Chaabouni, Habiba; Siala, Soumaya

    2002-03-01

    Retrocervical cystic hygroma is a congenital defect associated to chromosomic anomalies. We report a retrospective study about 35 cystic hygroma autopsies colliged in C.M.N.T in 10 years. Antenatal sonography has a sensibility 94.5%. Genetic abnormalities dominated by trisomie 13 Turner syndrome dad found in 11.5%. Medical abortion has done in 48.5%. A multidisciplinary management autorized to understand etiopathogeny of this defect.

  15. 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

  16. Giant mesenteric cyst: a rare cause of abdominal distension diagnosed with CT and managed with ultrasound-guided drainage

    PubMed Central

    Ma, Alexander; Ayre, Karyn; Wijeyekoon, Sanjaya

    2012-01-01

    This rare cystic lesion has an approximate incidence of 1 in 200 000. This patient complained of abdominal distension and was diagnosed with a giant mesenteric cyst measuring 26 cm in height using CT imaging. She underwent a successful ultrasound-guided drainage, which is not previously reported in the literature. This represents utilisation of an established procedure in a novel situation. PMID:22948982

  17. 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

  18. 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.

  19. 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

  20. Giant Cell Arteritis.

    PubMed

    Hoffman, Gary S

    2016-11-01

    This issue provides a clinical overview of giant cell arteritis, focusing on diagnosis, treatment, and practice improvement. The content of In the Clinic is drawn from the clinical information and education resources of the American College of Physicians (ACP), including MKSAP (Medical Knowledge and Self-Assessment Program). Annals of Internal Medicine editors develop In the Clinic in collaboration with the ACP's Medical Education and Publishing divisions and with the assistance of additional science writers and physician writers.

  1. Abnormal Ion Permeation through Cystic Fibrosis Respiratory Epithelium

    NASA Astrophysics Data System (ADS)

    Knowles, M. R.; Stutts, M. J.; Spock, A.; Fischer, N.; Gatzy, J. T.; Boucher, R. C.

    1983-09-01

    The epithelium of nasal tissue excised from subjects with cystic fibrosis exhibited higher voltage and lower conductance than tissue from control subjects. Basal sodium ion absorption by cystic fibrosis and normal nasal epithelia equaled the short-circuit current and was amiloride-sensitive. Amiloride induced chloride ion secretion in normal but not cystic fibrosis tissue and consequently was more effective in inhibiting the short-circuit current in cystic fibrosis epithelia. Chloride ion-free solution induced a smaller hyperpolarization of cystic fibrosis tissue. The increased voltage and amiloride efficacy in cystic fibrosis reflect absorption of sodium ions across an epithelium that is relatively impermeable to chloride ions.

  2. Collapsing benign cystic nodules of the thyroid gland: sonographic differentiation from papillary thyroid carcinoma.

    PubMed

    Ko, M-S; Jeong, K S; Shong, Y K; Gong, G Y; Baek, J H; Lee, J H

    2012-01-01

    The US features of benign and malignant nodules overlap, and benign thyroid lesions can mimic thyroid malignancy on US. Benign cystic nodules after spontaneous collapse or needle aspiration, can mimic malignant thyroid nodules. Our aim was to evaluate the US features of CBCNs of the thyroid that distinguish such nodules from malignant thyroid nodules. US and clinical findings in 13 patients, each with a single CBCN, were evaluated to determine if they showed >50% cystic content on initial US or CT and >30% decrease in maximum diameter on follow-up US. We compared these findings with those of 26 patients, each with a single surgically confirmed PTMC. US scans were analyzed for internal content, shape, margin, echogenicity, presence of echogenic dots suggesting micro- and macrocalcification, inner isoechoic rim, and low-echoic halo. Six of the 13 (46%) CBCNs were classified as malignant on US due to their marked hypoechogenicity, microcalcification, or spiculated margins. US features that differed between CBCNs and PTMCs were shape (ovoid-to-round versus taller-than-wide, P = .016); margins (ill-defined versus spiculated, P < .000); low-echoic halo (P < .000); inner isoechoic rim (P < .000) with high negative predictive values (100%, 91%, 91%, and 89%, respectively); and clinically acceptable diagnostic accuracy (59%, 80%, 82%, and 85%, respectively). US features helpful for differential diagnosis of CBCNs from PTMCs include shape, margin, and the presence of an inner isoechoic rim and a low-echoic halo. Familiarity with US features suggesting CBCNs may be helpful in reducing unnecessary repeated FNABs.

  3. General Information about Malignant Mesothelioma

    MedlinePlus

    ... form in the lining of the chest or abdomen. Malignant mesothelioma is a disease in which malignant ( ... that examine the inside of the chest and abdomen are used to detect (find) and diagnose malignant ...

  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. A canine case with cystic meningioma showing miraculous reduction of the cystic lesion

    PubMed Central

    WADA, Masae; HASEGAWA, Daisuke; HAMAMOTO, Yuji; ASAI, Atsushi; SHOUJI, Akane; CHAMBERS, James; UCHIDA, Kazuyuki; FUJITA, Michio

    2015-01-01

    A 12-year-old spayed female Labrador retriever was presented with forebrain signs. Brain MRI revealed a huge cystic lesion with the thickened falx in the frontal region. The brain parenchyma surrounding the lesion showed significant signs of a mass effect and also increased intracranial pressure. However, the dog suddenly became lucid after about two weeks, and an MRI scan one month after the initial study revealed a dramatically shrunken cystic lesion. The dog survived for over a year until it was euthanized for other reasons, and the brain lesion was diagnosed as a cystic meningioma histologically. To the authors’ knowledge, this is the first report that described the reduction of the cystic lesion of a cystic meningioma in dogs. PMID:26256491

  6. Can we preoperatively risk stratify ovarian masses for malignancy?

    PubMed

    Oltmann, Sarah C; Garcia, Nilda; Barber, Robert; Huang, Rong; Hicks, Barry; Fischer, Anne

    2010-01-01

    Given a 10% malignancy rate in pediatric ovarian masses, what preoperative factors are helpful in distinguishing those at higher risk to risk stratify accordingly? After institutional review board approval (IRB#022008-095), a 15(1/2)-year retrospective review of operative ovarian cases was performed. A total of 424 patients were identified, with a mean age 12.5 years (range, 1 day to 19 years), without an age disparity between benign (12.54 years, 89%) and malignant (11.8 years, 11%) cases. The 1- to 8-year age group had the highest percentage of malignancies (22%; odds ratio [OR], 3.02; 95% confidence interval [CI], 1.33-6.86). A chief complaint of mass or precocious puberty versus one of pain had an OR for malignancy of 4.84 and 5.67, respectively (95% CI, 2.48-9.45 and 1.60-20.30). Imaging of benign neoplasms had a mean size of 8 cm (range, 0.9-36 cm) compared with malignancies at 17.3 cm (6.2-50 cm, P < .001). An ovarian mass size of 8 cm or longer on preoperative imaging had an OR of 19.0 for malignancy (95% CI, 4.42-81.69). Ultrasound or computed tomographic findings of a solid mass, although infrequent, were most commonly associated with malignancy (33%-60%), compared with reads of heterogeneous (15%-21%) or cystic (4%-5%) lesions. The malignancies (n = 46) included germ cell (50%, n = 23), stromal (28%, n = 13), epithelial (17%, n = 8), and other (4%, n = 2). Tumor markers obtained in 71% of malignancies were elevated in only 54%, whereas 6.5% of those sent in benign cases were similarly elevated. Elevated beta-human chorionic gonadotropin (beta-HCG), alpha fetoprotein (alphaFP), and cancer antigen 125 (CA-125) were significantly associated with malignancy (P < .02) and an elevated carcinoembryonic antigen (CEA) was not (P = .1880). This reported series of pediatric ovarian masses demonstrates that preoperative indicators that best predict an ovarian malignancy are a complaint of a mass or precocious puberty, a mass exceeding 8 cm or a mass with solid

  7. Adenoid Cystic Carcinoma of Sublingual Salivary Gland Obstructing the Submandibular Salivary Gland Duct

    PubMed Central

    Kumar, Venkata Suneel; Prathi, Venkata Sarath; Manne, Rakesh Kumar; Beeraka, Swapna; Natarajan, Kannan

    2013-01-01

    Sublingual salivary gland malignancies are extremely rare and account for only 0.3-1% of all epithelial salivary gland tumors. Here, we report a case of adenoid cystic carcinoma (ACC) of the sublingual salivary gland that presented as a swelling in the right anterior floor of the mouth obstructing the submandibular duct. Sublingual salivary gland ACC obstructing the submandibular duct is rare and only three cases have been reported in the literature until date. We discuss the different patterns of ACC seen during the pathologic investigations and its radiologic features. PMID:24516773

  8. Bosniak classification of renal cystic lesions according to multidetector computed tomography findings*

    PubMed Central

    de Miranda, Christiana Maia Nobre Rocha; Maranhão, Carol Pontes de Miranda; dos Santos, Carla Jotta Justo; Padilha, Igor Gomes; de Farias, Lucas de Pádua Gomes; da Rocha, Milzi Sarmento

    2014-01-01

    Renal cystic lesions are usually diagnosed in the radiologists’ practice and therefore their characterization is crucial to determine the clinical approach to be adopted and prognosis. The Bosniak classification based on computed tomography findings has allowed for standardization and categorization of lesions in increasing order of malignancy (I, II, IIF, III and IV) in a simple and accurate way. The present iconographic essay developed with multidetector computed tomography images of selected cases from the archives of the authors’ institution, is aimed at describing imaging findings that can help in the diagnosis of renal cysts. PMID:25741060

  9. Procaine in Malignant Hyperpyrexia

    PubMed Central

    Moulds, R. F. W.; Denborough, M. A.

    1972-01-01

    The caffeine contracture of normal human muscle, which has been used as a model for malignant hyperpyrexia, is greatly potentiated by halothane. Prior administration of procaine markedly reduces the halothane-potentiated caffeine contracture, and procaine given at the height of the contracture induces relaxation. Lignocaine, on the other hand, produces a variable response and sometimes increases the contracture. The muscle from a patient with an inherited susceptibility to malignant hyperpyrexia contracted spontaneously with halothane alone, and this contracture was reversed by procaine. These experiments support the therapeutic use of procaine in malignant hyperpyrexia. PMID:4642792

  10. Unusual Giant Prostatic Urethral Calculus

    PubMed Central

    Bello, A.; Maitama, H. Y.; Mbibu, N. H.; Kalayi, G. D.; Ahmed, A.

    2010-01-01

    Giant vesico-prostatic urethral calculus is uncommon. Urethral stones rarely form primarily in the urethra, and they are usually associated with urethral strictures, posterior urethral valve or diverticula. We report a case of a 32-year-old man with giant vesico-prostatic (collar-stud) urethral stone presenting with sepsis and bladder outlet obstruction. The clinical presentation, management, and outcome of the giant prostatic urethral calculus are reviewed. PMID:22091328

  11. Giant bulla mimicking tension pneumothorax.

    PubMed

    Gökçe, Mertol; Saydam, Ozkan; Altin, Remzi; Kart, Levent

    2009-01-01

    In the chest X-ray, we observe tension pneumothorax (TPX) as wide radiolucent view in a hemithorax and pushing the mediastinal structures contralateral. Giant bulla may mimic TPX with wide radiolucent view and mediastinal shift. The present report includes giant pulmonary bulla in 35-year-old woman. The giant bulla was diagnosed as a TPX in emergency, and chest tube was performed. The differentiation between TPX and a giant bulla may be very difficult. The therapies of these two similar entities are completely different. So that, we must be careful about anamnesis, physical examination and radiology for true diagnosis.

  12. 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.

  13. 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.

  14. Malignant fibrous histiocytoma at the site of an alumina-on-alumina-bearing total hip arthroplasty mimicking infected trochanteric bursitis.

    PubMed

    Yoon, Pil Whan; Jang, Woo Young; Yoo, Jeong Joon; Yoon, Kang Sup; Kim, Hee Joong

    2012-02-01

    Although the incidence of malignant tumors in patients undergoing total hip arthroplasties (THAs) is known to be lower than the general population, there exist several reports on the development of malignant tumors at the site of THAs. We report another case of malignant fibrous histiocytoma at the site of a THA, which was developed in an older patient who presented a cystic mass around the total hip prosthesis using a ceramic-on-ceramic bearing system, even without evidence of osteolysis or loosening of implants. This is the second case associated with an aluminum oxide prosthesis in English literature.

  15. Asbestos-related malignancy

    SciTech Connect

    Antmann, K.; Aisner, J.

    1986-01-01

    This book contains 20 chapters. Some of the chapter titles are: The Radiology of Asbestosis and Related Neoplasms; Computed Tomography and Malignant Mesothelioma; Radiation Therapy for Pleural Mesothelioma; and Radiation Therapy of Peritoneal Mesothelioma.

  16. Gynecologic malignancy in pregnancy

    PubMed Central

    Ji, Yong Il

    2013-01-01

    Gynecologic malignancy during pregnancy is a stressful problem. For the diagnosis and treatment of malignancy during pregnancy, a multidisciplinary approach is needed. Patients should be advised about the benefits and risk of treatment. When selecting a treatment for malignancy during pregnancy, the physiologic changes that occur with the pregnancy should be considered. Various diagnostic procedures that do not harm the fetus can be used. Laparoscopic surgery or laparotomy may be safely performed. The staging approach and treatment should be standard. Systemic chemotherapy during the first trimester should be delayed if possible. Radiation therapy should preferably start postpartum. Although delivery should be delayed preferably until after 35 weeks of gestation, termination of pregnancy may be considered when immediate treatment is required. Subsequent pregnancies do not increase the risk of malignancy recurrence. PMID:24328018

  17. What Is Malignant Mesothelioma?

    MedlinePlus

    ... you learn about the treatment options and possible side effects, and point you to information and services to help you in your cancer journey. ... free PDFs of our malignant mesothelioma information ...

  18. [Case of giant liver cyst with angiogenesis mimicking hemangioma that was difficult to differentiate from cystadenocarcinoma of the liver].

    PubMed

    Uchiyama, Tetsuyuki; Akahane, Takehiro; Watanabe, Mika; Kitayama, Taku; Ise, Hideo

    2008-11-01

    The patient was a 72-year-old man who had a history of hepatic cyst, which had, however, not been followed up. It was noted that he had internal hemorrhage in the hepatic cyst as well as a contrast-enhanced tumorous lesion in the cystic wall. Further detailed examination could not rule out hepatic cystadenocarcinoma or cystadenoma, so a right hepatic lobectomy was performed. The lesion was histopathologically diagnosed as non-malignant cyst with angiogenesis simulating cavernous hemangioma and intra-cystic hemorrhage. Although this disease is difficult to diagnose, its diagnosis is not impossible as long as the disease is kept in mind.

  19. Emerging treatments in cystic fibrosis.

    PubMed

    Jones, Andrew M; Helm, Jennifer M

    2009-10-01

    There are a number of potential drugs for the treatment of cystic fibrosis (CF) currently undergoing clinical studies. A number of antibacterials formulated for delivery by inhalation are at various stages of study; these include dry-powder inhaler versions of colistin, tobramycin and ciprofloxacin, and formulations of azteonam, amikacin, levofloxacin, ciprofloxacin and fosfomycin/tobramycin for nebulization. Clinical trials of anti-inflammatory agents, including glutathione, phosphodiesterase-5 inhibitors such as sildenafil, oral acetylcysteine, simvastatin, methotrexate, docosahexaenoic acid, hydroxychloroquine, pioglitazone and alpha1-antitrypsin, are ongoing. Ion channel modulating agents, such as lancovutide (Moli1901, duramycin) and denufosol, which activate alternate (non-CF transmembrane regulator [CFTR]) chloride channels, and GS 9411, a sodium channel antagonist, are now at the stages of clinical study and if successful, will offer a new category of therapeutic agent for the treatment of CF. Correction of the underlying gene effect, either by agents that help to correct the dysfunctional CFTR, such as ataluren, VX-770 and VX-809, or by gene transfer (gene therapy), is a particularly exciting prospect as a new therapy for CF and clinical studies are ongoing. This article reviews the exciting potential drug treatments for CF currently being evaluated in clinical studies, and also highlights some of the challenges faced by research and clinical teams in assessing the efficacy of potential new therapies for CF.

  20. [Azithromycin therapy in cystic fibrosis].

    PubMed

    Máiz Carro, Luis; Cantón Moreno, Rafael

    2004-03-06

    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.

  1. 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)

  2. 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.

  3. 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)

  4. Pineal cysts and other pineal region malignancies: determining factors predictive of hydrocephalus and malignancy.

    PubMed

    Starke, Robert M; Cappuzzo, Justin M; Erickson, Nicholas J; Sherman, Jonathan H

    2016-10-21

    OBJECTIVE Cystic lesions of the pineal gland are most often uncomplicated benign lesions with typical MRI characteristics. The authors aimed to study pineal lesion characteristics on MRI to better distinguish benign pineal cysts from other pineal region malignancies as well as to determine which characteristics were predictive of the latter malignancies. They also aimed to study risk factors predictive of hydrocephalus or malignancy in patients harboring these lesions. METHODS The authors performed a retrospective review of a prospectively compiled database documenting the outcomes of patients with suspected pineal cysts on MRI who had presented in the period from 1998 to 2004. Inherent patient and lesion characteristics were assessed in a univariate logistic regression analysis to predict the following dependent variables: development of hydrocephalus, biopsy-confirmed malignancy, and intervention. Possible inherent patient and lesion characteristics included age, sex, T1 and T2 MRI signal pattern, contrast enhancement pattern, presence of cyst, presence of blood, complexity of lesion, presence of calcification, and duration of follow-up. Inherent patient and lesion characteristics that were predictive in the univariate analysis (p < 0.15) were included in the multivariable logistic regression analysis. RESULTS Of the 79 patients with benign-appearing pineal cysts, 26 (33%) were male and 53 (67%) were female, with a median age of 38 years (range 9-86 years). The median cyst radius was 5 mm (range 1-20 mm). Two patients (2.5%) had evidence of calcifications, 7 (9%) had multicystic lesions, and 25 (32%) had some evidence of contrast enhancement. The median follow-up interval was 3 years (range 0.5-13 years). Seven patients (9%) had an increase in the size of their lesion over time. Eight patients (10%) had a hemorrhage, and 11 patients (14%) developed hydrocephalus. Nine (11%) received ventriculoperitoneal shunts for the development of hydrocephalus, and 12 patients

  5. 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.

  6. Giant Nasolabial Cyst Treated Using Neumann Incision: Case Report

    PubMed Central

    Ordones, Alexandre Beraldo; Neri, Larissa; Oliveira, Ingrid Helena Lopes; Tepedino, Miguel Soares; Pinna, Fábio de Rezende; Voegels, Richard Louis

    2013-01-01

    Introduction A nasolabial cyst is an ectodermal development cyst. It presents as a fullness of canine fossa, nasal ala, or vestibule of the nose. It is rare and usually small. Treatment consists of complete surgical excision or transnasal endoscopic marsupialization. Objective To describe a giant nasolabial cyst case treated using Neumann incision. Case Report A 37-year-old man was referred to the otolaryngology department with nasal obstruction and nasal deformity. Computed tomography showed a nasal cystic lesion 4 × 4.5 × 5 cm wide. Surgical excision using Neumann incision was performed. Discussion Neumann incision provides wide access to the nasal cavity and may be useful in nasolabial cyst treatment. PMID:25992051

  7. Giant pleomorphic adenoma of the parotid gland: an unusual case presentation and literature review.

    PubMed

    Tarsitano, A; Pizzigallo, A; Giorgini, F; Marchetti, C

    2015-10-01

    Pleomorphic adenoma is the most common type of all salivary gland tumours. Although uncommon, cases of giant pleomorphic adenomas have been described in the medical literature, the majority involving the parotid gland. This paper describes an unusual case of a giant adenoma arising in the parotid gland. The patient underwent surgical resection of the giant tumour, which was one of the largest pleomorphic adenoma reported in recent literature. This case has prompted us to evaluate the behaviour of those benign tumours, which suggested that aesthetic and social morbidity is sufficient to justify, when possible, early tumour excision, despite the relatively low risk of malignant transformation. Management of this unusual tumour is discussed, and the literature on giant parotid tumours is reviewed.

  8. Cystic duct carcinoma mimicking a middle bile duct tumour

    PubMed Central

    Francisco, Elsa; Mendes, Miguel; Vale, Sílvio; Esteves, Joana

    2015-01-01

    Cystic duct carcinoma was defined by Farrar as a tumour restricted to the cystic duct, making it a rare disease. The authors describe a case of a cystic duct carcinoma that fulfils Farrar’s strict diagnostic criteria and that became clinically relevant by compressing the common hepatic duct, thus causing cholestasis. A cholecystectomy was performed with en bloc resection of the cystic and extrahepatic bile duct with a regional lymphadenectomy. PMID:25819819

  9. Challenges in pulmonary fibrosis. 3: Cystic lung disease.

    PubMed

    Cosgrove, Gregory P; Frankel, Stephen K; Brown, Kevin K

    2007-09-01

    Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans' cell histiocytosis and lymphangioleiomyomatosis) and focal or multifocal cystic lung disease are discussed.

  10. Challenges in pulmonary fibrosis · 3: Cystic lung disease

    PubMed Central

    Cosgrove, Gregory P; Frankel, Stephen K; Brown, Kevin K

    2007-01-01

    Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans' cell histiocytosis and lymphangioleiomyomatosis) and focal or multifocal cystic lung disease are discussed. PMID:17726170

  11. Cystic Meningioma Masquerading as a Metastatic Tumor: A Case Report

    PubMed Central

    Ramanathan, Nithya; Kamaruddin, Khairul Azmi; Othman, Aizzat; Mustafa, Fadhli; Awang, Mohamed Saufi

    2016-01-01

    Cystic meningioma is a rare form of intracranial meningioma. Meningiomas are typically solid tumors but may rarely have cystic components. The diagnosis of cystic meningioma is clinically challenging as the finding of multiple intra-axial tumors, including metastatic tumors, is relatively common. We report a case of cystic meningioma initially diagnosed as a metastatic tumor from a recurrence of acute lymphoid leukemia. However, postoperative histopathological examination demonstrated an atypical meningioma. PMID:27418876

  12. 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.

  13. 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.

  14. [A study on low grade malignant tumors arisen in the trachea and the bronchus].

    PubMed

    Yamazaki, K; Kubo, Y; Hirasawa, M; Kitada, M; Yatsuyanagi, E; Moriyama, H; Koshiko, S; Sugimoto, H; Hirata, S; Sasajima, T

    1997-10-01

    Twelve patients who suffered from low grade malignant tumors arisen in the trachea and the bronchus (6 of carcinoid, 4 of mucoepidermoid carcinoma, and 2 of adenoid cystic carcinoma) underwent surgical treatment from 1977 to 1996 in our department. Operations included 1 sleeve resection of the trachea, 1 patch plasty of the trachea, 3 sleeve lobectomies, and 7 lobectomies. Lymph node dissection was performed in 9 of 12 cases. Metastases in lymph nodes were not found in all 12 cases. Five year survival rate of low grade malignant tumors arisen in the trachea and the bronchus was 78.8% and better than that of stage I lung cancers.

  15. Giant pulmonary teratoma with huge splenic lymphangiomatosis: a very rare case.

    PubMed

    Alsubaie, Hemail M; Alsubaie, Khaled M; Mahfouz, Mohammed Eid

    2017-09-01

    Teratomas are tumors composed of tissues derived from more than one germ cell line. They manifested with a great variety of clinical and radiological features. We report a case of a giant left hemithorax teratoma in a female with huge spleen tumor and review the relevant literature. A 38-year-old female with progressively aggravating dyspnea at rest from a mild trauma. Absent breath sounds on the left side. There was splenomegaly. Computed tomography scan revealed a huge mass (20 × 15 × 18 cm), containing elements of heterogeneous density in the left hemithorax. The spleen tumor was occupying most of the spleen without any other abdominal manifestations. The patient underwent left thoracotomy and laparoscopic splenectomy. Histopathological examination revealed a benign mature teratoma and cystic lymphangiomatosis of the spleen. To the best of our knowledge and after reviewing the available literature this is the first case of huge mature pulmonary teratoma with large cystic spleen lymphangiomatosis.

  16. Giant left ventricular pseudoaneurysm.

    PubMed

    Prakash, Sumi; Garg, Nadish; Xie, Gong-Yuan; Dellsperger, Kevin C

    2010-01-01

    Left ventricular (LV) pseudoaneurysm (PS) is an uncommon, often fatal complication associated with myocardial infarction, cardiothoracic surgery, trauma, and, rarely, infective endocarditis. A 28-year-old man with prior history of bioprosthetic mitral valve replacement presented with congestive heart failure and bacteremia with Abiotrophia granulitica. Transesophageal echocardiogram showed bioprosthesis dysfunction, large vegetations, mitral regurgitation, and probable PS. Cardiac and chest CT confirmed a PS communicating with the left ventricle Patient had pulseless electrical activity and died. Autopsy showed a giant PS with layered thrombus and pseudo-endothelialized cavity. Our case highlights the importance of multimodality imaging as an important tool in management of PS.

  17. A Giant Urethral Calculus.

    PubMed

    Sigdel, G; Agarwal, A; Keshaw, B W

    2014-01-01

    Urethral calculi are rare forms of urolithiasis. Majority of the calculi are migratory from urinary bladder or upper urinary tract. Primary urethral calculi usually occur in presence of urethral stricture or diverticulum. In this article we report a case of a giant posterior urethral calculus measuring 7x3x2 cm in a 47 years old male. Patient presented with acute retention of urine which was preceded by burning micturition and dribbling of urine for one week. The calculus was pushed in to the bladder through the cystoscope and was removed by suprapubic cystolithotomy.

  18. [Cystic fibrosis in a 70-year-old woman].

    PubMed

    Bruun, Lene Søndberg; Jensen, Michael Skov

    2002-05-06

    Cystic fibrosis is usually diagnosed in early childhood, and patients rarely live beyond the age of 40. We present a case of a 70-year-old woman, in whom cystic fibrosis was diagnosed with the rare mutation, R117C. Cystic fibrosis should therefore also be considered in older patients.

  19. 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;…

  20. 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;…

  1. Analysis of orbital malignancies presenting in a tertiary care hospital in Pakistan

    PubMed Central

    Khan, Asad Aslam; Sarwar, Suhail; Sadiq, Mohammad Ali A; Ahmad, Imran; Tariq, Nayab; Sibghat-ul-Noor

    2017-01-01

    Objective: To determine the frequencies of various orbital malignancies amongst orbital lesions in patients presenting in a tertiary care hospital in Pakistan. Methods: A retrospective analysis of 666 orbital cases with an established histopathological diagnosis of malignant tumors treated in Mayo Hospital Lahore from 1996 to 2015 (20 years). Results: About 66% of the malignant tumors were primary, 25% secondary, 8% haematopoietic and 1% metastatic. Almost 50% of the cases were children. Retinoblastoma is the commonest tumor (43% overall and 87% among children). Squamous cell carcinoma is the second commonest (15.6% overall and 31% among adults). These are then followed by Adenoid cystic carcinoma of Lacrimal Gland (9%), Lymphoma/Leukaemia (8%) and Rhabdomyosarcoma (6.3%). Conclusion: Frequencies of various orbital malignancies show geographical variation in both paediatric and adult population. PMID:28367175

  2. [Primary malignant mesenchymoma of the lung].

    PubMed

    Kodolova, I M; Kogan, E A; Sekamova, S M; Iashunskaia, N Ia

    1988-01-01

    A case of a primary malignant mesenchymoma of the lung with elements of rhabdomyo-, leiomyo-, osteo-, fibro- and lipo-sarcoma is described. The tumor developed in a man of 58 with a long history of smoking, complaints of chest pains and cough with scanty sputum expectoration. The neoplastic process involved the upper lobe of the left lung and microscopically contained smooth muscle, endothelial, fibroblast-like cells, multinuclear giant cells resembling osteoclasts, strips of osteoid-like hyalinized connective tissue. Electron-microscopic examination revealed myoid-type cells with clusters of myofilaments and Z-type material, cells resembling fibroblasts, osteoclasts and lipocytes. It is suggested that there should be a common histogenesis of primary lung sarcomas arising from a stem cell precursor of mesenchymal origin in lung stroma, bronchial and vascular walls, pleura.

  3. 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.

  4. Benign Cystic Mesothelioma in a Child: Case Report and Review of the Literature

    PubMed Central

    Tuncer, Ahmet Ali; Narcı, Adnan; Dilek, Fatma Hüsniye; Embleton, Didem Baskın; Çetinkurşun, Salih

    2016-01-01

    Background: Benign cystic mesothelioma (BCM) is a rare tumor with benign characteristic. There are only 8 child cases reported in the English literature. In this report, we present this rare entity a brief review of the literature. Case Report: A two year-old boy presenting with abdominal swelling was hospitalized. Physical examination revealed a mass filling the abdomen. Laboratory findings were not specific. Abdominal computerized tomography showed a 15×13×11 cm cystic mass extending from the bladder to the liver with no solid components and no infiltration to adjacent organs. Operation revealed a cystic mass filled with yellow-green serous fluid which was attached to the right lobe of the liver with a 1 cm thick peduncle. Total excision of the mass was performed by clamping and cutting the narrow attachment. Recovery was uneventful. Pathology revealed multiple cysts lined with mesothelial cells. No recurrence was seen after 5 years of follow-up. Conclusion: BCM should be kept in mind as a rare cause of the abdominal mass in children, as it may lead to confusion in preoperative diagnosis. Although rare, patients should be followed throughout life because of the risk of recurrence and malignant degeneration. PMID:27403396

  5. Cystic degeneration of neuro endocrine tumor of pancreas and Crohn's disease: true or coincidental association?

    PubMed

    Hedfi, Mohamed; Abbasi, Imed; Intissar, Chibani; Salwa, Ammar; Chouchen, Adnen

    2016-01-01

    Pancreatic endocrine tumors (PETs) or islet cell tumors are rare lesions, the incidence of which is estimated to be less than 1 per 100,000 person-years in the general population. PETs can be divided into functional (exhibit a distinct clinical syndrome due to hormone hypersecretion) and non-functional tumors. The majority of PETs are non-functional. In spite of their rarity, cystic neoplasms of the pancreas are characterized by existing or potential malignancy that cannot be ignored during decisive process with regard to the choice of treatment. The purpose of this workis to find an association with Crohn's disease and cystic degeneration of a neuroendocrine tumor of the pancreas. Crohn's disease may affect extraintestinal organs, including the pancreas. In such cases, It seems certain that many patients diagnosed with Crohn disease (CD) are predisposed to a wider spectrum of cancers. We present a case of pancreatic cyst with no typical features of pseudocyst in the medical interview, with history of Crohn's disease, treated by caudal pancreatectomy. We tried to evaluate the clinical and morphological features of so-called cystic neoplasms associated with inflammatory bowel disease and to define their pathological characteristics.

  6. 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

  7. Self-management education for cystic fibrosis.

    PubMed

    Savage, Eileen; Beirne, Paul V; Ni Chroinin, Muireann; Duff, Alistair; Fitzgerald, Tony; Farrell, Dawn

    2011-07-06

    Self-management education may help patients with cystic fibrosis and their families to choose, monitor and adjust treatment requirements for their illness, and also to manage the effects of illness on their lives. Although self-management education interventions have been developed for cystic fibrosis, no previous systematic review of the evidence of effectiveness of these interventions has been conducted. To assess the effects of self-management education interventions on improving health outcomes for patients with cystic fibrosis and their caregivers We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register (date of the last search: 23 February 2011).We also searched databases through EBSCO (CINAHL; Psychological and Behavioural Sciences Collection; PsychInfo; SocINDEX) and Elsevier (EMBASE) and handsearched relevant journals and conference proceedings (date of the last searches: 30th March 2011). Randomised controlled trials, quasi-randomised controlled trials or controlled clinical trials comparing different types of self-management education for cystic fibrosis or comparing self-management education with standard care or no intervention. Two authors assessed trial eligibility and risk of bias. Three authors extracted data. Four trials (involving a total of 269 participants) were included. The participants were children with cystic fibrosis and their parents or caregivers in three trials and adults with cystic fibrosis in one trial. The trials compared four different self-management education interventions versus standard treatment: (1) a training programme for managing cystic fibrosis in general; (2) education specific to aerosol and airway clearance treatments; (3) disease-specific nutrition education; and (4) general and disease-specific nutrition education. Training children to manage cystic fibrosis in general had no statistically significant effects on weight after six to eight weeks, mean difference -7.74 lb (95% confidence

  8. Self-management education for cystic fibrosis.

    PubMed

    Savage, Eileen; Beirne, Paul V; Ni Chroinin, Muireann; Duff, Alistair; Fitzgerald, Tony; Farrell, Dawn

    2014-09-08

    Self-management education may help patients with cystic fibrosis and their families to choose, monitor and adjust treatment requirements for their illness, and also to manage the effects of illness on their lives. Although self-management education interventions have been developed for cystic fibrosis, no previous systematic review of the evidence of effectiveness of these interventions has been conducted. To assess the effects of self-management education interventions on improving health outcomes for patients with cystic fibrosis and their caregivers We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register (date of the last search: 22 August 2013).We also searched databases through EBSCO (CINAHL; Psychological and Behavioural Sciences Collection; PsychInfo; SocINDEX) and Elsevier (Embase) and handsearched relevant journals and conference proceedings (date of the last searches: 01 February 2014 ). Randomised controlled trials, quasi-randomised controlled trials or controlled clinical trials comparing different types of self-management education for cystic fibrosis or comparing self-management education with standard care or no intervention. Two authors assessed trial eligibility and risk of bias. Three authors extracted data. Four trials (involving a total of 269 participants) were included. The participants were children with cystic fibrosis and their parents or caregivers in three trials and adults with cystic fibrosis in one trial. The trials compared four different self-management education interventions versus standard treatment: (1) a training programme for managing cystic fibrosis in general; (2) education specific to aerosol and airway clearance treatments; (3) disease-specific nutrition education; and (4) general and disease-specific nutrition education. Training children to manage cystic fibrosis in general had no statistically significant effects on weight after six to eight weeks, mean difference -7.74 lb (i.e. 3.51 kg) (95

  9. [Cystic lesions--diagnosis and conservative treatment].

    PubMed

    Olas, Jacek; Bucka, Jolanta; Dworak, Agata; Golański, Bartłomiej

    2003-01-01

    Effective treatment of cystic lesions that we can apply in the outpatient clinic is the target for seeking a new solution in finding treatment that will produce a better percentage of recovered patients. At this moment non-surgical treatment of cystic lesions is compare with traditional methods of treatment i.e. cyst content aspiration and injection of steroids, with methods which lead to obliteration of cyst lumen and in this way closing the space being the reservoir for the cyst content. Local application of hialuronidase, aspiration, and then steroid injection is gaining more and more attention of clinicians. We also pay much more attention to local fibrinogen injection as a safe and effective method of treatment, which can be useful in the treatment of cystic lesions and topical treatment of bursitis.

  10. Giant cemento-ossifying fibroma of the maxilla.

    PubMed

    Unal, Adnan; Yurtsever Kum, Nurcan; Kum, Rauf Oguzhan; Erdogan, Aysun; Ciliz, Deniz Sozmen; Guresci, Servet; Ozcan, Muge

    2015-11-14

    Fibro-osseous lesions of the skull and facial bones are benign tumors, but they can be mistaken for malignant tumors due to their clinically aggressive behavior. Cemento-ossifying fibroma (COF) is a benign fibro-osseous lesion characterized by slow growth and fibrous and calcified tissue content. COFs are locally destructive lesions causing deformities in the bones. The recurrence risk is high if they are not completely removed. In this case report we describe a giant COF mimicking chondrosarcoma in the oral cavity of a 55-year-old woman causing significant facial deformity and feeding problems. Giant COF occurs rarely in the jaws and given that this lesion has similar imaging and clinical features to several other tumors, the diagnosis is always a challenge for clinicians, radiologists and pathologists.

  11. A rare case of bilateral cystic partially differentiated nephroblastoma recurring as bilateral cystic Wilms tumour.

    PubMed

    Kurian, Jujju Jacob; Ninan, Pradeep Joseph

    2015-04-15

    Childhood cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal neoplasm. Bilateral CPDN or CPDN co-existing with a cystic nephroma/Wilms tumour is extremely rare. Treatment of CPDN is by complete surgical excision. Although local recurrences are uncommon, distant metastases have not been described. We present a case of bilateral CPDN that, after complete excision, recurred as bilateral cystic Wilms tumour. To the best of our knowledge, this is the first reported case in the literature where a bilateral CPDN has recurred as bilateral Wilms tumour.

  12. Cystic angiomatosis, a heterogeneous condition

    PubMed Central

    Najm, Aurélie; Soltner-Neel, Elise; Le Goff, Benoît; Guillot, Pascale; Maugars, Yves; Berthelot, Jean-Marie

    2016-01-01

    Abstract Background: Cystic angiomatosis (CA) is a rare disorder causing bony cysts. It displays some similarity to Gorham–Stout disease (GSD), but has a much better local prognosis, despite the larger number of cysts. These 2 conditions also differ in terms of their location, visceral involvement, and response to treatment. Methods: We report 4 cases of CA, including 1 sclerosing form, which we compare with cases from a literature review performed with PRISMA methodology. Results: We reviewed 38 articles describing 44 other patients. Mean age at diagnosis for the 48 patients (our 4 patients + the 44 from the review) was 22.5 years, and 28 of the patients were men. The femur was involved in 81% (n = 39), the pelvis in 73% (n = 35), the humerus in 52% (n = 25), the skull in 48% (n = 23), and the vertebrae in 44% (n = 21). Visceral lymphangiomatosis (either clinical, or detected on autopsy) was also reported in 35% (n = 18) of the patients. The spleen was the most frequently involved organ (n = 12), followed by the lungs and pleura (n = 8). Liver cysts and/or chylothorax were rarely reported (5 cases), but were invariably fatal. Radiation therapy on bone or soft tissue masses was ineffective, as was interferon alpha, in the 2 patients in which this drug was tested. The efficacy of bisphosphonate was at best equivocal. Conclusion: The progression of CA is unpredictable and treatments effective against GSD, such as bisphosphonates and radiotherapy, have proved ineffective for this condition. New treatments are thus urgently required. PMID:27787381

  13. Venous thromboembolism in cystic fibrosis.

    PubMed

    Takemoto, Clifford M

    2012-02-01

    The incidence of venous thromboembolism (VTE) is increasing in the pediatric population. Individuals with cystic fibrosis (CF) have an increased risk of thrombosis due to central venous catheters (CVCs), as well as acquired thrombophilia secondary to inflammation, or deficiencies of anticoagulant proteins due to vitamin K deficiency and/or liver dysfunction. CVC-associated thrombosis commonly results in line occlusion, but may develop into serious life-threatening conditions such as deep venous thrombosis (DVT), superior vena cava syndrome or pulmonary embolism (PE). Post-thrombotic syndrome (PTS) may be a long complication. Local occlusion of the catheter tip may be managed with instillation of thrombolytics (such as tPA) within the lumen of the catheter; however, CVC-associated thrombosis involving the proximal veins is most often is treated with systemic anticoagulation. Initial treatment with heparin is a standard approach, but thrombolytic therapy, which may carry higher bleeding risks, should be considered for life and limb threatening episodes of VTE. Recommended duration of anticoagulation with low molecular weight heparin (LMWH) or warfarin ranges from 3 to 6 months for major removable thrombotic risks; longer anticoagulation is considered for recurrent thrombosis, major persistent thrombophilia, or the continued presence of a major risk factor such as a CVC. While CVCs are the most common risk for development of VTE in children, studies have not demonstrated a clear benefit with routine use of systemic thromboprophylaxis. The incidence and risk factors of VTE in CF patients will be reviewed and principles of diagnosis and management will be summarized.

  14. Cystic fibrosis in adult age.

    PubMed

    Lerín, M; Prados, C; Martínez, M T; Maíz, L; Girón, R; Solé, A; Cabanillas, J J; Alvarez-Sala, R

    2014-01-01

    To know the prevalence of the patients diagnosed of cystic fibrosis (CF) older than 18 years old of five specific Spanish Units and to analyze their clinical, genetic and microbiological characteristics. Observational, cross-sectional, descriptive study of patients diagnosed with CF at age or older than 18 years. The variables analyzed were: current age, age at diagnosis, sex, nationality, lung function parameters, pathologies presented at diagnosis, microbiological features and genetic findings. Eigthy nine patients (14.8% of the total of 600 CF patients followed at the participating units), of which 45 patients were female (50.6%) and 44 were males (49.4%), were included with a mean age at diagnosis of 36.4 years. Eigthy one patients (91%) were Spaniards. The sweat test was diagnostic in 77 (86.5%) of the patients studied. The sweat test was diagnostic in 77 of the 89 patients studied (86.5%). The most frequently detected mutations were F508del/other and G542X/other, and the most frequent clinical findings at diagnosis were the presence of bronchiectasis in 33 patients (37.1%) followed by sterility in 12 patients (13.5%). The most common colonizing organisms were meticillin-sensitive Staphylococcus aureus (S.aureus) (23.6%) and Pseudomonas aeruginosa (P. aeruginosa) (13.5%). Most patients presented a mild obstructive ventilatory defect and had no pancreatic involvement. The sweat test used to be indeterminate. CF is also a disease which diagnosis can be in adulthood. CF patients diagnosed in adulthood have a mild lung function and lower incidence of pancreatic involvement, so their prognosis tends to be favorable. Copyright © 2014 Elsevier España, S.L. All rights reserved.

  15. Cystic echinococcosis in Southern Israel.

    PubMed

    Ben-Shimol, Shalom; Sagi, Orli; Houri, Ohad; Bazarsky, Elina; Berkowitz, Anat; Bulkowstein, Shlomi; Barrett, Chiya; Greenberg, David

    2016-01-01

    The aim of this retrospective, population-based study was to characterize demographically and clinically cystic-echinococcosis (CE) in southern Israel, between 2005 and 2012. Newly-diagnosed (nd-CE) and past-diagnosed (pd-CE, diagnosed before the study) cases were defined. Two populations live in southern-Israel, receiving medical treatment at a single hospital: the Jewish and the Bedouin populations (resembling resource-rich and resource-poor populations, respectively). 126 CE cases were identified; 55 nd-CE and 71 pd-CE. Mean annual nd-CE incidence per 100,000 in the Bedouin and Jewish populations were 2.7 ± 1.2 and 0.4 ± 0.3, respectively (P<0.001). None of the Bedouin and 86.5% of the Jewish patients were born outside Israel. Liver and lung involvement were recorded in 85.7% and 15.1% of overall-CE, respectively. Abdominal pain, cough, fever, eosinophilia and asymptomatic disease were documented in 63.6%, 32.7%, 27.3%, 41.5% and 12.7% of nd-CE, respectively. Serology sensitivity for first test and any positive test were 67.3% and 83.3%, respectively. Computed tomography, ultrasonography and X-ray diagnosis were documented in 79.2%, 58.4% and 17.0% of overall-CE, respectively, with ultrasonography mainly used in liver-CE and X-ray in lung-CE. Treatment included surgery and albendazole in 50.0% and 55.3% of CE, respectively. We conclude that CE is endemic in southern-Israel among the Bedouin population, while disease is probably mainly imported in the Jewish population. Liver involvement and eosinophilia rates were high compared with those of other endemic regions, possibly due to differences in the timing of diagnosis. These findings may help developing treatment and prevention strategies.

  16. Enteral tube feeding for cystic fibrosis.

    PubMed

    Morton, Alison; Wolfe, Susan

    2015-04-09

    Enteral tube feeding is routinely used in many cystic fibrosis centres when oral dietary and supplement intake has failed to achieve an adequate nutritional status. The use of this method of feeding is assessed on an individual basis taking into consideration the patients age and clinical status. To examine the evidence that in people with cystic fibrosis, supplemental enteral tube feeding improves nutritional status, respiratory function, and quality of life without significant adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also contacted the companies that market enteral feeds and reviewed their databases.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 13 February 2015.Date of the most recent hand search of PubMed and conference abstract books: 13 February 2015. All randomised controlled trials comparing supplemental enteral tube feeding for one month or longer with no specific intervention in people with cystic fibrosis. The searches identified 38 trials; however, none were eligible for inclusion in this review. There are no trials included in this review. Supplemental enteral tube feeding is widely used throughout the world to improve nutritional status in people with cystic fibrosis. The methods mostly used, nasogastric or gastrostomy feeding, are expensive and may have a negative effect on self-esteem and body image. Reported use of enteral tube feeding suggests that it results in nutritional and respiratory improvement; but, efficacy has not been fully assessed by randomised controlled trials. It is acknowledged, however, that performing a randomised controlled trial would be difficult due to the ethics of withholding an intervention in a group of patients whose nutritional status necessitates it.

  17. Liver Disease in Cystic Fibrosis: an Update

    PubMed Central

    Parisi, Giuseppe Fabio; Di Dio, Giovanna; Franzonello, Chiara; Gennaro, Alessia; Rotolo, Novella; Lionetti, Elena; Leonardi, Salvatore

    2013-01-01

    Context Cystic fibrosis (CF) is the most widespread autosomal recessive genetic disorder that limits life expectation amongst the Caucasian population. As the median survival has increased related to early multidisciplinary intervention, other manifestations of CF have emergedespecially for the broad spectrum of hepatobiliary involvement. The present study reviews the existing literature on liver disease in cystic fibrosis and describes the key issues for an adequate clinical evaluation and management of patients, with a focus on the pathogenetic, clinical and diagnostic-therapeutic aspects of liver disease in CF. Evidence Acquisition A literature search of electronic databases was undertaken for relevant studies published from 1990 about liver disease in cystic fibrosis. The databases searched were: EMBASE, PubMed and Cochrane Library. Results CF is due to mutations in the gene on chromosome 7 that encodes an amino acidic polypeptide named CFTR (cystic fibrosis transmembrane regulator). The hepatic manifestations include particular changes referring to the basic CFTR defect, iatrogenic lesions or consequences of the multisystem disease. Even though hepatobiliary disease is the most common non-pulmonary cause ofmortalityin CF (the third after pulmonary disease and transplant complications), only about the 33%ofCF patients presents clinically significant hepatobiliary disease. Conclusions Liver disease will have a growing impact on survival and quality of life of cystic fibrosis patients because a longer life expectancy and for this it is important its early recognition and a correct clinical management aimed atdelaying the onset of complications. This review could represent an opportunity to encourage researchers to better investigate genotype-phenotype correlation associated with the development of cystic fibrosis liver disease, especially for non-CFTR genetic polymorphisms, and detect predisposed individuals. Therapeutic trials are needed to find strategies of

  18. Enteral tube feeding for cystic fibrosis.

    PubMed

    Conway, Steven; Morton, Alison; Wolfe, Susan

    2012-12-12

    Enteral tube feeding is routinely used in many cystic fibrosis centres when oral dietary and supplement intake has failed to achieve an adequate nutritional status. The use of this method of feeding is assessed on an individual basis taking into consideration the patients age and clinical status. To examine the evidence that in people with cystic fibrosis, supplemental enteral tube feeding improves nutritional status, respiratory function, and quality of life without significant adverse effects. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also contacted the companies that market enteral feeds and reviewed their databases.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 03 September 2012.Date of the most recent hand search of PubMed and conference abstract books: 15 June 2012. All randomised controlled trials comparing supplemental enteral tube feeding for one month or longer with no specific intervention in people with cystic fibrosis. Thirty-one trials were identified by the searches; however, none were eligible for inclusion in this review. There are no trials included in this review. Supplemental enteral tube feeding is widely used throughout the world to improve nutritional status in people with cystic fibrosis. The methods mostly used, nasogastric or gastrostomy feeding, are expensive and may have a negative effect on self-esteem and body image. Reported use of enteral tube feeding suggests that it results in nutritional and respiratory improvement; but, efficacy has not been fully assessed by randomised controlled trials. It is acknowledged, however, that performing a randomised controlled trial would be difficult due to the ethics of withholding an intervention in a group of patients whose nutritional status

  19. Giant extragenital Bowen's disease.

    PubMed

    Bakardzhiev, Ilko; Chokoeva, Anastasiya Atanasova; Tchernev, Georgi

    2015-12-01

    Giant extragenital forms of Morbus Bowen are extremely rare. The already described cases in the word literature are most commonly with periungual localization, as well as located on the foot and neck area. The clinical manifestation is presented most commonly by non-specific erythematous to erythematous-squamous plaques or papules, which is confusing to the clinician. From the pathogenic point of view, it is important to be confirmed or rejected the presence of human papilloma viruses (HPVs) in each case of affected patient, as this information is mandatory in respect to the adequate selection of the subsequent regimen. If HPVs are detected, systemic antiviral therapy could be initiated to reduce the size of the lesions before subsequent surgical eradication. A postoperative prevention through vaccination could be also considered additionally. In cases of HPV-negative giant extragenital forms of Morbus Bowen (as in the described patient), the focus should be on local immunomodulation by substances such as imiquimod, which reduce the size of the lesions, thereby creating optimal opportunities for their future surgical eradication. Other possible options described in the literature include topical application of 5-fluorouracil, photodynamic therapy, cryotherapy, and laser therapy (carbon dioxide laser). The choice of the most appropriate regimen should have been an individual decision of the clinician, considering also the location and the extent of the lesion.

  20. 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.

  1. 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

  2. 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.

  3. Giant Intradiverticular Bladder Tumor

    PubMed Central

    Noh, Mohamad Syafeeq Faeez Md; Aziz, Ahmad Fuad Abdul; Ghani, Khairul Asri Mohd; Siang, Christopher Lee Kheng; Yunus, Rosna; Yusof, Mubarak Mohd

    2017-01-01

    Patient: Male, 74 Final Diagnosis: Giant intradiverticular bladder tumor with metastasis Symptoms: Hematuria Medication:— Clinical Procedure: — Specialty: Urology Objective: Rare disease Background: Intradiverticular bladder tumors are rare. This renders diagnosis of an intradiverticular bladder tumor difficult. Imaging plays a vital role in achieving the diagnosis, and subsequently staging of the disease. Case Report: A 74-year-old male presented to our center with a few months history of constitutional symptoms. Upon further history, he reported hematuria two months prior to presentation, which stopped temporarily, only to recur a few days prior to coming to the hospital. The patient admitted to having lower urinary tract symptoms. However, there was no dysuria, no sandy urine, and no fever. Palpation of his abdomen revealed a vague mass at the suprapubic region, which was non tender. In view of his history and the clinical examination findings, an ultrasound of the abdomen and computed tomography (CT) was arranged. These investigations revealed a giant tumor that seemed to be arising from a bladder diverticulum, with a mass effect and hydronephrosis. He later underwent operative intervention. Conclusions: Intradiverticular bladder tumors may present a challenge to the treating physician in an atypical presentation; thus requiring a high index of suspicion and knowledge of tumor pathophysiology. As illustrated in our case, CT with its wide availability and multiplanar imaging capabilities offers a useful means for diagnosis, disease staging, operative planning, and follow-up. PMID:28246375

  4. Reinflating Giant Planets

    NASA Astrophysics Data System (ADS)

    Kohler, Susanna

    2017-01-01

    Two new, large gas-giant exoplanets have been discovered orbiting close to their host stars. A recent study examining these planets and others like them may help us to better understand what happens to close-in hot Jupiters as their host stars reach the end of their main-sequence lives.OversizedGiantsUnbinned transit light curves for HAT-P-65b. [Adapted from Hartman et al. 2016]The discovery of HAT-P-65b and HAT-P-66b, two new transiting hot Jupiters, is intriguing. These planets have periods of just under 3 days and masses of roughly 0.5 and 0.8 times that of Jupiter, but their sizes are whats really interesting: they have inflated radii of 1.89 and 1.59 times that of Jupiter.These two planets, discovered using the Hungarian-made Automated Telescope Network (HATNet) in Arizona and Hawaii, mark the latest in an ever-growing sample of gas-giant exoplanets with radii larger than expected based on theoretical planetary structure models.What causes this discrepancy? Did the planets just fail to contract to the expected size when they were initially formed, or were they reinflated later in their lifetimes? If the latter, how? These are questions that scientists are only now starting to be able to address using statistics of the sample of close-in, transiting planets.Unbinned transit light curves for HAT-P-66b. [Hartman et al. 2016]Exploring Other PlanetsLed by Joel Hartman (Princeton University), the team that discovered HAT-P-65b and HAT-P-66b has examined these planets observed parameters and those of dozens of other known close-in, transiting exoplanets discovered with a variety of transiting exoplanet missions: HAT, WASP, Kepler, TrES, and KELT. Hartman and collaborators used this sample to draw conclusions about what causes some of these planets to have such large radii.The team found that there is a statistically significant correlation between the radii of close-in giant planets and the fractional ages of their host stars (i.e., the stars age divided by its full

  5. The Cystic Fibrosis of Exocrine Pancreas

    PubMed Central

    Wilschanski, Michael

    2013-01-01

    The cystic fibrosis transmembrane conductance regulator (CFTR) protein is highly expressed in the pancreatic duct epithelia, and permits anions and water to enter the ductal lumen. This results in an increased volume of alkaline fluid allowing the highly concentrated proteins secreted by the acinar cells to remain in a soluble state. This work will expound on the pathophysiology and pathology caused by the malfunctioning CFTR protein with special reference to ion transport and acid-base abnormalities both in humans and animal models. We will also discuss the relationship between cystic fibrosis (CF) and pancreatitis, and outline present and potential therapeutic approaches in CF treatment relevant to the pancreas. PMID:23637307

  6. Cardiac lymphangioma presenting as intrapericardial cystic mass.

    PubMed

    Lone, Nazir A; Naikoo, Bashir A; Khan, Naseer A

    2016-01-01

    Cystic lymphangioma usually confined to head and neck is a well-recognized tumor that occurs during childhood. However, a cardiac lymphangioma is exceptionally uncommon and a particularly rare form of disease. We report a case of cystic lymphangioma arising from the right ventricular wall, and presenting as pericardial mass in a young female, who presented with a history of exercise intolerance in the form of breathlessness on exertion and palpitations. The management of such a case was a difficult task; however, she underwent near total resection of the mass, and is doing well for the last 2 years.

  7. Management Issues for Adolescents with Cystic Fibrosis

    PubMed Central

    Withers, Adelaide Lindsay

    2012-01-01

    The healthy adolescent will encounter major changes in biological and psychosocial domains. The adolescent period can be greatly affected by a chronic illness. Cystic fibrosis is a terminal illness that can significantly affect an adolescent's biological, mental and psychosocial health. This paper discusses general issues to consider when managing an adolescent with a chronic medical condition, and specifically how cystic fibrosis may impact upon puberty, body image, risk-taking behaviours, mental health, independence, nonadherence, reproductive health, transition, lung transplantation, and end of life care. PMID:22991662

  8. 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.

  9. 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.

  10. Precision genomic medicine in cystic fibrosis

    PubMed Central

    Chang, Eugene H.; Zabner, Joseph

    2015-01-01

    The successful application of precision genomic medicine requires an understanding of how a person’s genome can influence their 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. PMID:26073768

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

    PubMed

    Schmitz, Lars; Motani, Ryosuke; Oufiero, Christopher E; Martin, Christopher H; McGee, Matthew D; Gamarra, Ashlee R; Lee, Johanna J; Wainwright, Peter C

    2013-02-18

    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. 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. 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.

  12. 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

  13. 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

  14. Cystic lesions of the parotid gland: radiologic-pathologic correlation according to the latest World Health Organization 2017 Classification of Head and Neck Tumours.

    PubMed

    Takita, Hirotaka; Takeshita, Tohru; Shimono, Taro; Tanaka, Hiroko; Iguchi, Hiroyoshi; Hashimoto, Shigeo; Kuwae, Yuko; Ohsawa, Masahiko; Miki, Yukio

    2017-08-23

    The latest World Health Organization 2017 Classification of Head and Neck Tumours includes a new chapter on tumors and tumor-like lesions of the neck and lymph nodes. Tumor-like lesions include a variety of cystic lesions of the parotid gland. Cystic lesions of the parotid gland can be divided into three groups: non-neoplastic cysts, benign tumors with macrocystic change, and malignant tumors with macrocystic change. It is important to distinguish these lesions from one another because treatment and patient management differ among the three groups. The purpose of this review is to describe the magnetic resonance imaging and scintigraphy findings and the histopathologic characteristics of each parotid gland lesion based on the latest World Health Organization 2017 Classification of Head and Neck Tumours and to summarize the key points of differential diagnosis for cystic lesions of the parotid gland.

  15. 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.

  16. Malignant peritoneal mesothelioma

    PubMed Central

    Munkholm-Larsen, Stine; Cao, Christopher Q; Yan, Tristan D

    2009-01-01

    Malignant mesothelioma is a highly aggressive neoplasm. The incidence of malignant mesothelioma is increasing worldwide. Diffuse malignant peritoneal mesothelioma (DMPM) represents one-fourth of all mesotheliomas. Association of asbestos exposure with DMPM has been observed, especially in males. The great majority of patients present with abdominal pain and distension, caused by accumulation of tumors and ascitic fluid. In the past, DMPM was considered a pre-terminal condition; therefore attracted little attention. Patients invariably died from their disease within a year. Recently, several prospective trials have demonstrated a median survival of 40 to 90 mo and 5-year survival of 30% to 60% after combined treatment using cytoreductive surgery and perioperative intraperitoneal chemotherapy. This remarkable improvement in survival has prompted new search into the medical science related to DMPM, a disease previously ignored as uninteresting. This review article focuses on the key advances in the epidemiology, diagnosis, staging, treatments and prognosis of DMPM that have occurred in the past decade. PMID:21160794

  17. 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.

  18. Malignant Vestibular Schwannoma

    PubMed Central

    Gruber, B.; Petchenik, L.; Williams, M.; Thomas, C.; Luken, M.G.

    1994-01-01

    A 61-year-old woman underwent a translabyrinthine resection of a right intracanulicular acoustic neuroma, which had been detected in the work-up of sudden hearing loss. At the time of surgery, the tumor was roughly twice as large as indicated by the magnetic resonance scan taken only 2 months previously. The tumor eroded the vertical and transverse crests and extended well into the cerebellopontine angle. It was impossible to distinguish the facial nerve proximal to the geniculate ganglion. All visible tumor was resected, along with the facial nerve. Histological evaluation showed a highly cellular tumor, with many mitoses and areas of necrosis, meeting the criteria for malignant schwannoma. The patient has no stigmata of neurofibromatosis, and has no known relatives with that condition. This case is only the fourth reported of a malignant vestibular schwannoma. The relationships between vestibular schwannoma, neurofibromatosis, and malignancy are discussed. ImagesFigure 2Figure 3Figure 4Figure 5Figure 6 PMID:17171176

  19. Resolving quandaries: basaloid adenoid cystic carcinoma or breast cylindroma? The role of massively parallel sequencing

    PubMed Central

    Fusco, Nicola; Colombo, Pierre-Emmanuel; Martelotto, Luciano G.; De Filippo, Maria R.; Piscuoglio, Salvatore; Ng, Charlotte K.Y.; Lim, Raymond S.; Jacot, William; Vincent-Salomon, Anne; Reis-Filho, Jorge S.; Weigelt, Britta

    2015-01-01

    Aims The aims of this study were to perform a whole-exome sequencing analysis of a breast cylindroma and to investigate the role of molecular analyses in the differentiation between breast cylindroma, a benign tumor that displays MYB expression and CYLD gene mutations, and its main differential diagnosis, the breast solid-basaloid adenoid cystic carcinoma, a malignant tumor that is characterized by the presence of the MYB-NFIB fusion gene and MYB overexpression. Methods and Results A 66-year old female underwent quadrantectomy after an irregular dense shadow was discovered in the right breast at the screening mammogram. Histologically, the tumor displayed features suggestive of a solid-basaloid variant of adenoid cystic carcinoma with a differential diagnosis of cylindroma. Fluorescence in situ hybridization, reverse transcription PCR, immunohistochemistry and whole-exome sequencing revealed absence of the MYB-NFIB fusion gene, low levels of MYB protein expression and a clonal somatic CYLD splice site mutation associated with loss of heterozygosity of the wild-type allele. Conclusions The results of the histologic, immunohistochemical and molecular analyses were consistent with a diagnosis of breast cylindroma, providing a proof-of-principle that the integration of histopathologic and molecular approaches can help differentiate between a low-malignant potential and a benign breast tumor of triple-negative phenotype. PMID:25951887

  20. Giant Cell Tumor of Bone With Pseudosarcomatous Changes Leading to Premature Denosumab Therapy Interruption: A Case Report With Review of the Literature.

    PubMed

    Sanchez-Pareja, Andrea; Larousserie, Frédérique; Boudabbous, Sana; Beaulieu, Jean-Yves; Mach, Nicolas; Saiji, Essia; Rougemont, Anne-Laure

    2016-06-01

    Denosumab has shown promising results in the management of giant cell tumor of bone, a primary bone tumor with locally aggressive behaviour. We report a case of premature denosumab interruption due to radiological and clinical tumor expansion of a giant cell tumor of the distal ulna. Although denosumab is known to induce tumor regression, with progressive ossification and loss of the characteristic morphology of giant cell tumor of bone, the ulnar tumor specimen showed a moderately to highly cellular proliferation of short spindle-shaped cells, and no osteoclast-like giant cells. There were no abnormal mitotic figures. We considered the surgical specimen as a giant cell tumor of bone with partial regression after prematurely interrupted denosumab treatment. This case illustrates the diagnostic issues of an initially unfavourable evolution raising concern for malignancy, and the difficulties in histological assessment of a partially treated giant cell tumor of bone, that may mimic osteosarcoma.

  1. Management considerations for giant congenital melanocytic nevi in adults.

    PubMed

    Green, Margaret C; Mitchum, Marsha D; Marquart, Jason D; Bingham, Jonathan L

    2014-04-01

    Giant congenital melanocytic nevi (GCMN) are a rare type of melanocytic nevus that covers a large body surface, often with satellite nevi scattered on the rest of the skin. There are several complications associated with GCMN, including malignant melanoma and neurocutaneous melanosis. The management of GCMN is very complex because of the cosmetic appearance and the associated psychological distress, the risk of severe complications, and the need for long-term follow-up. We report a case of a 43-year-old active-duty female with a GCMN reporting new and symptomatic satellite lesions with atypical features on dermoscopy.

  2. [Intrathoracic giant peripheral nerve sheath tumor during Von Recklinghausen disease].

    PubMed

    Ngabou, U D; Mounguengui, D; Owono Mbouengou, J P; El Wali, A; Nguema Edzang, B; Boguikouma, J B; Tchoua, R; Aziz, N E

    2014-06-01

    We report the case of a patient aged 23, admitted for bilateral intrathoracic tumor, including a giant right. Surgery was performed by right sternothoracotomy. After 7 days, she presented an irreversible cardiac arrest. The malignant peripheral nerve sheath tumors are rare and aggressive. Their incidence is 0.001% in the general population and 0.16% in patients with neurofibromatosis type 1. These tumors are characterized by their risk of recurrence and poor prognosis. The treatment is the surgical resection. We analyze incidence, diagnosis and prognosis of these tumors.

  3. 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

  4. Envy and malignant envy.

    PubMed

    Shengold, L

    1994-10-01

    Envy begins early in psychic development and is initially characterized by destructive primal hatred. With maturation, envy becomes modified in intensity. Its primal murderous quality is attenuated, and, as object relationships develop, it becomes partly transformed into jealousy. Malignant envy is a retention of, or regression to, the original primal murderous affective mix. Clinically, one sees in malignant envy the phenomenon of the subject feeling with delusional intensity that what the envied one has is not only urgently wanted but has been stolen from the self--an intensity that is reacted to defensively by projection and delusion formation. This operates as a formidable resistance in analytic work.

  5. National trends in resection of cystic lesions of the pancreas

    PubMed Central

    Reames, Bradley N.; Scally, Christopher P.; Frankel, Timothy L.; Dimick, Justin B.; Nathan, Hari

    2016-01-01

    Background Management of cystic lesions of the pancreas (CLP) is controversial. In this study, we sought to evaluate national changes in the resection of CLP over time, to better understand the impact of evolving guidelines on CLP management. Methods We used Medicare data to examine CLP resection among patients undergoing pancreatic resection between 2001 and 2012. Patients with a diagnosis of CLP were identified and compared to patients with non-CLP indications. We then examined changes over time in patient and hospital characteristics and outcomes among patients with a CLP diagnosis. Results We identified 56,419 Medicare patients undergoing pancreatic resection, of which 2129 had a CLP diagnosis. The annual number of CLP resections, and proportion of all resections performed for CLP increased significantly during the period, from 2.1% (65/3072) resections in 2001, to 4.5% (286/6348) in 2012 (p < 0.001). The proportion of CLP resections with a malignant diagnosis did not change (15.5% in 2001–2003 vs. 13.1% in 2010–2012, p = 0.4). Overall rates of 30-day mortality decreased significantly during the period (9.6% in 2001–2003 vs. 5.5% in 2010–2012, p < 0.001). Discussion CLP resections were performed with increasing frequency in Medicare patients between 2001 and 2012, but this did not correspond to increased diagnosis of malignancy. Additional research is needed to understand the influence of recent guidelines on management of CLP. PMID:27037208

  6. On to the Ice Giants

    NASA Astrophysics Data System (ADS)

    Reh, Kim; Hofstdater, Mark; Simon, Amy; Elliott, John

    2017-04-01

    Voyager 2 mission flew by Uranus in 1986 and Neptune in 1989 resulting in stunning remote observations not previously accessible from the ground. There have been no follow-up space flight missions to examine ice giants and, as a result there are significant gaps in our understanding of planetary formation and evolution. This gap not only affects our understanding of our own solar system but also our understanding of exoplanets; the majority of planets discovered around other stars are thought to be ice giants. Ice Giants are likely to be far more abundant in our galaxy than previously thought. The U.S. 2011 Planetary Science Decadal Survey committee recognized the importance of Uranus and Neptune, and prioritized the exploration of the Ice Giants. Following from this, NASA and ESA have recently completed a study of candidate missions to Uranus and Neptune, the so-called ice giant planets. The intent was to examine what could be accomplished within the budget realities of the predictable future. This "Pre-Decadal Study," focused on opportunities for missions launching in the 2020's and early 2030's. This paper presents results from the Ice Giants study (science, architectures and technologies) and concludes that compelling and affordable missions to the Ice Giants are within our reach.

  7. Psychological interventions for cystic fibrosis.

    PubMed

    Glasscoe, C A; Quittner, A L

    2003-01-01

    As survival estimates for cystic fibrosis (CF) steadily increase long-term management has become an important focus for intervention. Psychological interventions are largely concerned with emotional and social adjustments, adherence to treatment and quality of life, however no systematic review of such interventions has been undertaken for this disease. To describe the extent and quality of effectiveness studies utilising psychological interventions for CF and whether these interventions provide significant psychosocial and physical benefits in addition to standard care. Relevant trials were identified from searches of Ovid MEDLINE, the Cochrane trial registers for CF and Depression, Anxiety and Neurosis Groups and PsychINFO; unpublished trials were located through professional networks and Listserves. Most recent search: April 2003. This review included RCTs and quasi-randomised trials. Study participants were children and adults diagnosed with CF, and their immediate family members. Psychological interventions were from a broad range of modalities and outcomes were primarily psychosocial, although physical outcomes and cost effectiveness were also considered. Two reviewers independently selected relevant trials and assessed their methodological quality. For binary and continuous outcomes a pooled estimate of treatment effect was calculated for each outcome. This review is based on the findings of eight studies, representing data from a total of 358 participants. Studies fell into four conceptually similar groups: (1) gene pre-test education counselling for relatives of those with CF (one study); (2) biofeedback, massage and music therapy to assist physiotherapy (three studies); (3) behavioural intervention to improve dietary intake in children up to 12 years (three studies); and (4) self-administration of treatments to improve quality of life in adults (one study). Interventions were largely educational or behavioural, targeted at specific treatment concerns

  8. The diagnosis of cystic fibrosis.

    PubMed

    De Boeck, Kris; Vermeulen, Francois; Dupont, Lieven

    2017-06-01

    Establishing the diagnosis of cystic fibrosis (CF) is straight forward in the majority of patients: they present with a clear clinical picture (most frequently chronic respiratory symptoms plus malabsorption), the sweat chloride value is>60mmol/L and two known disease causing CFTR mutations are identified. In less than 5% of subjects, mainly those with a milder or limited phenotype, the diagnostic process is more complex, because initial diagnostic test results are inconclusive: sweat chloride concentration in the intermediate range, less than 2 CF causing mutations identified or both. These patients should be referred to expert centers where bioassays of CFTR function like nasal potential difference measurement or intestinal current measurement can be done. Still, in some patients, despite symptoms compatible with CF and some indication of CFTR dysfunction (e.g. only intermediate sweat chloride value), diagnostic criteria are not met (e.g. only 1 CFTR mutation identified). For these subjects, the term CFTR related disorder (CFTR-RD) is used. Patients with disseminated bronchiectasis, congenital bilateral absence of the vas deferens and acute or recurrent pancreatitis may fall in this category. CF has a very wide disease spectrum and increasingly the diagnosis is being made during adult life, mainly in subjects with milder phenotypes. In many countries, nationwide CF newborn screening (NBS) has been introduced. In screen positive babies, the diagnosis of CF must be confirmed by a sweat test demonstrating a sweat chloride concentration above 60mmol/L. To achieve the benefit of NBS, every baby in whom the diagnosis of CF is confirmed must receive immediate follow-up and treatment in a CF reference center. CF NBS is not full proof: some diagnoses will be missed and in some babies the diagnosis cannot be confirmed nor ruled out with certainty. Screening algorithms that include gene sequencing will detect a high number of such babies that are screen positive with an

  9. Giant magnetofossils and hyperthermal events

    NASA Astrophysics Data System (ADS)

    Chang, Liao; Roberts, Andrew P.; Williams, Wyn; Fitz Gerald, John D.; Larrasoaña, Juan C.; Jovane, Luigi; Muxworthy, Adrian R.

    2012-10-01

    Magnetotactic bacteria biomineralize magnetic minerals with precisely controlled size, morphology, and stoichiometry. These cosmopolitan bacteria are widely observed in aquatic environments. If preserved after burial, the inorganic remains of magnetotactic bacteria act as magnetofossils that record ancient geomagnetic field variations. They also have potential to provide paleoenvironmental information. In contrast to conventional magnetofossils, giant magnetofossils (most likely produced by eukaryotic organisms) have only been reported once before from Paleocene-Eocene Thermal Maximum (PETM; 55.8 Ma) sediments on the New Jersey coastal plain. Here, using transmission electron microscopic observations, we present evidence for abundant giant magnetofossils, including previously reported elongated prisms and spindles, and new giant bullet-shaped magnetite crystals, in the Southern Ocean near Antarctica, not only during the PETM, but also shortly before and after the PETM. Moreover, we have discovered giant bullet-shaped magnetite crystals from the equatorial Indian Ocean during the Mid-Eocene Climatic Optimum (˜40 Ma). Our results indicate a more widespread geographic, environmental, and temporal distribution of giant magnetofossils in the geological record with a link to "hyperthermal" events. Enhanced global weathering during hyperthermals, and expanded suboxic diagenetic environments, probably provided more bioavailable iron that enabled biomineralization of giant magnetofossils. Our micromagnetic modelling indicates the presence of magnetic multi-domain (i.e., not ideal for navigation) and single domain (i.e., ideal for navigation) structures in the giant magnetite particles depending on their size, morphology and spatial arrangement. Different giant magnetite crystal morphologies appear to have had different biological functions, including magnetotaxis and other non-navigational purposes. Our observations suggest that hyperthermals provided ideal conditions for

  10. [Concurrent tumors in a patient with giant hydronephrosis].

    PubMed

    Pereira Arias, José Gregorio; Ullate Jaime, Vicente; Gutiérrez Díez, José Maria; Ateca Díaz-Obregón, Ricardo; Ramírez Rodríguez, María Mar; Pereda Martínez, Esther; Berreteaga Gallastegui, José Ramón

    2006-01-01

    Giant hydronephrosis, defined as the presence of a liquid volume over 1000 ml within the urinary collector system, is a rare clinical entity, the diagnosis of which is an excellent exercise because it lacks of a defined clinical presentation. We report the case of a 66-year-old male under study for a suspicious digestive tumor due to a long lasting clinical picture with severe cachexia, asthenia, anemia, constipation, and abdominal mass. Radiological tests showed a great right hydronephrosis secondary to a culculus in the ureteral-pelvic junction. Simple nephrectomy was performed, evacuating 7800 ml of serous-hematic liquid from the pyelocalicial system. A histological diagnosis revealed the presence of multiple foci of transitional cell carcinoma and renal cell carcinoma associated. We review the diagnostic and therapeutic features in the literature. Giant hydronephrosis represents a diagnostic dilemma. It may present as an asymptomatic process, with clinical features of abdominal organs compression (bowel or urinary obstruction) or simulate abdominal tumors, massive ascites, or cystic retroperitoneal lesions. Simple nephrectomy is the treatment of choice in most cases, due to the advanced deterioration of the renal unit. Nevertheless, in some cases, in compromised patients, percutaneous drainage may be necessary as previous or definitive treatment to avoid changes in the hemodynamic balance secondary to the sudden abdominal decompression.

  11. Malignant Pigmented Villonodular Synovitis-A Rare Entity.

    PubMed

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

    2014-01-01

    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. 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. 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.

  12. 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

  13. Craniofacial morphology in children with cystic fibrosis.

    PubMed

    Hellsing, E; Brattström, V; Strandvik, B

    1992-04-01

    Cystic fibrosis (CF) is a hereditary metabolic disorder with clinical symptoms of abnormal mucus production. This blocks the airways, gives pancreatic insufficiency, and increases sweat electrolytes. The progressive respiratory disease often leads to respiratory insufficiency and cor pulmonale. The aim of the present investigation was to examine the facial morphology in children with cystic fibrosis. The sample comprised 11 children with cystic fibrosis, who were divided in two groups, one with gastrointestinal disorders and the other with predominantly respiratory insufficiency. Eleven healthy children with normal occlusions were selected as controls. Lateral skull radiographs obtained in natural head posture were digitized, and linear and angular variables for the different groups calculated and compared statistically. The cystic fibrosis group showed open bite, decreased posterior facial height, increased mandibular and craniocervical inclination. Additionally, within the CF-group, the children with respiratory insufficiency differed more from the controls than the children with gastrointestinal disorders. Despite the small number of subjects, the facial morphology of the CF children showed a similar pattern to that of children with nasal respiratory obstruction due to enlarged adenoids or tonsils.

  14. 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

  15. Ivacaftor for patients with cystic fibrosis.

    PubMed

    Wainwright, Claire E

    2014-10-01

    Ivacaftor is an oral bioavailable potentiator of the cystic fibrosis transmembrane conductance regulator protein. It is the first therapeutic agent that has been registered for clinical use which targets the basic defect in people with cystic fibrosis who carry a G551D mutation or other rarer specific gating mutations. Clinical trials have shown consistent and impressive clinical benefit that appears to be sustained over time in people with cystic fibrosis who carry a G551D mutation and similar benefits have been seen in those who carry rarer gating mutations. Ivacaftor is orally administered twice daily with a dose that does not vary between children aged 6 years through to adult life in patients with G551D. It appears to be well tolerated although there are potential interactions with drugs that are metabolised through CYPP450 CYP3A. Ivacaftor is also currently being trialled in combination with correctors for patients with the most common mutation of cystic fibrosis transmembrane conductance regulator the F508del mutation.

  16. Cystic fibrosis year in review 2016.

    PubMed

    Savant, Adrienne P; McColley, Susanna A

    2017-08-01

    In this article, we highlight cystic fibrosis (CF) research and case reports published in Pediatric Pulmonology during 2016. We also include articles from a variety of journals that are thematically related to these articles, or are of special interest to clinicians. © 2017 Wiley Periodicals, Inc.

  17. Spontaneous hepatocellular adenoma with marked cystic degeneration.

    PubMed

    Kobayashi, M; Enzan, H; Araki, K; Kawai, S; Kitagawa, N

    1999-01-01

    We report a case of spontaneous hepatocellular adenoma with marked cystic degeneration in the non-cirrhotic liver. A 36 year-old Japanese woman with neither history of liver diseases nor use of oral contraceptives and steroids, complained of a 6 kg weight loss over 6 months. Barium meal study revealed an extramural compression along the fornix of the stomach. Abdominal ultrasonography (UC) and computed tomography (CT) demonstrated a mass in the left lateral segment of the liver that measured 11.6x9.5 cm with cystic lesions. Laboratory data on admission showed no significant findings. Celiac angiography revealed a hyper-vascular mass. Surgical exploration revealed a soft mass arising and protruding from the left lateral segment of the liver. Partial resection of the left lateral segment was performed. Histologically, the tumor was surrounded by a thin fibrous pseudocapsule. The neoplastic cells resembling normal hepatocytes around the tumor were large, pale and arranged in thick, irregular cords. Neither mitotic figures nor foci of dysplasia were present. The central portions of the tumor showed marked cystic degeneration. The tumor was histologically diagnosed as hepatocellular adenoma (HCA). HCA with cystic degeneration has been rarely reported.

  18. Exercise is medicine in cystic fibrosis.

    PubMed

    Wheatley, Courtney M; Wilkins, Brad W; Snyder, Eric M

    2011-07-01

    Exercise activates adrenergic and purinergic pathways that regulate activity of ion channels on airway epithelia cells and sweat glands. Therefore, we hypothesize that exercise is not only an important therapy for cystic fibrosis (CF) patients by facilitating systemic improvements but, more importantly, that exercise can improve the pathophysiological ion dysregulation at a cellular level, thereby enhancing quality of life in CF.

  19. Cystic echinococcosis: Future perspectives of molecular epidemiology

    USDA-ARS?s Scientific Manuscript database

    Human cystic echinococcosis (CE) has been conceived to be caused predominantly by Echinococcus granulosus sensu stricto (the dog-sheep strain). Recent molecular approaches on CE, however, have revealed that human cases are also commonly caused by another species, Echinococcus canadensis. All indices...

  20. Nutritional assessment in children with cystic fibrosis

    USDA-ARS?s Scientific Manuscript database

    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...

  1. Zinc supplementation in children with cystic fibrosis

    USDA-ARS?s Scientific Manuscript database

    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...

  2. 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.

  3. Dental treatment for people with cystic fibrosis.

    PubMed

    Harrington, N; Barry, P J; Barry, S M

    2016-06-01

    To describe the nature and consequences of the multi-system genetic condition cystic fibrosis with a view to ensuring optimal dental treatment planning for these patients. A literature search was conducted to identify the key medical and dental manifestations of cystic fibrosis. These findings are discussed and utilised to create recommendations for treatment planning in patients with cystic fibrosis for the practising dental practitioner. Cystic fibrosis is a complex, lethal, multisystem autosomal recessive disorder resulting from mutations on chromosome 7 which result in dysfunction of an ion channel that sits on epithelial surfaces. Respiratory disease remains the leading cause of mortality. Survival has greatly increased in recent decades secondary to improved treatment and specialist care. Specific dental manifestations of the disease may result from the condition itself or complications of treatment. Modification of patient management may be necessary to provide optimum patient care. The pathophysiology and clinical manifestations are relevant to practicing dental practitioners and inform recommendations to be utilised to ensure optimal treatment planning for these patients.

  4. [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.

  5. Bilateral Adenoid Cystic Carcinoma of the Orbit.

    PubMed

    Branson, Sara V; McClintic, Elysa; Yeatts, R Patrick

    A 41-year-old woman with adenoid cystic carcinoma of the left lacrimal gland underwent extended left orbital exenteration and postoperative external beam radiation therapy. She presented 1 year postoperatively with contralateral right orbital involvement. The authors report a rare case of adenocystic carcinoma of the lacrimal gland with bilateral orbital involvement.

  6. A giant Ordovician anomalocaridid.

    PubMed

    Van Roy, Peter; Briggs, Derek E G

    2011-05-26

    Anomalocaridids, giant lightly sclerotized invertebrate predators, occur in a number of exceptionally preserved early and middle Cambrian (542-501 million years ago) biotas and have come to symbolize the unfamiliar morphologies displayed by stem organisms in faunas of the Burgess Shale type. They are characterized by a pair of anterior, segmented appendages, a circlet of plates around the mouth, and an elongate segmented trunk lacking true tergites with a pair of flexible lateral lobes per segment. Disarticulated body parts, such as the anterior appendages and oral circlet, had been assigned to a range of taxonomic groups--but the discovery of complete specimens from the middle Cambrian Burgess Shale showed that these disparate elements all belong to a single kind of animal. Phylogenetic analyses support a position of anomalocaridids in the arthropod stem, as a sister group to the euarthropods. The anomalocaridids were the largest animals in Cambrian communities. The youngest unequivocal examples occur in the middle Cambrian Marjum Formation of Utah but an arthropod retaining some anomalocaridid characteristics is present in the Devonian of Germany. Here we report the post-Cambrian occurrence of anomalocaridids, from the Early Ordovician (488-472 million years ago) Fezouata Biota in southeastern Morocco, including specimens larger than any in Cambrian biotas. These giant animals were an important element of some marine communities for about 30 million years longer than previously realized. The Moroccan specimens confirm the presence of a dorsal array of flexible blades attached to a transverse rachis on the trunk segments; these blades probably functioned as gills.

  7. Giant Hedge-Hogs: Spikes on Giant Gravitons

    SciTech Connect

    Sadri, D

    2004-01-28

    We consider giant gravitons on the maximally supersymmetric plane-wave background of type IIB string theory. Fixing the light-cone gauge, we work out the low energy effective light-cone Hamiltonian of the three-sphere giant graviton. At first order, this is a U(1) gauge theory on R x S{sup 3}. We place sources in this effective gauge theory. Although non-vanishing net electric charge configurations are disallowed by Gauss' law, electric dipoles can be formed. From the string theory point of view these dipoles can be understood as open strings piercing the three-sphere, generalizing the usual BIons to the giant gravitons, BIGGons. Our results can be used to give a two dimensional (worldsheet) description of giant gravitons, similar to Polchinski's description for the usual D-branes, in agreement with the discussions of hep-th/0204196.

  8. Giant resonances: Progress, new directions, new challenges

    SciTech Connect

    Bertrand, J.R.; Beene, J.R.

    1989-01-01

    A review of some recent developments in the field of giant multipole resonances is presented. Particular emphasis is placed on directions that the authors feel will be followed in this field during the next several years. In particular, the use of high-energy heavy ions to excite the giant resonances is shown to provide exciting new capabilities for giant resonance studies. Among subjects covered are: Coulomb excitation of giant resonances, photon decay of giant resonances, the recent controversy over the identity of the giant monopole resonance, the most recent value for incompressibility of nuclear matter from analysis of giant monopole data, the isospin character of the 63 A/sup /minus/1/3/ GQR, agreement between (e,e/prime/) and (hadron, hadron/prime/) excitation of the giant quadrupole resonance, prospects for multiphonon giant resonance observation, and isolation of the isovector giant quadrupole resonance. 55 refs., 23 figs., 4 tabs.

  9. Osteogenic sarcoma. Malignant fibrous histiocytoma subtype.

    PubMed

    Ballance, W A; Mendelsohn, G; Carter, J R; Abdul-Karim, F W; Jacobs, G; Makley, J T

    1988-08-15

    A distinctly different entity from the now well-delineated malignant fibrous histiocytoma (MFH) of bone is the MFH histopathologic subtype of osteogenic sarcoma. Although uncommon, recently the authors have encountered six cases of this neoplasm, in each of which the soft tissue component was devoid of bone elements and was microscopically indistinguishable from MFH of bone or soft tissue. Neoplastic osteoid and woven bone were present in the osseous component of each tumor, however. Radiologically, the lesions generally were osteoblastic but focally osteolytic with features typical of osteogenic sarcoma. Pain was the most common presenting symptom. There was no age or sex predilection. Immunocytochemical staining showed strong positivity with alpha-1-antichymotrypsin within malignant bizarre giant cells and occasional neoplastic osteoblasts in five cases. The biological behavior followed a very aggressive course. Four of the six patients developed pulmonary metastases 6 to 12 months after initial surgery; one patient presented initially with pulmonary metastases. Adequate tumor sampling as well as optimal correlation with clinical and radiographic information are required to distinguish the MFH subtype of osteogenic sarcoma from MFH of bone, both being high-grade neoplasms, however.

  10. Giant fibroepithelial stromal polyp of the vulva: largest case reported

    PubMed Central

    2013-01-01

    Background Fibroepithelial stromal polyps are site-specific mesenchymal lesions that are commonly found in the vulvovaginal region in premenopausal females. These polyps usually are less than 5 cm in diameter and are most commonly identified during routine gynecological examination. Although the stromal polyp is benign, its differential diagnosis includes some malignant vulva lesions making it critical to ensure that an accurate pathologic diagnosis is made. Case We present a case of a 21 year old female with a giant fibroepithelial stromal polyp of the vulva. Upon review of the literature this is the largest reported fibroepithelial stromal polyp to date. Conclusion Fibroepithelial stromal polyps can grow as large as 390 grams and can be 18.5-cm in diameter. Microscopic evaluation of the polyp is critical in the exclusion of malignancy with this diagnosis. PMID:23842282

  11. 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

  12. Trichoepithelioma Arising in an Ovarian Mature Cystic Teratoma

    PubMed Central

    Nakayama, Satoru; Muto, Haruka; Shimizu, Shinichi; Otsuki, Yoshiro; Adachi, Hiroshi; Murakoshi, Takeshi

    2016-01-01

    Here, we report an extremely rare case of trichoepithelioma (TE)—a benign epithelial tumor originating from the outer root sheath of a hair follicle—arising in an ovarian mature cystic teratoma (MCT) with fluorodeoxyglucose-positron emission tomography (FDG-PET) findings. A 48-year-old Japanese woman presented to our hospital for her annual follow-up of adenomyosis. Ultrasonography and magnetic resonance imaging revealed a left ovarian tumor with irregular-shaped septum, which was suspicious of malignancy. However, tumor marker levels were within normal range. On FDG-PET, the maximum standardized uptake value (SUVmax) of the tumor was 2.9. Laparotomy with left salpingooophorectomy was performed. Pathologic examination revealed the probability of TE, rather than basal cell carcinoma (BCC), arising in an ovarian MCT. After five years of follow-up, the patient had no sign of recurrence. The FDG-PET SUVmax was low in TE, as with other benign tumor. However, future investigation is needed to evaluate the findings of FDG-PET imaging in TE cases. PMID:28127486

  13. Adenoid cystic carcinoma: emerging role of translocations and gene fusions

    PubMed Central

    Wysocki, Piotr T.; Izumchenko, Evgeny; Meir, Juliet; Ha, Patrick K.; Sidransky, David; Brait, Mariana

    2016-01-01

    Adenoid cystic carcinoma (ACC), the second most common salivary gland malignancy, is notorious for poor prognosis, which reflects the propensity of ACC to progress to clinically advanced metastatic disease. Due to high long-term mortality and lack of effective systemic treatment, the slow-growing but aggressive ACC poses a particular challenge in head and neck oncology. Despite the advancements in cancer genomics, up until recently relatively few genetic alterations critical to the ACC development have been recognized. Although the specific chromosomal translocations resulting in MYB-NFIB fusions provide insight into the ACC pathogenesis and represent attractive diagnostic and therapeutic targets, their clinical significance is unclear, and a substantial subset of ACCs do not harbor the MYB-NFIB translocation. Strategies based on detection of newly described genetic events (such as MYB activating super-enhancer translocations and alterations affecting another member of MYB transcription factor family-MYBL1) offer new hope for improved risk assessment, therapeutic intervention and tumor surveillance. However, the impact of these approaches is still limited by an incomplete understanding of the ACC biology, and the manner by which these alterations initiate and drive ACC remains to be delineated. This manuscript summarizes the current status of gene fusions and other driver genetic alterations in ACC pathogenesis and discusses new therapeutic strategies stemming from the current research. PMID:27533466

  14. Early malignant syphilis*

    PubMed Central

    Ortigosa, Yara Martins; Bendazzoli, Paulo Salomão; Barbosa, Angela Marques; Ortigosa, Luciena Cegatto Martins

    2016-01-01

    Early malignant syphilis is a rare and severe variant of secondary syphilis. It is clinically characterized by lesions, which can suppurate and be accompanied by systemic symptoms such as high fever, asthenia, myalgia, and torpor state. We report a diabetic patient with characteristic features of the disease showing favorable evolution of the lesions after appropriate treatment. PMID:28300925

  15. Malignant tumors of childhood

    SciTech Connect

    Brooks, B.J.

    1986-01-01

    This book contains 34 papers about malignant tumors. some of the titles are: Invasive Cogenital Mesoblastic Nephroma, Leukemia Update, Unusual Perinatal Neoplasms, Lymphoma Update, Gonadal Germ Cell Tumors in Children, Nutritional Status and Cancer of Childhood, and Chemotherapy of Brain tumors in Children.

  16. Giant Perineal Condyloma Acuminatum (Buschke-Lowenstein Tumour): A Case Report.

    PubMed

    Badiu, D C; Manea, C A; Mandu, M; Chiperi, V; Marin, I E; Mehedintu, C; Popa, C C; David, O I; Bratila, E; Grigorean, V T

    2016-01-01

    The giant condyloma acuminatum, also known as Buschke- Lowenstein tumor (BLT), is a rare sexually-transmitted disease. Moreover, the condyloma acuminatum produced by Human Papilloma Virus (HPV) infection becomes one the most common sexually-transmitted infection which affects the perineal region. Under such situations, the first therapeutic option is surgical excision. The objective of this article is to present a case in which the tumor has reached giant dimensions and also to stress the importance of an in toto resection, taking into account the high rate of recurrence and the significant potential of malignant transformation of BLT. Celsius.

  17. Cystic Renal Disease in the Domestic Ferret

    PubMed Central

    Jackson, Courtnye N; Rogers, Arlin B; Maurer, Kirk J; Lofgren, Jennifer LS; Fox, James G; Marini, Robert P

    2008-01-01

    Cystic renal diseases in domestic ferrets are a common anecdotal finding but have received scant systematic assessment. We performed a 17-y, case-control retrospective analysis of the medical records of 97 ferrets housed at our institution between 1987 and 2004, to determine the prevalence and morphotypes of cystic renal diseases in this species. Histologic sections stained with hematoxylin and eosin, Masson trichrome, or periodic acid–Schiff were evaluated by a comparative pathologist, and statistical analysis of hematologic and serum chemistry values was correlated with morphologic diagnosis. Of the 97 available records, 43 were eliminated due to lack of accompanying tissues. Of the 54 remaining cases, 37 (69% prevalence) had documented renal cysts, and 14 of the 54 ferrets (26%) had primary polycystic disease consisting of either polycystic kidney disease affecting renal tubules or, more commonly, glomerulocystic kidney disease. Secondary polycystic lesions were identified in 11 ferrets (20%), and 12 ferrets (22%) exhibited focal or isolated tubular cysts only as an incidental necropsy finding. Ferrets with secondary renal cysts associated with other developmental anomalies, mesangial glomerulopathy, or end-stage kidney disease had hyperphosphatemia and elevated BUN in comparison with those with primary cystic disease and elevated BUN compared with those without renal lesions. Although reflecting institutional bias, these results implicate primary and secondary cystic renal diseases as highly prevalent and underreported in the domestic ferret. In addition to the clinical implications for ferrets as research subjects and pets, these findings suggest a potential value for ferrets as a model of human cystic renal diseases. PMID:18524174

  18. Investigation of Burkholderia cepacia nosocomial outbreak with high fatality in patients suffering from diseases other than cystic fibrosis.

    PubMed

    Shehabi, Asem A; Abu-Al-Soud, Waleed; Mahafzah, Azmi; Khuri-Bulos, Najwa; Abu Khader, Ilham; Ouis, Ibn-Sina; Wadström, Torkel

    2004-01-01

    Over a 1-y period, 26 inpatients at the Jordan University Hospital in Amman were detected with bacteraemia (23 cases) or respiratory tract colonized with B. cepacia (3 cases). A combination of genetic identification and molecular typing has proved that all cases were caused by a single epidemic strain of B. cepacia genomovar IIIa. Nosocomial infections could be documented in 21/26 (81%) patients, mostly with severe underlying or malignant diseases other than cystic fibrosis, but the source of infection was undetected. The overall mortality related to infection with B. cepacia was 42%. All B. cepacia isolates were resistant to ampicillin, amikacin, carbenicillin and gentamicin; and mostly susceptible to piperacillin, chloramphenicol, cotri-moxazole, tetracycline, ceftazidime, and tazocin (62-88%). This study demonstrates the nosocomial and high fatality of B. cepacia genomovar IIIa in Jordanian patients suffering from diseases other than cystic fibrosis.

  19. Theories of Giant Planet Formation

    NASA Technical Reports Server (NTRS)

    Lissauer, Jack J.; Young, Richard E. (Technical Monitor)

    1998-01-01

    An overview of current theories of planetary formation, with emphasis on giant planets, is presented. The most detailed models are based upon observations of our own Solar System and of young stars and their environments. While these models predict that rocky planets should form around most single stars, the frequency of formation of gas giant planets is more difficult to predict theoretically. Terrestrial planets are believed to grow via pairwise accretion until the spacing of planetary orbits becomes large enough that the configuration is stable for the age of the system. Giant planets begin their growth as do terrestrial planets, but they become massive enough that they are able to accumulate substantial amounts of gas before the protoplanetary disk dissipates. Most models for extrasolar giant planets suggest that they formed as did Jupiter and Saturn (in nearly circular orbits, far enough from the star that ice could), and subsequently migrated to their current positions, although some models suggest in situ formation.

  20. Lichens On Galapagos Giant Tortoises.

    PubMed

    Hendrickson, J R; Weber, W A

    1964-06-19

    The association of Physcia picta with the giant Galdpagos tortoise is believed to be the first reported occurrence of lichens on land animals. The habitat is restricted to specific sites on the carapace of male tortoises.

  1. 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.

  2. What Is Giant Cell Arteritis?

    MedlinePlus

    ... 01, 2017 Giant cell arteritis (GCA) is an inflammation (swelling) of the arteries, which are the blood ... help nourish your eyes, reduced blood flow can cause sudden, painless vision loss. This condition is called ...

  3. 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.

  4. Giant sacrolumbar meningioma. Case report.

    PubMed

    Feldenzer, J A; McGillicuddy, J E; Hopkins, J W

    1990-06-01

    A case of giant sacral meningioma with presacral and lumbar extension is presented. The difficulties in diagnosis and management are emphasized including the staged multidisciplinary surgical approaches and preoperative tumor embolization.

  5. 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.

  6. Theories of Giant Planet Formation

    NASA Technical Reports Server (NTRS)

    Lissauer, Jack J.; Young, Richard E. (Technical Monitor)

    1998-01-01

    An overview of current theories of planetary formation, with emphasis on giant planets, is presented. The most detailed models are based upon observations of our own Solar System and of young stars and their environments. While these models predict that rocky planets should form around most single stars, the frequency of formation of gas giant planets is more difficult to predict theoretically. Terrestrial planets are believed to grow via pairwise accretion until the spacing of planetary orbits becomes large enough that the configuration is stable for the age of the system. Giant planets begin their growth as do terrestrial planets, but they become massive enough that they are able to accumulate substantial amounts of gas before the protoplanetary disk dissipates. Most models for extrasolar giant planets suggest that they formed as did Jupiter and Saturn (in nearly circular orbits, far enough from the star that ice could), and subsequently migrated to their current positions, although some models suggest in situ formation.

  7. 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.

  8. Giant retroperitoneal sarcomas.

    PubMed

    Ianoşi, G; Neagoe, Daniela; Buteică, Elena; Ianoşi, Simona; Drighiciu, Corina; Stănoiu, B; Burada, F; Mercuţ, D

    2007-01-01

    Retroperitoneal sarcomas are rare malignant tumors, which are developing from mesenchymal stem cells residing in muscle, fat, and connective tissues. Underlying the rarity of this kind of tumors in general population, the aim of this paper is to present three cases of retroperitoneal sarcomas operated in Surgical Department of Military Hospital of Craiova (a retroperitoneal liposarcoma, a dedifferentiated liposarcoma and a malignant fibrous histiocytoma). From clinical point of view, we note the poverty of symptoms and non-specificity of these and a great tolerability of retroperitoneal space that offers the possibility for a great development of the tumor. CT-scan and MRI are the best investigations for diagnosis but surgical exploration is the best way for a good evaluation of these tumors. From histological point of view, we try to present new features about these kinds of tumors in order to classify them. Results of surgery correlated with complementary therapies were good without per-operative mortality or postoperative morbidity but we noted a recidive of one tumor (with different histological pattern) 21 months after the surgical intervention. The rarity of retroperitoneal sarcomas, combined with the vast array of histologic subtypes, has complicated our understanding of these tumors and impeded the development of effective therapies.

  9. Giant cell arteritis: a review

    PubMed Central

    Patil, Pravin; Karia, Niral; Jain, Shaifali; Dasgupta, Bhaskar

    2013-01-01

    Giant cell arteritis is the most common vasculitis in Caucasians. Acute visual loss in one or both eyes is by far the most feared and irreversible complication of giant cell arteritis. This article reviews recent guidelines on early recognition of systemic, cranial, and ophthalmic manifestations, and current management and diagnostic strategies and advances in imaging. We share our experience of the fast track pathway and imaging in associated disorders, such as large-vessel vasculitis. PMID:28539785

  10. Novel CT-guided biopsy of isolated perineural spread of adenoid cystic carcinoma along the trigeminal nerve masquerading as chronic trigeminal neuropathy.

    PubMed

    Yong, Xian Zhang Eric; Dillon, Jonathan; Smith, Paul; Salinas-La Rosa, Cesar; Jhamb, Ashu

    2017-02-01

    The differential diagnoses for chronic peripheral neuropathy are broad and diagnosing a cause can be challenging. We present a case of isolated perineural spread of adenoid cystic carcinoma to the trigeminal nerve involving skull base foramina and Meckel's cave in the setting of chronic trigeminal neuropathy and no known prior malignancy. Computed tomography-guided core (CT) needle biopsy was needed to arrive at a diagnosis and a novel approach was required to obtain tissue from the trigeminal nerve lesion at foramen ovale.

  11. Giants in the Local Region

    NASA Astrophysics Data System (ADS)

    Luck, R. Earle; Heiter, Ulrike

    2007-06-01

    We present parameter and abundance data for a sample of 298 nearby giants. The spectroscopic data for this work have a resolution of R~60,000, S/N>150, and spectral coverage from 475 to 685 nm. Overall trends in the Z>10 abundances are dominated by Galactic chemical evolution, while the light-element abundances are influenced by stellar evolution, as well as Galactic evolution. We find several super-Li stars in our sample and confirm that Li abundances in the first giant branch are related to mixing depths. Once astration of lithium on the main sequence along with the overall range of main-sequence lithium abundances are taken into account, the lithium abundances of the giants are not dramatically at odds with the predictions of standard stellar evolution. We find the giants to be carbon-diluted in accord with standard stellar evolution and that the carbon and oxygen abundances determined for the local giants are consistent with those found in local field dwarfs. We find that there is evidence for systematic carbon variations in the red giant clump in the sense that the blue side of the clump is carbon-poor (more diluted) than the red side.

  12. Kuiper Prize: Giant Planet Atmospheres

    NASA Astrophysics Data System (ADS)

    Ingersoll, Andrew P.

    2007-10-01

    The study of giant planet atmospheres is near and dear to me, for several reasons. First, the giant planets are photogenic; the colored clouds are great tracers, and one can make fantastic movies of the atmosphere in motion. Second, the giant planets challenge us with storms that last for hundreds of years and winds that blow faster the farther you go from the sun. Third, they remind us of Earth with their hurricanes, auroras, and lightning, but they also are the link to the 200 giant planets that have been discovered around other stars. This talk will cover the past, present, and future (one hopes) of giant planet research. I will review the surprises of the Voyager and Galileo eras, and will discuss what we are learning now from the Cassini orbiter. I will review the prospects for answering the outstanding questions like: Where's the water? What is providing the colors of the clouds? How deep do the features extend? Where do the winds get their energy? What is the role of the magnetic field? Finally, I will briefly discuss how extrasolar giant planets compare with objects in our own solar system.

  13. Physical exercise training for cystic fibrosis.

    PubMed

    Radtke, Thomas; Nolan, Sarah J; Hebestreit, Helge; Kriemler, Susi

    2015-06-28

    Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of previously published reviews. To determine the effects of physical exercise training compared to no training on aerobic exercise capacity, forced expiratory volume in one second, health-related quality of life and other patient-relevant (secondary) outcomes in cystic fibrosis. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 10 March 2015. All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and duration with conventional care in people with cystic fibrosis. Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. Of the 48 studies identified, 13 studies which included 402 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; one study was in adults, six were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and nine studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows limited evidence from both short- and long-term studies that in people with cystic fibrosis aerobic or anaerobic physical exercise training or a combination of both has a positive effect on aerobic exercise capacity, pulmonary function and health-related quality of life. Although improvements are not consistent between studies and ranged

  14. Multidetector computed tomography analysis of benign and malignant nodules in patients with chronic lymphocytic thyroiditis.

    PubMed

    Zhu, Caisong; Liu, Wei; Yang, Jun; Yang, Jing; Shao, Kangwei; Yuan, Lixin; Chen, Hairong; Lu, Wei; Zhu, Ying

    2016-07-01

    The aim of the present study was to compare the multidetector computed tomography (MDCT) features of benign and malignant nodules in patients with chronic lymphocytic thyroiditis (CLT). MDCT findings, including the size, solid percentage, calcification, margin, capsule, anteroposterior-transverse diameter ratio as well as the mode and the degree of enhancement of 137 thyroid nodules in 127 CLT cases were retrospectively analyzed. Furthermore, the correlation between MDCT findings and pathological results combined with the CT perfusion imaging was analyzed for the differences between benign and malignant nodules. A total of 77.5% (31/40) of malignant nodules were completely solid, and 33% (32/97) of benign nodules were predominantly cystic. Compared with the benign nodules, micro-calcification and internal calcification were more frequently observed in the malignant nodules (P<0.05). MDCT features such as ill-defined margin, absence of capsule or incomplete capsule or homogeneous enhancement were more likely to be present in the malignant nodules (P<0.05). Nevertheless, no significant difference was observed in the enhancement degree at arterial or venous phase between benign and malignant nodules (P>0.05). MDCT features are useful in differentiating the benign and malignant nodules in CLT patients, and it may be essential for a radiologist to review the MDCT characteristics of nodules in the clinical practice.

  15. Malignant otitis externa.

    PubMed

    Karaman, Emin; Yilmaz, Mehmet; Ibrahimov, Metin; Haciyev, Yusuf; Enver, Ozgün

    2012-11-01

    Malignant external otitis is a severe infection of the external auditory canal and skull base, which most often affects elderly patients with diabetes mellitus. This disease is still a serious disease associated with cranial nerve complications and high morbidity-mortality rate. Malignant otitis externa requires urgent diagnosis and treatment. The most effective treatment is to control the diabetes and to fight infection with the proper antibiotic and debridement necrotic tissue; sometimes, aggressive surgical management is done. We present our 5-year institutional experience in the management of this disease. The aim of this study was to present our experience with the management of malignant otitis externa. All patients' records with malignant otitis externa during the last 5 years (2007-2012) were retrieved and reviewed. Diabetes mellitus profile, erythrocyte sedimentation rate, ear swab for culture and sensitivity, computed tomography, and scintigraphy using technetium 99 and gallium 67 were investigated for all patients. During the last 5 years (2007-2012), 10 patients with the diagnosis of malignant otitis externa were admitted to our clinic for investigation and treatment. There were 7 men and 3 women, all between 64 and 83 years of age, with severe persistent otalgia, purulent otorrhea, granulation tissue in the external auditory canal, and diffuse external otitis, and there were 4 patients with facial nerve palsy. Nine patients were confirmed to have a diabetes, and 4 of these 9 cases just had chronic renal failure and underwent dialysis; the remainder 1 case had no diabetes mellitus, but with chronic renal failure on dialysis. Ear swabs for culture and sensitivity usually revealed Pseudomonas aeruginosa. Local debridement and local and systemic antibiotic treatment were sufficient to control the disease. Facial nerve decompression was done in facial paralysis. Hyperbaric oxygen therapy was performed in facial nervy palsy cases. Malignant otitis externa is

  16. The Giant Magnetocaloric Effect

    NASA Astrophysics Data System (ADS)

    Pecharsky, Vitalij K.

    1998-03-01

    Since the discovery of the magnetocaloric effect in pure iron by E.Warburg in 1881, it has been measured experimentally on many magnetic metals and compounds. The majority of the materials studied order magnetically undergoing a second order phase transformation. The magnetocaloric effect, typically peaking near the Curie or the Néel temperature, generally ranges from 0.5 to 2 K (in terms of adiabatic temperature change) or at 1 to 4 J/kg K (in terms of isothermal magnetic entropy change) per 1 T magnetic field change. The giant magnetocaloric effect recently discovered in Gd_5(Si_xGe_1-x)4 alloys, where x <= 0.5, is associated with a first order magnetic phase transition and it reaches values of 3 to 4 K and 6 to 10 J/kg K per 1 T field change, respectively. The refrigerant capacity, which is the measure of how much heat can be transferred from a cold to a hot reservoir in one ideal thermodynamic cycle, is larger than that of the best second order phase transition materials by 25 to 100%. When the Gd_5(Si_xGe_1-x)4 alloys are compared with other known materials, which show first order magnetic phase transition, such as Dy, Ho, Er, HoCo_2, NdMn_2Si_2, Fe_0.49Rh_0.51, and (Hf_0.83Ta_0.17)Fe_2+x, only Fe_0.49Rh_0.51 has comparable magnetocaloric properties. However, the first order magnetic phase transition in Fe_0.49Rh_0.51 is irreversible, and the magnetocaloric effect disappears after one magnetizing/demagnetizing cycle. A study of the crystal structure, thermodynamics, and magnetism of the Gd_5(Si_xGe_1-x)4 alloys, where 0 <= x <= 1 allowed us to obtain a qualitative understanding of the basic relations between the composition, the crystal structure, and the change in thermodynamics and magnetocaloric properties, which occur in the Gd_5(Si_xGe_1-x)4 system, and which brings about the giant magnetocaloric effect when x <= 0.5.

  17. Polyomavirus (BK)-associated pleomorphic giant cell carcinoma of the urinary bladder: a case report.

    PubMed

    Alexiev, Borislav A; Papadimitriou, John C; Chai, Toby C; Ramos, Emilio; Staats, Paul N; Drachenberg, Cinthia B

    2013-04-01

    This report describes the morphological features of a pleomorphic giant cell carcinoma with focal trophoblastic differentiation of the urinary bladder in a male, 12 years post living related donor renal transplant. The voided urine cytology demonstrated rare decoy cells admixed with markedly atypical urothelial cell clusters, papillae and giant cells. Cystoprostatectomy demonstrated a nodular mass involving the trigone and right lateral-posterior wall, adjacent to the ureteral orifice. Hematoxylin-eosin stained sections showed two synchronous malignancies: (a) pleomorphic giant cell carcinoma with focal trophoblastic differentiation of the urinary bladder, metastatic to the omentum and (b) prostatic adenocarcinoma, Gleason score 3+4=7, involving the right prostate lobe. Strong diffuse expression of polyomavirus large T antigen was demonstrated in the primary and metastatic pleomorphic giant cell carcinoma, supporting a possible role for polyomavirus (BK) in the oncogenetic pathway. The prostatic adenocarcinoma was negative for polyomavirus large T antigen. Our findings of p63, CK7 and CK903 expression in pleomorphic giant cell carcinoma suggest that the tumor is of urothelial derivation. This is the first report describing the morphological features of urinary bladder pleomorphic giant cell carcinoma with trophoblastic differentiation, positive for polyomavirus large T antigen, arising in the background of BKV reactivation.

  18. Omega-3 fatty acids for cystic fibrosis.

    PubMed

    Oliver, Colleen; Jahnke, Nikki

    2011-08-10

    Studies suggest that a diet rich in omega-3 essential fatty acids may have beneficial anti-inflammatory effects for chronic conditions such as cystic fibrosis. To determine whether there is evidence that omega-3 polyunsaturated fatty acid supplementation reduces morbidity and mortality and to identify any adverse events associated with supplementation. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Authors and persons interested in the subject of the review were contacted.Date of last search: 10 March 2011. Randomised controlled trials in people with cystic fibrosis comparing omega-3 fatty acid supplements with placebo. Two authors independently selected studies for inclusion, extracted data and assessed the risk of bias of the studies. The searches identified 13 studies; four studies with 91 participants were included. Two studies compared omega-3 fatty acids to olive oil for six weeks. One study compared a liquid dietary supplement containing omega-3 fatty acids to one without for six months. One study compared omega-3 fatty acids and omega-6 fatty acids to a control (capsules with customised fatty acid blends) for three months. Only one short-term study (19 participants) comparing omega-3 to placebo reported a significant improvement in lung function and Shwachman score and a reduction in sputum volume in the omega-3 group. Another study (43 participants) demonstrated a significant increase in serum phospholipid essential fatty acid content and a significant drop in the n-6/n-3 fatty acid ratio following omega-3 fatty acid supplementation compared to control. The longer-term study (17 participants) demonstrated a significant increase in essential fatty acid content in neutrophil membranes and a significant decrease in the leukotriene B4 to leukotriene B5 ratio

  19. 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.

  20. Malignancy from Radium

    PubMed Central

    Loutit, J. F.

    1970-01-01

    Human experience of the toxicity of radium acts as a guide for the setting of occupationally permissible levels for radioactive nucleides, especially bone-seekers. Reviewing the published statements and photomicrographs in early reports especially those of Martland (1931) one can make a case that malignancy was induced in bone-marrow (leukaemia, malignant myelosclerosis) as well as in bone (osteosarcoma) by radium, especially with large doses. Three case reports of radium intoxication in Britons are noted as compatible with this suggestion, after revised interpretation in two of them. ImagesFigs. 9-10Fig. 4Fig. 5Figs. 11-12Figs. 6-7Fig. 8Figs. 13-14 PMID:5271269