Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Somatostatinomas are extremely rare endocrine tumors, and those with diameters above 2 cm are reported to increase the risk of metastasis significantly. We report a case of a large functional somatostatinoma in the pancreatic tail without metastases. A 46-year-old woman with a history of recurrent mild upper abdominal pain and diarrhea for 10 months was admitted to our hospital. Multiple-phase spiral computed tomography revealed a 10 cm x 8 cm, ill-defined, elliptic mass in the body and tail of the pancreas. There was a slightly heterogeneous enhancement on hepatic arterial phase and isodensity to the pancreatic parenchyma with small dotted necrosis within the middle region of the mass on hepatic portal venous and parenchymal phase, with patent splenic vein, dilated collaterals at the splenic hilum and no dilated pancreatic duct, resembling a diffuse infiltration tumor. To the best of our knowledge, this is the first description of multiple-phase spiral computed tomography findings of a functional somatostatinoma in the pancreatic tail and the largest thus far on reported computed tomography, with some differences compared with the previous reports.
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PMID:A large functional somatostatinoma in the pancreatic tail: atypical CT appearances. 2008 76

Somatostatinoma are rare well-differentiated endocrine tumors with malignant behavior arising from the pancreas and duodenum. They are defined by somatostatin positive immunostaining of the majority of tumor cells. The main clinical features are diabetes, diarrhea and biliary lithiasis related to somatostatin production. Somatostatinoma secreting both calcitonin and somatostatin may be unrecognized as a small number of such observations have been published. We report the case of a 57- year-old woman referred for weight loss, diarrhea and worsening diabetes. Computer tomography scan revealed multiple hypervascular liver lesions suggestive of metastases. High plasma calcitonin level was evidenced, with normal chromogranin-A value, and high plasma somatostatin results lately communicated. Calcitonin secretion of extra-thyroidal origin was suspected leading to the identification of a pancreatic mass by further multiphase CT. The patient underwent left pancreatectomy with surgical hepatic resection. Histological and immunostaining studies confirmed definitive diagnosis of somatostatinoma secreting both somatostatin and calcitonin. Plasma calcitonin should be measured in the assessment of duodeno-pancreatic endocrine neoplasm. Calcitonin determination is available, more reproducible than other specific pancreatic endocrine markers and could be effective for diagnosis and follow-up of such foregut-derived endocrine neoplasia.
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PMID:Hypercalcitonemia revealing a somatostatinoma. 2081 46

Acute pancreatitis may rarely be caused by papillary mass lesions such as adenocarcinomas and neuroendocrine tumours. Occasionally these papillary lesions may cause recurrent episodes of acute pancreatitis and patients presenting in this way require further pancreatic investigation. We believe this to be the first reported case of a duodenal papillary somatostatinoma causing recurrent acute pancreatitis. The patient was investigated with multiple imaging modalities, both at endoscopy and with more traditional radiology, and treated with resection by Whipple's pancreaticoduodenectomy. If diagnosed early in the absence of distant metastases the prognosis of papillary somatostatinoma with tumour resection is excellent.
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PMID:Case report: recurrent acute pancreatitis secondary to papillary somatostatinoma--a new association. 2356 78

We report a 51-year-old female patient with adult-onset type II citrullinemia (CTLN2) who had a history of pancreatoduodenectomy for duodenal somatostatinoma with metastases to regional lymph nodes at age 49 years, paying special attention to indications for liver transplantation. At age 50 years, she developed hepatic encephalopathy with elevation of plasma ammonia and citrulline levels. A diagnosis of CTLN2 was made by DNA analysis of the SLC25A13 gene and treatment with conservative therapies was begun, including a low-carbohydrate diet and supplementation with arginine and sodium pyruvate. However, despite these treatments, frequent attacks of encephalopathy occurred with markedly elevated plasma ammonia levels. While we were apprehensive regarding the risk of recurrence of somatostatinoma due to immunosuppressive therapy after liver transplantation, the patient was in a critical condition with CTLN2 and it was decided to perform living-donor liver transplantation using a graft obtained from her son. Her postoperative clinical course was uneventful and she has had an active life without recurrence of somatostatinoma for 2 years. This is the first case of CTLN2 with somatostatinoma. As the condition of CTLN2 patients with rapidly progressive courses is often intractable by conservative therapies alone, liver transplantation should be considered even after surgery for malignant tumors in cases with neither metastasis nor recurrence.
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PMID:Patient with adult-onset type II citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma: Indication for liver transplantation. 2361 80

Somatostatinomas are rare functioning neoplasms usually arising in the pancreas and duodenum. They are seldom associated with typical clinical symptoms. Their diagnosis is confirmed only by histological and immunohistochemical studies and the presence of specific hormones. Two distinct clinicopathological forms of somatostatinoma exist: duodenal and pancreatic somatostatinomas. Clinically, compared to pancreatic somatostatinomas, duodenal somatostatinomas are more often associated with nonspecific symptoms and neurofibromatosis, but less often with somatostatinoma syndrome or metastasis. We report a case of somatostatin-producing duodenal carcinoma in a 45-year-old female with neither neurofibromatosis nor somatostatinoma syndrome. Abdominal computed tomography showed a 18 mm mass in the duodenum which had given rise to multiple lymph node metastases. Although the endoscopic biopsies were free of malignancy, the patient subsequently underwent Whipple's operation for the duodenal mass. Immunohistochemical analysis confirmed the diagnosis of somatostatin-producing carcinoma.
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PMID:Somatostatin-producing duodenal carcinoma: clinicopathological description of a case. 2466 82

Among the neuroendocrine neoplasia, the pancreatic somatostatin-producing tumors are very rare. Usually functional, these tumors produce the somatostatinoma syndrome, which encompasses diabetes mellitus, diarrhea/steatorrhoea, and cholelithiasis. Other symptoms may include dyspepsia, weight loss, anemia, and hypochlorhydria. All theses symptoms are explained by the inhibitory actions of the somatostatin released by tumoral cells originated from pancreatic delta cells or endocrine cells of the digestive tract. The diagnosis is easy to overlook since these symptoms are commonly observed in other more common syndromes. Besides the clinical features, diagnosis is based on serum determination of somatostatin, and imaging exams, such as ultrasound, computer tomography and positron emission tomography. Pathologic examination is characterized by the positivity of immunohistochemical reaction for synaptophysin, chromogranin, and somatostatin. These tumors can be classified according to tumor size, mitotic index, neural or vascular invasion, and distant metastases. The authors describe the case of a 61-year-old female patient who sought medical care because of a 6-month history of watery diarrhea, weight loss, and depression. She was diagnosed with diabetes mellitus 3 years ago. Imaging examination revealed a tumoral mass of 4 cm in its longest axis in the topography of the head of the pancreas and calculous cholecistopathy. The patient's clinical status was unfavorable for a surgical approach. She died after 20 days of hospitalization. The definitive diagnosis was achieved with the autopsy findings, which disclosed a pancreatic somatostatinoma.
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PMID:Somatostatinoma syndrome: a challenging differential diagnosis among pancreatic tumors. 3152 95


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