1/31. Duodenal somatostatinoma presenting as upper gastrointestinal bleeding.Duodenal somatostatinoma is a rare cause of upper gastrointestinal bleeding. We report the case of a 55-yr-old woman who presented with upper gastrointestinal hemorrhage due to a duodenal somatostatinoma. We discuss the characteristics of these tumors, and review the literature on diagnostic workup and management.- - - - - - - - - - ranking = 1keywords = somatostatinoma (Clic here for more details about this article) |
2/31. Heterotopic pancreas, periampullary somatostatinoma and type I neurofibromatosis: a pathogenetic proposal.This case documents the association of ectopic pancreatic tissue with a duodenal somatostatinoma in a patient with type I neurofibromatosis. pancreatic ducts have been noted within the centres of somatostatinomas, but little significance has been attached to this finding. Here we describe a patient in whom a separate proliferation of somatostatin cells occurred in association with the ectopic pancreatic ductular epithelium. This lesion bore a striking resemblance to the ductulo-insular or ductulo-endocrine complexes that are seen in nesidioblastosis in the pancreas. We therefore postulate that the ducts, which are sequestered within somatostatinomas, are of pathogenetic significance. The somatostatin-producing cells arise from these ducts, very much in the fashion of ductulo-endocrine complexes in nesidioblastosis.- - - - - - - - - - ranking = 1.1666666666667keywords = somatostatinoma (Clic here for more details about this article) |
3/31. Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis.Somatostatinomas are rare functioning neoplasms usually arising in the pancreas and duodenum. We report a case of somatostatinoma in a 42-year-old male with neither neurofibromatosis nor somatostatinoma syndrome. A large tumor in the descending duodenum had given rise to multiple lymph node metastases. An additional 31 duodenal somatostatinoma cases were also reviewed. Most originated in the descending part of the duodenum, with the ampulla and peri-ampullary area as the most common sites (60%). Frequent manifestations were abdominal pain (25%), jaundice (25%), or cholelithiasis (19%), the latter two reflecting obstruction of the bile duct by tumors. Only two cases showed a possible somatostatinoma syndrome (6%). The tumors with metastases, lymph nodes (10) and liver (2), were significantly larger than average than those without (2.91 /- 1.49 cm vs 1.36 /- 0.71 cm, P < 0.05). With a cut-off point of 2.0 cm, diagnostic accuracy for metastasis was 77.78% with 87.50% specificity and 63.64% sensitivity. The smallest tumor with metastases was 0.8 cm and the largest without metastases was 3.0 cm. These results indicate that duodenal somatostatinomas are malignant by nature and the risk of metastasis significantly increases with tumors larger than 2.0 cm.- - - - - - - - - - ranking = 1.5keywords = somatostatinoma (Clic here for more details about this article) |
4/31. Duodenal somatostatinoma of the ampulla of vater diagnosed by endoscopic fine needle aspiration biopsy: a case report.BACKGROUND: Duodenal somatostatinoma is a rare neuroendocrine neoplasm. A better prognosis can be obtained if these tumors are resected at an early clinical stage. Endoscopic punch biopsy has been the method most commonly used for the preoperative diagnosis of neuroendocrine duodenal tumors. To the best of our knowledge, endoscopic fine needle aspiration biopsy (FNAB) of duodenal somatostatinoma has not been reported before. CASE: A 41-year-old, black female presented with upper gastrointestinal bleeding as well as elevated bilirubin, liver enzymes and glucose. Computed tomography, esophagogastroduodenoscopy and endoscopic retrograde cholangiopancreatography (ERCP) detected a mass at the region of the ampulla of vater partially obstructing the pancreatic duct. The initial punch biopsy yielded only intestinal mucosa. Subsequent endoscopic FNAB suggested the diagnosis of a neuroendocrine neoplasm, as confirmed by additional punch biopsies. Immunohistochemical and electron microscopic studies disclosed somatostatin production by the tumor, which was resected through a modified Whipple procedure. The patient recovered fully. CONCLUSION: This case demonstrates the usefulness of endoscopic FNAB in diagnosing submucosal gastrointestinal neuroendocrine tumors.- - - - - - - - - - ranking = 1keywords = somatostatinoma (Clic here for more details about this article) |
5/31. Duodenal somatostatinoma presenting with complete somatostatinoma syndrome.Somatostatinomas are the rarest pancreatic endocrine tumors and can arise in the pancreas or duodenum. Duodenal somatostatinomas are less common than, and are distinguished from, their pancreatic counterparts by a frequent association with type I neurofibromatosis, the presence of psammoma bodies, the less frequent presence of metastatic disease, and the absence of somatostatinoma syndrome (diabetes mellitus, steatorrhea, and cholelithiasis). We report a case of somatostatinoma with metastases and psammoma bodies presenting with all three features of the syndrome in a patient with neurofibromatosis. Although several reports have documented portions of the syndrome in patients with duodenal somatostatinomas, to our knowledge, this is the first report of the complete syndrome associated with a duodenal lesion.- - - - - - - - - - ranking = 2keywords = somatostatinoma (Clic here for more details about this article) |
6/31. exocrine pancreatic insufficiency and pancreatic fibrosis due to duodenal somatostatinoma in a patient with neurofibromatosis.A case of duodenal somatostatinoma is described in a patient with Von Recklinghausen neurofibromatosis. The patient presented with exocrine pancreatic insufficiency, probably due to distal obstruction of the pancreatic duct by the tumor. Preoperative evaluation with calcium-pentagastrin and tolbutamide stimulation tests were nondiagnostic. At laparotomy, local excision of the tumor was performed. Pathological findings were compatible with duodenal somatostatinoma, causing pancreatic fibrosis. Somatostatin extracted from the tumor coeluted with the somatostatin-14 standard on high performance liquid chromatography (HPLC).- - - - - - - - - - ranking = 1keywords = somatostatinoma (Clic here for more details about this article) |
7/31. Malignant duodenal somatostatinoma presenting in association with von Recklinghausen disease.Somatostatinomas are extremely rare periampullary malignant neuroendocrine tumors that may be associated with von Recklinghausen disease or type-I neurofibromatosis. Duodenal somatostatinomas are distinguished from pancreatic somatostatinomas by their frequent association with type-I neurofibromatosis and typically absence of somatostatinoma syndrome. We report a very rare and atypical case of malignant duodenal somatostatinoma presenting with somatostatinoma syndrome in association with type-I neurofibromatosis.- - - - - - - - - - ranking = 1.5keywords = somatostatinoma (Clic here for more details about this article) |
8/31. Von Recklinghausen's neurofibromatosis associated with duodenal somatostatinoma. A case report and review of the literature.Somatostatin producing duodenal carcinoids are rare, comprising a mere 2% of small bowel carcinoids and 5-10% of all duodenal tumors. Since the 1st case described by Kaneko in 1979 more than 50 cases have been reported in the world literature. From these reports, it is gradually emerging that duodenal somatostatinomas may show a strong association with von Recklinhausen's neurofibromatosis (VRNF) as a distinct neuroendocrine syndrome. A case of a patient affected by VRNF associated with duodenal somatostatinoma with consequent obstructive jaundice is reported. The authors discuss the characteristics of these tumors and review the literature. A total of 27 patients with Von Recklinghausen's disease associated with immunohistologically proved duodenal somatostatinoma have been identified and compared with 29 duodenal somatostatinoma not associated with VRNF, and with 32 cases of pancreatic somatostatinomas.- - - - - - - - - - ranking = 1.5keywords = somatostatinoma (Clic here for more details about this article) |
9/31. Large duodenal somatostatinoma in the third portion associated with severe glucose intolerance.A 51-year-old man was admitted with hyperglycemia and a duodenal tumor. Although his glycemic control was poor, basal c-peptide levels were not suppressed. Further examination revealed a mass measuring 7.8 cm in diameter in the third portion of the duodenum. Duodenectomy revealed a slow-growing sessile tumor located near Treitz's ligament. The immunohistochemical profile of sections of the specimen revealed the presence of somatostatin. The patient's serum somatostatin was elevated to 300 pg/ml preoperatively, but was reduced to 10 pg/ml postoperatively. Glycemic control also normalized after the operation.- - - - - - - - - - ranking = 0.66666666666667keywords = somatostatinoma (Clic here for more details about this article) |
10/31. Duodenal somatostatinoma associated with Von Recklinghausen's disease.Somatostatinomas are rare, malignant, somatostatin-producing neuroendocrine tumors with a prevalence of one in 40 million. The coincidence of Von Recklinghausen's disease and duodenal somatostatinoma has been known since 1982. We report the case of a 57-year-old female patient with Von Recklinghausen's disease and a tumor of the pancreatic head that was diagnosed due to painless icterus. Histopathological examination after pylorus-preserving pancreatoduodenectomy revealed the existence of a duodenal somatostatinoma with lymph node metastases. Characteristics of the association of von Recklinghausen's disease and somatostatinoma, and therapy and prognosis will be discussed. In patients with Von Recklinghausen's disease and an ampullary tumor, a somatostatinoma should be considered. In contrast to its pancreatic counterparts, duodenal somatostatinoma is frequently associated with Von Recklinghausen's disease, often contains psammoma bodies, is rarely associated with a recognizable "somatostatin syndrome", and is hardly ever associated with demonstrable metastases at the time of diagnosis. Small tumors arising in the duodenum may be treated with local excision, whereas larger tumors should be treated by total excision, which may entail a partial pancreatoduodenectomy.- - - - - - - - - - ranking = 1.5keywords = somatostatinoma (Clic here for more details about this article) |
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