Cases reported "Somatostatinoma"

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1/70. 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. ( info)

2/70. 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. ( info)

3/70. Ampullary somatostatinoma in a patient with Merkel cell carcinoma.

    A 59-yr-old white man with Merkel cell carcinoma of his right leg status post extensive skin resection and chemotherapy had dilated hepatic and common bile ducts on a routine follow-up abdominal CT scan. A 1.9-cm ampullary mass was appreciated on endoscopy. histology showed psammoma bodies and positive immunoperoxidase staining consistent with a somatostatinoma. Merkel cell tumors and somatostatinomas are extremely rare neuroendocrine tumors derived from neural crest cells. Associations have been found between somatostatinomas and other islet cell tumors with multiple endocrine neoplasia syndromes, but no reported association has been published between islet cell tumors and Merkel cell tumors. This patient represents the first documented case of Merkel cell carcinoma and somatostatinoma in a single patient. Such an occurrence may represent a previously undescribed neuroendocrine tumor syndrome, and this possibility should be considered when either tumor is diagnosed. ( info)

4/70. 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. ( info)

5/70. somatostatinoma of the ampulla of vater in celiac sprue.

    The increased incidence of gastrointestinal lymphoma and adenocarcinoma in patients with celiac sprue is well recognized, with 10% to 15% developing a gastrointestinal malignancy. Somatostatinomas are rare neuroendocrine tumors that occur most commonly within the pancreatic head or duodenum. Although fewer than 100 cases have been reported, somatostatinomas are often associated with multiple endocrine neoplasia-1 syndrome and von Recklinghausen's disease. The unusual case of a 43-year-old woman with celiac sprue in which a somatostatinoma involving the ampulla of vater was identified and resected is presented. To our knowledge, somatostatinomas have not been previously reported in patients with celiac sprue. ( info)

6/70. 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. ( info)

7/70. 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. ( info)

8/70. Case report: somatostatin producing teratoma, causing rapidly alternating extreme hyperglycemia and hypoglycemia, and ovarian somatostatinoma.

    A 54-year-old woman presented with extremely fluctuating and symptomatic blood glucose levels. Very high levels of somatostatin and low levels of insulin, c-peptide, gastric inhibitory peptide (GIP), and glucagon-like peptide-1 (GLP-1) in peripheral blood were constantly present. A benign somatostatinoma was localized by meta-iodobenzyl guanidine iodine 123 (MIBG-I(123)) scintigraphy and successfully removed encapsulated in an ovarian teratoma. The patient made a complete recovery. The case described is unique with regard to clinical symptomatology and anatomic localization of the tumor. ( info)

9/70. Periampullary pancreatic somatostatinoma.

    BACKGROUND: Somatostatinomas involving the gastrointestinal tract are extremely rare neoplasms that typically present with indolent, nonspecific symptoms in the absence of systemic neuroendocrine manifestations that characterize the somatostatinoma syndrome. Because of a relatively large size at the time of presentation (average diameter of 5 cm) and common location within the head of the pancreas, the Whipple procedure (pancreaticoduodenectomy) serves as the predominant modality for curative and palliative surgical approaches. methods: Two cases of somatostatinoma involving the minor duodenal papilla with concomitant pancreatic divisum were reviewed, with a general overview of this unique islet cell tumor. RESULTS: Unlike typical somatostatinomas, these two tumors were subcentimeter in size but were associated with synchronous regional metastasis. CONCLUSIONS: Somatostatinomas are often associated with regional and/or portal metastases at the time of diagnosis, and only 60% to 70% of surgical cases result in complete tumor resection. Predictors of an unfavorable prognosis include size >3 cm, poor cytological differentiation, regional and/or portal metastasis, and incomplete surgical resection. Even in the presence of synchronous metastases, the 5-year overall survival for patients with somatostatinoma is approximately 40%. Currently, there are no clinical trials demonstrating significant improvement in survival with the use of adjuvant therapy. ( info)

10/70. somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease.

    We report a case of somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease (VRD). A 64-year-old woman who had had recurrent attacks of acute pancreatitis and cholangitis was found, on gastroduodenal endoscopy, to have a tumor of the papilla of Vater and multiple submucosal tumors of the stomach and duodenum. Numerous submucosal tumors were observed in the stomach, duodenum, and jejunum, and total excision of the papilla of Vater and resection of the duodenal and jejunal submucosal tumors was performed. The tumor of the papilla of Vater showed the histologic appearance of a dense proliferation of tumor cells in acinar form, from the duodenal mucosa to the muscle layer, and psammoma bodies were revealed within the tumor. Immunohistologically, the tumor cells were intensely positive for somatostatin. The submucosal tumors of the duodenum and jejunum were negative for smooth muscle actin, s-100, and neuron-specific enolase (NSE), and positive for CD34 and c-kit, and they were diagnosed as gastrointestinal stromal tumors (GISTs) according to the strict definition. The only 25 cases of papilla of Vater somatostatinoma associated with VRD to have been reported in the English-language literature since 1982 are reviewed, as well as our own case. ( info)
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