1/12. 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.- - - - - - - - - - ranking = 1keywords = somatostatinoma (Clic here for more details about this article) |
2/12. 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.- - - - - - - - - - ranking = 0.375keywords = somatostatinoma (Clic here for more details about this article) |
3/12. 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 = 0.75keywords = somatostatinoma (Clic here for more details about this article) |
4/12. 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.- - - - - - - - - - ranking = 0.25keywords = somatostatinoma (Clic here for more details about this article) |
5/12. Somatostatin-producing neuroendocrine tumor of the ampulla (ampullary somatostatinoma). Evidence of prosomatostatin production.Two cases of somatostatin-producing ampullary neuroendocrine tumors (somatostatinoma) are reported. The authors have characterized their immunoreactivity using antibodies specific for the amino- and carboxyl-terminal portions of prosomatostatin, the precursor of somatostatin in the normal synthetic pathway. Cytoplasmic staining was found using each of these two antibodies in the tumor cells of both ampullary somatostatinomas as well as in the cytoplasm of cells in the hypothalamus, crypt cells of the duodenal mucosa, mucosal cells of the biliary tract, D cells of the pancreatic islets, and parafollicular cells of fetal thyroid. These studies suggest that the synthesis of somatostatin in ampullary somatostatinomas occurs through the normal pathway from the precursor prosomatostatin.- - - - - - - - - - ranking = 0.875keywords = somatostatinoma (Clic here for more details about this article) |
6/12. somatostatinoma: collision with neurofibroma and ultrastructural features.The clinical presentation, histopathology and immunoelectron microscopic features of two cases of duodenal somatostatinoma are described, one of which is a hitherto unreported example of a collision tumour with a neurofibroma. Ultrastructural morphometric immunoelectron microscopy studies revealed the presence of four types of cells in both tumours, but there was no difference in the proportions of these cells between the collision tumour and the non-collision tumour. Neurosecretory granules ranging in size from 255-815 nm were generally larger than those previously reported for somatostatinomas and somatostatin was identified in granules of all sizes across this range. Neither tumour was associated with the somatostatinoma syndrome comprising associated diabetes mellitis, steatorrhoea and cholelithiasis.- - - - - - - - - - ranking = 0.375keywords = somatostatinoma (Clic here for more details about this article) |
7/12. Incidental small ampullary somatostatinoma treated with ampullectomy 2 years after diagnosis.Somatostatinomas are rare tumors; ampullary somatostatinomas are very rare. We report a case of a small pure somatostatin-producing neuroendocrine tumor of ampulla of vater in a 54-year-old woman with neither neurofibromatosis nor somatostatinoma syndrome, "incidentally" discovered during an abdominal computed tomography. The patient initially refused other adjunctive exams but after 2 years she was admitted, presenting with itch, night sweats, severe fatigue, and unintentional weight loss. The size of the tumor (1.5 cm) and the other radiologic findings had not changed since the abdominal CT scan 2 years before. The somatostatin, gastrin, glucagons, serotonin, vasoactive intestinal peptide, dopamine, norepinephrine, epinephrine, and calcitonin plasma levels were normal. ERCP-obtained biopsies revealed a neuroendocrine tumor with psammoma bodies; immunohistochemical profile was positive for chromogranin and somatostatin. The patient underwent surgery; intraoperative histologic examination of lymph nodes sampling of perihepatic and periduodenal lymph nodes was negative for metastasis. We performed, therefore, a transduodenal ampullectomy. The patient continues to do well at 3 years' follow-up with no evidence of local or distance recurrence of disease.- - - - - - - - - - ranking = 0.75keywords = somatostatinoma (Clic here for more details about this article) |
8/12. Ampullary somatostatinoma. An immunohistochemical and ultrastructural study.Two new cases of ampullary somatostatinoma are reported. In one case the tumor is associated with an increase in somatostatin-positive cells in the adjacent duodenal mucosa. Both tumors show a predominant glandular pattern with many psammomatous calcified bodies. Such bodies seem to arise by calcium phosphate encrustation of intraluminal cellular debris. The neoplastic cells contain two distinct types of intermediate filaments: the first is located along the plasma membrane and reacts to keratin antiserum; the other, appearing as paranuclear aggregates, reacts to neurofilament antiserum. The neoplastic cells show signs of intestinal differentiation (microvilli, glycocalyceal bodies, filamentous core rootlets) as well as of neuroendocrine differentiation (secretory granules, whorls of neurofilaments).- - - - - - - - - - ranking = 0.625keywords = somatostatinoma (Clic here for more details about this article) |
9/12. Ampullary somatostatinoma: psammomatous variant of gastrointestinal carcinoid tumor--an immunohistochemical and ultrastructural study. Report of a case and review of the literature.A 28-year-old man presented with epigastric pain and obstructive jaundice associated with a histologically and immunologically unusual variant of carcinoid tumor involving the ampulla of vater. The tumor contained abundant psammoma bodies and exhibited immunoreactivity only for somatostatin. Immunoperoxidase studies for insulin, glucagon, vasoactive intestinal peptide, calcitonin, serotonin, and ACTH had negative results. In contrast to most somatostatinomas of pancreatic origin, clinically this ampullary somatostatinoma was not accompanied by features of the somatostatinoma syndrome. A literature review of the clinical and hormonal features in reported cases of gastrointestinal and pancreatic somatostatinomas is presented.- - - - - - - - - - ranking = 1keywords = somatostatinoma (Clic here for more details about this article) |
10/12. Ampullary somatostatinoma associated with von Recklinghausen's neurofibromatosis presenting as obstructive jaundice.A case of von Recklinghausen's disease associated with a somatostatinoma of the pancreas causing obstructive jaundice is described. Discussion on the association of von Recklinghausen's disease with somatostatinoma is presented.- - - - - - - - - - ranking = 0.75keywords = somatostatinoma (Clic here for more details about this article) |
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