1/17. Expression of vesicular monoamine transporters in endocrine hyperplasia and endocrine tumors of the oxyntic stomach.BACKGROUND: Gastric enterochromaffin-like (ECL) cells selectively express the vesicular monoamine transporter (VMAT) VMAT2, and enterochromaffin (EC) cells the VMAT1 isoform. Aims: We investigated whether VMAT isoform selection indicates the origin of endocrine hyperplasia and neoplasia from oxyntic ECL or EC cells and may be of prognostic significance in different types of gastric carcinoids. methods: Tissue from patients with chronic atrophic gastritis (CAG), Zollinger-Ellison-syndrome (ZES), gastric carcinoids and neuroendocrine carcinoma (NEC) was investigated by immunohistology and in situ hybridization. RESULTS: endocrine cells forming diffuse, linear, and micronodular hyperplasia in CAG and ZES, as well as oxyntic microcarcinoids expressed both VMAT2 and chromogranin a (CgA) but neither VMAT1 nor serotonin. In five of six sporadic carcinoids VMAT2 and CgA but not VMAT1 were detected. One carcinoid was copositive for VMAT1 and serotonin but negative for VMAT2. Electron microscopy confirmed the VMAT2-positive tumors as ECLoma and the VMAT1-immunoreactive carcinoid as EComa. CONCLUSIONS: VMAT2 and VMAT1 are reliable markers for differentiation of gastric endocrine hyperplasia and neoplasia from ECL and EC cells, respectively. The significance of VMAT2 and VMAT1 as prognostic markers lies in the relatively poor prognosis for EComa compared to ECLoma, characterized by VMAT2 positivity. The absence of both VMAT2 and VMAT1 in NEC may indicate poor prognosis.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
2/17. Mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with zollinger-ellison syndrome: an autopsy case with a 24-year survival period.We report an autopsy case of mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with zollinger-ellison syndrome. A 38-year-old Japanese male was found to have zollinger-ellison syndrome and pancreatic gastrinoma, and gastrectomy and resection of the pancreatic tumor were performed. However, hypergastrinemia persisted, and the patient died of disseminated carcinomatosis at 62 years of age, 24 years after the onset of zollinger-ellison syndrome. At autopsy, the main tumor was present in the residual pancreas, and metastases were noted in many organs. In the pancreas and other organs, ductal and endocrine carcinoma areas were mixed and there was a gradual transition between the two. No acinar differentiation was noted. The ductal elements were positive for mucins and carcinoembryonic antigen but negative for neuroendocrine markers, while endocrine elements were positive for chromogranin a and synaptophysin and to a lesser extent for gastrin, but negative for mucins and carcinoembryonic antigen. The ductal elements comprised about 30% of the tumor cells, and endocrine elements 70%. According to the revised world health organization classification, our case was diagnosed as mixed ductal-endocrine carcinoma. Our case is rare because the tumor manifested as gastrinoma with zollinger-ellison syndrome and the patient survived for 24 years. To the best of our knowledge, no such case has been reported. Our case suggests that pancreatic endocrine tumors may evolve into mixed ductal-endocrine carcinomas.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
3/17. association of zollinger-ellison syndrome with pancreatitis: report of five cases.In a retrospective analysis, five cases of zollinger-ellison syndrome were found in a typical urban inner-city teaching hospital. Chronic alcohol abuse and heavy smoking characterized these patients, and four of them also had pancreatitis, suggesting an association of gastrin-producing tumors and pancreatic inflammation. Ductal obstruction by neuroendocrine tumors has been reported to cause pancreatitis in a few cases. In this analysis, however, a nonobstructive gastrinoma was the surgical diagnosis in three patients, and it was suggested by imaging studies in the two other cases. The potential other pathomechanisms for a dual cause-effect relationship of gastrinoma and pancreatitis are discussed.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
4/17. Gastric collision between a papillotubular adenocarcinoma and a gastrinoma in a patient with zollinger-ellison syndrome.We report a unique case of a gastric collision tumor composed of an intramural gastrin-secreting tumor and a papillotubular adenocarcinoma of the intestinal type discovered at autopsy in a patient with zollinger-ellison syndrome. There was extensive metastatic dissemination of the neuroendocrine component to regional lymph nodes and to the liver. The unusual macroscopical, histological, and immunohistochemical features of this case and its specific clinical setting are discussed.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
5/17. Ultrastructural and immunocytochemical study of a primary gastrinoma of the liver.A primary hepatic gastrinoma found in a 13-year-old boy was studied by light microscopy, immunohistochemistry, electron microscopy, and immunoelectron microscopy. Results were consistent with a neuroendocrine neoplasm with abundant gastrin-immunoreactive cells. Unlike all previously reported cases of primary hepatic neuroendocrine tumors, which have been endocrinologically asymptomatic, the patient had a zollinger-ellison syndrome apparently cured by surgical resection of the tumor.- - - - - - - - - - ranking = 2keywords = neuroendocrine (Clic here for more details about this article) |
6/17. Hyperplastic polyposis and cancer of the colon with gastrinoma of the duodenum.BACKGROUND: A 64-year-old woman presented to the emergency room with a 3-month history of intermittent abdominal cramps, accompanied by nausea, vomiting, anorexia, and decreased bowel movements consistent with a partial intestinal obstruction. She had a 12-year history of peptic ulcers, which had been treated with histamine-2 blockers. INVESTIGATIONS: physical examination, abdominal X-ray, abdominal CT scan, colonoscopy and assessment of gastrin levels. diagnosis: Duodenal neuroendocrine neoplasm showing gastrin expression and stage III (T3N2M0), poorly differentiated adenocarcinoma of the cecum arising from hyperplastic polyposis. MANAGEMENT: Right-sided hemicolectomy with ileocolonic anastomosis, duodenal resection, leucovorin and 5-fluorouracil chemotherapy, annual colonoscopic surveillance, and polypectomy.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
7/17. Glycoprotein hormone alpha subunit in endocrine cells of human oxyntic mucosa. Studies on its relation to neuroendocrine tumors.Expression of the alpha-subunit of glycoprotein hormones is an acquired feature of the endocrine cells of the oxyntic mucosa in patients with sustained serum levels of gastrin, and may be related to the hyperplasia-carcinoid sequence occurring in these patients. In the present study we have investigated the intragastric cellular localization and the circulating levels of alpha-subunit in a patient with zollinger-ellison syndrome. In this patient we have found that: 1) endocrine cells accounted for 2.29% /- 1.44% of the total oxyntic mucosal volume (normal value: 0.9% /- 0.4%), with the ECL cells representing 63.22% /- 10.9% of the total endocrine cell volume (normal value: 29.8 /- 8.8%). 2) Cells immunoreactive for the alpha-subunit were found to correspond ultrastructurally to a subpopulation of enterochromaffin-like cells, indistinguishable from similar cells devoid of significant immuno-electron microscopic labeling. 3) Immunoreactive cells included a portion of oxyntic endocrine cells with punctate granules, a feature previously observed only in carcinoid tumors of the oxyntic mucosa. 4) In consecutive sections of freeze-dried vapor-fixed biopsies a fraction of alpha-subunit storing cells was found to co-express histamine. 5) The serum alpha-subunit levels were abnormally elevated and paralleled those of gastrin in a secretin-stimulation test. Analysis of similar curves in two other patients with zollinger-ellison syndrome, and five patients with hypergastrinemic atrophic gastritis, all presenting alpha-subunit containing oxyntic endocrine cells, showed significant alpha-subunit elevations only in the patients with ulcerogenic syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 4keywords = neuroendocrine (Clic here for more details about this article) |
8/17. Hyperprealbuminemia, euthyroid hyperthyroxinemia, Zollinger-Ellison-like syndrome and hypercorticism in a pancreatic endocrine tumour.prealbumin, one of the main thyroxine transport proteins, has recently been shown to be a valuable immunohistochemical marker of neuroendocrine tumours. We report the case of a multisecretory pancreatic endocrine tumour whose prealbumin secretion was so high that it produced a peak on routine serum protein electrophoresis and induced a euthyroid hyperthyroxinemia. The maximal binding capacity of prealbumin for thyroxine was indeed markedly increased, whereas its affinity for this hormone was normal. The tumour was associated with gastric hyperacidity and hypergastrinemia thereby evoking a zollinger-ellison syndrome. The secretin stimulation test and gastrin tumoural immunohistochemistry were, however, negative. We suggest that the concomitant tumoural production of gastrin-releasing peptide was responsible for the gastric hyperacidity and hypergastrinemia. This hormone probably also accounted for a moderate hypercorticism.- - - - - - - - - - ranking = 7.9174140862033keywords = neuroendocrine tumour, neuroendocrine (Clic here for more details about this article) |
9/17. Neuroendocrine tumours of the gut: long-term therapy with the somatostatin analogue SMS 201-995.Nine patients with metastatic neuroendocrine tumours of the gut were treated for over 100 days with the long-acting octapeptide of somatostatin, SMS 201-995. Two patients had unusually aggressive tumours (neuroendocrine tumours are typically slow-growing) with only modest elevation of tumour secretory products. SMS 201-995 was given in an attempt to slow tumour growth, but tumour size continued to increase. Three patients had glucagonomas and uncontrolled skin rashes, and four patients had VIPomas, which caused uncontrolled watery diarrhoea. SMS 201-995 was given principally to reduce hypersecretion of hormones from their tumours and to relieve symptoms. All the patients experienced rapid relief of their symptoms when SMS 201-995 treatment was introduced, and symptomatic control was maintained throughout treatment except in one patient, who required a supplementary procedure (pancreatic primary tumour embolization). Circulating peptide levels were initially suppressed, but continued suppression did not occur in every case despite good clinical control. It seems likely that SMS 201-995 has beneficial effects on target organs as well as suppressing the release of peptides from tumour tissue. The tumours did not grow during treatment. The analogue was well tolerated, most adverse effects being minor. somatostatin inhibited insulin release, but in only one patient was there a slight deterioration of glucose tolerance. SMS 201-995 is therefore a useful therapeutic tool in the long-term management of patients with symptoms due to uncontrolled hormone secretion from neuroendocrine tumours.- - - - - - - - - - ranking = 23.75224225861keywords = neuroendocrine tumour, neuroendocrine (Clic here for more details about this article) |
10/17. Perioperative use of long-acting somatostatin analog (SMS 201-995) in patients with endocrine tumors of the gastroenteropancreatic axis.The clinical manifestations of hormone excess caused by functioning neuroendocrine tumors of the gastroenteropancreatic (GEP) axis can be life threatening and frequently prove refractory to conventional antisecretory drugs. Administration of a long-acting somatostatin analog (SMS 201-995) proved effective in three patients with complex management problems related to GEP tumors. A patient with an insulinoma was maintained euglycemic intraoperatively with a single 100 micrograms dose of SMS given before surgery. Gastric suction in two patients with gastrinomas caused hypochlorhydric alkalosis that was preventable with preoperative SMS. Iatrogenic pancreatic fistula occurring after resection of a benign insulinoma healed within 4 days of SMS administration. This drug may be a useful adjunct in the perioperative management of patients with GEP endocrine tumors. Caution is advised regarding potential hazards related to malabsorption and gastric dysmotility.- - - - - - - - - - ranking = 1keywords = neuroendocrine (Clic here for more details about this article) |
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