Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Herein is presented the case of a malignant non-functioning endocrine tumor of the pancreas with oncocytic features, and a discussion on the high incidence of malignancy in oncocytic endocrine pancreatic tumors. The patient was a 65-year-old woman who showed no paraneoplastic symptoms produced by functioning pancreatic endocrine tumors. The primary tumor was located in the body and tail of the pancreas, and had metastasized to the liver. Tumor cells were arranged in a ribbon-like or trabecular pattern and had an abundant eosinophilic cytoplasm containing numerous mitochondria and neurosecretory granules. The cytoplasm of the tumor cells was intensely stained with an antimitochondrial antigen antibody. Most tumor cells stained positively with Grimelius stain and for chromogranin A. Some tumor cells also stained for synaptophysin. However, the tumor cells negatively stained for hormones such as insulin, glucagon, somatostatin, gastrin, vasoactive intestinal peptide and pancreatic polypeptide, for serotonin, and for pancreatic enzymes such as amylase and trypsin. Analysis of 18 oncocytic pancreatic endocrine tumors, consisting of those reported previously and that in the present case, suggests that the high incidence of malignancy in oncocytic endocrine tumors is associated with the high incidence of non-functioning endocrine tumors among them, most of which are malignant.
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PMID:Oncocytic non-functioning endocrine tumor of the pancreas. 1709 34

Gastrinoma tissue has been found frequently in lymph nodes located near the duodenum without a known primary tumor. Therefore, it has been suggested that a primary lymph node gastrinoma exists. We report on a 38-year-old woman suffering from multiple endocrine neoplasia type 1 (MEN1) confirmed by menin gene mutation analysis. MEN1 disease started with primary hyperparathyroidism followed by Cushing disease, the detection of tumors of the pituitary, adrenal cortex, and the pancreas and also an elevated serum gastrin level. An octreotide scan revealed 4 tumors in the upper abdomen. A selective arterial calcium stimulation test located the source of the hypergastrinemia to the area of the gastroduodenal and the superior mesenteric arteries. Total pancreatoduodenectomy was performed and conventional histopathologic examination revealed a well-differentiated cystic neuroendocrine tumor of the pancreas expressing glucagon and accompanied by several microadenomas. In addition, 3 suprapancreatic lymph nodes with gastrin-positive endocrine tissue were found. None of the pancreatic microadenomas expressed gastrin and no duodenal endocrine tumor was found despite careful macroscopic examination. Only after complete embedding of the duodenal and pancreatic tissue in 65 paraffin blocks, 2 microgastrinomas (0.45 and 0.8 mm in diameter) were identified in the duodenum. It is concluded that duodenal gastrinomas that give rise to lymph node metastases may be so tiny that they are easily overlooked in a routine examination and that systematic tissue monitoring is required to identify them.
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PMID:Primary lymph node gastrinoma or occult duodenal microgastrinoma with lymph node metastases in a MEN1 patient: the need for a systematic search for the primary tumor. 1852 Apr 36

The Zollinger-Ellison syndrome is due to an endocrine gastrin-secreting tumor, the gastrinoma. This tumor is often malignant and patients develop metastases in 25% of cases. The usual localizations of gastrinomas are at the head of the pancreas, the duodenal wall and the peripancreatic lymph nodes. Ectopic localizations, such as stomach, small bowel, gallbladder, liver or ovaries, are rare. We report the case of an intrahepatic gastrinoma, surgically treated by left hepatectomy. Upon review of scientific literature, we found 19 cases of intrahepatic gastrinoma. This diagnosis is always difficult to establish, even after a complete preoperative imaging and an extensive operative search for a possible primary tumor. The best evidence for diagnosis is the gastrinemia decreasing to a normal range after liver resection, and the absence of recurrence in long-term follow-up.
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PMID:[A case of intrahepatic gastrinoma]. 1916 91

Background. Gastrinoma-positive lymph nodes and failed localization of the primary tumor during surgical exploration are described. Specialists suppose that these lymph nodes are metastases rather than a primary gastrinoma. Methods. Case report with a five-year long-term followup. A 60-year-old patient with an confirmed gastrinoma was treated in our department. All preoperative evaluations including somatostatin-receptor-scintigraphy and F-Dopa PET failed to localize the gastrinoma. Explorative laparotomy revealed a gastrinoma in two peripancreatic lymph nodes. Despite extensive intraoperative exploration, no primary gastrinoma could be detected in typical localization. Results. Over a period of 5 years, the patient's gastrin level stayed in the normal range and the patient seems to be completely cured. Conclusion. A prophylactic partial pancreatoduodenectomy is not indicated to avoid recurrence, since complete biochemical cure by local resection of the lymph node gastrinoma is possible.
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PMID:Five-Year Long-Term Followup of a Primary Lymph node Gastrinoma: Is a Pancreaticoduodenectomy Justified? 1972 57

Zollinger-Ellison syndrome (ZES) is an endocrinopathy characterized by gastrin-secreting tumors, responsible for causing the formation of multiple, refractory, and recurrent peptic ulcers in the distal duodenum and proximal jejunum. Two main variants have been described, sporadic and those found in association with parathyroid and pituitary tumors, a genetic disorder known as multiple endocrine neoplasia-1 (MEN-1). Biochemical serum evaluation for elevated gastrin, followed by radiological or nuclear localization of the primary lesion, is mandated for establishing diagnosis. The mainstays of treatment include management of hypersecretory state with medical suppression of gastric acid production and surgical resection of primary tumor for the prevention of malignant transformation and metastatic complications. Medical therapy with proton pump inhibitors has virtually eliminated the need for acid-reducing surgical procedures. Surgical approach to sporadic and MEN-1-associated ZES varies based on our understanding of the natural history of the condition and the probability of cure; however, resection to a negative microscopic margin is indicated in both cases. Postoperative surveillance involves measurement of gastrin level, followed by imaging if elevation is detected. Re-excision of recurrent or resection of metastatic disease is a subject of controversy; however, at the present time aggressive cytoreductive approach is favored.
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PMID:Zollinger-Ellison syndrome: classical considerations and current controversies. 2431 20


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