Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01275 (glucagon)
 26,492 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The glucagonoma syndrome is a rare clinical condition characterized by a distinctive cutaneous eruption associated with a glucagon-secreting islet cell neoplasm of the pancreas. A 19-year-old woman manifested typical features of this condition: a polymorphous skin eruption with characteristic distribution of lesions in perioral and paragenital regions; lesions in sites of cutaneous trauma; a skin biopsy that showed epidermal cleavage; glossitis; weight loss; mild anemia; abnormal glucose tolerance test results. Plasma glucagon levels, determined by radioimmunoassay, were approximately five times normal. Angiography indicated a pancreatic tumor with liver metastases. Islet cell origin was confirmed histologically. It is hoped that wider recognition of the distinctive clinical features of this syndrome will result in earlier detection and possible surgical cure of the underlying malignancy.
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PMID:The glucagonoma syndrome. A distinctive cutaneous marker of systemic disease. 20 56

Goblet cell carcinoids are uncommon but distinctive tumours of the appendix. We have reviewed 11 cases diagnosed within the period 1976-1990. The mean age at presentation was 58 years (range 24-76), with a female:male ratio of 8:3. At presentation, in seven patients tumour was confined to the appendix or mesoappendix (mean age 51) and in four there was extension beyond the appendix (mean age 69). Of the seven patients with localized tumour, six are alive and without clinical disease after a mean follow-up period of 32 months and one died with recurrent tumour after 10 years. Of the four with more extensive disease, two died during follow-up (at 23 months with probable liver metastases and at 16 months with intestinal obstruction) and two are alive, one with disease and one clinically disease-free. Immunohistochemistry showed that all of the tumours stained positively for either neuron-specific enolase, chromogranin A or protein gene product 9.5. No tumour stained with antiserum to substance P and none showed glucagon-like immunoreactivity, but four cases stained positively for pancreatic polypeptide, an unusual feature in midgut carcinoids.
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PMID:Appendiceal goblet cell carcinoids: a clinicopathological and immunohistochemical study. 167 61

Dehiscence of pancreaticojejunostomy represent the main technical postoperative complication after duodenocephalopancreasectomy for periampullary carcinoma. The incidence of this complication is particularly high in cases of narrow duct and a tender pancreatic gland. In this case the authors suggest a technique of occlusion of the residual pancreatic stump using a fibrin sealant. This approach was utilized in 6 consecutive patients affected by resectable periampullary carcinoma. No postoperative mortality was observed. Pancreatic fistula developed in 5 cases and all of them resolved spontaneously in 1-4 months. The sixth patient underwent, at 3 months p-o, a CT-guided percutaneous aspiration of an intraabdominal fluid collection and with no further complications. 3 patients died at 3, 9 and 11 months because of liver metastases. Currently 3 patients are alive and apparently disease free at 25, 7 and 5 months. Pancreatic endocrine function was assessed in 5 patients at 3 months p-o. Blood glucose and insulin, glucagon and C-peptide plasma levels, all fasting and 1 our after a standard meal, revealed a normal glucose metabolism. The authors conclude that, since fibrin sealant avoids the pancreatic fibrosis which could be induced by non-absorbable polymers and the benign evolution of this type of pancreatic fistula, this method for handling the exocrine secretion is a safe and satisfactory approach which is particularly indicated in case of a pancreatic stump at risk for intestinal anastomoses.
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PMID:[Occlusion of the duct with a fibrin glue and preservation of the pylorus after resection of the duodenum and head of the pancreas for periampullary carcinoma]. 196 1

After an acute episode of pancreatitis, a 63-year-old man was found to have a pancreatic glucagonoma. The tumor was resected without evidence of metastases. Three years later he had symptoms of uncontrolled diabetes, no skin lesions, and diarrhea and was found to have a pancreatic pseudocyst and multiple hepatic metastases. Glucagon concentrations were raised but were suppressible by glucose and somatostatin and responded to arginine stimulation. He was treated for 6 months with octreotide (Sandostatin), which reduced his symptoms; the pseudocyst resolved, but liver metastases continued to grow. Although spontaneous resolution of the pseudocyst is possible, this case appears to illustrate differences in sensitivity of endocrine and exocrine tissues to suppression by Sandostatin.
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PMID:Somatostatin analogue in treatment of coexisting glucagonoma and pancreatic pseudocyst: dissociation of responses. 216 87

Eighty-four carcinoids of the colon and rectum were studied with emphasis on prognostic features, immunohistochemical characteristics, and pitfalls in diagnosis. Follow-up data were available on 35 patients. Tumors with adenocarcinomatous components, or those resembling small cell carcinomas of the lung, were excluded. Eighty-one tumors were in the rectum and three tumors were in the distal sigmoid colon. Neuron-specific enolase, chromogranin, and Leu-7 were positive in 87%, 58%, and 53% of the tumors, respectively. Hormones were positive in the following percentages: serotonin, 45%; pancreatic polypeptide, 46%; glucagon, 10%; gastrin, 3%; somatostatin, 3%; adrenocorticotrophic hormone, 1%; cholecystokinin, 0%; calcitonin, 0%; and insulin, 0%. Many tumors elaborated more than one hormone. Fifty-five percent of the tumors were argyrophil and 28% were argentaffin. Carcinoembryonic antigen was present in 24% of the tumors; 82% of the tumors contained prostatic acid phosphatase. Three patients had liver metastases; their tumors ulcerated, invaded muscularis propria, and had more than 2 mitoses per 10 high-power fields (HPF). One patient with a 2.5-cm tumor without mitoses had regional lymph node metastases. All non-metastasizing tumors had less than one mitosis in 10 HPF. We conclude that large bowel carcinoid tumors are essentially limited to the rectum and sigmoid, that they are indolent if mitotically inactive and smaller than 2 cm, and that most show production of a selected group of endocrine markers.
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PMID:Rectal and colonic carcinoids. A clinicopathologic study of 84 cases. 229 59

In this study, liver metastases from a patient with a pancreatic glucagonoma producing the syndrome have been investigated histologically, ultrastructurally, and immunocytochemically. A comparison has also been made between the metastases and the primary pancreatic tumor investigated in a parallel study. In the metastatic tissue, glucagon-, pancreatic polypeptide (PP)-, and somatostatin-containing cells were found together with a majority of cells without any immunoreactivity. Glucagon-positive cells were much more numerous than PP- and somatostatin-immunoreactive cells. As in the primary tumor, double immunogold staining of ultrathin sections demonstrated the co-existence of glucagon and PP immunoreactivities in most of the granulated cells, but PP immunolabeling was often faint, so that it probably could not be revealed by the PAP method in light microscopical sections. Such a finding, together with the histological and ultrastructural features, is consistent with an ontogenic and phylogenetic primitiveness of the metastatic cell population.
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PMID:A malignant tumor of the pancreas producing glucagonoma syndrome: immunocytochemistry and ultrastructure of liver metastases and comparison with the primary tumor. 254 78

The glucagon-producing pancreatic tumors or glucagonomas are among the rarest forms of islet cell tumors; most are malignant and usually produce a definite clinical syndrome. Mild diabetes mellitus, weight loss, and anemia usually accompany the syndrome. However, only the presence of a peculiar cutaneous rash (necrolytic migratory erythema) and the finding of hyperglucagonemia on assay are reliable diagnostic features of the syndrome. Selective, celiac axis arteriography is the most valuable preoperative technique for localizing these neoplasms and their common liver metastases. Immunohistochemical and ultrastructural examinations are particularly helpful in defining the tumor cell nature (alpha-2 islet cell) and the peptide content (glucagon). When the tumor is benign (less than 30%), complete operative removal results in lasting cure; for malignant forms, surgical therapy is mainly palliative, and adjunctive chemotherapy should be administered. In this report, the importance of clinical recognition and operative and chemotherapeutic responses is illustrated in two patients. In each case, the characteristic dermatitis, diabetes mellitus, weight loss, anemia, and elevated plasmatic glucagon were present. Both patients had their tumors localized by selective angiography and underwent operative removal of the primary pancreatic lesion. In the case of benign glucagonoma, surgical excision was curative. In the malignant one, cytoreductive surgery plus adjunctive chemotherapy (dimethyltriazenomidazole-carboxamide resulted in prolonged survival and significant clinical improvement. Follow-up with serum glucagon assay has been useful in monitoring recurrence.
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PMID:Response of glucagonomas to surgical excision and chemotherapy. Report of two cases and review of the literature. 254 27

We report a case of a 29-yr-old man with a rectal carcinoid that metastasized to the liver, secreting somatostatin. The patient first presented with an abdominal mass and mild diabetes, and, subsequently, rectal tumor and hypersomatostatinemia. Microscopic examination of the rectal tumor showed characteristic features of carcinoid. Multiple liver metastases were recognized. The plasma level of somatostatin was remarkably high (2,839 pg/ml). Plasma gut glucagon and pancreatic polypeptide (PP) increased with progress of the disease. He died of hepatic failure 20 months after his first admission. Immunoperoxidase staining showed that rectal tumor cells contained somatostatin-like-immunoreactivity. Electron microscopy of tumor tissue showed neurosecretory (D-cell) granules. Somatostatin content of the rectal tumor was very high (5,629 pg/mg).
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PMID:Somatostatinoma of the rectum. 257 69

Seven cases of dog islet cell carcinomas were studied by conventional and immunohistochemical light- and electron-microscopy. Antisera to insulin, pancreatic polypeptide, somatostatin and glucagon were used. In 6 tumours several hormones were demonstrated. Glucagon never occurred. Insulin was the only hormone present in every tumour, thus it seems to be a good marker for these neoplasms. Liver metastases contained less immunoreactive cells than primary tumours and cell types found in primary carcinomas were sometimes not present in liver metastases. In two cases a degenerative neuropathy occurred.
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PMID:Islet cell carcinomas in dogs. 298 30

Histologic, immunofluorescence and ultrastructural studies were performed in 17 cases of pancreatic carcinomas induced by the BK virus in Syrian hamsters, a unique model of experimentally induced malignant islet cell tumors. The tumors were composed of small, poorly differentiated cells mostly arranged in a trabecular structure. By immunofluorescence all four islet cell types were found in the tumors, though with different frequency. Insulin cells were present in 16 cases, glucagon cells in 11, somatostatin cells in 7, PP cells in 6. Thirteen tumors contained more than one cell type. Insulin cells were the most frequent cell type in 13 cases, and glucagon cells predominated in 1 case. Insulin-containing cells usually occupied a central position within tumor-cell aggregates, while the other cell types were mostly located in a peripheral position, a distribution reminiscent of that seen in normal islets. Gastrin and calcitonin immunoreactivities were not observed. Immunoreactive cells were more abundant in tumors with trabecular structure. Argyrophil cells revealed by the Grimelius method often exceeded the cumulative number of immunoreactive cells in the same tumor, which suggests that there were additional cell types. Multiple cell types were also found in liver metastases. Ultrastructurally most neoplastic cells were poorly granulated. The occurrence of many damaged cells suggests hormone leakage, which may account, at least in part, for the deregulated hormone release from the tumors.
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PMID:Histologic, immunofluorescence, and ultrastructural study of malignant islet-cell tumors of the pancreas induced in hamsters by BK human papovavirus. 298 71


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