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)

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

A malignant tumor of the pancreas producing the glucagonoma syndrome and associated with high plasma levels of glucagon and pancreatic polypeptide was studied histologically, ultrastructurally, and immunocytochemically. The histologic and ultrastructural features were closely similar to those of previously reported malignant glucagonomas. However, immunolabeling with specific antisera revealed that, in addition to cells having only glucagon- or only pancreatic polypeptide-immunoreactivity, other cells were also present, showing a co-existence of both peptides. These findings indicate that the tumor contains a cell population with a phenotype similar to that of intestinal L-cells rather than to pancreatic A-cells.
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PMID:A malignant tumor of the pancreas producing glucagonoma syndrome: coexistence of glucagon and pancreatic polypeptide (PP) in the tumor cells. 254 79

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 1 patient with a necrolytic migratory erythema, a high plasma glucagon concentration and a metastatic pancreatic endocrine tumor who has now been treated effectively for 33 months with the somatostatin analogue octreotide (SMS 201-995) (400 micrograms/day). The results of SMS 201-995 in the treatment of glucagonoma syndrome are reviewed.
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PMID:Octreotide (SMS 201-995) in the treatment of metastatic glucagonoma: report of one case and review of the literature. 254 11

Necrolytic migratory erythema is the distinctive skin rash of the glucagonoma syndrome. Its presence is virtually pathognomonic of a glucagon-producing pancreatic islet cell neoplasm. Results of a study of a patient with hyperglucagonemia and necrolytic migratory erythema complicating untreated celiac disease are reported. Whereas pancreatic glucagon was only mildly elevated, there was marked elevation of enteroglucagon. Immunofluorescence staining demonstrated numerous (19.6 cells per square millimeter of mucosa) enteroglucagon-positive small intestinal crypt cells. Treatment with gluten-free diet not only resulted in resolution of malabsorption and improvement in small intestinal histology but was paralleled by disappearance of necrolytic migratory erythema, normalization of plasma glucagon levels, and marked reduction in the number of enteroglucagon-producing crypt cells (0.2/mm2 mucosa). The findings demonstrate that necrolytic migratory erythema is not an exclusively paraneoplastic phenomenon and that it can occur in association with excess production of enteroglucagon by the intestinal mucosa.
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PMID:Necrolytic migratory erythema with elevated plasma enteroglucagon in celiac disease. 270 19

A patient with a metastatic islet cell tumour with clinical features of both insulin and glucagon excess is reported. This appears to be only the third such case in the literature and the first in whom the initial symptoms were of the glucagonoma syndrome. Radiologically and biochemically defined complete remission was induced within 9 months of hepatic arterial embolization and cyclical chemotherapy with 5-fluorouracil and streptozocin; complete remission has been maintained for a further 23 months without therapy.
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PMID:Metastatic islet cell tumour with clinical manifestations of insulin and glucagon excess: successful treatment by hepatic artery embolization and chemotherapy. 283 37

A 62-year-old man had necrolytic migratory erythema in association with hyperglucagonemia and multiple hepatic tumors. A diagnosis of metastatic glucagonoma was made. He was treated with human lymphoblastoid interferon given subcutaneously in the dose of 2.8 to 7.1 X 10(6) IU/day. This produced a considerable fall in plasma glucagon and resolution of the associated rash. The treatment was continued for 10 weeks over a 4-month period. Computed tomography demonstrated a reduction in hepatic tumor mass.
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PMID:Hepatic tumors with hyperglucagonemia. Response to treatment with human lymphoblastoid interferon. 284 25

A 53-year-old Japanese man with a skin eruption characteristic of glucagonoma syndrome had had misdiagnoses for 10 years. The plasma glucagon level was not abnormally high on the first admission, and 4 years later the level was elevated as determined by the 30 gm arginine tolerance test. An alpha cell carcinoma detected in the tail of the pancreas was associated with a lymph node metastasis; the skin eruption cleared up 10 days after the partial pancreatectomy with lymphadenectomy. The clinical features of glucagonoma syndrome should be given attention.
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PMID:Glucagonoma syndrome. 284 81

We report a case of pancreatic tumour metastatic to the liver in a patient with insulin-treated diabetes, anaemia, cheilitis, necrolytic migratory erythema, hypokalemia and chronic watery diarrhea, a picture suggesting combined glucagonoma and VIPoma syndromes. Immunocytochemistry of a biopsied hepatic metastatic nodule revealed both glucagon and vasoactive intestinal peptide (VIP) positive cells. Increased plasma glucagon and VIP levels were detected (values of 900 pmol/l and 277 pmol/l respectively). This is the first reported case showing not only immunocytochemical, but also clinical evidence of the combined secretion of these hormones.
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PMID:A combined glucagonoma and VIPoma syndrome. First pathologic and clinical report. 284 62

A case is described that represents the only reported patient with glucagonoma syndrome and morbid obesity. The diagnosis of glucagonoma should be considered in any patient with the classic criteria despite weight gain. The criteria for diagnosis of glucagonoma are 1) the presence of a glucagon-secreting tumor, 2) hyperglucagonemia, and 3) the clinical manifestations of either necrolytic migratory erythema, glucose intolerance, or hypoaminoacidemia.
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PMID:Glucagonoma presenting as morbid obesity. 284 56


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