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Report (EN)

2007 (EN)
Glucagonoma (EN)

Christopoulos C.,
Economopoulos P.,

SUMMARY Glucagonomas are rare endocrine pancreatic tumors manifested by a well-known clinical syndrome. They may occur either sporadically (87%) or as part of multiple endocrine neoplasia syndrome type 1 (MEN 1) (13%). Tumors, with some rare exceptions, are located exclusively in the pancreas (mainly in the tail, that is at the distribution site of the glucagon-secreting A cells) and secrete excessive amounts of glucagon. They are usually large and solitary. In the majority of the reported cases, glucagonomas are malignant and there is a close correlation between tumor size and malignancy. Liver and lymph nodes are common sites of metastases. Both sexes appear to be affected with equal frequency and the average age at the time of diagnosis is 52.5 years. Clinical manifestations include a skin rash, termed necrolytic migratory erythema, (although characteristic, it is not pathognomonic, since similar lesions have been reported in other situations), glossitis/stomatitis/chelitis, weight loss or cachexia often associated with anorexia and thromboembolic problems. Diarrhea, ophthalmic, neurologic or psychiatric manifestations have also been reported. Hyperglycagonemia, diabetes or glucose intolerance (73.3%), hypoaminoacidemia (41.2%), normochromic normocytic anemia (33-85%) and often elevated sedimentation rate are the major biochemical findings. Elevated levels of other polypeptide hormones have also been reported. Chronic hyperglycagonemia that produces hypoaminoacidemia, perhaps in combination with other non-known tumor products or metabolic disturbances are responsible for the skin eruption. The diagnosis may be delayed for many years and will be advocated in patients with unexplained chronic dermatitis, particularly if associated with buccomucosal inflammation, diabetes or glucose intolerance, weight loss or unexplained thomboembolic episodes. The existence of hyperglucagonemia (>1000 mg/ml) may establish the diagnosis. The localization of tumor or metastases may be made by imaging methods (mainly be endoscopic U/S and angiography) or by surgical exploration. Surgery (enucleation of the tumor, distal pancreatectomy, major pancreatic resection, debulking of unresectable or metastatic disease) is the treatment of choice. Embolization of hepatic artery, chemotherapy or somatostatin analogues (octerotide or lanreotide) are the alternative solutions. The prognosis, despite the malignant nature of the tumor, is relatively good, due to its slow growth. The 10-year survival rate is 51.6% in patients with metastases and 64.3% in those without metastases. (EN)




Hellenic Society of Gastroenterology (EN)

Annals of Gastroenterology; Volume 14, No 2 (2001) (EN)

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