Glucagonomas

Glucagonomas are among the rarest of the pancreatic endocrine tumors. Approximately 80% of cases are sporadic, and 20% are associated with MEN1. Although glucagonomas may be associated with diabetes mellitus, clinically significant hyperglycemia occurs in only half of such patients. Patients with glucagonomas are in fact frequently diagnosed by a dermatologist, after presenting with necrolytic migratory erythema. This rash, characterized by raised erythematous patches beginning in the perineum and subsequently involving the trunk and extremeties, is found in over two thirds of all patients.

Somatostatin analogs are generally successful in the initial management in patients with the glucagonoma syndrome. Patients who are refractory to somatostatin analogs may benefit from the intravenous infusion of amino acids. Surgical resection may be performed with curative intent in the rare patient with localized disease. More often, however, surgery is performed, to diminish systemic symptoms in patients with metastases.

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