Glucagonomas

Glucagonomas are rare tumors originating from the alpha cells of the pancreatic islets. Their incidence is estimated to be approximately 1 per 20 to 30 million person-years. There is a slight female predominance, with a mean age at diagnosis of 55 years. Glucagonomas generally present with a pathogno-monic rash called necrolytic migratory erythema. This desquamating pruritic rash usually begins on the extremities and intertriginous regions before

Figure 15-5. A small submucosal duodenal gastrinoma is enucleated.

involving the trunk and face (Figure 15-6). These patients may have been treated for psoriasis, pemphigus, zinc deficiency, or eczema for several years before the rash is recognized for what it is, prompting a proper diagnosis. These patients also have mild to moderate diabetes mellitus associated with anemia, weight loss, glossitis, and thrombophlebitis. The diagnosis can be proved by demonstration of an elevated serum glucagon level.

Most (88%) glucagonomas are solitary lesions and occur in the body or tail of the pancreas (Figures 15-7 and 15-8). Computed tomography (CT) or MRI is accurate and sensitive in the search for a primary lesion and evaluation of the liver. Angiography is generally reserved for patients in whom a tumor is not otherwise seen and is accurate in at least 80% of these tumors. There are no known provocative agents for a stimulation test. Complete resection of localized benign tumors is curative and results in rapid resolution of the necrolytic rash (Figure 15-9). Subtotal distal pancreatectomy is recommended for the rare cases caused by hyperplasia of the islets in the pancreatic tail.33 Seventy percent of these patients will unfortunately present with liver metastases. Surgical debulking can result in symptom improvement and may be indicated if significant reduction of the tumor mass can be achieved.

Figure 15-6. The pathognomonic rash for glucagonoma is necrolytic migratory erythema and is depicted here.
Figure 15-7. This patient's glucagonoma is identified in the tail of the pancreas near the spleen.

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