Pancreatic alpha cell tumors: case report and review of the literature.

Twenty-eight cases of alpha cell tumors of the pancreatic islets have been reported. The clinical features include typical skin rash (termed migratory necrolytic erythema) and stomatitis with anemia, abnormal glucose tolerance, and weight loss. The time course of the disease is variable but the clinical syndrome may be present for up to 15 years (median five years) before discovery of the tumor. In 3 patients, cure was achieved by surgical resection, and in 17 patients in whom metastatic sites were evaluable, 16 involved the liver. Six patients have received chemotherapy: 4 with streptozotocin (STZ); 1 with dimethyl triazeno imidazole carboxamide (DTIC); and 2 with 5-fluorouracil. All 4 patients receiving STZ responded to therapy with objective regression of the tumor and in 3 the dermatitis syndrome receded as well. The selectivity of the STZ for beta cells observed in animals is contrasted with the specific antitumor activity of STZ for alpha as well as beta and delta cell tumors in man.