[Glucagon-secreting malignant neuroendocrine tumor of the pancreas].

Background: Glucagonoma is a rare pancreatic tumor of islet alpha 2 cells. Fewer than 200 cases have been reported worldwide, with an estimated incidence of 1 in 20 million. In general, the disease is characterized by a well-defined clinical syndrome which typically shows as necrotic migratory erythema of the skin, weight loss, diabetes mellitus, anemia, cheilosis and stomatitis.