Glucagonoma syndrome. A clinicopathologic, immunocytochemical, and ultrastructural study

Necrolytic migratory erythema has been considered to be a paraneoplastic dermatosis. Although some individuals have had no associated neoplasm, a pancreatic glucagonoma is typically found in the vast majority. We describe a 71-year-old woman with findings strongly suggestive of the glucagonoma syndrome. The plasma glucagon level was modestly elevated. Special studies of the pancreas, including computerized axial tomography and selective angiography, did not demonstrate a tumor. Surgery, however, revealed a 5-cm tumor in the head of the pancreas. Although immunocytochemical stains showed only rare, weakly reactive cells, the electron microscopic study confirmed the diagnosis of an islet cell tumor and revealed characteristic morphologic features of glucagonoma.