The concurrence of duodenal epithelioid stromal sarcoma, pulmonary chondromatous hamartoma, and nonfunctioning pancreatic islet cell tumor. A possible analogue of Carney's triad?

Abstract

A 63-year-old man with non-insulin-dependent diabetes mellitus and peripheral vascular disease presented with acute pulmonary edema and ascites. He died after a brief illness. Coincidence of duodenal epithelioid stromal sarcoma, pulmonary chondromatous hamartoma, and pancreatic islet cell tumor was found at autopsy. This rare concurrence of three uncommon neoplasms may be a pathogenetic variant or an analogue of Carney's triad in an elderly individual. Since all three tumors expressed markers for neural differentiation, the coincidence of tumors with these features may represent a form of neurocristopathy.