The concurrence of duodenal epithelioid stromal sarcoma, pulmonary chondromatous hamartoma, and nonfunctioning pancreatic islet cell tumor. A possible analogue of Carney's triad?
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.
Ngadiman
S
S
Department of Pathology, New York Hospital-Cornell Medical Center, New York 10021.
Horenstein
M G
MG
Campbell
W G
WG
D007516:Adenoma, Islet Cell / Q000150:complications* / Q000473:pathology; D004379:Duodenal Neoplasms / Q000150:complications* / Q000473:pathology; D006222:Hamartoma / Q000150:complications* / Q000473:pathology; D006801:Humans; D008171:Lung Diseases / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000150:complications; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000473:pathology; D012509:Sarcoma / Q000150:complications* / Q000473:pathology