CLINICOPATHOLOGICAL RELEVANCE OF THE ASSOCIATION BETWEEN GASTROINTESTINAL AND SEBACEOUS NEOPLASMS - THE MUIR-TORRE SYNDROME

The association between sebaceous neoplasms of the skin and visceral c ancers, known as Muir-Torre syndrome, is described in three patients, including one with an extensive history of cancer in his family. The f irst patient, a 54-year-old man, developed multiple sebaceous adenomas , epitheliomas, and carcinomas in association with a colonic carcinoma 6 years after cardiac transplantation. Family history in this patient disclosed colon cancer in 17 relatives. The second patient was a 51-y ear-old man who had recurrent adenocarcinoma of the sigmoid colon, ade nocarcinoma arising in Barrett's esophagus, and sebaceous epithelioma during a period of 15 years. The third patient was a 90-year-old man w ith a sebaceous adenoma followed 5 months later by adenocarcinoma of t he sigmoid colon with liver metastases. Muir-Torre syndrome in 129 oth er patients published in the literature is reviewed. Although it is a rare disease, Muir-Torre syndrome requires recognition because skin le sions may be the first sign of the syndrome and this may lead to early diagnosis of associated visceral cancers. Moreover, because this synd rome appears to be inherited, family members should be screened for vi sceral cancer, especially colorectal adenocarcinoma. HUM PATHOL 26:422 -427. Copyright (C) 1995 by W.B. Saunders Company