The cancer family syndrome. Rare cutaneous phenotypic linkage of Torre's syndrome

Abstract

Sebaceous neoplasia have been observed in members of four families exhibiting the cancer family syndrome (CFS). This disorder is characterized by adenocarcinomas, particularly involving the (proximal) colon, endometrium, and ovary; an excess of multiple primary cancer; early age of cancer onset; and autosomal dominant pattern of inheritance. Multiple adenomatous polyps are lacking in this disorder. In four patients from three of these cancer-prone kindreds, cutaneous lesions were accompanied by multiple visceral adenocarcinomas, fulfilling the criteria for Torre's syndrome, a disease that heretofore has not shown notable familial clustering characteristic of the CFS. Therefore, the coexistence of rare sebaceous neoplasia and visceral cancer in CFS supports the notion that some cases of Torre's syndrome may in fact represent the more full phenotypic expression of the gene responsible for the CFS.