PHENOTYPIC VARIATION IN HEREDITARY NONPOLYPOSIS COLON CANCER SYNDROME- ASSOCIATION WITH INFILTRATIVE FIBROMATOSIS (DESMOID TUMOR)

Familial infiltrative fibromatosis (desmoid tumor) is a recognized com plication of familial adenomatous polyposis (FAP) but has not been des cribed in families without colonic polyposis. The authors describe a u nique family in which a predisposition to infiltrative fibromatosis an d nonpolyposis colon cancer was inherited dominantly through four gene rations. This report expands the range of phenotypic variation describ ed for the hereditary non-polyposis colon cancer (HNPCC) syndrome and adds to the extracolonic complications that are common with FAP and HN PCC.