We report a family manifesting the
cancer-family syndrome in which 11 family members had colonic
carcinomas (predominantly involving the proximal colon, in the absence of polyposis), with an average age at onset of 35 years. Three women had endometrial or endocervical
cancers. The kindred is notable in that its full evaluation was predicated upon the recognition of features consistent with the
cancer-family syndrome in only two sisters. The ascertainment and evaluation of the kindred demonstrates the clinical utility of regarding such criteria (early
cancer onset, multiple primary
cancers, proximal colonic involvement) as a basis for selecting cases for more thorough family-history evaluation. Although such selection criteria are not pathognomonic for the syndrome, identification of a more extensive family
cancer history sometimes enables the initiation of a highly specific
cancer surveillance program. Specific attention has been given to the problems of screening patients at risk for the development of proximal
colonic cancer, an important feature of the
cancer-family syndrome. Innovative operative management is also indicated, such as total
colectomy for initial
colonic cancer, and consideration of prophylactic
hysterectomy for women with
colonic cancer (because of the high risk of development of
endometrial carcinoma).