PATTERNS OF INHERITANCE OF OVARIAN-CANCER - AN ANALYSIS FROM AN OVARIAN-CANCER SCREENING-PROGRAM

Background. A variety of inheritance patterns for familial ovarian can cer have been proposed including an autosomal dominant inheritance, a breast-ovary cancer syndrome and Lynch Cancer Family Syndrome (involvi ng breast, bowel, ovary, and endometrial cancers). Methods. Women part icipating in an ovarian cancer screening study completed a questionnai re concerning their family history of ovarian and other malignancies ( in particular breast, bowel, and endometrial cancer). Confirmation of the diagnosis was sought when there was uncertainty. Results. Two hund red forty women with a first-degree relative with ovarian cancer parti cipated in the study. Nine percent of these women (representing 13 fam ilies) gave a definite history of two or more affected first-degree re latives. Two families had a pedigree consistent with an autosomal domi nant inheritance. A breast-ovary cancer family and a Lynch cancer fami ly syndrome were suspected in one family each, although 34% of all wom en gave a history of at least one other first-degree relative with eit her breast, bowel, or endometrial cancer. Conclusions. Only a small nu mber of women with a family history of ovarian cancer fit into the rec ognized hereditary patterns. Difficulty in recognizing the inheritance patterns and the lack of definitive genetic markers poses problems in providing adequate counseling regarding screening and prophylactic oo phorectomy.