Familial peritoneal ovarian carcinomatosis: a new clinical entity?

A familial form of ovarian carcinoma is now widely recognized. There are at least several ovarian cancer-prone genotypes, consistent with genetic heterogeneity. Prophylactic oophorectomy has been employed for women who were judged to be at 50% risk for this disease by virtue of their position in the pedigree. However, recent evidence has disclosed that a fraction of such patients who underwent prophylactic oophorectomy and who had ovaries which appeared to be histologically normal at surgical resection, subsequently developed intraabdominal carcinomatosis with histologic findings showing the lesions to be indistinguishable from ovarian carcinoma. Given the embryologic derivatives of the ovary, which comprise gonadal ridges composed of mesodermal cells covered by coelomic epithelium, we postulate that patients with hereditary predisposition to ovarian carcinoma harbor the first germinal hit in both the epithelial cells of the ovary as well as their derivatives in the coelomic mesothelium. These patients may then be inordinately susceptible to carcinogenesis from the second (somatic) hit in these same tissues.