Familial ovarian carcinoma has been recognized with increased frequency during the past decade commensurate with physician attention to family history. Putative autosomal dominant inheritance of this heterogeneous problem mandates attention to both paternal and maternal lineages. A family with probable paternal transmission of breast/ovarian carcinoma is presented. One family member had findings consonant with papillary serous adenocarcinoma of ovarian origin, which may have arisen directly from extra-ovarian pelvic mesothelium. These and other clinical nuances of familial ovarian carcinoma are discussed in order to aid physicians in understanding the natural history, surveillance, and management of familial ovarian carcinoma.
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