Hereditary Carcinomas of the Ovary, Fallopian Tube, and Peritoneum

Approximately 10% of ovarian cancers are associated with inherited germline mutations, most commonly of the BRCA1 or BRCA2 genes. The majority of BRCA1 and BRCA2 cancers are high-grade serous carcinomas diagnosed at an advanced stage, and there are as yet no histologic features that distinguish these tumors from sporadic serous cancers. Many women identified as being at high genetic risk undergo prophylactic salpingo-oophorectomy, and careful histopathological examination of these specimens may identify occult carcinoma, frequently in the distal fallopian tube. In addition, serous cancer precursors, including tubal intraepithelial carcinoma, have been increasingly recognized in distal and fimbrial epithelium. Little has been documented to date of the histopathological features of the cancers associated with the hereditary nonpolyposis colon cancer syndrome, but it appears these ovarian cancers may include a variety of histologic types, and in contrast to the BRCA cancers, are low grade and early stage.