Familial papillary serous carcinoma of the cervix, peritoneum, and ovary: A report of the first case

Abstract

Objective. We report an occult primary papillary serous carcinoma of the endocervix that was encountered in a woman whose mother and identical twin sister died of papillary serous carcinomas (PSCs) of the peritoneum and ovary, respectively. Methods. The medical records and the histologic material belonging to the patient, her sister, and her mother were reviewed. Results. The cervical PSC was histologically similar to the peritoneal and ovarian carcinomas. The patient has recurred with peritoneal carcinomatosis 24 months following surgery and postoperative radiotherapy and chemotherapy. Conclusions. Primary papillary serous carcinoma of the cervix is a very rare adenocarcinoma variant; there have been approximately 30 such cases reported, and, to our knowledge, this is the first documented case of familial peritoneal/ovarian/uterine papillary serous carcinoma. The prophylaxis achieved through bilateral oophorectomy in individuals with a family history of ovarian cancer does not address the risk of PSCs arising in the uterus, cervix, or peritoneum.