Familial Papillary Serous Carcinoma of the Cervix, Peritoneum, and Ovary: a Report of the First Case

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.