Extraovarian Primary Peritoneal Carcinoma

Extraovarian primary peritoneal carcinoma (EOPPC), a relatively newly defined disease that develops only in women, accounts for approximately 10% of cases with a presumed diagnosis of ovarian cancer. Characterized by abdominal carcinomatosis, uninvolved or minimally involved ovaries, and no identifiable primary, EOPPC has been reported following bilateral oophorectomy performed for benign disease or prophylaxis. Most cases are of serous histology; however, nonserous tumors have been observed. Although EOPPC is similar to serous ovarian carcinoma with respect to clinical presentation, histologic appearance, and response to chemotherapy, molecular and epidemiologic studies have indicated that it may be a separate entity. This review explores the clinical presentation, management, prognosis, and survival of EOPPC.

Extraovarian primary peritoneal carcinoma (EOPPC) is an adenocarcinoma that develops from the peritoneum lining the pelvis and abdomen and is characterized by abdominal carcinomatosis, uninvolved or minimally involved ovaries, and no identifiable primary tumor. This relatively newly defined disease entity occurs exclusively in women and has been reported following bilateral oophorectomy performed for benign disease or prophylaxis.[1] It accounts for approximately 10% of cases with a presumed diagnosis of ovarian cancer.[2]

Although most cases of EOPPC are of serous histology, nonserous tumors have been reported.[3] Different investigators have referred to EOPPC as serous surface papillary carcinoma,[1,4,5] primary peritoneal carcinoma,[6] peritoneal mesothelioma,[7] multiple focal extraovarian serous carcinoma,[8] primary peritoneal papillary serous adenocarcinoma,[3] serous surface carcinoma of the peritoneum,[9] extraovarian peritoneal serous papillary carcinoma,[10,11] extraovarian mllerian adenocarcinoma,[12] normal-sized ovary carcinoma syndrome,[13] papillary serous carcinoma of the peritoneum,[14-16] and peritoneal papillary carcinoma.[17]

Extraovarian primary peritoneal carcinoma is similar in clinical presentation, histologic appearance, and response to serous ovarian carcinoma. However, molecular and epidemiologic studies[18,19] suggest that EOPPC may be a separate entity. This review examines the current literature on EOPPC, with an emphasis on its clinical presentation, management, prognosis, and survival.

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