Ovarian cancer and Mayer-Rokitansky-Kuster-Hauser syndrome

A 48-year-old woman with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, right kidney aplasia, a pelvic-abdominal mass, and an elevated CA-125 level underwent bilateral salpingo-oophorectomy, omentectomy, and debulking for a presumed ovarian carcinoma. Intraoperative findings included a pelvic tumor on the surface of both ovaries. Pathological examination revealed a poorly differentiated ovarian carcinoma, mixed type, mainly of transitional and serous types, with minor components of clear cell and mucinous patterns. A stage III ovarian epithelial carcinoma, mixed type was diagnosed. The patient was treated with Paclitaxel and Carboplatin and was asymptomatic 24 months postoperatively. Rarely, ovarian carcinoma may be associated with MRKH syndrome.