Primary pleomorphic liposarcoma involving bilateral ovaries: Case report and literature review

Abstract Pleomorphic liposarcomas (PLPSs) commonly occur in the extremities or retroperitoneum. However, cases of primary PLPSs in the female reproductive system are rare, with only one reported case in the ovary. Herein, we describe the case of a patient with primary PLPS involving bilateral ovaries. She presented with a 2-month history of abdominal pain, and underwent total hysterectomy with bilateral salpingo-oophorectomy, omentectomy, and excision of surface lesions on the bladder and rectum. Intraoperatively, nitrogen mustard (2%) was used to rinse the abdominal cavity. One week postoperatively, she was administered the first cycle of postoperative cisplatin chemotherapy for intraperitoneal heat infusion chemotherapy plus intravenous liposomal paclitaxel. Postoperative histopathological examination revealed primary PLPS involving both ovaries. Therefore, a doxorubicin liposomal chemotherapy regimen was administered according to the soft tissue sarcoma NCCN guidelines. The patient’s CA-125 levels decreased from 987 to 9.8 U/mL; however, after two chemotherapy sessions, she declined further treatment. The patient was still being followed-up and had no signs of recurrence at the time of writing this report. PLPS tends to be misdiagnosed and underdiagnosed due to its complex pathology and the lack of specific molecular markers. The disease is infrequent in the female reproductive system, and there is no consensus on its diagnostic and therapeutic guidelines. Herein, we summarized the findings of published case reports of PLPSs in organs of the female reproductive system to raise awareness of the disease and discussed its diagnosis, clinical treatment, and prognosis.