A Rare Case of Primary Leiomyosarcoma of the Ovary in Association with Cellular Leiomyoma of the Broad Ligament and Uterus

Background: Primary ovarian leiomyosarcoma is an extremely rare tumor, contributing to <0.1% of all ovarian malignancies, and its association with cellular leiomyoma of the broad ligament and uterus is an even rarer presentation. There are very few reports of primary ovarian leiomyosarcoma in the literature. Case: This article reports on a case of a 55-year-old postmenopausal woman who presented with complaints of abdominal distention and pain of 5 months' duration, with dyspnea. On examination there was an intra-abdominal mass corresponding to 36 weeks of gestation. Abdominal ultrasonography (USG) revealed a huge abdominopelvic mass with solid and cystic areas. The patient's CA-125 level was 2.7 U/mL (normal 35 U/mL). Total abdominal hysterectomy, with left salpingo-oopherectomy and right ovariotomy, was performed. The broad-ligament fibroid was excised. Samples were sent for histology analysis. Results: On gross examination, the right ovary was found to be enlarged, with a broad ligament and intramural uterine fibroid. Histopathologic examination of the ovary showed features of leiomyosarcoma, while the broad ligament and uterine fibroid showed features of cellular leiomyoma. Conclusions: Primary ovarian leiomyosarcoma of nonteratomatous origin is an extremely rare tumor, with only few case reports existing in the literature. Microscopically, the tumor varies from being well-differentiated to being a highly pleomorphic sarcoma, with many differential diagnoses. A correct diagnosis is required for the recommended treatment, such as radical surgery followed by adjuvant chemotherapy or radiotherapy. (J GYNECOL SURG 30:363)