Adnexal leiomyosarcoma with intravascular extension to the inferior vena cava junction: a case report

Primary adnexal leiomyosarcoma is an extremely rare and aggressive malignancy. Only a few cases with intravascular extension into the ovarian vein or the inferior vena cava have been reported. When feasible, complete surgical resection offers the best chance for favorable outcomes. A 64-year-old Japanese woman (gravida 3, para 2) was found to have a large solid adnexal tumor with central necrosis and intravascular extension from the ovarian vein to the junction of the inferior vena cava on contrast-enhanced CT and MRI. A CT-guided biopsy confirmed the diagnosis of leiomyosarcoma. At laparotomy, a 20-cm right adnexal tumor extending into the right ovarian vein without invasion of the surrounding organs was identified. Gentle traction on the tumor enabled ligation and transection of the ovarian vein immediately proximal to its junction with the inferior vena cava. The tumor was successfully resected en bloc together with the right ovarian vein. Total hysterectomy, left salpingo-oophorectomy, and partial omentectomy were also performed. The resected specimen showed that the right ovary had been completely replaced by the tumor, which extended into the ovarian vein. Following surgery, the patient received six cycles of adjuvant docetaxel and gemcitabine chemotherapy. At 18 months after surgery, she remains recurrence-free and is under regular follow-up. We successfully achieved complete en bloc resection of a rare primary adnexal leiomyosarcoma with tumor thrombus extending from the ovarian vein to the junction with the inferior vena cava. In general, primary adnexal leiomyosarcoma is associated with a poor prognosis. When feasible, complete surgical resection remains the treatment of choice and may require a multidisciplinary surgical approach.