Leiomyosarcomas are an uncommon malignant subset of tumors accounting for approximately 20% of soft tissue sarcomas. Primary vascular leiomyosarcomas (PVLs) are a rare subset of leiomyosarcomas that may originate in the arterial or venous circulation but most commonly affect the inferior vena cava (IVC). PVLs more commonly affect women to men in a 2:1 ratio and most frequently occur in the fourth to sixth decades of life. Few reports have described this infrequent pathologic state in the setting of advanced pregnancy. Presented is a case of a 44-year-old 30-week pregnant woman who presented with a PVL of the retrohepatic IVC, which was complicated by occlusion of the IVC and tumor thrombus extension into the hepatic veins and right atrium. Herein, we describe our multidisciplinary management of this rare problem with successful surgical resection of her tumor and IVC reconstruction.
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