A rare case of primary leiomyosarcoma of the inferior vena cava with an intra- and extravascular component

Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare vascular tumor. It is frequently seen in the sixth decade of life with a female predominance. The diagnosis is often challenging as patients present with nonspecific complaints such as dyspnea, abdominal discomfort, or back pain. Computed tomography (CT) and magnetic resonance imaging individually or in combination with cavography, ultrasonography, and echocardiography allow an early preoperative diagnosis. Herein, we present a rare case report of primary leiomyosarcoma of the IVC in a 65-year-old female patient who presented with vague abdominal discomfort. Ultrasound of the abdomen detected a mass in the retroperitoneum with mixed echogenicity. CT scan revealed a 10.5 cm × 8.5 cm × 6.3 cm lobulated, heterogeneously enhancing mass with areas of necrosis and involving IVC. The diagnosis of a retroperitoneal mass involving IVC with a possibility of an IVC tumor was made. En bloc resection of the tumor with primary closure of the IVC was performed after careful dissecting from the surrounding structures. Specimen grossly revealed a soft pedunculated tissue mass projecting into the IVC and a large lobulated mass of 13 cm × 12 cm × 10 cm adherent to the surrounding structures. Histopathological report confirmed the diagnosis of primary leiomyosarcoma of the IVC.