Current update on IVC leiomyosarcoma

Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare soft tissue sarcoma associated with poor prognosis. Patients are often asymptomatic or present with nonspecific abdominal symptoms, which delays initial diagnosis and contributes to poor oncologic outcome. Key imaging modalities include ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI). Characteristic imaging features include imperceptible caval lumen, dilation of the IVC, heterogeneous enhancement of the tumor, and development of extensive collateral circulation. Surgical resection is the mainstay of treatment, while chemotherapy and/or radiation may serve as therapy adjuncts. This article reviews the pathology, clinical findings, imaging features and management of IVC leiomyosarcoma.