Abstract
Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is an extremely rare malignancy with <400 cases reported. We present a 42-year-old woman with a 3-day history of vague and non-specific abdominal pain. Examination revealed mild tenderness to the epigastrium and right upper quadrant with no other findings. Abdominal ultrasound was performed, which revealed a large hypoechoic mass overlying the IVC. Abdominal computed tomography (CT) was performed which revealed an 8.9 × 7.9 × 9 cm multilobulated lesion encasing the IVC. A CT-guided biopsy was performed which revealed a primary LMS of the IVC. Surgical en bloc excision was performed with an end-to-end Dacron graft for IVC reconstruction. Histopathology confirmed LMS of the vessel wall with negative surgical margins.