Abstract
We present a case of a 70-year-old female with Leiomyosarcoma (LMS) of the inferior vena cava (IVC). Although this is an extremely rare entity, in contradistinction, it is also the most common primary malignancy of the IVC [5]. The patient has a history of sarcoidosis, hypertension, diabetes mellitus type two, and chronic obstructive pulmonary disease (COPD). She presented with a complaint of bilateral lower extremity edema and was admitted where a computerized tomography (CT) scan of the abdomen and pelvis showed a large mass filling the IVC, a finding confirmed by magnetic resonance imaging. Radical resection of the retroperitoneal tumor was carried out including portions of the inferior vena cava with en bloc radical right nephrectomy and right adrenalectomy. The pathologic diagnosis of inferior venal caval leiomyosarcoma (IVC LMS) was made with positive immunostains for desmin, vimentin and smooth muscle actin. The rarity of this entity, clinical presentation along with concomitant sarcoidosis makes this an interesting case.