Huge primary pleomorphic leiomyosarcoma in the right ventricle with impending obstruction of both inflow and outflow tracts

Primary cardiac leiomyosarcoma is an extremely rare disease entity that is associated with very poor prognosis. We describe here a 45-year-old man who had a huge pleomorphic leiomyosarcoma in the right ventricle (RV) that presented with signs of acute pressure and volume overload and impending obstruction of both outflow and inflow tracts of the RV. The tumor was attached to the RV apex and the interventricular septum, and extended into the main pulmonary trunk just above the pulmonary valve as well as into the right atrium through the tricuspid valve. We evaluated the extent of the tumor using various imaging modalities including transthoracic and transesophageal echocardiography, magnetic resonance imaging, computed tomography, and positron emission tomography-computed tomography. Although he underwent urgent debulking surgery to relieve the obstruction, the tumor could not be resected completely because of its extensive local invasion. The tumor has shown aggressive regrowth after surgery despite adjuvant chemotherapy.