One-stage surgical removal of intravenous leiomyomatosis with right heart extension is safe.

Intravenous leiomyomatosis is a rare smooth muscle tumor. We report the case of a 42-year-old woman with both intravenous and intracardiac extension of leiomyomatosis who underwent 3 operations within 9 years. During the last admission, she underwent a successful single-stage surgical approach while under cardiopulmonary bypass with circulatory arrest. A postoperative histopathologic examination of the resected specimen confirmed the diagnosis.