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OBJECTIVE The aim of this study was to outline the surgical management and outcomes for patients diagnosed with intravenous leiomyomatosis with intracardiac extension at a single institution. STUDY DESIGN This was a retrospective review of patients diagnosed with intravenous leiomyomatosis with intracardiac extension between 2002–2008. RESULTS Four patients were identified. The surgical approach in 3 (75%) patients was a single-stage operation. Four (100%) patients presented with cardiac symptoms: 3 (75%) with syncope and 1 (25%) with an abnormal electrocardiogram. Mean age at presentation was 48 years (range, 42–58 years). Complete resection of tumor was obtained in 1 (25%) patient and 3 (75%) patients experienced incomplete resection. Mean follow-up, including surveillance imaging, was 25.5 months (range, 8–57 months) and all 4 patients (100%) are currently free of recurrence. CONCLUSION Surgical excision remains an effective therapy for treating patients with benign metastasizing leiomyomatosis. Incomplete surgical resection may result in favorable response.

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