Recurrent primary cardiac malignant fibrous histiocytoma following orthotopic heart transplantation.

Malignant fibrous histiocytoma (MFH) is an extremely rare primary cardiac tumor. We describe a young patient who underwent orthotopic heart transplantation for an unresectable right ventricular MFH and presented 7 years later with a local recurrence in the native right atrium. This was treated by complete resection of the right atrial tumor and adjuvant chemotherapy. This case represents the only reported long-term survival following cardiac transplantation for MFH and describes our management strategy for local recurrence in this patient.