Total orthotopic heart transplantation for primary cardiac rhabdomyosarcoma: factors influencing long-term survival.

BACKGROUND: Primary cardiac sarcomas are uncommon and rare, with an unequal distribution in the population. A dismal prognosis is usually admitted that is related to a high propensity to develop distant metastasis with survival rarely exceeding 2 years. We report a case of a patient with a primary cardiac rhabdomyosarcoma characterized by an exceptional long-term survival after surgical treatment by a total orthotopic heart transplantation. From this limited experience, we reviewed factors that may influence survival to optimize therapeutic strategy.

METHODS: A 33-year-old man was found to have a 10-cm primary cardiac rhabdomyosarcoma located in the right atrium and extending to the atrioventricular groove; therefore, resection was not possible. Since no metastases were detected, the patient was scheduled for urgent cardiac transplantation, which was performed after adjuvant radiotherapy.

RESULTS: Postoperative outcome was uneventful and the patient is still alive, with regular follow-up, at 102 months.

CONCLUSIONS: In a case of primary rhabdomyosarcoma, heart transplantation, despite immunosuppressive therapy, can provide long-term survival and can be considered for selected patients after rigorous analysis of predictors of survival.