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.