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 tha t is related to a high propensity to develop distant metastasis with surviv al rarely exceeding 2 years. We report a case of a patient with a primary c ardiac rhabdomyosarcoma characterized by an exceptional long-term survival after surgical treatment by a total orthotopic heart transplantation From t his 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 rhabdo myosarcoma located in the right atrium and extending to the atrioventricula r groove; therefore, resection was not possible. Since no metastases were d etected, the patient was scheduled for urgent cardiac transplantation, whic h was performed after adjuvant radiotherapy. Results. Postoperative outcome was uneventful and the patient is still aliv e, 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 b e considered for selected patients after rigorous analysis of predictors of survival. (Ann Thorac Surg 2001;71:1438-41) (C) 2001 by The Society of Tho racic Surgeons.