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

摘要: Background. Primary cardiac sarcomas are uncommon and rare, with an unequal distribution in the population. A dismal prognosis is usually admitted that related to a high propensity develop distant metastasis survival rarely exceeding 2 years. We report case of patient primary rhabdomyosarcoma characterized by exceptional long-term after surgical treatment total orthotopic heart transplantation. From this limited experience, we reviewed factors may influence optimize therapeutic strategy. Methods. 33-year-old man was found have 10-cm located right atrium extending atrioventricular groove; therefore, resection not possible. Since no metastases were detected, scheduled for urgent transplantation, which performed adjuvant radiotherapy. Results. Postoperative outcome uneventful still alive, regular follow-up, at 102 months. Conclusions. In rhabdomyosarcoma, despite immunosuppressive therapy, can provide be considered selected patients rigorous analysis predictors survival.