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.
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.
Authors: Uladzimir U Andrushchuk; Youry P Ostrovsky; Andrei V Valentsiukevich; Liana G Shestakova; Siarhei G Amelchanka; Valery G Krutau; Olga A Yudina; Pavel F Chernoglaz; Irina I Grinchuk; Andrei Smalenski Journal: Kardiochir Torakochirurgia Pol Date: 2017-12-20