Objective: The prognosis for patients with primary cardiac sarcoma is poor. Median survival is less than 10 months, especially when complete surgical excision is not feasible. Removal of all cardiopulmonary structures involved by tumor followed by orthotopic allotransplantation has been proposed to improve long-term survival.
Methods: From 1996 through 1999, we performed combined heart and lung resection followed by en bloc heart and bilateral lung transplantation in 4 patients (2 men and 2 women): 2 with inoperable pulmonary arterial sarcoma and 2 with left atrial sarcoma extending into the pulmonary vein.
Results: Median age at diagnosis was 39 years (range 37-45 years). All 4 patients were given chemotherapy before transplantation: doxorubicin and ifosfamide in 2 cases, and doxorubicin, ifosfamide, mesna, and dacarbazine in 2 cases. There were no operative deaths. Median survival after transplantation was 31 months (range 5-49 months). All patients had tumor recurrence: local recurrence in the chest (n = 1) and distant metastases in the brain (n = 2) and abdomen (n = 1). One patient remains alive 49 months after disease progression with cerebral metastasis as the only site of recurrence treated with whole-brain irradiation, resection, and stereotactic radiosurgery.
Conclusions: Combined heart and lung transplantation is a technically feasible treatment for highly selected patients with localized advanced primary cardiac sarcomas. The high incidence of metastatic disease, however, limits its utility.