We report on our experience with six patients with malignant sarcoma of the heart and show that long-term survival is possible after radical resection. At presentation, the 6 patients (3 male, 3 female) were 45.8 +/- 20 years old. Three patients are alive without evidence of metastases since 29.6 +/- 36.8 months, three patients died after 38 +/- 50.2 months due to distant metastases. Precise preoperative localization of the tumor by means of imaging techniques is very important. In some cases, radical surgery requires an ex situ procedure (autotransplantation). If necessary, the right heart can be resected almost completely, and reconstructed in the form of a Fontan-type circulation. A heart transplantation, as suggested by others, is not justified from our point of view, since prognosis is not better and donor organs are too rare. The results of radical resection are promising, but new concepts for treatment--in particular chemotherapy concepts--for these mostly middle-aged or young patients are required.