Primary angiosarcoma of the heart.

A case report on a 27 year old woman with an inoperable angiosarcoma of the right atrium is presented. The tumor localization was established by echocardiography, computed tomography and cinecardiography. There was no evidence of distant metastases at diagnosis. After exploratory thoracotomy with surgical biopsy a radiotherapy was started covering the whole heart and the mediastinum. Superior Vena Cava Syndrome was improved quickly and reducing the target volume to the right atrium after 40 Gy radiotherapy was continued up to a total dose of 60 Gy. A complete remission was documented by echocardiography and computed tomography. Chemotherapy ("VAPAC") for distant metastases led to partial remission. The patient died 15 months after diagnosis from brain metastases. Autopsy revealed no macroscopic evidence of tumor in the right atrium. A combined modality approach with surgical tumor mass reduction followed by high dose locoregional radiotherapy (60 Gy) and combination chemotherapy (e.g. "VAPAC") is suggested.