Primary malignant sarcomas of the heart and great vessels - a single center experience

Abstract

9044 Background: Sarcomas of the heart and the great vessels are very rare entities. The most common subtypes included leiomyo- and angiosarcoma. Prognosis is very poor because of locally advanced or metastatic stage at diagnosis. Methods: Between 6/2001 and 10/2003 12 patients (pts) have been identified in the sarcoma registry of Tuebingen University. Results: Median age was 43 years (22 –72). All pts were symptomatic with reduction of vitality, anorexia, dyspnoea, or neurological symptoms at the time of reception. Tumors were located in atrium [(n=5), left (3)/right (2)], Art. pulmonalis (n=3), Aorta (n=2), pericardium or left chamber, (n=1 each). Six pts presented with localized disease and 5 pts underwent curative intended resection (n=1 considered as irresectable). Six pts had advanced disease including brain mets (4 pts), lymph nodes involvement (2), lung (3) and liver mets (2). Five of these 6 pts underwent palliative resection to relieve the physiologic effects of the tumor. Three out of 5 pts with curative intended surgery have subsequently developed recurrence within 6, 8 and 25 mos despite adjuvant treatment. Palliative chemotherapy including adriamycin and ifosfamide has been applied in 7 pts and 4 pts attained some response to treatment (n=1 PR, n=3 SD). Palliative radiation included 3 pts with brain metastases and a single pt with progressive primary tumor. After a median follow up 13 mos (2 –28) 2 pts had NED, 3 pts are alive with disease and 7 pts have died due to tumor progression. Conclusions: Pts with primary sarcomas of the heart and the great vessels were often of young age, and half of them presented with locally or distant advanced disease. These pts should preferentially be referred to a tertiary Cancer Center immediately after diagnosis without preceding treatment attempts. Although the majority of pts cannot be treated curatively due to the delay in diagnosis, an optimized treatment approach including neoadjuvant chemo-/radiotherapy might enhanced the rate of complete resection depending on the histological subtype and responsiveness to treatment. No significant financial relationships to disclose.