Abstract
Background Primary cardiac sarcoma (CS) is an extremely rare disease. This study aims to identify
possible prognostic factors for long-term survival.
Methods A total of 17 consecutive patients who were treated for primary CS between 2003 und
2018 at two cardiac centers were investigated. Clinical data and histological characteristics
of the tumors were analyzed. Long-term follow-up of all patients were performed.
Results The median age was 54 years (range: 23–74). The tumors originated from the left side
of the heart in nine patients. Histologically, there were four angiosarcomas, three
intimal sarcomas, and three synovial sarcomas. One- and 7-year survivals were 81.9
and 18.2%, respectively. Low expression levels of Ki-67 tended to be associated with increased survival (log-rank p = 0.06). Adjuvant chemotherapy but not radiotherapy regardless of existing metastases
was associated with significantly increased survival (log-rank p = 0.001).
Conclusion Angiosarcoma was the most common type of CS. The survival of CS patients is poor
but prognostic factors, such as Ki-67, may help estimate the course of the disease. Survival could be improved significantly
with chemotherapy.
Keywords
sarcoma - outcomes - histopathology
