Abstract 1470
Background
Primary cardiac sarcoma (PCS) is a rare but often fatal disease. The current study aimed to analyze the impact of baseline demographics, local and systemic therapies in a contemporary cohort.
Methods
Clinical records of PCS across five institutions in three continents were reviewed and collected. Kaplan-Meier method was used to estimate survival. Cox proportional hazard model was used to associate variables to progression-free survival (PFS) or overall survival (OS).
Results
47 pts with PCS (1996-2016) with a median follow-up time of 12.9 months (ms) were identified. The median age at diagnosis was 41 (range 18-79); 43% (n = 20) presented with metastatic disease. Tumor equally originated from right- (n = 23) and left-sided heart (n = 23). The common histologies were angiosarcoma (n = 18, 38%), intimal sarcoma (n = 8, 17%), and sarcoma NOS (n = 10, 21%). 66% (n = 31) had surgical (S) treatment for PCS, and only 4 (13%) pts had R0 resection. The median primary lesion size was 49 mm (20-84 mm). 70% (n = 33) of pts received at least one line of chemotherapy (C), and 51% (n = 24) received multi-modality treatment (45% S + C, 4% S + XRT, 2% S + C + XRT). The median OS was 17.7 ms (95% CI 12.4-21.8 ms). For all pts, age ≥ 65 was the only significant negative prognostic factor (HR 7.43, p
Conclusions
The prognosis of PCS remains poor without significant improvement in OS compared to historical levels. Further research is required for this rare entity.
Clinical trial identification
Not applicable
Legal entity responsible for the study
Not applicable
Funding
None
Disclosure
All authors have declared no conflicts of interest.