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.
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).
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
The prognosis of PCS remains poor without significant improvement in OS compared to historical levels. Further research is required for this rare entity.
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All authors have declared no conflicts of interest.