Abstract
BackgroundPrimary 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.MethodsClinical records of PCS across six institutions in three continents were reviewed. Kaplan‐Meier method was used to estimate survival. Cox proportional hazard model was used to determine variables impacting progression‐free survival (PFS) or overall survival (OS).ResultsSixty‐one patients with PCS (1996‐2016) were identified. The median age at diagnosis was 46 (range 18‐79); 36% (n = 22) presented with metastatic disease. The most common histology was angiosarcoma (n = 24, 39%). A total of 46 patients received surgery (75%) but only 5 (8%) patients achieved R0 resection. Multi‐modality treatment to the primary tumor was given to 28 patients (46%; localized disease 23/39 (59%); metastatic disease 5/22 (23%)). The median OS for the entire cohort was 17.5 months (95% CI 9.5‐20.6), with seven (11%) patients surviving longer than 36 months. On multi‐variate analysis, age <65 (P = 0.01) was the only significant favorable prognostic factor. For first‐line palliative chemotherapy, the median PFS was 4.4 months (95% CI 2.9‐7.7 months). The best response for first‐line chemotherapy was 32% (CR = 1, PR = 9). No significant improvement in OS was identified in patients presenting throughout the 20‐year period of this review.ConclusionYounger age at diagnosis was associated with improved outcome although the prognosis of PCS remains poor. Given the lack of improvement in survival, further dedicated research is required.
Highlights
Soft tissue sarcomas (STS) are a heterogeneous collection of rare tumors of mesenchymal origin which represent less than 1% of adult cancers.[1]
No significant improvement in overall survival (OS) was identified in patients presenting throughout the 20‐year period of this review
For patients presenting with localized disease, multi‐modality treatment (MMT) was given to 23 patients (59%; surgery and chemotherapy (n = 16, 70%); surgery and radiation (n = 2, 8%); surgery, radiation, and chemotherapy (n = 5, 22%))
Summary
Soft tissue sarcomas (STS) are a heterogeneous collection of rare tumors of mesenchymal origin which represent less than 1% of adult cancers.[1] Management of STS is challenging due to their wide variety of histological sub‐types with differing clinical, phenotypical, and genomic characteristics that impact their sensitivity to treatment.[2] Primary cardiac sarcoma (PCS) is an extremely rare subset of STS, with an estimated incidence of 0.001%‐0.03%.3-5. It is distinguished from a diverse and more common group of other cardiac tumors, including benign tumors (ie, myxomas), and secondary neoplasms metastasizing to the heart (eg, lung, breast, renal, melanoma), by arising from pluripotent mesenchymal cells within the heart.[6]. The median OS for the entire cohort was 17.5 months (95% CI 9.5‐20.6), with seven (11%) patients surviving longer than
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