Multimodality treatment of primary cardiac angiosarcoma: A systematic literature review

Primary cardiac angiosarcoma (PCA) is the most prevalent histological type of cardiac sarcoma but its rarity poses a challenge for standardizing treatment protocols. Moreover, published studies are limited by small patient numbers and lack of randomization, making it challenging to establish evidence-based treatment strategies. This systematic review aims to consolidate the heterogeneous published data and identify factors related to the treatment outcome of PCA patients.The PubMed and Scopus bibliographic databases were systematically searched for original articles reporting clinical, treatment and outcome data for PCA patients. Kaplan-Meier analysis was used to calculate the time to progression and survival. The Log-Rank test was used to compare progression-free and overall survival data. The Cox proportional hazards regression model was used for univariate and multivariate analysis of survival data.A total of 127 studies containing data for 162 patients were analyzed. The median age of the patient cohort was 45 years, with males being 1.5 times more frequently affected than females. Tumors were primarily located on the right side of the heart, with a median size of 6 cm. Median progression-free and overall survival of 5 months and 12 months, respectively, were calculated. Age, sex, and resection margins did not have a significant impact on PCA survival, as determined by both univariate and multivariate analyses. The presence of metastases at diagnosis was associated with lower overall survival in univariate analysis, although this effect was not significant in multivariate analysis. Multimodality treatment that incorporated surgery and adjuvant chemo-radiotherapy was associated with a statistically significant survival benefit. Median overall survival increased from 6 months with surgery alone to 13 months and 27 months with adjuvant chemotherapy and chemo-radiotherapy, respectively.Multimodality treatment including surgery and chemo-radiotherapy was found to offer the greatest survival benefit for PCA patients.