WEI-WU CHENLoong H.H.Srikanthan A.Zer A.Barua R.Butany J.Cusimano R.J.Liang Y.-C.Chang C.-H.Iakobishvili Z.Razak A.R.A.Lewin J.2022-03-152022-03-1520192045-7634https://www.scopus.com/inward/record.uri?eid=2-s2.0-85060546100&doi=10.1002%2fcam4.1897&partnerID=40&md5=02a93a82949a60a3cd822eef7e95c905https://scholars.lib.ntu.edu.tw/handle/123456789/597544Background: 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 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). Results: Sixty-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. Conclusion: Younger 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. ? 2018 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.primary cardiac sarcoma; radiotherapy; sarcoma chemotherapy; surgery[SDGs]SDG3adult; age; aged; angiosarcoma; Article; cancer chemotherapy; cancer prognosis; cardiovascular system cancer; clinical feature; female; histology; human; major clinical study; male; overall survival; palliative therapy; primary cardiac sarcoma; primary tumor; priority journal; progression free survival; retrospective study; survival rate; trend study; tumor localization; tumor volume; adolescent; heart tumor; middle aged; pathology; sarcoma; survival analysis; treatment outcome; young adult; Adolescent; Adult; Aged; Female; Heart Neoplasms; Humans; Male; Middle Aged; Retrospective Studies; Sarcoma; Survival Analysis; Treatment Outcome; Tumor Burden; Young Adultjournal article10.1002/cam4.1897305753092-s2.0-85060546100