@article{2a558f934f3748108ce057c481a21f45,
title = "Primary cardiac sarcomas: A multi-national retrospective review",
abstract = "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 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.",
keywords = "primary cardiac sarcoma, radiotherapy, sarcoma chemotherapy, surgery",
author = "Chen, {Tom Wei Wu} and Loong, {Herbert H.} and Amirrtha Srikanthan and Alona Zer and Reeta Barua and Jagdish Butany and Cusimano, {Robert J.} and Liang, {Yun Chieh} and Chang, {Chin Hao} and Zaza Iakobishvili and Razak, {Albiruni R.Abdul} and Jeremy Lewin",
note = "Publisher Copyright: {\textcopyright} 2018 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.",
year = "2019",
month = jan,
doi = "10.1002/cam4.1897",
language = "אנגלית",
volume = "8",
pages = "104--110",
journal = "Cancer Medicine",
issn = "2045-7634",
publisher = "John Wiley and Sons Inc.",
number = "1",
}