Primary cardiac sarcomas: A multi-national retrospective review
AuthorChen, TW-W; Loong, HH; Srikanthan, A; Zer, A; Barua, R; Butany, J; Cusimano, RJ; Liang, Y-C; Chang, C-H; Iakobishvili, Z; ...
Source TitleCancer Medicine
University of Melbourne Author/sLewin, Jeremy
AffiliationSir Peter MacCallum Department of Oncology
Document TypeJournal Article
CitationsChen, T. W. -W., Loong, 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. (2019). Primary cardiac sarcomas: A multi-national retrospective review. CANCER MEDICINE, 8 (1), pp.104-110. https://doi.org/10.1002/cam4.1897.
Access StatusOpen Access
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.
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