TY  - JOUR
AU  - Chen, TW-W
AU  - Loong, HH
AU  - Srikanthan, A
AU  - Zer, A
AU  - Barua, R
AU  - Butany, J
AU  - Cusimano, RJ
AU  - Liang, Y-C
AU  - Chang, C-H
AU  - Iakobishvili, Z
AU  - Razak, ARA
AU  - Lewin, J
Y2  - 2020/12/10
Y1  - 2019/01/01
SN  - 2045-7634
UR  - http://hdl.handle.net/11343/253782
AB  - 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.
LA  - English
PB  - WILEY
T1  - Primary cardiac sarcomas: A multi-national retrospective review
DO  - 10.1002/cam4.1897
IS  - Cancer Medicine
VL  - 8
IS  - 1
SP  - 104-110
L1  - /bitstream/handle/11343/253782/PMC6346258.pdf?sequence=1&isAllowed=y
ER  -