1490P - Primary cardiac sarcoma (PCS): A Multi-national Retrospective Review of Clinical Experience

Date 11 September 2017
Event ESMO 2017 Congress
Session Poster display session
Topics Soft Tissue Sarcomas
Sarcoma
Presenter Tom Chen
Citation Annals of Oncology (2017) 28 (suppl_5): v521-v538. 10.1093/annonc/mdx387
Authors T.W. Chen1, H.H. Loong2, A. Zer3, R. Barua4, J. Buttany5, R.J. Cusimano6, Y. Liang7, C. Chang8, Z. Iakobishvili3, A.R.A. Razak9, J. Lewin9
  • 1Department Of Oncology, National Taiwan University Hospital, 100 - Taipei/TW
  • 2Department Of Clinical Oncology, The Chinese University of Hong Kong, 00000 - Hong Kong/HK
  • 3Rabin Medical Center, Tel Aviv University, Tel Aviv - Tel Aviv/IL
  • 4Department Of Medicine, University of Toronto, Toronto/CA
  • 5Division Of Pathology, University Health Network, Toronto/CA
  • 6Division Of Cardiovascular Surgery, University Health Network, Toronto/CA
  • 7National Taiwan University College Of Public Health, Institute of Epidemiology and Preventive Medicine, Taipei/TW
  • 8Department Of Medical Research, National Taiwan University Hospital, Taipei/TW
  • 9Department Of Medical Oncology And Hematology, Princess Margaret Cancer Centre, Toronto/CA

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 five institutions in three continents were reviewed and collected. Kaplan-Meier method was used to estimate survival. Cox proportional hazard model was used to associate variables to progression-free survival (PFS) or overall survival (OS).

Results

47 pts with PCS (1996-2016) with a median follow-up time of 12.9 months (ms) were identified. The median age at diagnosis was 41 (range 18-79); 43% (n = 20) presented with metastatic disease. Tumor equally originated from right- (n = 23) and left-sided heart (n = 23). The common histologies were angiosarcoma (n = 18, 38%), intimal sarcoma (n = 8, 17%), and sarcoma NOS (n = 10, 21%). 66% (n = 31) had surgical (S) treatment for PCS, and only 4 (13%) pts had R0 resection. The median primary lesion size was 49 mm (20-84 mm). 70% (n = 33) of pts received at least one line of chemotherapy (C), and 51% (n = 24) received multi-modality treatment (45% S + C, 4% S + XRT, 2% S + C + XRT). The median OS was 17.7 ms (95% CI 12.4-21.8 ms). For all pts, age ≥ 65 was the only significant negative prognostic factor (HR 7.43, p 

Conclusions

The prognosis of PCS remains poor without significant improvement in OS compared to historical levels. Further research is required for this rare entity.

Clinical trial identification

Not applicable

Legal entity responsible for the study

Not applicable

Funding

None

Disclosure

All authors have declared no conflicts of interest.