Introduction
Primary cardiac sarcomas (PCS) are rare tumours of dismal prognosis.
Methods
Data of 124 patients with PCS referred to institutions of the French Sarcoma Group (FSG) from 1977 and 2010 were reviewed.
Results
Median age was 48.8 years. PCS were poorly-differentiated sarcomas (N = 45, 36.3%), angiosarcomas (N = 40, 32.3%), leiomyosarcomas (N = 16, 12.9%) and others (N = 23, 18.6%). At diagnosis, 100 patients (80.6%) were localised and 24 (19.4%) metastatic. Tumours were located in the right (N = 47, 38.8%), left atrial cavities (N = 45, 37.2%) or encompassed several locations in nine cases (7.4%). Surgery was performed in 81 cases (65.3%). Heart transplant was performed in five patients. Radiotherapy adjuvant (N = 18, 14.5%) or alone (N = 6, 4.8%) was performed in non-metastatic patients only (N = 24, 19.4%). With a median follow-up of 51.2 months, median overall survival (OS) was 17.2 months for the entire cohort, 38.8 months after complete resection versus 18.2 after incomplete resection and 11.2 months in non-resected patients. Radiotherapy was associated with improved progression-free survival (PFS) on multivariate analysis. Chemotherapy was significantly associated with better OS only in non-operated patients but not in operated patients. In non-metastatic patients, surgery (hazard ratio [HR] = 0.42, p < 0.001), male gender (HR = 0.56, p = .032) was associated with better OS and surgery (HR = 0.61; p = .076), radiotherapy (HR = 0.43; p = .004) and chemotherapy (HR = 0.30, p = .003) improved PFS.
Conclusion
Only surgical resection is associated with a perspective of prolonged survival. Chemotherapy is associated with a better outcome in non-resected patients.
