TY - JOUR
T1 - Primary cardiac sarcomas
T2 - A retrospective study of the French Sarcoma Group
AU - Isambert, Nicolas
AU - Ray-Coquard, Isabelle
AU - Italiano, Antoine
AU - Rios, Maria
AU - Kerbrat, Pierre
AU - Gauthier, Mélanie
AU - Blouet, Aurélien
AU - Chaigneau, Loïc
AU - Duffaud, Florence
AU - Piperno-Neumann, Sophie
AU - Kurtz, Jean Emmanuel
AU - Girard, Nicolas
AU - Collard, Olivier
AU - Bompas, Emmanuelle
AU - Penel, Nicolas
AU - Bay, Jacques Olivier
AU - Guillemet, Cécile
AU - Collin, Françoise
AU - Blay, Jean Yves
AU - Le Cesne, Axel
AU - Thariat, Juliette
PY - 2014/1/1
Y1 - 2014/1/1
N2 - 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.
AB - 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.
KW - Cardiac
KW - Chemotherapy
KW - Radiotherapy
KW - Sarcoma
KW - Surgery
UR - http://www.scopus.com/inward/record.url?scp=84891630248&partnerID=8YFLogxK
U2 - 10.1016/j.ejca.2013.09.012
DO - 10.1016/j.ejca.2013.09.012
M3 - Article
C2 - 24135684
AN - SCOPUS:84891630248
SN - 0959-8049
VL - 50
SP - 128
EP - 136
JO - European Journal of Cancer
JF - European Journal of Cancer
IS - 1
ER -