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Article Dans Une Revue European Journal of Cancer Année : 2013

Primary cardiac sarcomas: A retrospective study of the French Sarcoma Group.

Nicolas Isambert
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Isabelle Ray-Coquard
Antoine Italiano
Maria Rios
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Pierre Kerbrat
Mélanie Gauthier
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Aurélien Blouet
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Loïc Chaigneau
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Florence Duffaud
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Sophie Piperno-Neumann
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Jean-Emmanuel Kurtz
Nicolas Girard
Olivier Collard
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Emmanuelle Bompas
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Nicolas Penel
Jacques-Olivier Bay
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Cécile Guillemet
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Françoise Collin
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Jean-Yves Blay
Axel Le Cesne
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Résumé

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 48.8years. 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 51.2months, median overall survival (OS) was 17.2months for the entire cohort, 38.8months after complete resection versus 18.2 after incomplete resection and 11.2months 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.
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hal-00875589 , version 1 (22-10-2013)

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Nicolas Isambert, Isabelle Ray-Coquard, Antoine Italiano, Maria Rios, Pierre Kerbrat, et al.. Primary cardiac sarcomas: A retrospective study of the French Sarcoma Group.. European Journal of Cancer, 2013, epub ahead of print. ⟨10.1016/j.ejca.2013.09.012⟩. ⟨hal-00875589⟩
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