Primary cardiac sarcomas: a retrospective study of the French Sarcoma Group.
Fiche publication
Date publication
janvier 2014
Auteurs
Membres identifiés du Cancéropôle Est :
Dr CHAIGNEAU Loïc, Dr COLLIN Françoise, Pr KURTZ Jean-Emmanuel, Dr RIOS Maria
Tous les auteurs :
Isambert N, Ray-Coquard I, Italiano A, Rios M, Kerbrat P, Gauthier M, Blouet A, Chaigneau L, Duffaud F, Piperno-Neumann S, Kurtz JE, Girard N, Collard O, Bompas E, Penel N, Bay JO, Guillemet C, Collin F, Blay JY, Le Cesne A, Thariat J
Lien Pubmed
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
Référence
Eur J Cancer. 2014 Jan;50(1):128-36