Published June 28, 2018
| Version v1
Journal article
Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study
Creators
- Bompas, Emmanuelle
- Campion, Loïc
- Italiano, Antoine
- Le Cesne, Axel
- Chevreau, Christine
- Isambert, Nicolas
- Toulmonde, Maud
- Mir, Olivier
- Ray-Coquard, Isabelle
- Piperno-Neumann, Sophie
- Saada-Bouzid, Esma
- Rios, Maria
- Kurtz, Jean-Emmanuel
- Delcambre, Corinne
- Dubray-Longeras, Pascale
- Duffaud, Florence
- Karanian, Marie
- Le Loarer, Francois
- Soulié, Patrick
- Penel, Nicolas
- Blay, Jean-Yves
Contributors
Others:
- CRLCC René Gauducheau
- Institut Bergonié [Bordeaux] ; UNICANCER
- Sarcome ; Département de médecine oncologique [Gustave Roussy] ; Institut Gustave Roussy (IGR)-Institut Gustave Roussy (IGR)
- Institut Universitaire du Cancer de Toulouse - Oncopole (IUCT Oncopole - UMR 1037) ; Université Toulouse III - Paul Sabatier (UT3) ; Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Institut National de la Santé et de la Recherche Médicale (INSERM)
- Centre Régional de Lutte contre le cancer Georges-François Leclerc [Dijon] (UNICANCER/CRLCC-CGFL) ; UNICANCER
- Centre Léon Bérard [Lyon]
- Institut Curie [Paris]
- Centre de Lutte contre le Cancer Antoine Lacassagne [Nice] (UNICANCER/CAL) ; UNICANCER-Université Côte d'Azur (UCA)
- Institut de Cancérologie de Lorraine - Alexis Vautrin [Nancy] (UNICANCER/ICL) ; UNICANCER
- Les Hôpitaux Universitaires de Strasbourg (HUS)
- Centre Régional de Lutte contre le Cancer François Baclesse [Caen] (UNICANCER/CRLC) ; Normandie Université (NU)-UNICANCER-Tumorothèque de Caen Basse-Normandie (TCBN)
- Centre Jean Perrin [Clermont-Ferrand] (UNICANCER/CJP) ; UNICANCER
- Assistance Publique - Hôpitaux de Marseille (APHM)
- Institut de Cancérologie de l'Ouest [Angers/Nantes] (UNICANCER/ICO) ; UNICANCER
- Centre Régional de Lutte contre le Cancer Oscar Lambret [Lille] (UNICANCER/Lille) ; Université de Lille-UNICANCER
- Service d'Oncologie Médicale ; Centre Léon Bérard [Lyon]
Description
Five-year overall survival (OS) of localized RMS exceeds 70% in children (<18) but is very poor in adult patients. We analyzed the outcome and prognostic factors (PF) of a national series of adult patients with RMS in a large study. The study population consisted of two different cohorts: a retrospective cohort (157 adult patients treated in 13 reference centers between 05/1981 and 02/2010) and the prospective cohort (292 patients with RMS diagnosed and treated between 01/2010 and 12/2014 in France) included in the NetSarc database. A descriptive analysis of patients' characteristics and prognostic factors was conducted on both series which were compared. In the retrospective series, histological subtypes were embryonal (E-RMS) for 21% of patients, alveolar (A-RMS) for 35% of patients, and "adult-type" P-RMS (pleomorphic, spindle cell RMS, not otherwise specified) (P) for 44% patients. This distribution significantly differed in the prospective cohort: A-RMS: 18%; E-RMS: 17%; and P-RMS 65%. With a median follow-up of 8.5 years, 5-year OS for localized RMS and advanced RMS (with nodes and/or metastases) was 43% and 5%, respectively, (P < 0.0001), and median OS was 51, 33, and 16 months for E-RMS, A-RMS, and P-RMS, respectively, in the retrospective cohort. The median OS was less than 40 months for the prospective nationwide cohort for the entire population. In a multivariate analysis of the retrospective study, independent prognostic factors for OS were A-RMS, R0 resection, and adjuvant radiotherapy (RT). For localized RMS, age and use of pediatric chemotherapy (CT) regimen are independent prognostic factors. Adult patients with RMS have a poorer overall survival than pediatric patients, and survival varies considerably across histological subtypes.
Abstract
IF 3.202Abstract
International audienceAdditional details
Identifiers
- URL
- https://hal-univ-bourgogne.archives-ouvertes.fr/hal-01978221
- URN
- urn:oai:HAL:hal-01978221v1
Origin repository
- Origin repository
- UNICA