Atypical Teratoid and Rhabdoid Tumors in Children: the French Experience Since 1998 : O.058

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ID Serval
serval:BIB_CEF92AED638B
Type
Actes de conférence (partie): contribution originale à la littérature scientifique, publiée à l'occasion de conférences scientifiques, dans un ouvrage de compte-rendu (proceedings), ou dans l'édition spéciale d'un journal reconnu (conference proceedings).
Sous-type
Abstract (résumé de présentation): article court qui reprend les éléments essentiels présentés à l'occasion d'une conférence scientifique dans un poster ou lors d'une intervention orale.
Collection
Publications
Institution
Titre
Atypical Teratoid and Rhabdoid Tumors in Children: the French Experience Since 1998 : O.058
Titre de la conférence
41st Annual Conference of the International Society of Paediatric Oncology
Auteur(s)
Beaugrand Annick, Quidot Marion, Varlet Pascale, Bourdeaut Franck, Andre Nicolas, Palenzuela Gilles, Frappaz Didier, Leblond Pierre, Fouyssac Fanny, Couteau Nathalie, Bertozzi Anne Isabelle, Kanold Justyna, Carvalho Alice, Edan Christine, Riffaud Laurent, Popovic Maja, Grill Jacques, Le Deley Marie Cecile, Dufour Christelle
Adresse
Sao Paulo, Brazil, October 5-9, 2009
ISBN
1545-5009
Statut éditorial
Publié
Date de publication
2009
Peer-reviewed
Oui
Volume
53
Série
Pediatric Blood and Cancer
Pages
728
Langue
anglais
Notes
Purpose: To describe clinical features and therapeutic approaches and to identify prognostic factors in children with ATRT of the CNS.
Method: Observational study including all patients aged less than 18 years, diagnosed with CNS ATRT in France between January 1998 and July 2008, identified from hospital files and French Pediatric Cancer registry. Pathology review included histological and immunohistochemical analysis, including INI-1 staining. Impact of clinical characteristics (age, sex, site of primary tumor and metastatic status) on the overall survival (OS) was assessed using Cox models.
Results: seventy out of the 71 patients identified with ATRT over this 10-year period were included in the study (1 patient excluded due to incomplete clinical data). Median age was 2.8 years (range, 15 days-12.8 years). Primary tumor site was supratentorial (ST) in 34, posterior fossa (PF) in 30, mixed (STþPF) in 2 and medullar in 4 patients. The disease was disseminated at diagnosis in 22 patients. Five patients had non-CNS disease associated with CNS disease. Surgical resection was complete in 41 patients. Adjuvant therapy included chemotherapy in 55 cases and radiotherapy in 20 patients. Chemotherapy regimens were not standardized more than the study period: ATRT04, PNET High Risk and BB SFOP protocols were most frequently used. Median follow-up was 52 months (range, 13 months-10 years). Disease progression or relapse occurred in 51 children. Median time to progression/relapse was 4.4 months. Median survival time was 9.9 months. One-year progression-free survival and OS were 21% and 42%, respectively. Metastatic status at diagnosis was the only prognostic factor (Hazard ratio for death: 2.1, 95%CI: 1.2-3.8, p¼0.01).
Conclusion: Children with ATRT of the CNS have a dismal prognosis. Innovative therapeutic are urgently needed.
Web of science
Création de la notice
08/12/2009 15:31
Dernière modification de la notice
20/08/2019 15:49
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