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Toward Improved Diagnosis Accuracy and Treatment of Children, Adolescents, and Young Adults With Ependymoma: The International SIOP Ependymoma II Protocol.
Leblond, Pierre; Massimino, Maura; English, Martin; Ritzmann, Timothy A; Gandola, Lorenza; Calaminus, Gabriele; Thomas, Sophie; Pérol, David; Gautier, Julien; Grundy, Richard G; Frappaz, Didier.
Affiliation
  • Leblond P; Institut d'Hématologie et d'Oncologie Pédiatrique, Lyon, France.
  • Massimino M; Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
  • English M; Birmingham Children's Hospital, Birmingham, United Kingdom.
  • Ritzmann TA; Children's Brain Tumour Research Centre, Medical School, Queen's Medical Centre, Nottingham, United Kingdom.
  • Gandola L; Pediatric Radiotherapy Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
  • Calaminus G; Department of Pediatric Hematology and Oncology, University Hospital of Bonn, Bonn, Germany.
  • Thomas S; Children's Brain Tumour Research Centre, Medical School, Queen's Medical Centre, Nottingham, United Kingdom.
  • Pérol D; Department of Paediatric Neuropsychology, Nottingham Children's Hospital, Queen's Medical Centre, Nottingham, United Kingdom.
  • Gautier J; Department of Clinical Research and Innovation, Centre Léon Bérard, Lyon, France.
  • Grundy RG; Department of Clinical Research and Innovation, Centre Léon Bérard, Lyon, France.
  • Frappaz D; Children's Brain Tumour Research Centre, Medical School, Queen's Medical Centre, Nottingham, United Kingdom.
Front Neurol ; 13: 887544, 2022.
Article in En | MEDLINE | ID: mdl-35720069
ABSTRACT

Background:

The clinical management of ependymoma in childhood and adolescence is complex and the clinicobiopathological correlates of outcome remain poorly understood. This international SIOP Ependymoma II (SIOP EPII) trial aims to improve the outcome of patients with ependymoma.

Methods:

SIOP EPII includes any patient <22 years at diagnosis with ependymoma, stratified by age, tumor location, and outcome of the initial surgery. Centralized pathology and imaging is required for diagnosis confirmation. SIOP EPII included three randomized studies according to age, postoperative residue, and suitability to receive radiotherapy. Patients ineligible for interventional strata are followed-up in an observational study. The staging phase aims to determine if central neurosurgical and radiological postoperative MRI reviews increase the resection rate. Patients ≥12 months with (i) no residual disease are randomly assigned in a phase III trial to evaluate the efficacy of post-radiation 16-week chemotherapy (VEC + CDDP) on PFS (stratum I); (ii) centrally confirmed measurable inoperable residual disease are allocated to randomized frontline chemotherapy phase II study (VEC vs. VEC + high-dose methotrexate) and considered for a second-look surgery (stratum II). If second-look surgery is not feasible or tumor residuum remains, patients receive 8 Gy-boost radiotherapy after conformal radiotherapy (phase I). (iii) Patients < 12 months (18 months in the UK) or not eligible to receive radiotherapy are randomized in a phase II study to receive chemotherapy (alternated myelosuppressive and nonmyelosuppressive chemotherapy), with or without valproate (stratum III). To overcome the limitations encountered in the preliminary conclusions of the ACNS-0831 study, a SIOP EPII dedicated on-study amendment has been planned to definitively conclude the relevance of maintenance chemotherapy in stratum I. Secondary outcomes include overall survival, quality of life, neuropsychological and neuroendocrine outcomes, safety, and identification of key prognostic biomarkers (BIOMECA). Clinical Trial Registration ClinicalTrials.gov, identifier NCT02265770.
Key words

Full text: 1 Collection: 01-internacional Database: MEDLINE Type of study: Clinical_trials / Diagnostic_studies / Guideline / Observational_studies Aspects: Patient_preference Language: En Journal: Front Neurol Year: 2022 Document type: Article Affiliation country: France

Full text: 1 Collection: 01-internacional Database: MEDLINE Type of study: Clinical_trials / Diagnostic_studies / Guideline / Observational_studies Aspects: Patient_preference Language: En Journal: Front Neurol Year: 2022 Document type: Article Affiliation country: France