title: Primary CNS lymphoma in children and adolescents: a descriptive analysis from the International Primary CNS Lymphoma Collaborative Group (IPCG).
abstract: OBJECTIVE
  To describe the demographic and clinical features and outcomes for children and adolescents with primary CNS lymphoma (PCNSL).
  
  
  METHODS
  A retrospective series of children and adolescents with PCNSL was assembled from 10 cancer centers in 3 countries.
  
  
  RESULTS
  Twenty-nine patients with a median age of 14 years were identified. Sixteen (55%) had Eastern Cooperative Oncology Group (ECOG) performance status (PS) of 1 or greater. Frontline therapy consisted of chemotherapy only in 20 patients (69%), while 9 (31%) had chemotherapy plus cranial radiotherapy. Most patients received methotrexate (MTX)-based regimens. Overall response rate was 86% (complete remission 69%, partial remission 17%). The 2-year progression-free survival (PFS) and overall survival (OS) rates were 61% and 86%, respectively; the 3-year OS was 82%. Univariate analyses were conducted for age (≤ 14 vs. >14 years), PS (0 or 1 vs. >1), deep brain lesions, MTX dose, primary treatment with chemotherapy alone, intrathecal chemotherapy, and high-dose therapy. Primary treatment with chemotherapy alone was associated with better overall response rates with an odds ratio (OR) of 0.125 (P = 0.02). There was a marginally significant relationship between higher doses of MTX and response (OR = 1.5, P = 0.06). ECOG-PS of 0 to 1 was the only factor associated with better outcome with hazard ratios of 0.136 (P = 0.017) and 0.073 (P = 0.033) for PFS and OS, respectively.
  
  
  CONCLUSIONS
  This is the largest series collected of pediatric PCNSL. The outcome of children and adolescents seems to be better than in adults. PS of 0 to 1 is associated with better survival.
authors.affiliation: Division of Haematology/Oncology, Department of Paediatrics, Hospital for Sick Children, University of Toronto, Toronto, Canada. oussama.abla@sickkids.ca
authors.forename: Oussama
authors.initials: O
authors.lastname: Abla
authors.forename: Sheila
authors.initials: S
authors.lastname: Weitzman
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Blay
authors.forename: Brian Patrick
authors.initials: BP
authors.lastname: O'Neill
authors.forename: Lauren E
authors.initials: LE
authors.lastname: Abrey
authors.forename: Edward
authors.initials: E
authors.lastname: Neuwelt
authors.forename: Nancy D
authors.initials: ND
authors.lastname: Doolittle
authors.forename: Joachim
authors.initials: J
authors.lastname: Baehring
authors.forename: Kamnesh
authors.initials: K
authors.lastname: Pradhan
authors.forename: S Eric
authors.initials: SE
authors.lastname: Martin
authors.forename: Michael
authors.initials: M
authors.lastname: Guerrera
authors.forename: Shafqat
authors.initials: S
authors.lastname: Shah
authors.forename: Hervé
authors.initials: H
authors.lastname: Ghesquieres
authors.forename: Michael
authors.initials: M
authors.lastname: Silver
authors.forename: Rebecca A
authors.initials: RA
authors.lastname: Betensky
authors.forename: Tracy
authors.initials: T
authors.lastname: Batchelor
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D016543:Central Nervous System Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008223:Lymphoma / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult