title: Metastatic ependymoma: a multi-institutional retrospective analysis of prognostic factors.
abstract: OBJECTIVE
  Metastatic ependymoma is exceedingly rare at diagnosis with variable prognosis reported in the literature. The purpose of this study was to identify prognostic factors in children with metastatic ependymoma.
  
  
  METHODS
  Data regarding diagnosis, treatment and follow-up for 40 patients from eight institutional cohorts were collected.
  
  
  RESULTS
  Twenty-nine (72%) patients were less than 36 months of age at the time of diagnosis, 28% were females, and 90% of the patients had posterior fossa tumors. Gross total resection (GTR) of the primary tumor was achieved in 16 patients (40%). Adjuvant therapy was variable and included craniospinal irradiation (CSRT), chemotherapy, and chemotherapy with focal irradiation. The 5-year event free survival (EFS) and overall survival (OS) from the time of diagnosis were 29% (+/-7%) and 43% (+/-8%), respectively. Age at diagnosis was associated significantly with both EFS and OS (P < 0.001 for EFS, and P = 0.01 for OS). Patients who were 24-35 months of age at diagnosis had a 5-year EFS of 66% and a 5-year OS of 73%; both survival rates were superior to those of patients younger than 24 months of age or older than 36 months. Patients with GTR achieved a 5-year EFS of 35% and OS of 59%, compared to a 5-year EFS of 25% and OS of 32% for patients who did not achieve GTR (P = 0.12 for EFS, P = 0.03 for OS).
  
  
  CONCLUSIONS
  GTR should be attempted in patients with metastatic ependymoma. A subgroup of patients between 24 and 35 months might have a favorable outcome.
authors.affiliation: Department of Pediatrics, The Royal Marsden Hospital, Sutton, Surrey, UK. stergios.zacharoulis@rmh.nhs.uk
authors.forename: Stergios
authors.initials: S
authors.lastname: Zacharoulis
authors.forename: Lingyun
authors.initials: L
authors.lastname: Ji
authors.forename: Ian F
authors.initials: IF
authors.lastname: Pollack
authors.forename: Patricia
authors.initials: P
authors.lastname: Duffner
authors.forename: Russel
authors.initials: R
authors.lastname: Geyer
authors.forename: Jacques
authors.initials: J
authors.lastname: Grill
authors.forename: Steven
authors.initials: S
authors.lastname: Schild
authors.forename: Tang-Her
authors.initials: TH
authors.lastname: Jaing
authors.forename: Maura
authors.initials: M
authors.lastname: Massimino
authors.forename: Jonathan
authors.initials: J
authors.lastname: Finlay
authors.forename: Richard
authors.initials: R
authors.lastname: Sposto
mesh_terms: D001932:Brain Neoplasms / Q000473:pathology* / Q000601:surgery / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004806:Ependymoma / Q000473:pathology* / Q000556:secondary* / Q000601:surgery / Q000628:therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D009362:Neoplasm Metastasis; D016016:Proportional Hazards Models; D012189:Retrospective Studies