title: Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database.
abstract: OBJECTIVE
  Reports on spinal cord ependymoma in children are rare. The aim of this study was to evaluate the clinical spectrum, treatment, and outcome of children with primary ependymoma of the spinal cord who were registered in the database of the pediatric German brain tumor studies Hirntumor (HIT) '91 and HIT 2000.
  
  
  METHODS
  Between 1991 and 2007, 29 patients (12 male and 17 female, median age at diagnosis 13.6 years) with primary spinal cord ependymoma (myxopapillary ependymoma WHO Grade I, II, and III tumors in 6, 17, and 6 patients, respectively) were identified. Four patients had neurofibromatosis Type 2.
  
  
  RESULTS
  With a median follow-up of 4.2 years (range 0.48-15 years), 28 patients (96.6%) were alive. Seven patients (24.1%) developed progressive disease or relapse, 2 after gross-total resection (GTR) and 5 after incomplete resection or biopsy. One patient with anaplastic ependymoma (WHO Grade III) died 65 months after diagnosis of disease progression. Primary adjuvant treatment (radiotherapy, chemotherapy, or both) was used in 8 (50%) of 16 patients following GTR and in 9 (82%) of 11 patients who underwent less than a GTR. Three additional patients were treated adjuvantly following progression. Estimated progression-free survival and overall survival rates at 5 years were 72.3% (95% CI 50%-86%) and 100%, respectively. Progression-free survival at 5 years is 84.4% (95% CI 50%-96%) for patients following GTR compared with 57.1% (95% CI 25%-69%) for patients who achieved a less than GTR (p = 0.088, log-rank test). A high relapse incidence (4 of 6) was observed among patients with myxopapillary ependymoma.
  
  
  CONCLUSIONS
  Gross-total resection is the mainstay of treatment for patients with primary spinal cord ependymoma and may be achieved in about 50% of the patients using modern surgical techniques. Primary adjuvant treatment was commonly used in children with spinal cord ependymoma irrespective of the extent of resection or tumor grade. The impact of adjuvant treatment on progression-free and overall survival has to be investigated in a prospective trial.
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria. martin.benesch@klinikum-graz.at
authors.forename: Martin
authors.initials: M
authors.lastname: Benesch
authors.forename: Daniela
authors.initials: D
authors.lastname: Weber-Mzell
authors.forename: Nicolas U
authors.initials: NU
authors.lastname: Gerber
authors.forename: Katja
authors.initials: K
authors.lastname: von Hoff
authors.forename: Frank
authors.initials: F
authors.lastname: Deinlein
authors.forename: Jürgen
authors.initials: J
authors.lastname: Krauss
authors.forename: Monika
authors.initials: M
authors.lastname: Warmuth-Metz
authors.forename: Rolf-Dieter
authors.initials: RD
authors.lastname: Kortmann
authors.forename: Torsten
authors.initials: T
authors.lastname: Pietsch
authors.forename: Pablo Hernáiz
authors.initials: PH
authors.lastname: Driever
authors.forename: Franz
authors.initials: F
authors.lastname: Quehenberger
authors.forename: Christian
authors.initials: C
authors.lastname: Urban
authors.forename: Stefan
authors.initials: S
authors.lastname: Rutkowski
mesh_terms: D000293:Adolescent; D001317:Austria; D001706:Biopsy; D017024:Chemotherapy, Adjuvant; D002648:Child; D003131:Combined Modality Therapy; D004185:Disability Evaluation; D018450:Disease Progression; D004806:Ependymoma / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005858:Germany; D006801:Humans; D008297:Male; D016518:Neurofibromatosis 2 / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D011183:Postoperative Complications / Q000175:diagnosis / Q000401:mortality; D011446:Prospective Studies; D018714:Radiotherapy, Adjuvant; D012042:Registries; D013120:Spinal Cord Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D015996:Survival Rate