Journal of Neuro-Oncology

, Volume 102, Issue 3, pp 459–469

Outcome of 11 children with ependymoblastoma treated within the prospective HIT-trials between 1991 and 2006

  • Nicolas U. Gerber
  • Katja von Hoff
  • André O. von Bueren
  • Wiebke Treulieb
  • Monika Warmuth-Metz
  • Torsten Pietsch
  • Niels Soerensen
  • Andreas Faldum
  • Angela Emser
  • Paul G. Schlegel
  • Frank Deinlein
  • Rolf-Dieter Kortmann
  • Stefan Rutkowski
Clinical Study – Patient Study

DOI: 10.1007/s11060-010-0347-x

Cite this article as:
Gerber, N.U., von Hoff, K., von Bueren, A.O. et al. J Neurooncol (2011) 102: 459. doi:10.1007/s11060-010-0347-x

Abstract

Ependymoblastoma is a rare malignant brain tumor of early childhood. Data on clinical behavior and optimal treatment strategies are scarce. We report on 11 consecutively treated children with centrally confirmed diagnosis of CNS ependymoblastoma, registered between February 1994 and October 2006 to the prospective GPOH-HIT multicenter brain tumor trials, and treated by multimodal regimens. Median age at diagnosis was 3.5 years (range, 1.8–5.6 years), and the median follow-up of survivors was 5.9 years (range, 2.2–12.7 years). Initial stage was M0 in 9, and M0/1 (no cerebrospinal fluid examination done) in 2 patients. Gross-total tumor resection was achieved in 7 patients, incomplete resection in 4 patients. Further primary therapy included chemotherapy in all patients, craniospinal radiotherapy in 5 patients and high-dose chemotherapy in 2 patients. Tumor response to chemotherapy was observed in 1 of 4 evaluable patients. Tumor progression occurred in 7 patients after a median time of 5.0 months (range, 2.5–19.2 months). Five-year progression-free survival was 36.4% (±14.5%), 5-year overall survival 30.3% (±15.9%). Of 4 survivors, 3 had gross-total tumor resection, and all were treated by either craniospinal radiotherapy and/or high-dose chemotherapy with autologous blood stem cell rescue. Prognosis of children with ependymoblastoma is poor, but sustained remissions have been achieved after multimodal treatment. Considerable diagnostic discrepancies between local and central pathologists underscore the importance of central review. Further studies are needed to improve survival of children with this rare malignant central nervous system tumor.

Keywords

EpendymoblastomaCentral nervous system primitive neuroectodermal tumorChildrenBrain tumorCancer

Copyright information

© Springer Science+Business Media, LLC. 2011

Authors and Affiliations

  • Nicolas U. Gerber
    • 1
    • 2
  • Katja von Hoff
    • 2
    • 8
  • André O. von Bueren
    • 2
    • 8
  • Wiebke Treulieb
    • 2
    • 8
  • Monika Warmuth-Metz
    • 3
  • Torsten Pietsch
    • 4
  • Niels Soerensen
    • 5
  • Andreas Faldum
    • 6
  • Angela Emser
    • 6
  • Paul G. Schlegel
    • 2
  • Frank Deinlein
    • 2
  • Rolf-Dieter Kortmann
    • 7
  • Stefan Rutkowski
    • 2
    • 8
  1. 1.Department of OncologyUniversity Children’s HospitalZurichSwitzerland
  2. 2.Department of PediatricsUniversity of WuerzburgWuerzburgGermany
  3. 3.Department of NeuroradiologyUniversity of WuerzburgWuerzburgGermany
  4. 4.Institute of NeuropathologyUniversity of BonnBonnGermany
  5. 5.Department of Pediatric NeurosurgeryUniversity of WuerzburgWuerzburgGermany
  6. 6.Institute for Medical Biostatistics, Epidemiology and InformaticsUniversity Medical CenterMainzGermany
  7. 7.Department of Radiation OncologyUniversity of LeipzigLeipzigGermany
  8. 8.Department of Pediatric Hematology and OncologyUniversity Medical Center Hamburg-EppendorfHamburgGermany