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

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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.

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Authors and Affiliations

  1. Department of Oncology, University Children’s Hospital, 8032, Zurich, Switzerland

    Nicolas U. Gerber

  2. Department of Pediatrics, University of Wuerzburg, 97080, Wuerzburg, Germany

    Nicolas U. Gerber, Katja von Hoff, André O. von Bueren, Wiebke Treulieb, Paul G. Schlegel, Frank Deinlein & Stefan Rutkowski

  3. Department of Neuroradiology, University of Wuerzburg, 97080, Wuerzburg, Germany

    Monika Warmuth-Metz

  4. Institute of Neuropathology, University of Bonn, 53105, Bonn, Germany

    Torsten Pietsch

  5. Department of Pediatric Neurosurgery, University of Wuerzburg, 97080, Wuerzburg, Germany

    Niels Soerensen

  6. Institute for Medical Biostatistics, Epidemiology and Informatics, University Medical Center, 55101, Mainz, Germany

    Andreas Faldum & Angela Emser

  7. Department of Radiation Oncology, University of Leipzig, 04103, Leipzig, Germany

    Rolf-Dieter Kortmann

  8. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany

    Katja von Hoff, André O. von Bueren, Wiebke Treulieb & Stefan Rutkowski

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  1. Nicolas U. Gerber
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Correspondence to Nicolas U. Gerber.

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Gerber, N.U., von Hoff, K., von Bueren, A.O. et al. Outcome of 11 children with ependymoblastoma treated within the prospective HIT-trials between 1991 and 2006. J Neurooncol 102, 459–469 (2011). https://doi.org/10.1007/s11060-010-0347-x

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  • DOI: https://doi.org/10.1007/s11060-010-0347-x

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