Journal of Neuro-Oncology

, Volume 97, Issue 2, pp 257–265

Clinical and epidemiological characteristics of pediatric gliosarcomas

  • Michael Karremann
  • Ulrike Rausche
  • Gudrun Fleischhack
  • Michaela Nathrath
  • Torsten Pietsch
  • Christof M. Kramm
  • Johannes E. A. Wolff
Clinical Study - Patient Study

DOI: 10.1007/s11060-009-0021-3

Cite this article as:
Karremann, M., Rausche, U., Fleischhack, G. et al. J Neurooncol (2010) 97: 257. doi:10.1007/s11060-009-0021-3

Abstract

Gliosarcoma (GS) is a glioblastoma with a sarcomatous component that is presumed to be a metaplastic differentiation of glioma cells. We studied the clinical relevance of this histological glioblastoma subentity within the pediatric population. We obtained patient data from the German HIT-GBM database, which contains clinical data for more than 600 pediatric patients with centrally reviewed high-grade gliomas. By applying defined inclusion criteria (diagnosis of GS proven by central neuropathological review; patient age 0 to 21 years), four patients were identified. In addition, after a review of the English medical scientific literature, 19 additional cases were found. The relative frequency of GS in the German HIT-GBM database was only 1.9%. In the whole series of 23 pediatric GS patients, including previously reported cases, the male-to-female-ratio was 1.2:1. GS was found in all pediatric age groups with a median age of 11 years, but there was an unexpectedly high accumulation in infants (6 of 23 <3 years of age, 26%). GS showed a strong predilection of the cerebral hemispheres (22 out of 23 cases). Increased intracranial pressure was the leading symptom of a short clinical history with a median duration of 0.7 month. Interestingly, six patients (26%) were reported with a history of cranial radiotherapy prior to GS diagnosis. In 60% of the GS patients in our series, gross total resection was achieved. Median overall (OS) and event-free survivals (EFS) of the total cohort were 12.1 and 9.8 months, respectively. In conclusion, GS is a very rare tumor entity in children. Literature review suggests a relatively higher incidence in infants and in patients with a previous history of radiotherapy.

Keywords

GliosarcomaGlioblastoma multiformeChildrenInfantsCase reportHIT-GBM trialClinical courseReview

Copyright information

© Springer Science+Business Media, LLC. 2009

Authors and Affiliations

  • Michael Karremann
    • 1
    • 2
  • Ulrike Rausche
    • 1
  • Gudrun Fleischhack
    • 3
  • Michaela Nathrath
    • 4
    • 5
  • Torsten Pietsch
    • 6
  • Christof M. Kramm
    • 1
  • Johannes E. A. Wolff
    • 7
    • 8
  1. 1.Department of Pediatrics and Adolescence MedicineMartin Luther University Halle-WittenbergHalleGermany
  2. 2.Department of PediatricsUniversity Hospital MannheimMannheimGermany
  3. 3.Department of PediatricsUniversity Hospital BonnBonnGermany
  4. 4.Department of PediatricsTechnische Universität MünchenMunichGermany
  5. 5.Clinical Cooperation Group OsteosarcomaHelmholtz Center MunichMunichGermany
  6. 6.Institute of NeuropathologyUniversity of BonnBonnGermany
  7. 7.Division of Pediatrics, Department of Pediatric Neuro-Oncology, Unit 87The University of Texas M.D. Anderson Cancer CenterHoustonUSA
  8. 8.Department of BiostatisticsThe University of Texas M.D. Anderson Cancer CenterHoustonUSA