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Clinical and epidemiological characteristics of pediatric gliosarcomas

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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.

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Acknowledgments

The ongoing support of the Deutsche Kinderkrebsstiftung, Bonn, Germany, is greatly acknowledged. Without this support performance of clinical trials as well as quality control measures like central neuropathological and neuroradiological review and a central review of radiotherapy planning would not be possible within the HIT network. We also thank all colleagues who contributed patients and their data to the HIT-GBM studies.

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Authors and Affiliations

  1. Department of Pediatrics and Adolescence Medicine, Martin Luther University Halle-Wittenberg, Halle, Germany

    Michael Karremann, Ulrike Rausche & Christof M. Kramm

  2. Department of Pediatrics, University Hospital Mannheim, Mannheim, Germany

    Michael Karremann

  3. Department of Pediatrics, University Hospital Bonn, Bonn, Germany

    Gudrun Fleischhack

  4. Department of Pediatrics, Technische Universität München, Munich, Germany

    Michaela Nathrath

  5. Clinical Cooperation Group Osteosarcoma, Helmholtz Center Munich, Munich, Germany

    Michaela Nathrath

  6. Institute of Neuropathology, University of Bonn, Bonn, Germany

    Torsten Pietsch

  7. Division of Pediatrics, Department of Pediatric Neuro-Oncology, Unit 87, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX, 77030, USA

    Johannes E. A. Wolff

  8. Department of Biostatistics, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA

    Johannes E. A. Wolff

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  1. Michael Karremann
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  2. Ulrike Rausche
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  7. Johannes E. A. Wolff
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Correspondence to Johannes E. A. Wolff.

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Karremann, M., Rausche, U., Fleischhack, G. et al. Clinical and epidemiological characteristics of pediatric gliosarcomas. J Neurooncol 97, 257–265 (2010). https://doi.org/10.1007/s11060-009-0021-3

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  • DOI: https://doi.org/10.1007/s11060-009-0021-3

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