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Anaplastic ganglioglioma in children

  • Clinical study - patient study
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Abstract

Purpose Anaplastic gangliogliomas (AGG) are gangliogliomas with areas of pronounced hypercellularity, vascular proliferation, necrosis, and many mitotic figures. As very few pediatric patients have been studied, we analyzed the cases registered in the HIT-GBM database. Patients and Methods Patient data were obtained from the German HIT-GBM database. Inclusion criteria were diagnosis of AGG proven by a central neuropathological review and patient age 0 to 17 years. Eight patients (five male and three female) were identified. Results Patients’ median age was 10 years. The median history of disease was 9 months (range, 1.0–43.0 months). Initial symptoms included signs of raised intracranial pressure, seizures, and, in the case of spinal cord tumor, bladder dysfunction. In five cases, AGGs were localized supratentorially with three patients having multiple lobes involved. The tumors affected the frontal (n = 3 cases), parietal (n = 2), temporal (n = 2), and occipital lobes (n = 1), as well as the brainstem (n = 1) and the spinal cord (n = 2). Gross total tumor resection was achieved in six patients. The estimated 5-year overall survival rate ± standard error was 88 ± 12%, and the event-free survival rate was 63 ± 17%. While gender and tumor location did not affect survival rates, gross total tumor resection provided a better overall survival than non-total resection. Conclusion The prognosis of pediatric patients with AGG is good, especially for those who undergo gross total tumor resection.

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Acknowledgments

The ongoing support of the Deutsche Kinderkrebsstiftung, Bonn, Germany, is greatly appreciated. We also thank our colleagues who contributed patient 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 & Christof M. Kramm

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

    Michael Karremann

  3. Department of Neuropathology, University of Bonn, Bonn, Germany

    Torsten Pietsch

  4. Department of Pediatric Oncology, Hematology and Immunology, Heinrich-Heine-University, Düsseldorf, Germany

    Gisela Janssen

  5. 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

  6. 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. Torsten Pietsch
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  5. Johannes E. A. Wolff
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Corresponding author

Correspondence to Johannes E. A. Wolff.

Additional information

Christof M. Kramm and Johannes E. A. Wolff are contributed equally to this work.

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Karremann, M., Pietsch, T., Janssen, G. et al. Anaplastic ganglioglioma in children. J Neurooncol 92, 157–163 (2009). https://doi.org/10.1007/s11060-008-9747-6

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  • DOI: https://doi.org/10.1007/s11060-008-9747-6

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