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Anaplastic ganglioglioma in children

  • Michael Karremann
  • Torsten Pietsch
  • Gisela Janssen
  • Christof M. Kramm
  • Johannes E. A. Wolff
Clinical study - patient study

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.

Keywords

Gliomas Pediatrics Central nervous system neoplasms Ganglioglioma Brain cancer 

Notes

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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Copyright information

© Springer Science+Business Media, LLC. 2008

Authors and Affiliations

  • Michael Karremann
    • 1
    • 2
  • Torsten Pietsch
    • 3
  • Gisela Janssen
    • 4
  • Christof M. Kramm
    • 1
  • Johannes E. A. Wolff
    • 5
    • 6
  1. 1.Department of Pediatrics and Adolescence MedicineMartin-Luther-University Halle-WittenbergHalleGermany
  2. 2.Department of PediatricsUniversity Hospital MannheimMannheimGermany
  3. 3.Department of NeuropathologyUniversity of BonnBonnGermany
  4. 4.Department of Pediatric Oncology, Hematology and ImmunologyHeinrich-Heine-UniversityDüsseldorfGermany
  5. 5.Department of Pediatric Neuro-Oncology, Unit 87The University of Texas M. D. Anderson Cancer CenterHoustonUSA
  6. 6.Department of BiostatisticsThe University of Texas M. D. Anderson Cancer CenterHoustonUSA

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