gms | German Medical Science

60th Annual Meeting of the German Society of Neurosurgery (DGNC)
Joint Meeting with the Benelux countries and Bulgaria

German Society of Neurosurgery (DGNC)

24 - 27 May 2009, Münster

From glioblastoma to gangliocytoma – a peculiar pediatric path?

Meeting Abstract

  • M. Holling - Klinik für Neurochirurgie, Universitätsklinikum Münster
  • A. Jeibmann - Institut für Neuropathologie, Universitätsklinikum Münster
  • A. Brentrup - Klinik für Neurochirurgie, Universitätsklinikum Münster
  • T. Niederstadt - Institut für klinische Radiologie, Universitätsklinikum Münster
  • H. Wassmann - Klinik für Neurochirurgie, Universitätsklinikum Münster
  • R. Sträter - Abteilung für pädiatrische Onkologie und Hämatologie, Universitätsklinikum Münster
  • M. Frühwald - Abteilung für pädiatrische Onkologie und Hämatologie, Universitätsklinikum Münster

Deutsche Gesellschaft für Neurochirurgie. 60. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit den Benelux-Ländern und Bulgarien. Münster, 24.-27.05.2009. Düsseldorf: German Medical Science GMS Publishing House; 2009. DocP12-05

DOI: 10.3205/09dgnc379, URN: urn:nbn:de:0183-09dgnc3794

Published: May 20, 2009

© 2009 Holling et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.


Outline

Text

Objective: Glioblastoma multiforme (WHO grade IV) is an infrequent tumor entity in neonates and is genetically distinct from its adult counterpart, but despite recent therapeutic advances the prognosis of pediatric glioblastoma remains poor. Spontaneous or therapy-induced maturation or differentiation has been described in individual cases in medulloblastomas but not yet in glioblastoma.

Methods: Here we report the case of a two-months-old girl presenting with accelerated growth of head circumference and symptoms of intracranial pressure caused by the presence of a large tumor located within the left hemisphere. MRI revealed growth of the lesion raising suspicion of a malignant CNS tumor. Biopsy revealed a glioblastoma multiforme (WHO grade IV). Chemotherapy based on carboplatin and etoposide was initiated. The clinical status of the child stabilized. MRI scans showed consolidation of tumor size and pronounced decrease of contrast uptake. Upon a last course of chemotherapy second-look-surgery was performed and a near-complete resection was achieved. Histopathological examination now revealed a gangliocytoma (WHO grade I). The child remained in excellent clinical condition, achieving the usual milestones of early childhood development. Neurological examination exhibits mild hemiparesis, but no gross motor deficit.

Results: To our knowledge, such gangliocytic maturation is exceptional and has not yet been described in glioblastomas. In medulloblastomas several cases of gangliocytic maturation are on record and have been thought to reflect spontaneous or therapy-induced changes. The present case might prompt a closer examination of frequency and prognostic significance of neural differentiation in pediatric glioblastomas within ongoing and future clinical trials.

Conclusions: Pediatric glioblastoma may show maturation into gangliocytoma upon adjuvant chemotherapy, although the biological mechanisms underlying this specific sensitivity remain to be elucidated.