Child's Nervous System

, Volume 29, Issue 5, pp 839–847

Fourth ventricle rosette-forming glioneuronal tumour in children: an unusual presentation in an 8-year-old patient, discussion and review of the literature

  • Benjamin Thurston
  • Roxana Gunny
  • Glenn Anderson
  • Simon Paine
  • Dominic Thompson
  • Thomas Jacques
  • Jessica Ternier
Case Report

DOI: 10.1007/s00381-012-1993-4

Cite this article as:
Thurston, B., Gunny, R., Anderson, G. et al. Childs Nerv Syst (2013) 29: 839. doi:10.1007/s00381-012-1993-4

Abstract

Introduction

Rosette-forming glioneuronal tumour of the fourth ventricle is a rarely described entity. While usually having an indolent course and hence classified as a WHO grade 1 tumour, the precise characteristics and risk of recurrence of this tumour are still unknown. In addition, the preferred treatment modality remains unclear.

Discussion

We present a case of an 8-year old with an early recurrence of 9 months after undergoing a sub-total resection of her tumour. Following further resection, there was no tumour present on the 3-month follow-up. In order to better characterise this tumour entity, we performed a review of the available literature on the subject. We found that it mainly affected young adults and had a female predominance. While initially these tumours were described in the fourth ventricle, the current literature suggests that they may be found in a larger variety of sites within the brain and spinal cord. There are several reports of recurrence occurring between 9 months and 10 years following surgery. There is as yet no feature of the tumour that appears to predict the risk of recurrence.

Conclusion

This phenomenon warrants further examination to discover if there is a sub-section of tumours that is likely to recur, and until this is established, all patients should be followed up at regular intervals.

Keyword

Fourth ventricleRosette-forming glioneuronal tumourRecurrencePaediatric neurosurgeryAstrocytique tumourUnusual

Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Benjamin Thurston
    • 1
  • Roxana Gunny
    • 2
  • Glenn Anderson
    • 3
  • Simon Paine
    • 3
    • 4
  • Dominic Thompson
    • 1
  • Thomas Jacques
    • 3
    • 4
  • Jessica Ternier
    • 1
  1. 1.Paediatric NeurosurgeryGreat Ormond Street Hospital NHS Foundation TrustLondonUK
  2. 2.NeuroradiologyGreat Ormond Street Hospital NHS Foundation TrustLondonUK
  3. 3.HistopathologyGreat Ormond Street Hospital NHS Foundation TrustLondonUK
  4. 4.Neural Development UnitUCL Institute of Child HealthLondonUK