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

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.