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

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.