Institutional experience of paediatric high-grade central nervous system tumours: An analysis of 74 patients and review of the literature

Abstract

Aim of the studyAlthough the survival for children with certain central nervous system (CNS) tumour types has improved through current surgical and adjuvant treatment modalities, the prognosis of many high-grade tumours remains poor despite aggressive treatment. The aim of this study is to analyse patients with high-grade brain tumours in our institution to determine the histopathology, clinical characteristics, treatment modalities, and survival.Material and methodsA total of 74 patients with a diagnosis of high-grade brain tumour were analysed. There were a total of 31 patients with embryonal tumours, 27 patients with high-grade glial tumours, 12 patients with brain stem gliomas and 4 patients with other high-grade brain tumours.ResultsThere were 48 (65%) boys and 26 (35%) girls (ratio: 1.85) with a median age of 99.7 months (range = 2-204 months). The median follow-up period was 19 months (range = 1-204 months). Tumour recurrence was observed in 38 patients (51.4%). The overall survival rate and event-free survival rate of our patients were 27% and 19.5%, respectively.ConclusionsPediatric high-grade CNS tumours have a very aggressive behaviour and a significant number of children eventually succumb to disease despite multimodal treatment. There is a need of more effective therapeutic approaches for these tumours with poor prognosis. The future improvement in childhood high-grade brain tumour management depends on a better understanding of the molecular genetics and biology of brain tumours.

A pediatric bithalamic high grade glioma with concomitant H3K27M and EGFR mutations

“…However, our knowledge about the biology of childhood HGG tumors is increasing, especially in line with the developments in molecular biology in recent years. [1][2][3][4]15 Another problem in childhood HGG tumors is that the desired treatment outcomes have not been achieved yet. Poor outcome and failure to achieve the desired success in treatment increases the interest in investigating the biological behavior of childhood HGG tumors.…”

“…High grade glial (HGG) tumors (Grades III and IV) constitute approximately 44.5 % of astrocytomas and 10% of all childhood CNS cancers. [1][2][3][4][5] Despite many treatment approaches, survival rates in HGG are still not at the desired level. One of the possible causes of this failure is that although having similar histologic features, they may display different biological behaviors depending on molecular heterogeneity.…”