Cerebellar location may predict an unfavourable prognosis in paediatric high-grade glioma

Abstract

Background:High-grade glioma (HGG) of the cerebellum accounts for only 5% of paediatric HGG. Since little is known about these tumours, the present study aimed at their further characterisation.Methods:Twenty-nine paediatric patients with centrally reviewed cerebellar HGG were identified from the HIT-GBM/HIT-HGG database. Clinical and epidemiological data were compared with those of 180 paediatric patients with cortical HGG.Results:Patients with cerebellar tumours were younger (median age of 7.6 vs 11.7 years, P=0.028), but both groups did not differ significantly with regard to gender, tumour predisposing syndromes, secondary HGG, primary metastasis, tumour grading, extent of tumour resection, chemotherapy regimen, or radiotherapy. Except for an increased incidence of anaplastic pilocytic astrocytoma (APA) in the cerebellar subset (20.7% vs 3.3% P<0.001), histological entities were similarly distributed in both groups. As expected, tumour grading had a prognostic relevance on survival. Compared with cortical HGG, overall survival in the cerebellar location was significantly worse (median overall survival: 0.92±0.02 vs 2.03±0.32 years; P=0.0064), and tumour location in the cerebellum had an independent poor prognostic significance as shown by Cox-regression analysis (P=0.019).Conclusion:High-grade glioma represents a group of tumours with an obviously site-specific heterogeneity associated with a worse survival in cerebellar location.

Unique genetic and epigenetic mechanisms driving paediatric diffuse high-grade glioma

“…By contrast, supratentorial tumours predominantly occur during adolescence (median 11–12 years) 3, 5 , though they may also be found in other age groups including infants (< 3 years). Tumours in the infant age group appear to respond considerably better to chemotherapeutic regimens and have improved clinical outcomes 12 , hinting at a distinct biology.…”

“…Diffuse high-grade lesions in children, although rare (approximately 1/100,000) 1 , retain the same dismal prognosis as those that arise in adults (median survival 12–15 months), particularly for those arising in the pons (median survival 9–12 months) 2 . In addition to the supratentorial locations in which the vast majority of adult tumours occur, children frequently present with histologically similar WHO grade IV glioblastoma in midline structures including the thalamus (13%) 3 and spinal cord (3%) 4 as well as the cerebellum (5%) 5 (Figure 1). A diverse spectrum of grade III lesions may also occur (Text Box 1).…”

Genetic Analysis of Diffuse High‐Grade Astrocytomas in Infancy Defines a Novel Molecular Entity

“…Although pediatric cases do not differ histopathologically from their adult counterparts, significant differences concerning their clinical features have been observed. Although curative therapy options are still absent and long‐term survivors are an exception in adults as well as in children , infants (<3 years of age) with high‐grade gliomas (iHGGs) appear to have a better clinical outcome and long‐term survival over 20 years after diagnosis was described . In the past few years, integrated molecular genetic profiling of pediatric high‐grade gliomas (pHGGs) improved the understanding of obviously significant differences concerning the pathogenetic mechanisms of these tumors as well as their putative cell of origin.…”

Effect of radiotherapy and chemotherapy on levels of serum S100B, IL-6, and IL-17 in patients with malignant glioma

“…In the classification of tumors of the central nervous system by WHO, gliomas are divided as I-IV grade in which the gliomas of grades III and IV are malignant ones, accounting for 77.5% of all. 6,7 Its clinical features are mainly characterized by symptoms and signs of intracranial hypertension and neurological deficit. 8 A variety of drugs have been applied singly or in combination with chemotherapy for malignant glioma.…”