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High-grade gliomas in children look like adult glioblastoma under the microscope but are driven by different genes, so they are now classified separately. Surgery and radiotherapy remain the mainstay and chemotherapy adds little; the real gains are in small subsets with a targetable gene change, such as BRAF V600E tumours and the fusion-driven tumours of infants.
WHO 2021 separates paediatric-type diffuse high-grade gliomas from adult glioblastoma into four types: diffuse midline glioma, H3 K27-altered (covered on its own page); diffuse hemispheric glioma, H3 G34-mutant, of adolescents and young adults with TP53 and ATRX mutations; diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype, with PDGFRA amplification, MYCN amplification or EGFR alteration defining methylation subgroups; and infant-type hemispheric glioma, defined by NTRK, ALK, ROS1 or MET fusions in children under about three. Hypermutant tumours arising in constitutional mismatch repair deficiency and radiation-induced gliomas after treatment of another childhood cancer are distinct further groups. Methylation profiling is essential because morphology cannot separate these entities and some low-grade-appearing tumours prove high grade molecularly.
Treatment is maximal safe resection followed by focal radiotherapy, with temozolomide during and after radiotherapy adopted from adult practice although the ACNS0126 study did not show it clearly improved on historical outcomes; the HERBY trial (2018) showed adding bevacizumab did not help. Radiotherapy is deferred or avoided in infants, whose tumours are chemosensitive and in whom fusion-directed drugs work: larotrectinib and entrectinib for NTRK fusions, alectinib or lorlatinib for ALK and ROS1 fusions, with responses that can allow less surgery. In BRAF V600E-mutant high-grade glioma dabrafenib plus trametinib produced durable responses in the paediatric cohort reported in 2023, and the combination has a tumour-agnostic approval for BRAF V600E solid tumours from the age of six. Constitutional mismatch repair deficiency tumours respond to PD-1 blockade.
The outlook for H3 G34-mutant and H3- and IDH-wildtype tumours has changed little; the pipeline includes CDK4/6 inhibition with temozolomide after radiotherapy, IDH inhibitors for the rare IDH-mutant adolescent tumours, CAR-T cells directed at GD2 and B7-H3 delivered into the ventricles, oncolytic viruses and focused-ultrasound opening of the blood-brain barrier. Late effects of radiotherapy on the developing brain weigh on every decision.
A minority of childhood brain tumours but a leading cause of death among them; most children with hemispheric high-grade glioma die within a few years of diagnosis, while infants with fusion-driven tumours can do far better.
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Lactotroph pituitary neuroendocrine tumour (prolactinoma), Somatotroph pituitary neuroendocrine tumour (acromegaly), Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour), Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma), Thyrotroph pituitary neuroendocrine tumour (TSH-secreting), Pineocytoma and pineal parenchymal tumour of intermediate differentiation, Pineoblastoma, Papillary tumour of the pineal region, Choroid plexus carcinoma, Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), Ependymoma, Craniopharyngioma, Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma, Brain and spinal cord tumours (all types), Astrocytoma, IDH-mutant (grades 2 to 4), Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, Meningioma, Brain metastases (secondary brain tumours), Vestibular schwannoma (acoustic neuroma), Central nervous system germ cell tumours (germinoma and non-germinomatous), Spinal cord tumours (intramedullary and intradural), WNT-activated medulloblastoma, SHH-activated medulloblastoma, Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Maximal safe resection, focal radiotherapy and temozolomide during and after radiotherapy by extrapolation from adult practice; molecular profiling of every tumour.
Surgery and fusion-directed therapy: larotrectinib or entrectinib for NTRK fusions, alectinib or lorlatinib for ALK or ROS1 fusions; chemotherapy to defer radiotherapy.
Dabrafenib plus trametinib (paediatric high-grade glioma cohort 2023; tumour-agnostic approval for BRAF V600E solid tumours from age six).
PD-1 blockade (nivolumab or pembrolizumab) for hypermutant tumours; germline counselling for the family.
No standard; re-resection, re-irradiation, clinical trials including CAR-T and oncolytic virus studies.
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BRAF V600E testing is mandatory in childhood high-grade glioma and the combination is a standard at relapse and, increasingly, alongside or before radiotherapy in newly diagnosed disease; it supported the tumour-agnostic approval in children.
Every brain tumour page on this site uses these names and grades; a tumour called glioblastoma before 2021 may now be an IDH-mutant astrocytoma with a different outlook and treatment.
The 2021 WHO paediatric-type diffuse high-grade glioma categories, and the practice of profiling every childhood glioma for a targetable fusion or mutation, rest on this landscape.
Query for this cancer: (TITLE:"Paediatric high-grade glioma" OR ABSTRACT:"Paediatric high-grade glioma" OR TITLE:"excluding diffuse midline glioma" OR ABSTRACT:"excluding diffuse midline glioma" OR TITLE:"pHGG" OR ABSTRACT:"pHGG" OR TITLE:"Childhood high-grade glioma" OR ABSTRACT:"Childhood high-grade glioma" OR TITLE:"Paediatric glioblastoma" OR ABSTRACT:"Paediatric glioblastoma" OR TITLE:"Diffuse hemispheric glioma, H3 G34-mutant" OR ABSTRACT:"Diffuse hemispheric glioma, H3 G34-mutant") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Paediatric high-grade glioma (excluding diffuse midline glioma), not a curated reading list.
Sposto and colleagues, the first randomised evidence for adjuvant chemotherapy in children.
Schwartzentruber and colleagues (Nature) and Wu and colleagues (Nature Genetics) find H3.3 K27M and G34 mutations.
Grill and colleagues (JCO).
Hargrave and colleagues (JCO) report durable responses in the paediatric cohort.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
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Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Take with food; exposure roughly triples with a high-fat meal, and the trials dosed with food.
Dabrafenib: take on an empty stomach. Trametinib: take on an empty stomach; both cause pyrexia.
Avoid grapefruit.
See all on the product pages:AlectinibDabrafenibDabrafenib + trametinibEntrectinibLorlatinibNivolumabPembrolizumabTemozolomideTrametinib·Printable cards in the navigator
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