The first 60 days: Paediatric high-grade glioma (excluding diffuse midline glioma)
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. Below, week by week, is what OnCo's record of Paediatric high-grade glioma (excluding diffuse midline glioma) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
- Newly diagnosed, child over about three yearsNCI PDQ: childhood astrocytomas, other gliomas and glioneuronal tumours
Maximal safe resection, focal radiotherapy and temozolomide during and after radiotherapy by extrapolation from adult practice; molecular profiling of every tumour.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Newly diagnosed, child over about three years, Infant-type hemispheric glioma with a fusion, Constitutional mismatch repair deficiency.
- SurgeonNamed in the standard of care for: Newly diagnosed, child over about three years, Infant-type hemispheric glioma with a fusion, Recurrence.
- Medical oncologistNamed in the standard of care for: Newly diagnosed, child over about three years, Infant-type hemispheric glioma with a fusion, BRAF V600E-mutant, Constitutional mismatch repair deficiency.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Newly diagnosed, child over about three years, Infant-type hemispheric glioma with a fusion, Recurrence.
- Transplant and cell therapy teamNamed in the standard of care for: Recurrence.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
- 1.Infant-type hemispheric glioma with a fusionNCI PDQ: childhood astrocytomas, other gliomas and glioneuronal tumours
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.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example H3 G34 mutationand, to exclude diffuse midline glioma, H3 K27 status, IDH1/IDH2, NTRK, ALK, ROS1 and MET fusions in infants, BRAF V600E with CDKN2A deletion, PDGFRA, MYCN and EGFR amplification), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Diffuse hemispheric glioma, H3 G34-mutant, Diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype, Infant-type hemispheric glioma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Newly diagnosed, child over about three years
- For my situation (newly diagnosed, child over about three years), which of the standard options do you recommend and why?Guideline options include: Maximal safe resection, focal radiotherapy and temozolomide during and after radiotherapy by extrapolation from adult practice; molecular profiling of every tumour.
- Am I a candidate for Temozolomide, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Infant-type hemispheric glioma with a fusion
- For my situation (infant-type hemispheric glioma with a fusion), which of the standard options do you recommend and why?Guideline options include: Surgery and fusion-directed therapy: larotrectinib or entrectinib for NTRK fusions, alectinib or lorlatinib for ALK or ROS1 fusions; chemotherapy to defer radiotherapy.
- Am I a candidate for Larotrectinib, Entrectinib, Alectinib or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
BRAF V600E-mutant
- For my situation (braf v600e-mutant), which of the standard options do you recommend and why?Guideline options include: Dabrafenib plus trametinib (paediatric high-grade glioma cohort 2023; tumour-agnostic approval for BRAF V600E solid tumours from age six).
- Am I a candidate for Dabrafenib + trametinib, Dabrafenib, Trametinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Constitutional mismatch repair deficiency
- For my situation (constitutional mismatch repair deficiency), which of the standard options do you recommend and why?Guideline options include: PD-1 blockade (nivolumab or pembrolizumab) for hypermutant tumours; germline counselling for the family.
- Am I a candidate for Nivolumab, Pembrolizumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Recurrence
- For my situation (recurrence), which of the standard options do you recommend and why?Guideline options include: No standard; re-resection, re-irradiation, clinical trials including CAR-T and oncolytic virus studies.
Any stage
- Are there clinical trials I could join, for example of Dabrafenib + trametinib, Larotrectinib, Entrectinib, CAR-T for glioma (IL13Rα2, GD2, EGFRvIII, multi-target)?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “No systemic therapy has improved survival in H3 G34-mutant or H3- and IDH-wildtype tumours”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “How long to continue fusion or BRAF inhibitors in children who respond, and what happens on stopping”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Paediatric high-grade glioma (excluding diffuse midline glioma): the full pageHigh-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.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- H3 K27M (diffuse midline glioma): A single change in a histone protein that defines diffuse midline glioma, the childhood brain tumour with the fewest treatment options, and now the target of the first approved drug for it.
- Re-irradiation: Giving radiotherapy again to a region that has already been treated, once thought impossible because normal tissues remember the first dose.
- Late effects and survivorship toxicity: Health problems appearing months or decades after treatment ends: heart damage, infertility, second cancers, lymphoedema, dry mouth, memory problems, weak bones.
Every term links to the glossary.