The first 60 days: Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
Group 3 and group 4 medulloblastoma are the two commonest forms of this cerebellar brain tumour and the ones without a druggable driver. Group 3 strikes young children, often with extra copies of MYC and spread through the spinal fluid; group 4 affects older boys. Both get surgery, craniospinal radiotherapy and chemotherapy; trials showed the radiation dose cannot be cut for young children. Below, week by week, is what OnCo's record of Group 3 and group 4 medulloblastoma (non-WNT/non-SHH) 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.
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.
- SurgeonNamed in the standard of care for: Average risk (aged 3 or over, no metastases, residual under 1.5 square centimetres).
- Medical oncologistNamed in the standard of care for: Average risk (aged 3 or over, no metastases, residual under 1.5 square centimetres), High risk (metastatic, residual disease or MYC amplification), Infants under three, Relapsed and 1 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Average risk (aged 3 or over, no metastases, residual under 1.5 square centimetres), High risk (metastatic, residual disease or MYC amplification), Infants under three, Relapsed.
- Transplant and cell therapy teamNamed in the standard of care for: Average risk (aged 3 or over, no metastases, residual under 1.5 square centimetres), High risk (metastatic, residual disease or MYC amplification), Infants under three, Relapsed.
- Palliative and supportive care teamNamed in the standard of care for: Survivorship.
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.Infants under threeNCI PDQ: Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (health professional version)
Intensive chemotherapy with methotrexate, then high-dose consolidation with stem cell rescue, to avoid or delay craniospinal radiotherapy.
- 2.High risk (metastatic, residual disease or MYC amplification)NCI PDQ: Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (health professional version)
36 Gy craniospinal radiotherapy with boost, daily carboplatin during radiotherapy for group 3 (ACNS0332), then cisplatin, vincristine and cyclophosphamide.
- 3.Average risk (aged 3 or over, no metastases, residual under 1.5 square centimetres)NCI PDQ: Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (health professional version)
Maximal safe resection, 23.4 Gy craniospinal radiotherapy with tumour-bed boost (ACNS0331 showed 18 Gy is inferior), then cisplatin, vincristine and cyclophosphamide or lomustine.
- 4.RelapsedNCI PDQ: Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (health professional version)
Temozolomide with irinotecan, bevacizumab, re-irradiation or high-dose chemotherapy; almost never curative; early-phase trials.
- 5.SurvivorshipNCI PDQ: Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (health professional version)
Neurocognitive, endocrine, hearing, vascular and second-tumour follow-up for life.
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 DNA methylation profiling, MYC and MYCN amplification, Isochromosome 17q, Chromosome 11 loss and whole chromosome 17 gain, Metastatic staging by spinal MRI and CSF cytology), 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 Group 3 medulloblastoma, MYC-amplified, Group 3 medulloblastoma without MYC amplification, Group 4 medulloblastoma, low risk.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Average risk (aged 3 or over, no metastases, residual under 1.5 square centimetres)
- For my situation (average risk (aged 3 or over, no metastases, residual under 1.5 square centimetres)), which of the standard options do you recommend and why?Guideline options include: Maximal safe resection, 23.4 Gy craniospinal radiotherapy with tumour-bed boost (ACNS0331 showed 18 Gy is inferior), then cisplatin, vincristine and cyclophosphamide or lomustine.
- Am I a candidate for Cisplatin, Vincristine, Cyclophosphamide or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of COG ACNS0331 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
High risk (metastatic, residual disease or MYC amplification)
- For my situation (high risk (metastatic, residual disease or myc amplification)), which of the standard options do you recommend and why?Guideline options include: 36 Gy craniospinal radiotherapy with boost, daily carboplatin during radiotherapy for group 3 (ACNS0332), then cisplatin, vincristine and cyclophosphamide.
- Am I a candidate for Carboplatin, Cisplatin, Vincristine or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Infants under three
- For my situation (infants under three), which of the standard options do you recommend and why?Guideline options include: Intensive chemotherapy with methotrexate, then high-dose consolidation with stem cell rescue, to avoid or delay craniospinal radiotherapy.
- Am I a candidate for Methotrexate, Cyclophosphamide, Vincristine or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Relapsed
- For my situation (relapsed), which of the standard options do you recommend and why?Guideline options include: Temozolomide with irinotecan, bevacizumab, re-irradiation or high-dose chemotherapy; almost never curative; early-phase trials.
- Am I a candidate for Temozolomide, Irinotecan (and liposomal irinotecan), Bevacizumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Survivorship
- For my situation (survivorship), which of the standard options do you recommend and why?Guideline options include: Neurocognitive, endocrine, hearing, vascular and second-tumour follow-up for life.
Any stage
- Are there clinical trials I could join, for example of DNA methylation profiling, Proton therapy, Temozolomide, Bevacizumab?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 “MYC and MYCN have no inhibitor, and relapse is almost always fatal”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Craniospinal radiotherapy dose cannot be reduced in young children without losing cures”. 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.
- Group 3 and group 4 medulloblastoma (non-WNT/non-SHH): the full pageGroup 3 and group 4 medulloblastoma are the two commonest forms of this cerebellar brain tumour and the ones without a druggable driver. Group 3 strikes young children, often with extra copies of MYC and spread through the spinal fluid; group 4 affects older boys. Both get surgery, craniospinal radiotherapy and chemotherapy; trials showed the radiation dose cannot be cut for young children.
- 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.
- Medulloblastoma molecular groups (WNT, SHH, group 3, group 4): Medulloblastoma is four diseases under one microscope: the WNT group is almost always cured, the SHH group depends on age and TP53, and groups 3 and 4 carry most of the deaths, so the group now steers how much radiation and chemotherapy a child receives.
- 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.