The first 60 days: WNT-activated medulloblastoma
WNT-activated medulloblastoma is the rarest and most curable of the four molecular groups of medulloblastoma, a brain tumour of the cerebellum. It is driven by a mutation in the beta-catenin gene that switches the WNT growth pathway on. Almost every child is cured with standard therapy, so current trials are asking how much radiotherapy and chemotherapy can be taken away. Below, week by week, is what OnCo's record of WNT-activated medulloblastoma 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.
- PathologistNamed in the standard of care for: Non-metastatic, standard therapy, Non-metastatic, de-escalation trials.
- SurgeonNamed in the standard of care for: Non-metastatic, standard therapy.
- Medical oncologistNamed in the standard of care for: Non-metastatic, standard therapy, Non-metastatic, de-escalation trials, Metastatic or residual disease, Survivorship.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Non-metastatic, standard therapy, Non-metastatic, de-escalation trials, Metastatic or residual disease.
- Transplant and cell therapy teamNamed in the standard of care for: Non-metastatic, standard therapy, Non-metastatic, de-escalation trials, Metastatic or residual disease.
- 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.Non-metastatic, standard therapyNCI PDQ: Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (health professional version)
Maximal safe resection, 23.4 Gy craniospinal radiotherapy with tumour-bed boost (protons where available), then cisplatin, vincristine and cyclophosphamide or lomustine.
- 2.Non-metastatic, de-escalation trialsNCI PDQ: Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (health professional version)
15 Gy (SJMB12) or 18 Gy (ACNS1422) craniospinal radiotherapy with reduced boost and fewer chemotherapy cycles, judged against historical survival.
- 3.Metastatic or residual diseaseNCI PDQ: Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (health professional version)
High-risk therapy: 36 Gy craniospinal radiotherapy with boost and intensified chemotherapy, as for other groups.
- 4.SurvivorshipNCI PDQ: Childhood Medulloblastoma and Other CNS Embryonal Tumors Treatment (health professional version)
Neurocognitive, audiological, endocrine 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 Nuclear beta-catenin immunohistochemistry, CTNNB1 exon 3 mutation, Monosomy 6, DNA methylation profiling, APC germline testing), 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 WNT-activated medulloblastoma, CTNNB1-mutant with monosomy 6, WNT-activated medulloblastoma with germline APC mutation, Metastatic WNT-activated medulloblastoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Non-metastatic, standard therapy
- For my situation (non-metastatic, standard therapy), 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 (protons where available), 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.
Non-metastatic, de-escalation trials
- For my situation (non-metastatic, de-escalation trials), which of the standard options do you recommend and why?Guideline options include: 15 Gy (SJMB12) or 18 Gy (ACNS1422) craniospinal radiotherapy with reduced boost and fewer chemotherapy cycles, judged against historical survival.
- Am I a candidate for Cisplatin, Vincristine, Cyclophosphamide, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Metastatic or residual disease
- For my situation (metastatic or residual disease), which of the standard options do you recommend and why?Guideline options include: High-risk therapy: 36 Gy craniospinal radiotherapy with boost and intensified chemotherapy, as for other groups.
- Am I a candidate for Carboplatin, Cisplatin, Cyclophosphamide or related drugs, 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, audiological, endocrine and second-tumour follow-up for life.
Any stage
- Are there clinical trials I could join, for example of Proton therapy, DNA methylation profiling, Germline (hereditary) testing?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 “How far craniospinal radiotherapy can be reduced without losing cures”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Whether adult and metastatic WNT tumours share the childhood prognosis”. 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.
- WNT-activated medulloblastoma: the full pageWNT-activated medulloblastoma is the rarest and most curable of the four molecular groups of medulloblastoma, a brain tumour of the cerebellum. It is driven by a mutation in the beta-catenin gene that switches the WNT growth pathway on. Almost every child is cured with standard therapy, so current trials are asking how much radiotherapy and chemotherapy can be taken away.
- 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.