The first 60 days: Atypical teratoid/rhabdoid tumour (ATRT)

Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.

• Newly diagnosed, any ageCOG ACNS0333 (JCO 2020); EU-RHAB

  Maximal safe resection followed by an intensive multimodal protocol: ACNS0333-style induction, high-dose chemotherapy with autologous stem-cell rescue, and age-adapted focal radiotherapy; or the EU-RHAB regimen with intraventricular methotrexate. Enrolment in SIOPE ATRT01 or a COG successor where available.

  Autologous stem cell transplant (high-dose therapy)MethotrexateCyclophosphamideCisplatinCarboplatinVincristineProton therapyACNS0333

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: Germline SMARCB1 or SMARCA4 alteration, Relapsed or refractory.
• RadiologistNamed in the standard of care for: Germline SMARCB1 or SMARCA4 alteration.
• SurgeonNamed in the standard of care for: Newly diagnosed, any age.
• Medical oncologistNamed in the standard of care for: Newly diagnosed, any age, Relapsed or refractory.
• Clinical oncologist (radiotherapy)Named in the standard of care for: Newly diagnosed, any age, Relapsed or refractory.
• Transplant and cell therapy teamNamed in the standard of care for: Newly diagnosed, any age.
• Palliative and supportive care teamNamed in the standard of care for: Relapsed or refractory.

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. 1.Germline SMARCB1 or SMARCA4 alterationNCI PDQ: childhood CNS atypical teratoid/rhabdoid tumour

   Genetic counselling and testing of parents and siblings; surveillance imaging for synchronous or second rhabdoid tumours in the kidney and soft tissue.

   Germline (hereditary) testing
2. 2.Relapsed or refractory

   Aurora kinase A inhibition and re-irradiation where feasible, with enrolment in a relapse trial the preferred route; EZH2 inhibition with tazemetostat was used in trials and on compassionate grounds until the drug was withdrawn from all markets in March 2026. No regimen is standard at relapse, and symptom and supportive care run alongside from the start.

   TazemetostatEarly integrated palliative care

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.

Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.

• NCI-COG Pediatric MATCH (APEC1621)Platform trial · active · NCT03155620Relapsed or refractory solid tumours, non-Hodgkin lymphomas and histiocytic disorders, age 1-21: tumour sequencing then assignment to one of a dozen single-agent targeted-therapy phase 2 arms

Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.

• Free in oncologyFree testing, helplines, rides and lodging, second opinions and trial travel help, with who is eligible.
• Financial helpManufacturer programmes, national schemes and charities by country and product.
• Second opinionExpert centres for this cancer in your country and how referral works.

• Atypical teratoid/rhabdoid tumour (ATRT): the full pageATRT is an aggressive brain tumour of babies and toddlers caused by loss of a single gene, SMARCB1, part of the machinery that opens and closes DNA. Intensive chemotherapy with stem-cell rescue, and radiotherapy where age allows, now cure a meaningful share of children who once had little chance, and drugs aimed at the epigenetic consequence of SMARCB1 loss (EZH2 inhibitors) are in trials.
• 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.