OnCo

Atypical teratoid/rhabdoid tumour (ATRT): lines of therapy

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3 standard-of-care settings across 3 lines and 3 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

LineAll comersFRαGermline
Advanced, first line1··
Third line and beyond·1·
Other settings··1

Advanced, first line

SubgroupSettingApproachProducts and trialsEvidence
All comersNewly diagnosed, any ageMaximal 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.87

Third line and beyond

SubgroupSettingApproachProducts and trialsEvidence
FRαRelapsed or refractoryNo standard; tazemetostat (EZH2 inhibitor) in trials and compassionate use, aurora kinase A inhibition, re-irradiation where feasible; early palliative care.66

Other settings

SubgroupSettingApproachProducts and trialsEvidence
GermlineGermline SMARCB1 or SMARCA4 alterationGenetic counselling and testing of parents and siblings; surveillance imaging for synchronous or second rhabdoid tumours in the kidney and soft tissue.94

Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.