Atypical teratoid/rhabdoid tumor (ATRT)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-ATRT |
|---|---|
| Type | Disease |
| Aliases | AT/RTATRTAtypical teratoid/rhabdoid tumorCNS rhabdoid tumorEmbryonal CNS tumor with rhabdoid featuresАтиповий тератоїдний/рабдоїдний пухлина (ATRT) |
| Status | reviewed 2026-05-19 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-NCCN-CNS-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 9508/3 |
|---|---|
| icd_o_3_topography | ["C71"] |
| icd_10 | C71.9 |
| who_classification | WHO CNS5 (2021): Atypical teratoid/rhabdoid tumor (ATRT) — molecularly defined by SMARCB1 (INI1) loss OR SMARCA4 (BRG1) loss; subgroups ATRT-TYR, ATRT-SHH, ATRT-MYC |
Notes
Aggressive multimodal therapy is the standard: max-safe surgical resection + adjuvant intensified chemotherapy + (in patients >3 y who tolerate) craniospinal RT. Tazemetostat (EZH2 inhibitor) is FDA-approved for relapsed/refractory adult and pediatric INI1- negative epithelioid sarcoma + ATRT (limited pediatric data). Surveillance for RTPS carriers: brain MRI q3mo from infancy to age 3-4, then q6mo to age 5; whole-body MRI q3-6mo to age 5 to detect extracranial MRT of kidney + soft tissue.
Used By
No reverse references found in the YAML corpus.