Atlas One · Host Biology · Scale 07 — System

Atypical Teratoid/Rhabdoid Tumor

AT/RT (atypical teratoid/rhabdoid tumor) is an aggressive CNS WHO grade 4 pediatric tumor defined by SMARCB1 biallelic LOF (~95%) or SMARCA4 LOF (~5%); peak <3 years; three molecular subgroups (TYR, SHH, MYC); multimodal therapy; 2-year OS ~40-50%; germline SMARCB1 → RTPS1.

Also known as: AT/RT, atypical teratoid rhabdoid tumor, ATRT, rhabdoid tumor brain, INI1-deficient CNS tumor, SMARCB1 brain tumor, SMARCB1-deficient tumor, pediatric CNS rhabdoid, rhabdoid tumor predisposition

Scale 07 — SystemScale
draftStatus
2026-06-06Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
atypical-teratoid-rhabdoid-tumor

Atypical Teratoid/Rhabdoid Tumor

AT/RT (atypical teratoid/rhabdoid tumor) is an aggressive CNS WHO grade 4 pediatric tumor defined by SMARCB1 biallelic LOF (~95%) or SMARCA4 LOF (~5%); peak <3 years; three molecular subgroups (TYR, SHH, MYC); multimodal therapy; 2-year OS ~40-50%; germline SMARCB1 → RTPS1.

Entry Metadata

FieldValue
IDatypical-teratoid-rhabdoid-tumor
NameAtypical Teratoid/Rhabdoid Tumor
Statusdraft
Last reviewed2026-06-06
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toSmarcb1 connects-toEzh2 connects-toMyc connects-toP53 connects-toCdkn2A connects-toBrain connects-toMedulloblastoma connects-toSynovial Sarcoma connects-toKidney connects-toSchwannomatosis connects-toWilms Tumor connects-toRhabdomyosarcoma connects-toDiffuse Midline Glioma

Sources