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 connects-toPhoton connects-toNeuroblastoma connects-toRetinoblastoma connects-toNeuron connects-toAstrocyte connects-toGlioblastoma connects-toPd 1 connects-toNervous System connects-toLi Fraumeni Syndrome connects-toMtor connects-toT Cytotoxic Cell connects-toAutophagy connects-toArid1A connects-toMicroglia connects-toNatural Killer Cell connects-toCdk4 6 connects-toDendritic Cell connects-toOligodendrocyte connects-toOxygen connects-toMacrophage connects-toVegf connects-toCalcium connects-toLiver connects-toBone Marrow connects-toElectron connects-toLung connects-toEye connects-toAntibody connects-toMagnesium connects-toNeutrophil connects-toReproductive System connects-toErythrocyte connects-toMusculoskeletal System connects-toIgf 1 connects-toRegulatory T Cell connects-toChordoma connects-toEpilepsy connects-toMeningioma connects-toEndothelial Cell connects-toStat3 connects-toNf Kb connects-toSepsis connects-toAspergillus Fumigatus connects-toCkd connects-toHeart Failure connects-toNeuropathic Pain connects-toAnemia Of Chronic Disease connects-toMajor Depressive Disorder connects-toWound Healing connects-toEndocrine System connects-toGeneralized Anxiety Disorder connects-toRenal System connects-toIntegumentary System connects-toCardiovascular System connects-toImmune System connects-toRespiratory System connects-toDigestive System connects-toCancer Chemotherapy connects-toTargeted Therapy connects-toEwing Sarcoma connects-toCar T connects-toCheckpoint Inhibitors connects-toHippocampus connects-toAlveolus connects-toHepatic Lobule connects-toOsteosarcoma connects-toOvarian Clear Cell Carcinoma connects-toRenal Cell Carcinoma connects-toCortical Bone connects-toCyclin D1 connects-toWnt Beta Catenin connects-toIdh Mutant Glioma connects-toAkt connects-toHif 1Alpha connects-toTert connects-toSmo connects-toNotch connects-toCcl2 connects-toPtch1 connects-toCxcl12 connects-toCaspase 3 connects-toCgas Sting connects-toDnmt3A connects-toFgfr connects-toE2F1 connects-toPik3Ca connects-toBcl 2 connects-toSufu connects-toPten connects-toErk1 2 connects-toJak1 2 connects-toTgf Beta connects-toGalectin 3 connects-toRb1 connects-toStat1 connects-toSmad4 connects-toFoxo connects-toPerforin connects-toS100A8 A9 connects-toGsk 3B connects-toMdm2 connects-toSrc Kinase connects-toAmpk connects-toCcr5 connects-toYap1 connects-toIl 1B connects-toIl 33 connects-toIl 6 connects-toIl 17A connects-toComplement C3 connects-toCalcineurin connects-toMhc Class Ii connects-toIl 2 connects-toCtla 4 connects-toHemoglobin connects-toTroponin Complex connects-toXanthine Oxidase connects-toProton connects-toIl 10 connects-toNitric Oxide connects-toIl 4 connects-toIl 13 connects-toIron connects-toProton connects-toPhoton connects-toPdgf connects-toLeptin connects-toAdiponectin connects-toHeart connects-toIfn Gamma connects-toIl 12 connects-toResistin connects-toIl 5 connects-toIl 23 connects-toIge connects-toT Helper Cell connects-toB Cell connects-toMast Cell connects-toComplement C5 connects-toC5Ar1 connects-toFactor H connects-toTslp connects-toBradykinin connects-toErythropoietin connects-toHistamine connects-toMelatonin connects-toC1 Esterase Inhibitor connects-toTestosterone connects-toSerotonin connects-toProlactin connects-toOxytocin connects-toVasopressin connects-toSelenium connects-toSodium connects-toPotassium connects-toZinc connects-toIodine connects-toCopper connects-toPhosphorus connects-toCarbon connects-toChloride connects-toHydrogen connects-toGlp 1 connects-toAngiotensin Ii connects-toRankl connects-toFibronectin connects-toActivin A connects-toCgrp connects-toCalcitonin connects-toSubstance P connects-toInsulin Receptor connects-toAldosterone connects-toAndrogen Receptor connects-toNorepinephrine connects-toAdrenomedullin connects-toBdnf connects-toOsteopontin connects-toEpinephrine connects-toRenin connects-toMyostatin

Sources