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Disease · MONDO_0020560

atypical teratoid rhabdoid tumor

6 sourcesdiseases-2026-09-18-0.1.0

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ConductScience Library. atypical teratoid rhabdoid tumor. Diseases, record MONDO_0020560. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0020560

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Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name
atypical teratoid rhabdoid tumor
Open Targets
Open Targets ID
MONDO_0020560
Open Targets
Other names
AT/RT, ATRT, ATT/RHT, CNS rhabdoid neoplasm, CNS rhabdoid tumor, CNS rhabdoid tumour, atypical teratoid/rhabdoid tumor (WHO grade IV), atypical teratoid/rhabdoid tumor (morphologic abnormality), atypical teratoid/rhabdoid tumour (WHO grade IV), atypical teratoid/rhabdoid tumour (morphologic abnormality), central nervous system rhabdoid neoplasm, central nervous system rhabdoid tumor, central nervous system rhabdoid tumour, malignant brain rhabdoid neoplasm, malignant brain rhabdoid tumor, malignant brain rhabdoid tumour, malignant rhabdoid neoplasm of brain, malignant rhabdoid neoplasm of the brain, malignant rhabdoid tumor of brain, malignant rhabdoid tumor of the brain, malignant rhabdoid tumour of brain, malignant rhabdoid tumour of the brain, primary malignant brain rhabdoid neoplasm, primary malignant brain rhabdoid tumor, primary malignant brain rhabdoid tumour, primary malignant rhabdoid neoplasm of brain, primary malignant rhabdoid neoplasm of the brain, primary malignant rhabdoid tumor of brain, primary malignant rhabdoid tumor of the brain, primary malignant rhabdoid tumour of brain, primary malignant rhabdoid tumour of the brain, rhabdoid neoplasm of CNS, rhabdoid neoplasm of central nervous system, rhabdoid neoplasm of the CNS, rhabdoid neoplasm of the central nervous system, rhabdoid tumor of CNS, rhabdoid tumor of central nervous system, rhabdoid tumor of the CNS, rhabdoid tumor of the central nervous system, rhabdoid tumour of CNS, rhabdoid tumour of central nervous system, rhabdoid tumour of the central nervous system
Open Targets
Disease Ontology
DOID:2129
Disease Ontology
NCI Thesaurus
C6906
NCI Thesaurus
Orphanet
ORPHA:99966
Orphanet
Highest stage of its drugs
Phase 3, for 9 of its 37 drugs, from ClinicalTrials.gov
Open Targets

Drugs37

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

DrugChEMBL IDTypeOpen Targets' stageStage reported byReportsOf them trialsTrials shown hereSource
CYCLOPHOSPHAMIDECHEMBL1200796Small moleculePhase 3ClinicalTrials.gov555Drugs and targets
METHOTREXATECHEMBL34259Small moleculePhase 3ClinicalTrials.gov555Drugs and targets
ETOPOSIDECHEMBL44657Small moleculePhase 3ClinicalTrials.gov444Drugs and targets
VINCRISTINECHEMBL90555Small moleculePhase 3ClinicalTrials.gov444Drugs and targets
CISPLATINCHEMBL11359Small moleculePhase 3ClinicalTrials.gov333Drugs and targets
TEMOZOLOMIDECHEMBL810Small moleculePhase 3ClinicalTrials.gov333Drugs and targets
FILGRASTIMCHEMBL1201567ProteinPhase 3ClinicalTrials.gov222Drugs and targets
CARBOPLATINCHEMBL1351Small moleculePhase 3ClinicalTrials.gov111Drugs and targets
THIOTEPACHEMBL671Small moleculePhase 3ClinicalTrials.gov111Drugs and targets
TAZEMETOSTATCHEMBL3414621Small moleculePhase 2ClinicalTrials.gov333Drugs and targets

1 to 10 of 37

See all 37 drugs

Targets161

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

TargetProteinIts drugs that act on itMechanismsHighest stage of those drugsGene in GenesSource
HDAC1Histone deacetylase2Histone deacetylase 1 inhibitor; Histone deacetylase inhibitorPhase 1/2https://conductscience.org/library/genes/3065Drugs and targets
HDAC2Histone deacetylase2Histone deacetylase 2 inhibitor; Histone deacetylase inhibitorPhase 1/2https://conductscience.org/library/genes/3066Drugs and targets
HDAC3Histone deacetylase2Histone deacetylase 3 inhibitor; Histone deacetylase inhibitorPhase 1/2https://conductscience.org/library/genes/8841Drugs and targets
HDAC6Histone deacetylase2Histone deacetylase 6 inhibitor; Histone deacetylase inhibitorPhase 1/2https://conductscience.org/library/genes/10013Drugs and targets
POLA1DNA polymerase (alpha/delta/epsilon)2DNA polymerase (alpha/delta/epsilon) inhibitorPhase 2https://conductscience.org/library/genes/5422Drugs and targets
POLA2DNA polymerase (alpha/delta/epsilon)2DNA polymerase (alpha/delta/epsilon) inhibitorPhase 2https://conductscience.org/library/genes/23649Drugs and targets
POLD1DNA polymerase (alpha/delta/epsilon)2DNA polymerase (alpha/delta/epsilon) inhibitorPhase 2https://conductscience.org/library/genes/5424Drugs and targets
POLD2DNA polymerase (alpha/delta/epsilon)2DNA polymerase (alpha/delta/epsilon) inhibitorPhase 2https://conductscience.org/library/genes/5425Drugs and targets
POLD3DNA polymerase (alpha/delta/epsilon)2DNA polymerase (alpha/delta/epsilon) inhibitorPhase 2https://conductscience.org/library/genes/10714Drugs and targets
POLD4DNA polymerase (alpha/delta/epsilon)2DNA polymerase (alpha/delta/epsilon) inhibitorPhase 2https://conductscience.org/library/genes/57804Drugs and targets

1 to 10 of 161

See all 161 targets

Trials21

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

TrialTitlePhaseParticipantsResults first postedConditionsDrugsLinked bySource
NCT00085202Treatment of Patients With Newly Diagnosed Medulloblastoma, Supratentorial Primitive Neuroectodermal Tumor, or Atypical Teratoid Rhabdoid TumorPhase 34162014-02-27Brain and Central Nervous System TumorsCISPLATIN, CYCLOPHOSPHAMIDE, FILGRASTIM, VINCRISTINEIts record names the diseaseClinicalTrials.gov
NCT02601950A Study of Tazemetostat in Adult Participants With Soft Tissue SarcomaPhase 22672025-03-11
Malignant Rhabdoid Tumors (MRT), Rhabdoid Tumors of the Kidney (RTK), Atypical Teratoid Rhabdoid Tumors (ATRT), Selected Tumors With Rhabdoid Features, Synovial Sarcoma, INI1-negative Tumors, Malignant Rhabdoid Tumor of Ovary, Renal Medullary Carcinoma, Epithelioid Sarcoma, Poorly Differentiated Chordoma (or Other Chordoma With Sponsor Approval), Any Solid Tumor With an EZH2 GOF Mutation
DOXORUBICIN, TAZEMETOSTATIts record names the diseaseClinicalTrials.gov
NCT02601937EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial SarcomaPhase 11092024-10-03Rhabdoid Tumors, INI1-negative Tumors, Synovial Sarcoma, Malignant Rhabdoid Tumor of OvaryTAZEMETOSTATIts record names the diseaseClinicalTrials.gov
NCT00084838Chemotherapy Combined With Radiation Therapy for Newly Diagnosed CNS AT/RTPhase 2252015-12-24Central Nervous System Tumor, PediatricCISPLATIN, CYCLOPHOSPHAMIDE, CYTARABINE, DACTINOMYCIN, DOXORUBICIN, ETOPOSIDE, FILGRASTIM, HYDROCORTISONE, LEUCOVORIN CALCIUM, METHOTREXATE, TEMOZOLOMIDE, VINCRISTINEIts record names the diseaseClinicalTrials.gov
NCT00003469Antineoplaston Therapy in Treating Children With Rhabdoid Tumor of the Central Nervous SystemPhase 242017-03-07Rhabdoid Neoplasm of CNSANTINEOPLASTON A10Its record names the diseaseClinicalTrials.gov
NCT01356290Antiangiogenic Therapy for Children With Recurrent Medulloblastoma, Ependymoma, ATRT and Rare CNS TumorsPhase 2232Medulloblastoma Recurrent, Ependymoma Recurrent, ATRT Recurrent, Rare CNS Tumor RecurrentBEVACIZUMAB, CELECOXIB, CYCLOPHOSPHAMIDE, CYTARABINE, ETOPOSIDE, FENOFIBRATE, THALIDOMIDEIts record names the diseaseClinicalTrials.gov
NCT02114229Phase 2 Study of Alisertib Therapy for Rhabdoid TumorsPhase 2125Malignant Rhabdoid Tumor, Atypical Teratoid Rhabdoid TumorALISERTIBIts record names the diseaseClinicalTrials.gov
NCT00392886Combination Chemotherapy With or Without Etoposide Followed By an Autologous Stem Cell Transplant in Treating Young Patients With Previously Untreated Malignant Brain TumorsPhase 3120Brain and Central Nervous System TumorsCARBOPLATIN, CISPLATIN, CYCLOPHOSPHAMIDE, ETOPOSIDE, METHOTREXATE, TEMOZOLOMIDE, THIOTEPA, VINCRISTINEOpen Targets' reading of its recordClinicalTrials.gov
NCT03838042INFORM2 Study Uses Nivolumab and Entinostat in Children and Adolescents With High-risk Refractory MalignanciesPhase 1/291CNS Tumor, Solid TumorENTINOSTAT, NIVOLUMABIts record names the diseaseClinicalTrials.gov
NCT00217412Vorinostat With or Without Isotretinoin in Treating Young Patients With Recurrent or Refractory Solid Tumors, Lymphoma, or LeukemiaPhase 160
Childhood Acute Promyelocytic Leukemia (M3), Childhood Atypical Teratoid/Rhabdoid Tumor, Childhood Burkitt Lymphoma, Childhood Chronic Myelogenous Leukemia, Childhood Diffuse Large Cell Lymphoma, Childhood Immunoblastic Large Cell Lymphoma, Juvenile Myelomonocytic Leukemia, Recurrent Childhood Acute Lymphoblastic Leukemia, Recurrent Childhood Acute Myeloid Leukemia, Recurrent Childhood Grade III Lymphomatoid Granulomatosis, Recurrent Childhood Large Cell Lymphoma, Recurrent Childhood Lymphoblastic Lymphoma, Recurrent Childhood Medulloblastoma, Recurrent Childhood Small Noncleaved Cell Lymphoma, Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor, Recurrent Neuroblastoma, Recurrent/Refractory Childhood Hodgkin Lymphoma, Relapsing Chronic Myelogenous Leukemia, Unspecified Childhood Solid Tumor, Protocol Specific
ISOTRETINOIN, VORINOSTATIts record names the diseaseClinicalTrials.gov

1 to 10 of 21

See all 21 trials

Comparisons in its trials1

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

ComparisonGroup 1, with the outcomeGroup 2, with the outcomeParticipantsTrial
Average-Risk Group vs High-Risk Group: serious adverse events17 of 26917 of 144413NCT00085202

Papers from its trials15

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMIDListed asTrials listing itSource
37883081RESULT1PubMed
19064966RESULT1PubMed
18447671RESULT1PubMed
17019740RESULT1PubMed
42745490DERIVED1PubMed
38501690DERIVED1PubMed
36291912DERIVED1PubMed
36151773DERIVED1PubMed
34714708DERIVED1PubMed
33743477DERIVED1PubMed

Data dates

The date of each source this release read.

Open Targets
2026-09-18
Open Targets
Drug links and their checks, from Drugs and targets
2026-09-18
Drugs and targets
ClinicalTrials.gov, from Trials
2026-11-12
Trials
Comparisons, from Trial comparisons
2026-03-23
Trial comparisons
PubMed, for the checks
2026-11-06
PubMed
MeSH, for the checks
2026-11-10
MeSH

Sources 6

  • ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
  • ChEMBL, through Open TargetsCC BY-SA 3.0
  • ClinicalTrials.govNo license stated
  • NLM's MeSHNLM's terms
  • Open TargetsCC0 1.0
  • PubMedNLM's terms
Each source's terms
  • ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
  • ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
  • ClinicalTrials.govnone stated
  • NLM's MeSHNLM's
  • Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
  • PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0020560

GET https://conductscience.org/api/diseases/v1/MONDO_0020560/drugs?page=2