Disease · MONDO_0002728

rhabdoid tumor

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. rhabdoid tumor. Diseases, record MONDO_0002728. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0002728

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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: rhabdoid tumor; Open Targets
Open Targets ID: MONDO_0002728; Open Targets
Other names: malignant rhabdoid tumor, malignant rhabdoid tumour, rhabdoid sarcoma; Open Targets
Disease Ontology: DOID:3672; Disease Ontology
MeSH: D018335; MeSH
NCI Thesaurus: C3808; NCI Thesaurus
Orphanet: ORPHA:69077; Orphanet
Highest stage of its drugs: Phase 2, for 12 of its 20 drugs, from ClinicalTrials.gov and TTD; Open Targets

Drugs20

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.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
TAZEMETOSTAT	CHEMBL3414621	Small molecule	Phase 2	ClinicalTrials.gov and TTD	4	3	3	Drugs and targets
ALISERTIB	CHEMBL483158	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
NIVOLUMAB	CHEMBL2108738	Antibody	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
SELINEXOR	CHEMBL3545185	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
CARBOPLATIN	CHEMBL1351	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
DACTINOMYCIN	CHEMBL1554	Protein	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
DOXORUBICIN	CHEMBL53463	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
DOXORUBICIN HYDROCHLORIDE	CHEMBL359744	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
ETOPOSIDE	CHEMBL44657	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

1 to 10 of 20

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Targets38

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.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
TOP2A	DNA topoisomerase 2-alpha	3	DNA topoisomerase II alpha inhibitor; DNA topoisomerase II inhibitor	Phase 2	https://conductscience.org/library/genes/7153	Drugs and targets
TOP1	DNA topoisomerase 1	2	DNA topoisomerase I inhibitor	Phase 2	https://conductscience.org/library/genes/7150	Drugs and targets
AURKA	Aurora kinase A	1	Serine/threonine-protein kinase Aurora-A inhibitor	Phase 2	https://conductscience.org/library/genes/6790	Drugs and targets
BRAF	Serine/threonine-protein kinase B-raf	1	Serine/threonine-protein kinase B-raf inhibitor	Phase 1/2	https://conductscience.org/library/genes/673	Drugs and targets
CD274	Programmed cell death 1 ligand 1	1	Programmed cell death 1 ligand 1 inhibitor	Phase 1/2	https://conductscience.org/library/genes/29126	Drugs and targets
CDK4	Cyclin-dependent kinase 4	1	Cyclin-dependent kinase 4 inhibitor	Phase 1	https://conductscience.org/library/genes/1019	Drugs and targets
CDK6	Cyclin-dependent kinase 6	1	Cyclin-dependent kinase 6 inhibitor	Phase 1	https://conductscience.org/library/genes/1021	Drugs and targets
CTLA4	Cytotoxic T-lymphocyte protein 4	1	Cytotoxic T-lymphocyte protein 4 inhibitor	Phase 1/2	https://conductscience.org/library/genes/1493	Drugs and targets
EZH2	Histone-lysine N-methyltransferase EZH2	1	Histone-lysine N-methyltransferase EZH2 inhibitor	Phase 2	https://conductscience.org/library/genes/2146	Drugs and targets
FLT1	Vascular endothelial growth factor receptor	1	Vascular endothelial growth factor receptor inhibitor	Phase 1/2	https://conductscience.org/library/genes/2321	Drugs and targets

1 to 10 of 38

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Trials12

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.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT00335556	Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors	Phase 2	291	2017-06-14	Childhood Renal Cell Carcinoma, Clear Cell Renal Cell Carcinoma, Clear Cell Sarcoma of the Kidney, Papillary Renal Cell Carcinoma, Rhabdoid Tumor of the Kidney, Stage I Renal Cell Cancer, Stage I Renal Wilms Tumor, Stage II Renal Cell Cancer, Stage II Renal Wilms Tumor, Stage III Renal Cell Cancer, Stage III Renal Wilms Tumor, Stage IV Renal Cell Cancer, Stage IV Renal Wilms Tumor	CARBOPLATIN, CYCLOPHOSPHAMIDE, DACTINOMYCIN, DOXORUBICIN HYDROCHLORIDE, ETOPOSIDE, IRINOTECAN, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT02601950	A Study of Tazemetostat in Adult Participants With Soft Tissue Sarcoma	Phase 2	267	2025-03-11	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, TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT01154816	Alisertib in Treating Young Patients With Recurrent or Refractory Solid Tumors or Leukemia	Phase 2	118	2017-06-05	Hepatoblastoma, Previously Treated Childhood Rhabdomyosarcoma, Recurrent Childhood Acute Lymphoblastic Leukemia, Recurrent Childhood Acute Myeloid Leukemia, Recurrent Childhood Kidney Neoplasm, Recurrent Childhood Malignant Germ Cell Tumor, Recurrent Childhood Rhabdomyosarcoma, Recurrent Childhood Soft Tissue Sarcoma, Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Recurrent Neuroblastoma, Recurrent Osteosarcoma	ALISERTIB	Open Targets' reading of its record	ClinicalTrials.gov
NCT02601937	EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma	Phase 1	109	2024-10-03	Rhabdoid Tumors, INI1-negative Tumors, Synovial Sarcoma, Malignant Rhabdoid Tumor of Ovary	TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT01747876	Study of Safety and Efficacy in Patients With Malignant Rhabdoid Tumors (MRT) and Neuroblastoma	Phase 1	32	2019-02-28	Malignant Rhabdoid Tumors (MRT), Neuroblastoma	RIBOCICLIB	Open Targets' reading of its record	ClinicalTrials.gov
NCT02114229	Phase 2 Study of Alisertib Therapy for Rhabdoid Tumors	Phase 2	125		Malignant Rhabdoid Tumor, Atypical Teratoid Rhabdoid Tumor	ALISERTIB	Its record names the disease	ClinicalTrials.gov
NCT05468359	Safety and Efficacy of Cyclophosphamide, Sorafenib, Bevacizumab, and Atezolizumab in Pediatric Solid Tumor Patients	Phase 1/2	64		Refractory Solid Tumor, Hepatocellular Carcinoma, Malignant Solid Tumor, Pediatric Cancer, Pediatric Solid Tumor, Fibrolamellar Carcinoma	ATEZOLIZUMAB, BEVACIZUMAB, CYCLOPHOSPHAMIDE, SORAFENIB	Its record names the disease	ClinicalTrials.gov
NCT05407441	Tazemetostat+Nivo/Ipi in INI1-Neg/SMARCA4-Def Tumors	Phase 1/2	49		Atypical Teratoid Rhabdoid Tumor, INI1 (SMARCB1)-Deficient Primary CNS Malignant Tumors, SMARCA4-deficient Primary CNS Malignant Tumors, Malignant Rhabdoid Tumor (MRT), Rhabdoid Tumor of the Kidney (RTK), Epithelioid Sarcoma, Chordoma	IPILIMUMAB, NIVOLUMAB, TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT05985161	A Study of Selinexor in People With Wilms Tumors and Other Solid Tumors	Phase 2	45		Wilms Tumor, Rhabdoid Tumor, Malignant Peripheral Nerve Sheath Tumors, MPNST, Nephroblastoma, XPO1 Gene Mutation, Solid Tumor	SELINEXOR	Its record names the disease	ClinicalTrials.gov
NCT06622941	Study to ONO-4538 in Patients With Rhabdoid Tumor	Phase 2	23		Rhabdoid Tumor	NIVOLUMAB	Its record names the disease	ClinicalTrials.gov

1 to 10 of 12

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Comparisons in its trials0

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.

None in this release.

Papers from its trials5

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

PMID	Listed as	Trials listing it	Source
38501690	DERIVED	1	PubMed
38396300	DERIVED	1	PubMed
34435684	DERIVED	1	PubMed
33035459	DERIVED	1	PubMed
30777875	DERIVED	1	PubMed

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_0002728

GET https://conductscience.org/api/diseases/v1/MONDO_0002728/targets?page=2

API reference For AI assistants