Rhabdoid tumor of the kidney
diseaseOn this page
Also known as kidney rhabdoid tumorkidney rhabdoid tumourmalignant rhabdoid tumor of kidneymalignant rhabdoid tumor of the kidneymalignant rhabdoid tumour of kidneymalignant rhabdoid tumour of the kidneyMRTKrenal rhabdoid neoplasmrenal rhabdoid tumorrenal rhabdoid tumourrhabdoid neoplasm of kidneyrhabdoid neoplasm of the kidneyrhabdoid tumor of kidneyrhabdoid tumor of the kidney (RTK)rhabdoid tumour of kidneyrhabdoid tumour of the kidney (RTK)
Summary
Rhabdoid tumor of the kidney (MONDO:0002729) is a cancer and 11 clinical trials. Top therapeutic interventions include dactinomycin, doxorubicin hydrochloride, and tazemetostat. A subtype of rhabdoid tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Clinical trials: 11
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | rhabdoid tumor of the kidney |
| Mondo ID | MONDO:0002729 |
| EFO | EFO:1000512 |
| DOID | DOID:3674 |
| NCIT | C8715 |
| UMLS | C0854917 |
| MedGen | 208930 |
| GARD | 0023224 |
| Anatomy (UBERON) | UBERON:0002113 |
| Is cancer (heuristic) | yes |
Also known as: kidney rhabdoid tumor · kidney rhabdoid tumour · malignant rhabdoid tumor of kidney · malignant rhabdoid tumor of the kidney · malignant rhabdoid tumour of kidney · malignant rhabdoid tumour of the kidney · MRTK · renal rhabdoid neoplasm · renal rhabdoid tumor · renal rhabdoid tumour · rhabdoid neoplasm of kidney · rhabdoid neoplasm of the kidney · rhabdoid tumor of kidney · rhabdoid tumor of the kidney · rhabdoid tumor of the kidney (RTK) · rhabdoid tumour of kidney · rhabdoid tumour of the kidney (RTK)
Data availability: 26 cell lines.
Disease family
This is a subtype of rhabdoid tumor. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › embryonal neoplasm › rhabdoid tumor › rhabdoid tumor of the kidney
Related subtypes (4): striated muscle rhabdoid tumor, familial rhabdoid tumor, atypical teratoid rhabdoid tumor, extrarenal rhabdoid tumor
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 11.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 5 |
| PHASE2 | 3 |
| PHASE3 | 2 |
| PHASE1/PHASE2 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT00945009 | PHASE3 | ACTIVE_NOT_RECRUITING | Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor |
| NCT03793166 | PHASE3 | ACTIVE_NOT_RECRUITING | Immunotherapy With Nivolumab and Ipilimumab Followed by Nivolumab or Nivolumab With Cabozantinib for Patients With Advanced Kidney Cancer, The PDIGREE Study |
| NCT04284774 | PHASE2 | ACTIVE_NOT_RECRUITING | Tipifarnib for the Treatment of Advanced Solid Tumors, Lymphoma, or Histiocytic Disorders With HRAS Gene Alterations, a Pediatric MATCH Treatment Trial |
| NCT04416568 | PHASE2 | ACTIVE_NOT_RECRUITING | Study of Nivolumab and Ipilimumab in Children and Young Adults With INI1-Negative Cancers |
| NCT05407441 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Tazemetostat+Nivo/Ipi in INI1-Neg/SMARCA4-Def Tumors |
| NCT00335556 | PHASE2 | COMPLETED | Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors |
| NCT00898365 | Not specified | COMPLETED | Study of Kidney Tumors in Younger Patients |
| NCT01118078 | Not specified | COMPLETED | Biomarkers in Tissue Samples From Patients With High-Risk Wilms Tumor |
| NCT01453465 | Not specified | WITHDRAWN | Biomarker Study in Samples From Patients With Malignant Rhabdoid Tumor of the Kidney or Atypical Teratoid Rhabdoid Tumor |
| NCT01642095 | Not specified | WITHDRAWN | Studying Biomarkers in Samples From Younger Patients With Kidney Cancer |
| NCT03874455 | Not specified | NO_LONGER_AVAILABLE | Tazemetostat Expanded Access Program for Adults With Solid Tumors |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| DACTINOMYCIN | 4 | 2 |
| DOXORUBICIN HYDROCHLORIDE | 4 | 2 |
| TAZEMETOSTAT | 4 | 2 |
| CABOZANTINIB | 4 | 1 |
| IPILIMUMAB | 4 | 1 |
| NIVOLUMAB | 4 | 1 |
| TIPIFARNIB | 3 | 1 |
| CHEMBL4748391 | 0 | 2 |
| CHEMBL5398431 | 0 | 2 |
| CHEMBL4215501 | 0 | 1 |
| CHEMBL4849721 | 0 | 1 |
| EXELIXIS | 0 | 1 |
Related Atlas pages
- Drugs: Dactinomycin, Doxorubicin, Tazemetostat, Cabozantinib, Ipilimumab, Nivolumab, Tipifarnib