Kidney neoplasm

Summary

Kidney neoplasm (MONDO:0021163) is a cancer (an umbrella term covering 8 Mondo subtypes) with 1 cohort gene (1 CIViC-evidence somatic driver; 1 ClinVar predisposition record) and 166 clinical trials. Top therapeutic interventions include sunitinib, axitinib, and doxorubicin hydrochloride.

At a glance

• Classification: Cancer
• Umbrella term: 8 Mondo subtypes
• Cohort genes: 1
• ClinVar variants: 1
• Clinical trials: 166

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	kidney neoplasm
Mondo ID	MONDO:0021163
EFO	EFO:0003865
NCIT	C3150
SNOMED CT	126880001
UMLS	C0022665
MedGen	5967
Anatomy (UBERON)	UBERON:0002113
Is cancer (heuristic)	yes

Also known as: kidney neoplasm · kidney neoplasm (disease) · kidney tumor · kidney tumour · neoplasm of kidney · neoplasm of the kidney · renal neoplasm · renal tumor · renal tumors · renal tumour · renal tumours · tumor of kidney · tumor of the kidney · tumour of kidney · tumour of the kidney

Data availability: 1 ClinVar variant · 2 cell lines.

Disease family

An umbrella term covering 8 Mondo subtypes.

Classification path: disease › human disease › disease by body system or component › urinary system disorder › kidney disorder › kidney neoplasm

Related subtypes (56): renal hypertension, kidney failure, nephritis, impaired renal function disease, nephrocalcinosis, atheroembolism of kidney, renal artery disease, nephrosis, cystic kidney disease, anuria, stricture or kinking of ureter, proteinuria, renal infectious disease, diabetes insipidus, orthostatic proteinuria, kidney hypertrophy, chronic kidney disease, hydronephrosis, renal tubular transport disease, kidney cortex necrosis, kidney papillary necrosis, perinephritis, renal aminoaciduria, autosomal dominant progressive nephropathy with hypertension, nephrolithiasis, X-linked diffuse leiomyomatosis-Alport syndrome, tubulointerstitial nephritis and uveitis syndrome, distal renal tubular acidosis, oligomeganephronia, duplication of urethra, renal tubular dysgenesis, exstrophy-epispadias complex, fetal lower urinary tract obstruction, IgG4-related kidney disease, congenital primary megaureter, renal nutcracker syndrome, renal hypoplasia, renal dysplasia, congenital megacalycosis, glomerular disorder, congenital renal artery stenosis, renal tubule disorder, pyonephrosis, Arnold stickler bourne syndrome, C1q nephropathy, hypertensive nephropathy, atypical Fanconi syndrome-neonatal hyperinsulinism syndrome, idiopathic non-lupus full-house nephropathy, lachiewicz sibley syndrome, crush syndrome, obstructive nephropathy, inherited kidney disorder, acute tubulointerstitial nephritis, kidney cortex disease, non-syndromic supernumerary kidneys, neonatal renal venous thrombosis

Subtypes (8): kidney hemangiopericytoma, kidney cancer, mixed epithelial stromal tumor of the kidney, kidney benign neoplasm, childhood kidney neoplasm, renal pelvis neoplasm, multiloculated renal cyst, mesonephric neoplasm

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

ClinVar germline variants

1 retrieved; paginated sample, class counts are floors:

1 pathogenic/likely pathogenic

ClinVar	Variant (HGVS)	Gene	Classification	Review
976001	NM_003000.3(SDHB):c.572G>A (p.Cys191Tyr)	SDHB	Pathogenic/Likely pathogenic	criteria provided, multiple submitters, no conflicts

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 7 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Somatic driver evidence (intOGen + CIViC, cohort fanout)

Gene	intOGen role	Cancer types	CIViC
SDHB			CIViC #5177

Orphanet rare-disease linkage (cohort genes)

Gene	Orphanet ID	Rare disease
SDHB	Orphanet:139411	Carney triad
SDHB	Orphanet:201	Cowden syndrome
SDHB	Orphanet:276621	Sporadic pheochromocytoma/secreting paraganglioma
SDHB	Orphanet:29072	Hereditary pheochromocytoma-paraganglioma
SDHB	Orphanet:3208	Isolated succinate-CoQ reductase deficiency
SDHB	Orphanet:44890	Gastrointestinal stromal tumor
SDHB	Orphanet:97286	Carney-Stratakis syndrome

Cohort genes → proteins

1 cohort genes, 1 distinct canonical proteins.

Evidence partition

Subset	Genes
multi_evidence	1

Cohort genes (full)

Symbol	HGNC	Ensembl	UniProt	Name	Evidence
SDHB	HGNC:10681	ENSG00000117118	P21912	Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial	clinvar

Cohort function summary

Lead sentence per gene, UniProt-curated.

Symbol	Protein name	Function (lead sentence)
SDHB	Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial	Iron-sulfur protein (IP) subunit of the succinate dehydrogenase complex (mitochondrial respiratory chain complex II), responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).

Protein-family classification

Druggable: 1 · Difficult: 0 · Unknown: 0 · Druggable fraction: 1.0

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

Family	Genes	Fold	FDR
Enzyme (other)	1	12.0×	0.083

Per-gene assignment

Symbol	Family	Druggable?	EC	InterPro (top 3)
SDHB	Enzyme (other)	yes	1.3.5.1	2Fe-2S_ferredoxin-type, Succ_DH/fum_Rdtase_Fe-S, 2Fe2S_fd_BS

Expression context

Cohort genes with no expression data: 0.

1 cohort gene are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

Bucket	Genes
narrow (1-5 tissues)	0
moderate (6-20)	0
broad (>20)	1
unknown	0

Top tissues across cohort

Tissue	Cohort genes
apex of heart	1
cardiac ventricle	1
heart left ventricle	1

Per-gene tissue summary (top 30)

Symbol	Bgee breadth	FANTOM5 breadth	SCXA	Top tissues
SDHB	293	ubiquitous	marker	heart left ventricle, cardiac ventricle, apex of heart

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

Symbol	Interactor count
SDHB	5,471

Structural data

PDB: 1 · AlphaFold-only: 0 · No structure: 0

Cohort genes with PDB structures (top 30)

Symbol	UniProt	PDB entries
SDHB	P21912	6

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 5. Enrichment computed across 1 evidence-associated genes (1 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 1 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

Pathway	Cohort genes	Fold	FDR	Sample cohort genes
Maturation of TCA enzymes and regulation of TCA cycle	1	571.0×	0.006	SDHB
Citric acid cycle (TCA cycle)	1	423.0×	0.006	SDHB
Respiratory electron transport	1	95.2×	0.014	SDHB
Aerobic respiration and respiratory electron transport	1	88.5×	0.014	SDHB
Metabolism	1	11.6×	0.086	SDHB

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 1 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO term	Cohort genes	Fold	FDR	Sample cohort genes
succinate metabolic process	1	3370.4×	9e-04	SDHB
mitochondrial electron transport, succinate to ubiquinone	1	3370.4×	9e-04	SDHB
respiratory electron transport chain	1	842.6×	0.002	SDHB
tricarboxylic acid cycle	1	510.7×	0.003	SDHB
proton motive force-driven mitochondrial ATP synthesis	1	263.3×	0.004	SDHB
aerobic respiration	1	247.8×	0.004	SDHB

Therapeutics

Drugs indicated for this disease

0 approved, 10 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Aldesleukin	Phase 3 (in late-stage trials)
Axitinib	Phase 3 (in late-stage trials)
Bevacizumab	Phase 3 (in late-stage trials)
Everolimus	Phase 3 (in late-stage trials)
Ipilimumab	Phase 3 (in late-stage trials)
Nivolumab	Phase 3 (in late-stage trials)
Sorafenib	Phase 3 (in late-stage trials)
St. John’S Wort	Phase 3 (in late-stage trials)
Sunitinib	Phase 3 (in late-stage trials)
Thalidomide	Phase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Acetaminophen, Capecitabine, Hydromorphone, Ibuprofen, Ketorolac, MUROMONAB-CD3, Motexafin Gadolinium, Oxycodone, Patupilone, Pazopanib, Pembrolizumab.

Drug target analysis

Approved (phase 4): 0 · Phase ≥3: 0 · Phased (≥1): 0 · Undrugged: 1

Druggability breadth: 1 of 1 evidence-associated genes (100%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Top cohort targets by molecule count

Symbol	Molecules	Max phase
SDHB	0	0

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 1.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

Symbol	Assays	Type breakdown
SDHB	4	Binding:4

Cohort enzymes (BRENDA EC)

Symbol	EC numbers	Names
SDHB	1.3.5.1	succinate dehydrogenase

Pharmacogenomics

Cohort genes with a PharmGKB record: 1; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Drug repurposing candidates

0 approved/phased drugs hit cohort targets but don’t yet appear in disease-level clinical trials. Target-inhibition rationale is strongest for cancer driver genes; a bioactivity hit is a screening signal, not a treatment claim.

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

Tier	Definition	Genes	Symbols
A	Approved (phase 4 drug)	0
B	Phased (≥1) drug, not yet approved	0
C	Druggable family + PDB, no drug	1	SDHB
D	Druggable family + AlphaFold only, no drug	0
E	Difficult family or no structure, no drug	0

Undrugged target profiles

1 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

Symbol	ChEMBL assays	Drugged partners (top 3)
SDHB	4	—

Clinical trials & evidence

Clinical trials

Clinical trials: 166.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	101
PHASE2	24
PHASE1	20
PHASE1/PHASE2	8
PHASE3	6
PHASE4	5
PHASE2/PHASE3	1
EARLY_PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06903312	PHASE4	RECRUITING	Primary Tumor Ablation and Outcome in Metastatic Renal Cell Carcinoma Treated With Immunotherapy Combinations.
NCT01413607	PHASE4	TERMINATED	The Use of Self Retaining Sutures in Open and Laparoscopic Partial Nephrectomy
NCT03444766	PHASE4	COMPLETED	Study of Nivolumab for Advanced Cancers in India
NCT04513522	PHASE4	COMPLETED	A Study to Evaluate the Safety and Efficacy of Nivolumab With Ipilimumab in Participants With Untreated Advanced Kidney Cancer Conducted in India
NCT05068180	PHASE4	UNKNOWN	Low-dose Neuroleptanalgesia for Postoperative Delirium in Elderly Patients
NCT03091192	PHASE3	ACTIVE_NOT_RECRUITING	Savolitinib vs. Sunitinib in MET-driven PRCC.
NCT06467097	PHASE3	RECRUITING	Systemic Treatment Alone Versus Systemic Treatment Plus Radiotherapy in Oligometastatic Renal Cell Carcinoma
NCT00065468	PHASE3	COMPLETED	Study Evaluating Interferon And CCI-779 In Advanced Renal Cell Carcinoma
NCT00221728	PHASE2/PHASE3	TERMINATED	Comparison Between Surgery and Radiofrequency for Treatment of Renal Tumors
NCT00375674	PHASE3	COMPLETED	A Clinical Trial Comparing Efficacy And Safety Of Sunitinib Versus Placebo For TheTreatment Of Patients At High Risk Of Recurrent Renal Cell Cancer
NCT00678392	PHASE3	COMPLETED	Axitinib (AG 013736) As Second Line Therapy For Metastatic Renal Cell Cancer
NCT00920816	PHASE3	COMPLETED	Axitinib (AG-013736) For the Treatment of Metastatic Renal Cell Cancer
NCT04258462	PHASE2	ACTIVE_NOT_RECRUITING	Hyperpolarized 13C Pyruvate MRI Scan in Predicting Tumor Aggressiveness in Patients With Renal Tumors
NCT05842044	PHASE2	RECRUITING	NSAID Use After Robotic Partial Nephrectomy
NCT05944237	PHASE1/PHASE2	RECRUITING	HTL0039732 in Participants With Advanced Solid Tumours
NCT06680089	PHASE2	RECRUITING	CD70-targeted immunoPET Imaging of Kidney Cancer
NCT07053462	PHASE1/PHASE2	RECRUITING	CRISPR-Edited HLA Donor Kidney Transplant to Reduce Rejection Risk
NCT07225114	PHASE2	RECRUITING	Translation of Acoustic Angiography: Contrast Enhanced Super Resolution (CESR) Imaging
NCT07227415	PHASE1/PHASE2	RECRUITING	Symbiotic-GU-08: A Study to Learn About the Medicine Called PF-08634404 Dosed Alone and in Combination With Other Anticancer Therapies in Adults With Locally Advanced or Metastatic Renal Cell Cancer
NCT07397611	PHASE2	RECRUITING	Pre-NEOSHIFT-RCC: Neoadjuvant HIF-Inhibitor Immunotherapy in RCC
NCT00035243	PHASE2	COMPLETED	EPO906 Therapy in Patients With Advanced Kidney Cancer
NCT00054886	PHASE2	COMPLETED	Study of the Safety and Efficacy of SU-011,248 in Adult Patients With Advanced Kidney Cancer
NCT00059735	PHASE2	COMPLETED	A Safety, Tolerability and Efficacy Study of E7070 in Patients With Renal Cell Carcinoma
NCT00065572	PHASE2	SUSPENDED	Exploratory Study to Assess the Biological Activity of ZD6126 in Subjects With Newly Diagnosed Metastatic Renal Cell Carcinoma (Stage IV)
NCT00069160	PHASE2	COMPLETED	Tariquidar and Docetaxel to Treat Patients With Lung, Ovarian, Renal and Cervical Cancer
NCT00076011	PHASE2	COMPLETED	Anti-angiogenesis Agent AG-013736 in Patients With Metastatic Renal Cell Carcinoma
NCT00101608	PHASE2	COMPLETED	Vinflunine in Patients With Locally Advanced or Metastatic Transitional Cell Carcinoma of the Urothelium
NCT00123773	PHASE2	COMPLETED	Study of F-Fluorodeoxyglucose (FluGlucoScan) in Patients With Known or Suspected Cancers of Low Incidence
NCT00134186	PHASE2	COMPLETED	Study of Motexafin Gadolinium for the Treatment of Renal Cell (Kidney) Cancer
NCT00226980	PHASE2	COMPLETED	A Trial of Thalidomide and Capecitabine in Metastatic Renal Cell Carcinoma
NCT00282048	PHASE2	COMPLETED	Study Of AG-013736 In Patients With Refractory Metastatic Renal Cell Cancer
NCT00651482	PHASE2	TERMINATED	Treatment of Refractory Metastatic Renal Cell Carcinoma With Bevacizumab and RAD001 (Everolimus)
NCT01633255	PHASE1/PHASE2	WITHDRAWN	[18]F-Fluciclatide for Kidney Cancer Imaging Studies and Treatment
NCT01688999	PHASE2	COMPLETED	Cabozantinib for Advanced Urothelial Cancer
NCT01712685	PHASE2	TERMINATED	Imaging Studies of Kidney Cancer Using 18F-VM4-037
NCT01961583	PHASE2	TERMINATED	[18F]Fluciclatide PET Imaging of Pazopanib Response
NCT01967407	PHASE1/PHASE2	UNKNOWN	IReversible Electroporation of Kidney Tumors Before Partial Nephrectomy.
NCT02581384	PHASE1/PHASE2	TERMINATED	Stereotactic Body Radiotherapy (SBRT) for Pulmonary Metastases in Ewing Sarcoma, Rhabdomyosarcoma, and Wilms Tumors
NCT02689167	PHASE2	UNKNOWN	Study to Assess Various Sunitinib Schedules in Renal Cell Carcinoma
NCT02862288	PHASE1/PHASE2	COMPLETED	Preliminary Study of Microwave Tumoral Ablation Performances for the Treatment of Pulmonary, Renal and Bone Neoplasia.

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
SUNITINIB	4	4
AXITINIB	4	3
DOXORUBICIN HYDROCHLORIDE	4	2
NIVOLUMAB	4	2
SORAFENIB	4	2
ALPELISIB	4	1
CABOZANTINIB	4	1
HYDROMORPHONE	4	1
IPILIMUMAB	4	1
MUROMONAB-CD3	4	1
TECHNETIUM TC 99M SESTAMIBI	4	1
TEMSIROLIMUS	4	1
URSODIOL	4	1
VINBLASTINE	4	1
VINFLUNINE	4	1
ENTINOSTAT	3	2
VALSPODAR	3	2
FLUORIDE ION F-18	3	1
GUADECITABINE	3	1
INTERFERON ALFA	3	1
MOTEXAFIN GADOLINIUM	3	1
PATUPILONE	3	1
SASANLIMAB	3	1
SAVOLITINIB	3	1
TARIQUIDAR	3	1
ZIMBERELIMAB	3	1
CASDATIFAN	2	1
INDISULAM	2	1
MUBRITINIB	2	1
PROPACETAMOL	2	1