Renal pelvis/ureter urothelial carcinoma

Summary

Renal pelvis/ureter urothelial carcinoma (MONDO:0020654) is a cancer and 101 clinical trials. Top therapeutic interventions include pembrolizumab, enfortumab vedotin, and gemcitabine. A subtype of urothelial carcinoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: Unknown (Worldwide) [Orphanet-validated]
• Clinical trials: 101

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 100 000	1.5	Worldwide	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	renal pelvis/ureter urothelial carcinoma
Mondo ID	MONDO:0020654
Orphanet	598216
NCIT	C7716
GARD	0009376
Anatomy (UBERON)	UBERON:0036295
Is cancer (heuristic)	yes

Also known as: renal pelvis and ureter TCC · renal pelvis and ureter transitional cell cancer · renal pelvis and ureter transitional cell carcinoma · renal pelvis and ureter urothelial carcinoma · TCC of renal pelvis and ureter · TCC of the renal pelvis and ureter · transitional cell cancer of renal pelvis and ureter · transitional cell cancer of the renal pelvis and ureter · transitional cell carcinoma of renal pelvis and ureter · transitional cell carcinoma of the pelvis and ureter · transitional cell carcinoma of the upper urinary tract · upper tract urothelial carcinoma · urothelial carcinoma of the renal pelvis and ureter · UTUC

Data availability: 6 cell lines · 21 intOGen driver records.

Disease family

This is a subtype of urothelial carcinoma. 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 › cancer › malignant urinary system neoplasm › urothelial carcinoma › renal pelvis/ureter urothelial carcinoma

Related subtypes (4): urethra transitional cell carcinoma, bladder transitional cell carcinoma, non-papillary transitional cell carcinoma of the bladder, pure squamous carcinoma of the urothelial tract

Subtypes (2): ureter transitional cell carcinoma, renal pelvis urothelial carcinoma

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: 101.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	57
Not specified	21
PHASE1	9
PHASE3	6
PHASE1/PHASE2	4
PHASE2/PHASE3	3
PHASE4	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06764095	PHASE4	RECRUITING	Enfortumab Vedotin and Pembrolizumab With Cystectomy and/or Ureterectomy for Locally Advanced or Metastatic Bladder and Upper Urothelial Tract Cancer, CAST-AI Trial
NCT03244384	PHASE3	ACTIVE_NOT_RECRUITING	Testing MK-3475 (Pembrolizumab) After Surgery for Localized Muscle-Invasive Bladder Cancer and Locally Advanced Urothelial Cancer
NCT04620239	PHASE3	RECRUITING	ENdoluminal LIGHT ActivatED Treatment of Upper Tract Urothelial Cancer (ENLIGHTED) Study
NCT04628767	PHASE2/PHASE3	RECRUITING	Testing the Addition of MEDI4736 (Durvalumab) to Chemotherapy Before Surgery for Patients With High-Grade Upper Urinary Tract Cancer
NCT06124976	PHASE2/PHASE3	RECRUITING	Multicenter Clinical Trial of ST-02 for Ablation of Upper Tract Urothelial Carcinoma
NCT00003376	PHASE3	COMPLETED	Two-Drug Combination Chemotherapy Compared With Four-Drug Combination Chemotherapy in Treating Patients With Advanced Cancer of the Urothelium
NCT00014274	PHASE2/PHASE3	COMPLETED	Combination Chemotherapy in Treating Patients With Transitional Cell Cancer of the Urothelium
NCT00022191	PHASE3	COMPLETED	Cisplatin Plus Gemcitabine With or Without Paclitaxel in Treating Patients With Stage IV Urinary Tract Cancer
NCT04006691	PHASE3	WITHDRAWN	Efficacy and Safety of UGN-101 in Recurrent Patients
NCT04197986	PHASE3	TERMINATED	Oral Infigratinib for the Adjuvant Treatment of Subjects With Invasive Urothelial Carcinoma With Susceptible FGFR3 Genetic Alterations
NCT04724018	PHASE1/PHASE2	RECRUITING	Sacituzumab Govitecan Plus EV in Metastatic UC
NCT05775471	PHASE2	RECRUITING	Pembrolizumab and Enfortumab Vedotin With Pembrolizumab Prior to and After Radical Nephroureterectomy for High-Risk Upper Tract Urothelial Cancer
NCT05917158	PHASE2	RECRUITING	A Study of RC48-ADC Combined with JS001 for Postoperative Adjuvant Treatment of Upper Tract Urothelial Carcinoma
NCT06823427	PHASE2	RECRUITING	9MW2821 + Toripalimab vs 9MW2821 for 1st Line Locally Advanced or Metastatic Urothelial Carcinoma
NCT06927128	PHASE2	NOT_YET_RECRUITING	Neoadjuvant Chemotherapy in High - Risk Upper Tract Urothelial Carcinoma
NCT06948552	PHASE2	RECRUITING	Photodynamic Diagnosis of Upper Tract Urothelial Carcinoma Using Fluorescence Endoscopy and Oral 5-ALA
NCT06983210	PHASE2	RECRUITING	Clinical Trial of AM80 in Combination With Gemcitabine, Cisplatin, and Nivolumab in Patients With Urothelial Carcinoma
NCT07087860	PHASE2	RECRUITING	Therapeutic Plasma Exchange With Enfortumab Vedotin and Pembrolizumab for Treatment of Bladder Cancers
NCT07126119	PHASE2	RECRUITING	Neoadjuvant Tislelizumab in Combination With Nab-Paclitaxel for UTUC
NCT07225374	PHASE2	RECRUITING	Cisplatin-Based Chemotherapy Followed by Maintenance Avelumab in UTUC
NCT07412171	PHASE2	NOT_YET_RECRUITING	NECTAR Study: Neoadjuvant Toripalimab + 9MW2821 for Local UTUC
NCT07498907	PHASE2	NOT_YET_RECRUITING	Disitamab Vedotin Plus Radiotherapy for Adjuvant Treatment of HER2-Expressing Cisplatin-Ineligible Upper Tract Urothelial Carcinoma
NCT07584733	PHASE2	NOT_YET_RECRUITING	Biomarker-Enriched Kidney-Preserving Strategy With Disitamab Vedotin Plus Tislelizumab in HER2-Positive High-Risk Upper Tract Urothelial Carcinoma
NCT00002914	PHASE2	COMPLETED	Piritrexim in Treating Patients With Advanced Cancer of the Urinary Tract
NCT00003105	PHASE1/PHASE2	COMPLETED	Combination Chemotherapy in Treating Patients With Metastatic or Locally Advanced Bladder Cancer
NCT00003175	PHASE2	COMPLETED	Fluorouracil in Treating Patients With Recurrent or Metastatic Bladder Cancer
NCT00004203	PHASE2	COMPLETED	Oxaliplatin in Treating Patients With Metastatic Bladder Cancer
NCT00004223	PHASE2	COMPLETED	Docetaxel and Gemcitabine in Treating Patients With Progressive Regional or Metastatic Bladder Cancer
NCT00004856	PHASE2	TERMINATED	Trastuzumab in Treating Patients With Previously Treated, Locally Advanced, or Metastatic Cancer of the Urothelium
NCT00005644	PHASE2	COMPLETED	Combination Chemotherapy in Treating Patients With Advanced Cancer of the Urothelium and Decreased Kidney Function
NCT00005831	PHASE2	COMPLETED	Trastuzumab and Combination Chemotherapy in Treating Patients With Locally Recurrent or Metastatic Urinary Tract Cancer
NCT00005958	PHASE2	UNKNOWN	Combination Chemotherapy Plus Filgrastim in Treating Patients With Locally Recurrent or Advanced Urothelium Cancer
NCT00006026	PHASE2	COMPLETED	Nitrocamptothecin in Treating Patients With Metastatic Cancer of the Urinary Tract
NCT00006111	PHASE2	COMPLETED	Surgery Plus Combination Chemotherapy and Radiation Therapy in Treating Patients With Cancer of the Urinary Tract
NCT00006351	PHASE2	COMPLETED	SCH 66336 Plus Gemcitabine in Treating Patients With Advanced Cancer of the Urinary Tract
NCT00006376	PHASE2	COMPLETED	R115777 in Treating Patients With Advanced Bladder Cancer
NCT00009867	PHASE2	COMPLETED	Arsenic Trioxide in Treating Patients With Urothelial Cancer
NCT00014144	PHASE2	COMPLETED	S0031, ZD 1839 in Treating Patients With Advanced Cancer of the Urinary Tract
NCT00021099	PHASE2	COMPLETED	Ixabepilone in Treating Patients With Advanced Urinary Tract Cancer
NCT00022633	PHASE2	TERMINATED	S0028, Gemcitabine and Paclitaxel in Treating Patients With Advanced or Recurrent Cancer of the Urinary Tract

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
PEMBROLIZUMAB	4	7
ENFORTUMAB VEDOTIN	4	4
GEMCITABINE	4	4
SUNITINIB MALATE	4	4
VINBLASTINE	4	3
CARBOPLATIN	4	2
GEFITINIB	4	2
INFIGRATINIB	4	2
PACLITAXEL	4	2
PAZOPANIB HYDROCHLORIDE	4	2
SORAFENIB	4	2
AMINOLEVULINIC ACID	4	1
ARSENIC TRIOXIDE	4	1
CISPLATIN	4	1
DOXORUBICIN HYDROCHLORIDE	4	1
DURVALUMAB	4	1
ERDAFITINIB	4	1
FLUDEOXYGLUCOSE F 18	4	1
IXABEPILONE	4	1
LONAFARNIB	4	1
MITOMYCIN	4	1
NIVOLUMAB	4	1
PEGFILGRASTIM	4	1
ROMIDEPSIN	4	1
TEMSIROLIMUS	4	1
VANDETANIB	4	1
VORINOSTAT	4	1
PIRARUBICIN	3	2
VELIPARIB	3	2
DISITAMAB VEDOTIN	3	1