raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:598216

Upper tract urothelial carcinoma

Synonyms: Transitional cell carcinoma of the pelvis and ureter · Transitional cell carcinoma of the upper urinary tract · UTUC

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
No data available
Status
active

A rare urinary tract tumor characterized by a malignant neoplasm arising from urothelial cells of the pyelocaliceal cavities and ureter. The tumor may be multifocal and bilateral in some cases and is more common in males than in females. Cigarette smoking and occupational exposure are major risk factors. Patients most commonly present with visible or non-visible hematuria and/or flank pain due to obstruction of the flow of urine by tumor tissue, or (less often) to local tumor growth. Systemic symptoms such as anorexia, weight loss, malaise, fatigue, fever, night sweats, or cough can be associated with advanced stages.

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Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enUpper tract urothelial carcinomaTransitional cell carcinoma of the pelvis and ureter · Transitional cell carcinoma of the upper urinary tract · UTUC
frCancer urothélial des voies supérieuresCarcinome urothélial des voies supérieures · Carcinome à cellules transitionnelles des voies urinaires supérieures · Carcinome à cellules transitionnelles du pelvis et de l'uretère
deUrothelkarzinom des oberen HarntraktesTransitionalzellkarzinom des Nierenbeckens und der Harnleiter · UTUC
esCarcinoma urotelial de las vías urinarias superioresCU-VUS · Carcinoma de células transicionales de la pelvis y el uréter · Carcinoma de células transicionales de las vías urinarias superiores
itCarcinoma uroteliale del tratto superioreCarcinoma della pelvi e dell'uretere a cellule transizionali · Carcinoma delle vie urinarie superiori a cellule transizionali · UTUC
nlUrotheelcarcinoom van hogere urinewegenOvergangscelcarcinoom van hogere urinewegen · Overgangscelcarcinoom van pelvis en ureter · Overgangsepitheelcarcinoom van bekken en urineleider · UTUC
ptCarcinoma urotelial do tracto superiorCarcinoma de células transicionais da pélvis e ureter · Carcinoma de células transicionais do tracto urinário superior
plRak urotelialny górnych dróg moczowychUTUC · Rak przejściowokomórkowy górnych dróg moczowych
csUroteliální karcinom horních cest močových= en
ukУротеліальна карцинома верхніх відділів травного трактуПерехідно-клітинна карцинома малого тазу та сечоводу · Перехідно-клітинна карцинома верхніх сечових шляхів · UTUC
zhUpper tract urothelial carcinoma= en
trUpper tract urothelial carcinoma= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C65BTNTattributedvalidated
ICD-10C66BTNTattributedvalidated
ICD-112C91.0NTBTattributedvalidated
ICD-112C92.0NTBTattributedvalidated
MONDO0020659Evalidated
UMLSC0220648Evalidated
GARD9376Evalidated
MedDRA10044407Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

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Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET
Annual incidence1-9 / 100 0001.5 / 100 000WorldwideValue and classValidated28543959[PMID]_32593530[PMID]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

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HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

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Sources for this card