raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:418951

Undifferentiated carcinoma of esophagus

Synonyms: Undifferentiated esophageal carcinoma

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adult, Elderly
Status
active

A rare, aggressive, malignant, epithelial carcinoma of the esophagus characterized, macroscopically, by an exophytic mass with central ulceration located on the esophagus and, histologically, by a sheet-like growth of neoplastic cells without significant glandular, squamous or neuroendocrine differentiation. Patients may present with progressive dysphagia, long-standing history of gastroesophageal reflux, weight loss, anemia, abdominal or chest pain/pressure, dyspnea, and/or hematemesis. Presence or history of Barrett esophagus is frequently associated.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enUndifferentiated carcinoma of esophagusUndifferentiated esophageal carcinoma
frCarcinome indifférencié de l'oesophage
deÖsophaguskarzinom, undifferenziertesUndifferenzierter Speiseröhrenkrebs
esCarcinoma indiferenciado de esófagoCarcinoma esofágico indiferenciado
itCarcinoma non differenziato dell'esofagoCarcinoma esofageo non differnziato
nlOngedifferentieerd carcinoom van oesofagusOngedifferentieerd oesofageaal carcinoom
ptCarcinoma indiferenciado do esófago
plNiezróżnicowany rak przełyku= en
csNediferencovaný karcinom jícnuNediferencovaný ezofageální karcinom
ukНедиференційована карцинома стравоходуНедиференційована карцинома стравоходу
zh食管未分化癌未分化型食管癌
trÖzofagusun farklılaşmamış karsinomuFarklılaşmamış özofageal karsinomu

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C15.0BTNTattributedvalidated
ICD-10C15.1BTNTattributedvalidated
ICD-10C15.2BTNTattributedvalidated
ICD-10C15.3BTNTattributedvalidated
ICD-10C15.4BTNTattributedvalidated
ICD-10C15.5BTNTattributedvalidated
ICD-10C15.8BTNTattributedvalidated
ICD-112B70.YNTBTattributedvalidated
MONDO0018481Evalidated
UMLSC2188058Evalidated

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

3

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

24 records
TypeClassValueAreaQualificationValidationSource
Annual incidence<1 / 1 000 0000.044 / 100 000EuropeValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.029 / 100 000AustriaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.077 / 100 000BelgiumValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.027 / 100 000BulgariaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.006 / 100 000CroatiaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence1-9 / 1 000 0000.115 / 100 000Czech RepublicValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.037 / 100 000EstoniaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.002 / 100 000FinlandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence1-9 / 1 000 0000.118 / 100 000FranceValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.042 / 100 000GermanyValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.085 / 100 000IcelandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.012 / 100 000IrelandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.041 / 100 000ItalyValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.087 / 100 000LatviaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.021 / 100 000LithuaniaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.038 / 100 000NorwayValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence1-9 / 1 000 0000.101 / 100 000PolandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.021 / 100 000PortugalValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.082 / 100 000SlovakiaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.013 / 100 000SloveniaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.051 / 100 000SpainValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.023 / 100 000SwitzerlandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.025 / 100 000NetherlandsValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.039 / 100 000United KingdomValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]

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

6

HPO phenotypes

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

7

Sources for this card