raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:423968

Squamous cell carcinoma of the small intestine

Synonyms: Squamous cell carcinoma of the small bowel

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

Squamous cell carcinoma of the small intestine is an extremely rare, malignant, epithelial tumor of the small intestine (most often localized in the duodenum). Presenting symptoms are often nonspecific, such as weight loss, epigastric pain, anorexia, weakness, fatigue, vomiting and abdominal distension, and vary depending on localization of the tumor. Gastrointestinal bleeding and perforation may occur in advanced cases.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enSquamous cell carcinoma of the small intestineSquamous cell carcinoma of the small bowel
frCarcinome épidermoïde de l'intestin grêle
dePlattenepithelkarzinom des DünndarmsSquamöses Karzinom des Dünndarms
esCarcinoma de células escamosas de intestino delgado
itCarcinoma a cellule squamose dell'intestino tenueCarcinoma a cellule squamose del piccolo intestino
nlPlaveiselcelcarcinoom van dunne darm
ptCarcinoma de células escamosas do intestino delgado
plRak łuskowaty jelita cienkiego= en
csDlaždicobuněčný karcinom tenkého střeva= en
ukПлоскоклітинний рак тонкої кишкиПлоскоклітинний рак тонкого кишечника
zh小肠鳞状细胞癌小肠鳞状细胞癌
trİnce bağırsakta skuamöz hücreli karsinomİnce bağırsakta skuamöz hücreli karsinom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C17.0BTNTattributedvalidated
ICD-10C17.1BTNTattributedvalidated
ICD-10C17.2BTNTattributedvalidated
ICD-10C17.3BTNTattributedvalidated
ICD-10C17.8BTNTattributedvalidated
ICD-112B80.YNTBTattributedvalidated
MONDO0018509Evalidated
UMLSC4707899Evalidated

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

20 records
TypeClassValueAreaQualificationValidationSource
Annual incidence<1 / 1 000 0000.008 / 100 000EuropeValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.008 / 100 000AustriaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.008 / 100 000BelgiumValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.002 / 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 incidence<1 / 1 000 0000.013 / 100 000Czech RepublicValue 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 incidence<1 / 1 000 0000.008 / 100 000FranceValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.004 / 100 000GermanyValue 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 0000.007 / 100 000ItalyValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.022 / 100 000LatviaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.003 / 100 000NorwayValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.003 / 100 000PolandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.02 / 100 000PortugalValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.005 / 100 000SlovakiaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.027 / 100 000SpainValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.011 / 100 000SwitzerlandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.002 / 100 000NetherlandsValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.012 / 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