raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:300557

Carcinoma of the ampulla of Vater

Synonyms: Ampulloma · Ampullary carcinoma

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

Carcinoma of the ampulla of Vater is a rare malignant tumor originating from the ampulla of Vater that can present with symptoms of general fatigue, loss of appetite, weight loss, nausea, vomiting, abdominal pain and, most commonly, painless obstructive jaundice. The tumor is believed to arise from duodenal, biliary or pancreatic epilthelium, resulting in the respective histological types. In general, carcinoma of the ampulla of Vater has a better prognosis (5-year survival rate of 45%) than cancers of the distal bile duct and pancreas.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enCarcinoma of the ampulla of VaterAmpulloma · Ampullary carcinoma
frCarcinome de l'ampoule de VaterAmpullome · Ampullome vatérien
deKarzinom der Ampulla VateriAmpullom
esCarcinoma de la ampolla de VaterAmpuloma · Carcinoma ampular
itCarcinoma dell'ampolla di VaterAmpulloma · Carcinoma ampollare
nlCarcinoom van ampul van VaterAmpullair carcinoom · Ampulloom
ptCarcinoma da ampola de Vater
plRak brodawki VateraAmpulloma (rak brodawki Vatera) · Rak typu ampulloma (rak brodawki Vatera)
csKarcinom Vaterovy papilyAmpulární karcinom · Ampulom
ukКарцинома ампули ВатераАмпульна карцинома · Ампулома
zhVater壶腹癌壶腹癌
trVater ampulla karsinomuAmpulloma · Ampuller karsinom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C24.1NTBTattributedvalidated
ICD-112C16Especificvalidated
MONDO0017590Evalidated
UMLSC0262401Evalidated
MedDRA10048853Evalidated

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 (2):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

3 records
TypeClassValueAreaQualificationValidationSource
Annual incidence1-9 / 1 000 0000.59 / 100 000FranceValue and classValidated10656214[PMID]
Point prevalence1-9 / 100 000—EuropeClass onlyNot yet validatedORPHANET
Annual incidence1-9 / 1 000 0000.57 / 100 000United KingdomValue and classValidated3664230[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.

6

HPO phenotypes

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

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Sources for this card