raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:424046

Acinar cell carcinoma of pancreas

Synonyms: Pancreatic acinar cell carcinoma

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

A very rare, malignant, epithelial tumor of the pancreas characterized, macroscopically, by a usually large, well-circumscribed, fully or partially encapsulated, solid mass, often with hemorrhage, necrosis and cystic changes, in any portion of the pancreas and, histologically, by neoplastic cells with variable degrees of differentiation and morphology, ranging from acinar structures similar to normal pancreatic acini to large sheets of poorly differentiated neoplastic cells. Presenting symptoms are typically non-specific and include abdominal pain, weight loss, vomiting, nausea, and/or, less commonly, jaundice. Immunohistochemical evidence of acinar-specific products is observed. Association with Lynch syndrome, familial adenomatous polyposis, and pancreatic panniculitis has been reported.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enAcinar cell carcinoma of pancreasPancreatic acinar cell carcinoma
frCarcinome à cellules acineuses du pancréasCarcinome acinaire du pancréas · Carcinome acinaire pancréatique · Carcinome pancréatique à cellules acineuses
deAzinuszellkarzinom des PankreasPankreaskarzinom, azinäres
esCarcinoma de células acinares de páncreasCarcinoma pancreático de células acinares
itCarcinoma a cellule acinose del pancreasCarcinoma pancreatico a cellule acinose
nlAcinuscelcarcinoom van pancreasAcinuscelcarcinoom van alvleesklier · Pancreatisch acinuscelcarcinoom
ptCarcinoma de células acinares do pâncreas
plRak z komórek groniastych trzustki= en
csKarcinom pankreatu z acinárních buněkPankreatický karcinom z acinárních buněk
ukАцинарно-клітинний рак підшлункової залозиАцинарно-клітинна карцинома підшлункової залози
zh胰腺腺泡细胞癌胰腺腺泡细胞癌
trAsiner hücreli pankreas karsinomuPankreatik asiner hücreli karsinom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C25.0BTNTattributedvalidated
ICD-10C25.1BTNTattributedvalidated
ICD-10C25.2BTNTattributedvalidated
ICD-10C25.7BTNTattributedvalidated
ICD-10C25.8BTNTattributedvalidated
ICD-112C10.0NTBTindexvalidated
MONDO0006346Evalidated
UMLSC0279661Evalidated
MedDRA10073363Evalidated

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

21 records
TypeClassValueAreaQualificationValidationSource
Annual incidence<1 / 1 000 0000.029 / 100 000EuropeValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.016 / 100 000United KingdomValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.039 / 100 000SwitzerlandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.017 / 100 000NetherlandsValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.049 / 100 000AustriaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.039 / 100 000BelgiumValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.034 / 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 0000.044 / 100 000Czech RepublicValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.083 / 100 000EstoniaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.024 / 100 000FinlandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.01 / 100 000FranceValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.058 / 100 000GermanyValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.028 / 100 000IrelandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.039 / 100 000ItalyValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.07 / 100 000LatviaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.027 / 100 000NorwayValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.005 / 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 0000.05 / 100 000SlovakiaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.024 / 100 000SpainValue 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