Familial pancreatic carcinoma

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Familial pancreatic carcinoma

ORPHA:1333Disease

Also called Familial pancreatic cancer

What it is

Familial pancreatic carcinoma is defined by the presence of pancreatic cancer (PC) in two or more first-degree relatives.

Key facts

Prevalence
1-9 / 1 000 000 (Europe)
Age of onset
Adult
Inheritance
Autosomal dominant, Multigenic/multifactorial
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

BRCA1Major susceptibility factor
BRCA2Major susceptibility factor
CDKN2AMajor susceptibility factor
KRASMajor susceptibility factor
PALB2Major susceptibility factor
PALLDMajor susceptibility factor
RABL3Disease-causing germline mutation(s)
SMAD4Major susceptibility factor
TP53Major susceptibility factor

ICD-10 codes

C25filed under a broader ICD-10 category

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 4206MESH C535837MONDO 0015278OMIM 260350OMIM 606856OMIM 613347OMIM 613348OMIM 614320UMLS C2931038

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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