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Start free with EleplanFamilial adenomatous polyposis
ORPHA:733Disease
Also called Colorectal adenomatous polyposis · FAP · Familial polyposis coli
What it is
A rare intestinal polyposis syndrome characterized by the development of up to hundreds to thousands of adenomas in the large bowel. It is a multi-system disorder with important extracolonic manifestations.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- Adult
- Inheritance
- Autosomal dominant, Autosomal recessive
- 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
Very common80–99%
6Common30–79%
7Sometimes5–29%
10- Abnormality of the cementum
- Abnormality of the thyroid gland
- Adrenocortical adenoma
- Angiofibromas
- Duodenal adenocarcinoma
- Neoplasm of the adrenal gland
- Odontoma
- Papillary thyroid carcinoma
and 2 more in this range
Rare1–4%
22- Adenocarcinoma of the small intestine
- Astrocytoma
- Biliary tract obstruction
- Brain neoplasm
- Cholangiocarcinoma
- Ependymoma
- Epidermoid cyst
- Fibroma
and 14 more in this range
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.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
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
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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