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Serrated polyposis syndrome

ORPHA:157798· ICD-10 D12.6

Definition

A rare, genetic intestinal disease characterized by the presence of multiple (usually large) hyperplastic/serrated colorectal polyps, usually with a pancolonic distribution. Histology reveals hyperplastic polyps, sessile serrated adenomas (most common), traditional serrated adenomas or mixed polyps. It is associated with an increased personal and familial (first-degree relatives) risk of colorectal cancer.

Prevalence
1-9 / 100 000
Inheritance
Autosomal dominant, Multigenic/multifactorial, Unknown
Age of onset
Adult, Elderly