(Familial adenomatous polyposis, PAF)

Autosomal dominant transmission with complete penetrance of a mutation in the APC gene (Adenomatous Polyposis Coli)   on locus 5q2. Association of multiple osteomas (especially on the face),  of epidermoid cysts, cutaneous fibromas, digestive desmoid tumors and adenomatous polyps (at the level of the colon, especially)  with frequent malignant degeneration. Turcot syndrome (see this term) is one of the clinical forms.

Anesthetic implications: 

no special precautions.

Updated: April 2019