Classical form of FAP

Attenuated form of FAP

Gardner syndrome

Turcot syndrome

Gene

APC: 5q21

APC: 5q21

N-terminal mutation

APC: 5q21

APC: 5q21

Transmission

Autosomal dominant

Autosomal dominant

Autosomal dominant

Autosomal dominant

Colic manifestations

Adenomatous polyps

Adenomatous polyps, late onset, low number

Adenomatous polyps

Adenomatous polyps

Extra-colic manifestations

Absent

Absent

HCEPR, epidermoid cysts, pilomatrixoma, fibrous hyperplasia, desmoid tumour, multiple osteomas

various digestive tumours

and extra digestive

Brain tumour:

glioblastoma, medulloblastoma.

Basal cell carcinoma.

Brown spots.

Evolution

Colonic adenocarcinoma

More or less favorable

Appearance of polyps: second and third decades.

Cancers: third and fourth decades.

Colon cancer – Nervous system disorders.