Guidelines for the clinical management of familial adenomatous polyposis (FAP)
- Vasen, H. F. A.
- Moeslein, G.
- Alonso, A.
- Aretz, S.
- Bernstein, I.
- Bertario, L.
- Blanco, I.
- Bulow, S.
- Burn, J.
- Capella, G.
- Colas, C.
- Engel, C.
- Frayling, Ian
- Friedl, W.
- Hes, F. J.
- Hodgson, S.
- Jarvinen, H.
- Mecklin, J-P
- Moller, P.
- Myrhoi, T.
- Nagengast, F. M.
- Parc, Y.
- Phillips, R.
- Clark, S. K.
- de Leon, M. Ponz
- Renkonen-Sinisalo, L.
- Sampson, J. R.
- Stormorken, A.
- Tejpar, S.
- Thomas, H. J. W.
- Wijnen, J.
DOIAbstract
Background: Familial adenomatous polyposis (FAP) is a well-described inherited syndrome, which is responsible for <1% of all colorectal cancer (CRC) cases. The syndrome is characterised by the development of hundreds to thousands of adenomas in the colorectum. Almost all patients will develop CRC if they are not identified and treated at an early stage. The syndrome is inherited as an autosomal dominant trait and caused by mutations in the APC gene. Recently, a second gene has been identified that also gives rise to colonic adenomatous polyposis, although the phenotype is less severe than typical FAP. The gene is the MUTYH gene and the inheritance is autosomal recessive. In April 2006 and February 2007, a workshop was organised in Mallorca ...
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