TY - JOUR
T1 - Management of familial adenomatous polyposis and MUTYH-associated polyposis; new insights
AU - Aelvoet, Arthur S.
AU - Buttitta, Francesco
AU - Ricciardiello, Luigi
AU - Dekker, Evelien
N1 - Funding Information: Evelien Dekker has endoscopic equipment on loan of FujiFilm and Olympus, received a research grant from FujiFilm, received honorarium for consultancy from FujiFilm, Olympus, Tillots, GI Supply, CPP-FAP, PAION and Ambu, and speakers' fees from Olympus, Roche, GI Supply, Norgine and FujiFilm. Publisher Copyright: © 2022 The Authors
PY - 2022/6/1
Y1 - 2022/6/1
N2 - Familial adenomatous polyposis (FAP) and MUTYH-associated polyposis (MAP) are rare inherited polyposis syndromes with a high colorectal cancer (CRC) risk. Therefore, frequent endoscopic surveillance including polypectomy of relevant premalignant lesions from a young age is warranted in patients. In FAP and less often in MAP, prophylactic colectomy is indicated followed by lifelong endoscopic surveillance of the retained rectum after (sub)total colectomy and ileal pouch after proctocolectomy to prevent CRC. No consensus is reached on the right type and timing of colectomy. As patients with FAP and MAP nowadays have an almost normal life-expectancy due to adequate treatment of colorectal polyposis, challenges in the management of FAP and MAP have shifted towards the treatment of duodenal and gastric adenomas as well as desmoid treatment in FAP. Whereas up until recently upper gastrointestinal surveillance was mostly diagnostic and patients were referred for surgery once duodenal or gastric polyposis was advanced, nowadays endoscopic treatment of premalignant lesions is widely performed. Aiming to reduce polyp burden in the colorectum as well as in the upper gastrointestinal tract, several chemopreventive agents are currently being studied.
AB - Familial adenomatous polyposis (FAP) and MUTYH-associated polyposis (MAP) are rare inherited polyposis syndromes with a high colorectal cancer (CRC) risk. Therefore, frequent endoscopic surveillance including polypectomy of relevant premalignant lesions from a young age is warranted in patients. In FAP and less often in MAP, prophylactic colectomy is indicated followed by lifelong endoscopic surveillance of the retained rectum after (sub)total colectomy and ileal pouch after proctocolectomy to prevent CRC. No consensus is reached on the right type and timing of colectomy. As patients with FAP and MAP nowadays have an almost normal life-expectancy due to adequate treatment of colorectal polyposis, challenges in the management of FAP and MAP have shifted towards the treatment of duodenal and gastric adenomas as well as desmoid treatment in FAP. Whereas up until recently upper gastrointestinal surveillance was mostly diagnostic and patients were referred for surgery once duodenal or gastric polyposis was advanced, nowadays endoscopic treatment of premalignant lesions is widely performed. Aiming to reduce polyp burden in the colorectum as well as in the upper gastrointestinal tract, several chemopreventive agents are currently being studied.
KW - Chemoprevention
KW - Colorectal cancer
KW - Colorectal surgery
KW - Endoscopic surveillance
KW - Familial adenomatous polyposis
KW - MUTYH-Associated polyposis
UR - http://www.scopus.com/inward/record.url?scp=85126527204&partnerID=8YFLogxK
U2 - https://doi.org/10.1016/j.bpg.2022.101793
DO - https://doi.org/10.1016/j.bpg.2022.101793
M3 - Review article
C2 - 35988966
SN - 1521-6918
VL - 58-59
JO - Best practice & research. Clinical gastroenterology
JF - Best practice & research. Clinical gastroenterology
M1 - 101793
ER -