• Br J Surg · May 2024

    Practice Guideline

    Updated European guidelines for clinical management of familial adenomatous polyposis (FAP), MUTYH-associated polyposis (MAP), gastric adenocarcinoma, proximal polyposis of the stomach (GAPPS) and other rare adenomatous polyposis syndromes: a joint EHTG-ESCP revision.

    • Gloria Zaffaroni, Alessandro Mannucci, Laura Koskenvuo, Borja de Lacy, Anna Maffioli, Tanya Bisseling, Elizabeth Half, Giulia Martina Cavestro, Laura Valle, Neil Ryan, Stefan Aretz, Karen Brown, Francesco Buttitta, Fatima Carneiro, Oonagh Claber, Ruth Blanco-Colino, Maxime Collard, Emma Crosbie, Miguel Cunha, Triantafyllos Doulias, Christina Fleming, Henriette Heinrich, Robert Hüneburg, Julie Metras, Iris Nagtegaal, Ionut Negoi, Maartje Nielsen, Gianluca Pellino, Luigi Ricciardiello, Abdurrahman Sagir, Luis Sánchez-Guillén, Toni T Seppälä, Peter Siersema, Benedikt Striebeck, Julian R Sampson, Andrew Latchford, Yann Parc, John Burn, and Gabriela Möslein.
    • Center for Hereditary Tumors, Bethesda Hospital, Duisburg, Germany.
    • Br J Surg. 2024 May 3; 111 (5).

    BackgroundHereditary adenomatous polyposis syndromes, including familial adenomatous polyposis and other rare adenomatous polyposis syndromes, increase the lifetime risk of colorectal and other cancers.MethodsA team of 38 experts convened to update the 2008 European recommendations for the clinical management of patients with adenomatous polyposis syndromes. Additionally, other rare monogenic adenomatous polyposis syndromes were reviewed and added. Eighty-nine clinically relevant questions were answered after a systematic review of the existing literature with grading of the evidence according to Grading of Recommendations, Assessment, Development, and Evaluation methodology. Two levels of consensus were identified: consensus threshold (≥67% of voting guideline committee members voting either 'Strongly agree' or 'Agree' during the Delphi rounds) and high threshold (consensus ≥ 80%).ResultsOne hundred and forty statements reached a high level of consensus concerning the management of hereditary adenomatous polyposis syndromes.ConclusionThese updated guidelines provide current, comprehensive, and evidence-based practical recommendations for the management of surveillance and treatment of familial adenomatous polyposis patients, encompassing additionally MUTYH-associated polyposis, gastric adenocarcinoma and proximal polyposis of the stomach and other recently identified polyposis syndromes based on pathogenic variants in other genes than APC or MUTYH. Due to the rarity of these diseases, patients should be managed at specialized centres.© The Author(s) 2024. Published by Oxford University Press on behalf of BJS Foundation Ltd.

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