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

Zaffaroni, Gloria, Mannucci, Alessandro, Koskenvuo, Laura, de Lacy, Borja, Maffioli, Anna, Bisseling, Tanya, Half, Elizabeth, Cavestro, Giulia Martina, Valle, Laura, Ryan, Neil, Aretz, Stefan, Brown, Karen, Buttitta, Francesco, Carneiro, Fatima, Claber, Oonagh, Blanco-Colino, Ruth, Collard, Maxime, Crosbie, Emma, Cunha, Miguel, Doulias, Triantafyllos, Fleming, Christina, Heinrich, Henriette, Hüneburg, Robert, Metras, Julie, Nagtegaal, Iris, Negoi, Ionut, Nielsen, Maartje, Pellino, Gianluca, Ricciardiello, Luigi, Sagir, Abdurrahman, Sánchez-Guillén, Luis, Seppälä, Toni T., Siersema, Peter, Striebeck, Benedikt, Sampson, Julian R. ORCID: https://orcid.org/0000-0002-2902-2348, Latchford, Andrew, Parc, Yann, Burn, John and Möslein, Gabriela 2024. 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. British Journal of Surgery 111 (5) , znae070. 10.1093/bjs/znae070

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Abstract

Background Hereditary adenomatous polyposis syndromes, including familial adenomatous polyposis and other rare adenomatous polyposis syndromes, increase the lifetime risk of colorectal and other cancers. Methods A 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%). Results One hundred and forty statements reached a high level of consensus concerning the management of hereditary adenomatous polyposis syndromes. Conclusion These 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.

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Medicine
Publisher:	Oxford University Press
ISSN:	0007-1323
Date of First Compliant Deposit:	16 August 2024
Date of Acceptance:	25 February 2024
Last Modified:	19 Aug 2024 09:15
URI:	https://orca.cardiff.ac.uk/id/eprint/171439

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