Tuesday, June 18, 2024

New Guidelines for Managing Hereditary Adenomatous Polyposis Syndromes Released

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In an effort to enhance the clinical management of hereditary adenomatous polyposis syndromes, which significantly increase the risk of colorectal and other cancers, a comprehensive review led by a group of 38 experts has culminated in the release of updated guidelines. These guidelines aim to provide clear, evidence-based recommendations for healthcare providers handling these complex conditions.

Updating Clinical Management Practices

The review process involved updating the European recommendations that were last set in 2008. This update not only revisited familial adenomatous polyposis but also expanded to include other rare monogenic adenomatous polyposis syndromes. The group of experts executed a systematic review of the literature, answering eighty-nine clinically relevant questions and employing the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology to ensure the reliability of the evidence.

During the Delphi rounds used for achieving consensus among the experts, two levels of agreement were established: a consensus threshold and a high threshold. The former required at least 67% of the voting members to either “Strongly agree” or “Agree,” whereas a high threshold demanded a consensus of at least 80%.

Consensus and Recommendations

The rigorous review process resulted in 140 statements that achieved a high level of consensus. These statements now form the backbone of the updated guidelines, addressing various aspects of managing hereditary adenomatous polyposis syndromes. They cover surveillance, treatment, and patient management, including newer identified polyposis syndromes due to pathogenic variants in genes other than APC or MUTYH.

Key Insights from Updated Guidelines

  • Integration of recommendations for MUTYH-associated polyposis and gastric adenocarcinoma.
  • Emphasis on the management of proximal polyposis of the stomach.
  • Guidelines suggest management should occur at specialized centers due to the rarity and complexity of these diseases.

The updated guidelines also reflect a significant advancement in understanding the genetic foundations of these syndromes, which is crucial for the effective treatment and management of patients. The inclusion of rare syndromes in the guidelines ensures that a broader spectrum of patients receives informed and up-to-date healthcare interventions.

By focusing on evidence-based practices and consensus among leading experts, the guidelines promise to steer clinical practices towards more effective and specialized management of patients with these challenging syndromes.

Original Article: Br J Surg. 2024 May 3;111(5):znae070. doi: 10.1093/bjs/znae070.

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