Gastrointestinal aspects of Peutz-Jeghers syndrome

A R Latchford; S K Clark

doi:10.1016/j.bpg.2022.101789

Gastrointestinal aspects of Peutz-Jeghers syndrome

Best Pract Res Clin Gastroenterol. 2022 Jun-Aug:58-59:101789. doi: 10.1016/j.bpg.2022.101789. Epub 2022 Apr 6.

Authors

A R Latchford¹, S K Clark²

Affiliations

¹ Polyposis Registry, St Mark's Hospital, Harrow, UK; Department of Surgery and Cancer, Imperial College, London, UK. Electronic address: andrew.latchford@nhs.net.
² Polyposis Registry, St Mark's Hospital, Harrow, UK; Department of Surgery and Cancer, Imperial College, London, UK.

PMID: 35988959
DOI: 10.1016/j.bpg.2022.101789

Abstract

There are two main problems in the clinical management of the gastrointestinal (GI) tract in patients with Peutz-Jeghers syndrome (PJS), namely long-term cancer risk and managing polyp related complications (of which the most important clinically is intussusception). Given the rarity of this condition, the evidence base upon which to make recommendations is small. Furthermore, controversies persist regarding the relationship between PJ polyps, cancer development and cancer risk. In this article we will explore some of these controversies, to put into context the recommendations for clinical management of these patients. We will provide an overview, particularly focusing on clinical data, and on the recommendations for clinical management and surveillance of the GI tract in PJS. We highlight knowledge gaps which need to be addressed by further research.

Keywords: Endoscopy; Intussusception; Peutz-Jeghers; Polyp; Surveillance.

Publication types

Review

MeSH terms

Humans
Neoplasms* / complications
Peutz-Jeghers Syndrome* / complications
Peutz-Jeghers Syndrome* / diagnosis