Gastrointestinal aspects of Peutz-Jeghers syndrome

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.

Automated summary

The clinical management of GI tract in Peutz-Jeghers syndrome (PJS) patients faces two main problems: long-term cancer risk and complications related to polyps. The evidence base for making recommendations is limited due to the rarity of this condition. Controversies remain regarding the relationship between PJ polyps, cancer development, and cancer risk. This article provides an overview of PJS clinical management and surveillance recommendations, emphasizing the need for further research to address knowledge gaps.