Peutz-Jeghers syndrome

Patients should be advised that they will require routine periodic examinations for gastrointestinal and extra-intestinal cancer surveillance. American Association for Cancer Research (AACR) guidelines recommend education of patients’ families about the signs and symptoms of intussusception and when to seek urgent care.[39]MacFarland SP, Becktell K, Schneider KW, et al. Pediatric cancer screening in hereditary gastrointestinal cancer risk syndromes: an update from the AACR Childhood Cancer Predisposition Working Group. Clin Cancer Res. 2024 Oct 15;30(20):4566-71. https://aacrjournals.org/clincancerres/article/30/20/4566/748802/Pediatric-Cancer-Screening-in-Hereditary http://www.ncbi.nlm.nih.gov/pubmed/39190470?tool=bestpractice.com ​ See Intussusception. 

Women with Peutz-Jeghers syndrome (PJS) should be asked to begin self-breast examinations at age 18 years.[18]Boland CR, Idos GE, Durno C, et al. Diagnosis and management of cancer risk in the gastrointestinal hamartomatous polyposis syndromes: recommendations from the US Multi-Society Task Force on Colorectal Cancer. Gastroenterology. 2022 Jun;162(7):2063-85. https://www.gastrojournal.org/article/S0016-5085(22)00151-2/fulltext http://www.ncbi.nlm.nih.gov/pubmed/35487791?tool=bestpractice.com ​ Patients may benefit from involvement in disease-specific patient advocacy groups. An online support group has been established for PJS. Peutz-Jeghers syndrome: patient information Opens in new window