Lynch Syndrome: Current management In 2019

Publication date: Available online 21 August 2019Source: Journal of Visceral SurgeryAuthor(s): B. Menahem, A. Alves, J.M. Regimbeau, C. SabbaghSummaryNearly 5% of colorectal cancers are related to constitutional genetic abnormalities. In Lynch Syndrome (LS), the abnormality is a mutation of the deoxyribonucleic acid (DNA) repair system. The goal of this update is to update indications and surgical strategies for patients with LS. Different spectra of disease are associated with LS. The narrow spectrum includes cancers with a high relative risk: colorectal cancer (CRC), endometrial cancer, urinary tract cancers and small intestinal cancer. The broader spectrum includes ovarian tumors, glioblastoma, cutaneous tumors (keratoacanthomas and sebaceous tumors), biliary duct tumors, and gastric tumors. The clinical diagnosis of LS was initially based on the Amsterdam I and II Criteria published in the 1990s and subsequently on the revised Bethesda Criteria, which expanded the criteria and identified patients who should be screened for LS. For patients with LS, learned societies recommend early and regular endoscopic screening because of the high incidence of CRC, i.e., every one to two years from the age of 25 and then annually from the age of 40 or starting 10 years before the age of appearance of the youngest case of CRC in the family. Professional recommendations on prophylactic surgery to prevent cancers in patients with genetic predisposition were published in 2009 under the aus...
Source: Journal of Visceral Surgery - Category: Surgery Source Type: research