Hereditary Diffuse Gastric Cancer Syndrome and the Role of CDH1: A Review.
Inherited variants in the tumor suppressor gene CDH1 are associated with an increased risk of gastric and breast cancers. This review aims to address the most current topics in management of the hereditary diffuse gastric cancer syndrome attributed to CDH1.Consensus management guidelines have broadened genetic testing criteria for CDH1. Prophylactic total gastrectomy is recommended for any pathogenic or likely pathogenic CDH1 variant carrier starting at the age of 20 years. Annual surveillance endoscopy is recommended to those who defer prophylactic total gastrectomy. Women with a CDH1 variant should initiate magnetic resonance imaging breast surveillance starting at age 30 years. Further research is needed to understand the pathogenesis of early-stage gastric cancers (T1a), which are pathognomonic of hereditary diffuse gastric cancer syndrome, that lead to advanced gastric cancer to develop both treatment and prevention strategies for this patient population.The heritable CDH1 gene mutation is of importance to today’s surgeons because it is associated with a substantial increased risk of developing both gastric and breast cancers. Management of this cancer syndrome currently uses prophylactic surgery and enhanced cancer surveillance strategies.
This is an abstract of the clinical research article “Hereditary Diffuse Gastric Cancer Syndrome and the Role of CDH1: A Review.” This clinical research article was published in the medical journal JAMA surgery on 2021-01-06 and has been categorised as belonging to the clinical specialty of General Surgery. To read the full clinical research article or obtain a PDF (if available) use the links directly above. To discover more of the latest General Surgery clinical research articles from the medical journal JAMA surgery please click the link below. For more of the latest General Surgery research articles from other leading medical journals click the link that says General Surgery next to the stethoscope icon at the top of the page. You can further filter clinical research articles by sub-specialties within General Surgery using the navigation menu at the top of the page.