Abstract
Abstract Background Patients with CDH1 (E-cadherin) gene mutations have a 60–80% increased lifetime risk of hereditary diffuse gastric cancer (HDGC). Although prophylactic total gastrectomy may reduce long-term risk of gastric cancers, the associated morbidity and mortality remains unclear. This systematic review aims to characterise postoperative surgical outcomes in patients choosing to undergo a prophylactic total gastrectomy. Methods A systematic literature search was conducted for studies reporting endoscopic surveillance, surgical outcomes and quality of life (QoL) for patients with a CDH1 mutation undergoing a total gastrectomy. Results A total of 991 patients were looked at across 33 studies, with 96% harbouring a CDH1 mutation (n=948). Seven hundred and seventy six patients had endoscopy results reported across 27 studies, with 72% presenting without evidence of cancer foci (n=555). Of these patients, 60% proceeded to have a total gastrectomy (n=333) and histological analysis revealed cancer foci in 83% of the gastrectomy specimens (n=276). Six hundred and forty nine patients were reported to have a total gastrectomy across 33 studies. Pathology outcomes were reported for 528 patients across 26 studies, of which 87% had pT1aN0M0 disease (n=458). Surgical complications were reported for 406 patients across 20 studies. Only 3 studies reported 0 complications. A total of 188 complications were reported across 17 studies, with the most common presentations being anastomotic strictures (23%), pulmonary complications (13%), wound infections (12%) and anastomotic leaks (11%). A total of 5 mortalities were reported in 23 studies over the follow-up period. Conclusions Prophylactic total gastrectomy is recommended for patients with a CDH1 mutation. Currently, reliable endoscopic surveillance is lacking and total gastrectomy remains the definitive method for preventing advanced diffuse gastric cancer. Patients choosing to undergo surgery should be provided with adequate counselling, with careful consideration of the associated morbidity.
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