Preemptive total gastrectomy for hereditary gastric cancer.

Hereditary gastric cancer is a recently described clinical syndrome, associated with truncating mutation of the E-cadherin gene, named CDH1. It is characterized by autosomal dominant transmission, presentation at an early age, and with diffuse type of gastric adenocarcinoma. Clinical management of these patients is challenging and includes intense endoscopic surveillance or prophylactic gastrectomy, which is associated with short- and long-term morbidity. We report four patients submitted to a prophylactic gastrectomy performed in members of three families with hereditary gastric cancer in a tertiary referral center in Mexico City. These are the first Hispanic families with hereditary gastric cancer reported in the literature.