Introduction: Germ line mutations in the E-cadherin gene account for approximately 30% to 50% of cases of hereditary diffuse gastric cancer syndrome. The estimated cumulative risk of gastric cancer by the age of 80 years in hereditary diffuse gastric cancer families is 67% for men and 83% for women, both of which carry a very poor prognosis if an early diagnosis is not made.
Materials and methods: Two siblings with a strong family history of gastric cancer over at least 2 generations, including an aunt testing positive for the E-cadherin gene mutation, presented with positive results of the same mutation. Both siblings underwent a laparoscopic total gastrectomy. The postoperative period was uneventful. A 7-trocar totally laparoscopic surgical approach was used. The esophagojejunal anastomosis was created using a linear stapler in a lateral side-to-side manner. The biliopancreatic limb was 50 cm and the Roux limb was 75 cm in length.
Results: Both siblings recovered without complications. Histologic evaluation revealed benign gastric tissue with no evidence of malignancy. Margins were clear of gastric mucosa. Prophylactic total gastrectomy eliminates the risk of gastric cancer in individuals with E-cadherin gene mutations. This procedure can be performed safely with a laparoscopic approach, resulting in excellent recovery and prognosis. Totally laparoscopic, lateral side-to-side esophagojejunal anastomosis using linear stapler creates an emerged lumen of both the esophagus and the jejunum that carries less risk of stricture, and reflux is prevented by a 75-cm-long Roux limb.