Prophylactic total gastrectomy for hereditary diffuse gastric cancer: surgical and pathological results

In this study, we examined the results of prophylactic total gastrectomy for patients with germline CDHI (E-cadherin) mutation. The 8 patients (3 male, 5 female) had a median age of 42 (range 38-51). All patients had an upper endoscopy or chromoendoscopy prior to surgery with only one patient identified as having a focus of diffuse gastric cancer. All patients underwent a total gastrectomy with Roux-en-Y esophagojejunostomy. For pathological analysis, the entire gastric mucosa was examined microscopically, which required up to 490 sections. Only one patient had unremarkable pathology. The other 7 patients had 1-77 foci of noninvasive cancer, and two of these patients had 4-12 foci of T1 invasive cancer. Thus, the majority of patients with germline CDHI mutation have foci of noninvasive or invasive gastric cancer by middle age. Serial endoscopy provides inadequate screening, and total prophylactic gastrectomy is the procedure of choice for definitive treatment.