To the Editors:

In response to the letter by Dr. Liakakos, we agree that evidence suggests that total gastrectomy should be performed for asymptomatic patients with a family history of gastric cancer and identified CDH1 mutations. Surgery can be performed safely with good results. One cannot rely on surveillance endoscopy and other methods of early diagnosis because all but one patient who underwent surgical resection had gastric cancer, and only two were diagnosed preoperatively. Further, if one waits until symptoms to perform surgery, it is too late because these patients did worse.

However, our study did not clearly define the timing of gastrectomy in patients with CDH1 mutation. There may be a long latency period before the cancer becomes incurable by surgery. Certainly, we would like to preserve gastric function for as long as possible in these young patients. Further, as Liakakos points out, a better understanding of the pathophysiology of CDH1 mutation––induced gastric cancer may result in specific drugs to alter the development of cancer, eliminating the need for total gastrectomy. For now, we agree that our study suggests that total gastrectomy should be performed early; that it is not prophylactic, but therapeutic; and that the presence of symptoms portends a poor prognosis.