Indication for the prophylactic total gastrectomy in the hereditary diffuse gastric cancer in carriers of E-Cadherin mutation. Two observations.

Aim : The germ-line truncating mutation in the E-Cadherin, CDH1,is identified in 30% of hereditary diffuse gastric cancer (CGDH).The tumors are characterized by a high penetrance with an autosomaldominant pattern of inheritance, predominantly in youngpeople. The endoscopic surveillance, by current methods, is largelyineffective because most tumors are growing up diffusely andquickly. We report the outcomes of 2 asymptomatic patients withthe CDH1 mutation who have undergone prophylactic total gastrectomy(GTP).Patients and methods: Two brothers 22 and 33 years old, belongingto a family severely altered by CGD, underwent prophylactictotal gastrectomy with Roux en Y reconstruction.Results: Pathologic examination of the stomach shows no evidenceof in situ or invasive carcinoma despite multiple samples. Therewere no operative complications. A 5-5 kg weight loss and diarrheastabilised in a 3 month period. There were no more complications inthe long term.Discussion: Four points are discussed: 1/ The preoperative endoscopicscreening for visualisation of carcinoma microfoci and ofgastric ectopia in oesophagus. 2/ The interest of the chromoendoscopicsurveillance unfortunately limited by size of tumoral focinever detectable if inferior to 4mm. 3/ The precision of the surgicalprocedure needing a complete removal of gastric mucosa thatmeans GTP in every case. 4/ Regular screening for malignancies ofother organs related to the CDH1 mutation (breast, colon-rectum,prostate). 5/ A proposition of therapeutic decisions for CGDH.Conclusion. The GTP indication is often difficult to promote forasymptomatic patients, but the operation avoids any risk of gastriccancer. Nevertheless the GTP cannot dispense patients of regularpolyvisceral surveillance.