“…2 Signet-ring cell carcinoma arises in the stomach in 90 per cent of cases; in 10 per cent of cases, it can occur in other organs, including the breast, gallbladder, pancreas, urinary bladder, and colon. This tumor is extremely uncommon in the duodenum with less than 15 cases reported; furthermore, most duodenal signet-ring cell carcinomas are reported to arise in the ampulla, whereas only three cases in the worldwide literature occurred in the rest of the duodenum: two Spanish cases in the duodenal first portion 2,3 and one Japanese case in the third portion. 4 Our case is the first report of duodenal signet-ring cell carcinoma of the second portion; furthermore, a synchronous ampullary intestinal-type adenocarcinoma was diagnosed, and for the first time, an immunohistochemical and genetic analysis of the tumors was performed detecting microsatellite instability with loss of expression of the hMLH1 protein.…”