Signet-ring-cell carcinoma of the Vater's ampulla.

CONTEXT: Most tumors affecting Vater's Ampulla are adenocarcinomas and other histological variants are less frequent. A review of the literature revealed only seven previously reported cases of signet ring cell carcinoma of the ampulla of Vater. The presence of this kind of tumor has no clear histological explanation. Two possible theories have been proposed: the presence of gastric heterotopia in the ampulla of Vater or the existence of a perivaterian duodenal heterotopia of ulcerous etiology as the origin of a signet ring cell tumor which secondarily invades the ampulla of Vater.

CASE REPORT: We performed a pancreatoduodenectomy in a 67-year-old woman with a T2N0M0 ampulla tumor. A histologic study revealed a signet ring cell neoplasm.

CONCLUSION

Etiology and survival of signet ring cell carcinoma of Vater's ampulla is not well-defined in the literature due to the extreme rarity of this disease. Duodenopancreatectomy with pylorus preservation is the treatment of choice.