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The scientific literature on adenocarcinoma of the ampulla (papilla) of Vater suggests that it either represents a distinct entity or is more closely related to small bowel adenocarcinoma than to the biliary malignancies. The ambiguity surrounding this rare cancer has kindled research exploring its immunohistochemistry aspects and gene expression profiling. While the basis of management for resectable disease remains surgical intervention, the role of adjuvant chemotherapy is not clear. A recent large phase 3 clinical trial conducted in patients with resected ampulla of Vater adenocarcinoma favored adjuvant chemotherapy over observation alone. The standards of therapy for the advanced small bowel adenocarcinoma and biliary cancer are fluoropyrimidine derivatives and gemcitabine-based combinations, respectively. In addition, new biologic and targeted agents may enhance clinical results seen in this cancer type. Therefore, diligently designed clinical trials are necessary to establish its optimal treatment strategies. We describe herein a patient with ampulla of Vater adenocarcinoma who had an exceptional response to fluoropyrimidine-based chemotherapy. We further include a discussion reviewing the clinicopathologic aspects of this neoplasm as well as focus on currently available and future therapeutic options.

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