Adenocarcinoma of the ampulla of Vater: What treatment options are available?

Abstract

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.

Synchronous gastric and ampullary adenocarcinomas in a hairy cell leukemia patient treated with pentostatin eight years prior

“…While one case reported primary signet ring adenoacarcinoma of the stomach in a patient with HCL, 30 to our knowledge, this is the first case reporting two simultaneous gastrointestinal cancers in a patient with HCL eight years after being treated with pentostatin. This eight-year interval is within the range of several reports of second malignancies, but even more highlights the fact that immune defects caused by either HCL or its treatments can exist for many years after achieving remission.…”

“…He was treated with FOLFOX as this regimen is an acceptable first-line therapy for gastric cancer and the preferred therapy for ampullary-small bowel cancer. 30 Trastuzumab was added as the patient had HER-2/neu positive gastric cancer. Nonetheless, the response was short, consistent with a limited prognosis in our patient.…”

Therapeutic options for ampullary carcinomas. A review

“…26 Recently, a case report has also demonstrated a good response to 5-FU in a patient with advanced ampullary adenocarcinoma. 27…”

A Comparative Study of Laparoscopic versus Open Pancreaticoduodenectomy for Ampulla of Vater Carcinoma

“…Ampulla of Vater cancer (AVC) is defined as a malignancy that arises within the ampullary complex, distal to the confluence of the distal common bile duct and the pancreatic duct. AVC is a rare malignancy and accounts for 0.2% of all digestive malignancies, with an annual incidence of 0.4–0.5 cases per 100,000 people per year [ 1 , 2 ].…”