2020 Volume 34 Issue 5 Pages 861-870
We herein present a rare case of a carcinoma that originated from the peribiliary gland within the ampulla of Vater. A 77-year-old male with epigastralgia was referred to our hospital for further examination and possible surgery. Under a strong suspicion of a carcinoma of the ampulla of Vater, pancreatoduodenectomy was performed. Microscopically, the tumor was a moderately to poorly differentiated adenocarcinoma. The tumor was distributed primarily in the submucosal layer of the duodenum, and not in the mucosa of either the bile duct or pancreatic duct. Immunohistochemical analysis of the tumor showed a pancreatobiliary phenotype, indicating that the tumor was not likely derived from the duodenal mucosa. Based on these findings, a carcinoma of the ampulla of Vater arising from the peribiliary gland was diagnosed. This reported case appears to be a very rare case of a carcinoma of the ampulla of Vater from the viewpoint of tumor development and extension.