The histopathology and staging of carcinoma of the ampulla of Vater.

Review of 26 resected ampullary carcinomas revealed intestinal type adenocarcinoma in all but one and overtly papillary carcinoma in only one case. Co-existing adenoma of the ampulla was present in 11 cases, over half of which were low-grade carcinomas. Flat duct epithelial dysplasia was present in a further eight cases, the adenocarcinoma in only two of these being well differentiated. The estimated 5 year survival rate, overall, was 52% and, with well differentiated adenocarcinoma, 75%. We recommend a new staging system, based on extent of local and lymph node spread: I = invasion confined to wall of common bile duct; II = infiltration of duodenal or retroperitoneal tissues, excluding pancreas; III = infiltration of pancreas; IV = metastasis to nearby lymph nodes. Long-term survival correlates inversely with stage, both by univariate analysis and independently of grade, so that a simple scoring system, based on a combination of both grade and stage, is an excellent predictor of the long-term outcome, defining two groups, with 5 year survival rates of 79% and 0% (P less than 0.001).