Most patients with ampullary carcinoma present at advanced age (usually beyond 60 years). There is a slight male predominance. Some cases are seen in the setting of familial adenomatous polyposis or neurofibromatosis type 1. The diagnostic modalities of choice are CT and endoscopic ultrasound which usually show dilation of the common bile duct or pancreatic duct. The tumor itself may not be apparent on the imaging studies. The final diagnosis rests on the examination of cytologic material, endoscopic biopsy, or transduodenal biopsy. The biopsy should be deep enough so as not to miss any areas of malignant change at the base of the lesion. The image shows a specimen resulting from Whipple procedure - the usual method of treating invasive ampullary carcinomas. Given the significant mortality and morbidity associated with this procedure, tumors lacking an invasive component are treated by transduodenal resection. Image courtesy of: Dr. Terri Crook, Dallas, Texas.