Abstract
Ampullary carcinoma is an extremely rare type of gastrointestinal cancer that originates at the ampulla of Vater, distal to the junction between the pancreatic duct and the common bile duct (CBD). There are three subtypes depending on the histological findings: pancreatobiliary, intestinal, and mixed subtype. Symptoms can mimic other pathologies related to biliary obstruction, such as jaundice, diarrhea, steatorrhea, and weight loss. In this report, we present a case of a 40-year-old male who presented with painless jaundice and dizziness. Magnetic resonance cholangiopancreatography (MRCP) showed choledocholithiasis and CBD dilatation. Endoscopic ultrasound showed a 24 x 14 mm ampulla mass. Subsequently, he underwent the Whipple procedure that revealed an intestinal-type periampullary adenocarcinoma characterized as stage III (T3bN2M0), with lymphovascular and perineural invasion. He was lost to follow-up but was later found to have metastatic pancreatic adenocarcinoma to the lung and liver. In this report, we also discuss the clinical presentation, pathogenesis, and current evidence-based therapeutic options in the management of this tumor, highlighting the importance of treatment choice depending on the tumor type.