Periampullary Cancer: Clinical Presentation and Diagnostic Strategies

Periampullary tumors include both benign and malignant neoplasms arising at or near the ampulla of Vater. The hallmark symptom of these tumors is painless obstructive jaundice, arising from their common location. The vast majority of periampullary tumors are malignant, with pancreatic adenocarcinoma being the most prevalent, followed by cancers of the ampulla of Vater, distal common bile duct, and duodenum, respectively. Surgical resection is the mainstay of therapy for periampullary tumors. Almost all resectable malignant and most benign tumors in this area are ultimately managed with pancreaticoduodenectomy. The most useful modalities for staging periampullary malignancies are dual-phase computed tomography and endoscopic ultrasound. In patients with unresectable disease, relief of biliary obstruction, through either biliary stenting or surgical biliary bypass, is essential for palliation. Determining the exact etiology of a periampullary malignancy can be challenging for both the surgeon and the pathologist. The clinical presentation, preoperative imaging studies, and intraoperative findings may not allow differentiation of the specific site of origin.