Ampullary Adenocarcinoma, Version 1.2023, NCCN Clinical Practice Guidelines in Oncology
E. Gabriela Chiorean, Marco Del Chiaro, Margaret A. Tempero, Mokenge P. Malafa, Al B. Benson, Dana B. Cardin, Jared A. Christensen, Vincent Chung, Brian G. Czito, Mary Dillhoff, Timothy R. Donahue, Efrat Dotan, Christos Fountzilas, Evan S. Glazer, Jeffrey M. Hardacre, William G. Hawkins, Kelsey Klute, Andrew H. Ko, John W. Kunstman, Noelle K. LoConte, Andrew M. Lowy, Ashiq Masood, Cassadie Moravek, Eric K. Nakakura, Amol Narang, Lorenzo Nardo, Jorge Obando, Patricio M. Polanco, Sushanth Reddy, Marsha Reyngold, Courtney L. Scaife, Jeanne Shen, Mark J. Truty, Charles M. Vollmer, Robert A. Wolff, Brian M. Wolpin, Beth McCullough, Senem Lubin, Susan Darlow
Abstract
Ampullary cancers refer to tumors originating from the ampulla of Vater (the ampulla, the intraduodenal portion of the bile duct, and the intraduodenal portion of the pancreatic duct), while periampullary cancers may arise from locations encompassing the head of the pancreas, distal bile duct, duodenum, or ampulla of Vater. Ampullary cancers are rare gastrointestinal malignancies, and prognosis varies greatly based on factors such as patient age, TNM classification, differentiation grade, and treatment modality received. Systemic therapy is used in all stages of ampullary cancer, including neoadjuvant therapy, adjuvant therapy, and first-line or subsequent-line therapy for locally advanced, metastatic, and recurrent disease. Radiation therapy may be used in localized ampullary cancer, sometimes in combination with chemotherapy, but there is no high-level evidence to support its utility. Select tumors may be treated surgically. This article describes NCCN recommendations regarding management of ampullary adenocarcinoma.