Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Nov 15, 2018; 10(11): 370-380
Published online Nov 15, 2018. doi: 10.4251/wjgo.v10.i11.370
Ampulla of Vater carcinoma: Molecular landscape and clinical implications
Antonio Pea, Giulio Riva, Riccardo Bernasconi, Elisabetta Sereni, Rita Teresa Lawlor, Aldo Scarpa, Claudio Luchini
Antonio Pea, Elisabetta Sereni, Department of Surgery, University and Hospital Trust of Verona, Verona 37134, Italy
Giulio Riva, Riccardo Bernasconi, Aldo Scarpa, Claudio Luchini, Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona, Verona 37134, Italy
Rita Teresa Lawlor, ARC-Net Research Center, University and Hospital Trust of Verona, Verona 37134, Italy
Author contributions: Luchini C, Pea A, Scarpa A and Lawlor RT conceived of and designed the study; Pea A, Riva G, Lawlor RT and Luchini C performed the literature review; all authors contributed to the literature analysis and interpretation; Pea A, Scarpa A and Luchini C wrote the manuscript; all authors performed the final editing and gave approval of the manuscript in its present form.
Supported by the Italian Association for Cancer Research, No. 12182.
Conflict-of-interest statement: No potential conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Claudio Luchini, MD, PhD, Assistant Professor, Department of Diagnostics and Public Health, University and Hospital Trust of Verona, Piazzale L.A. Scuro 10, Verona 37134, Italy.
Telephone: +39-45-8124835 Fax: +39-45-8027136
Received: June 1, 2018
Peer-review started: June 2, 2018
First decision: August 1, 2018
Revised: August 8, 2018
Accepted: October 8, 2018
Article in press: October 8, 2018
Published online: November 15, 2018

Ampulla of Vater is a peculiar anatomical structure, characterized by the crossroad of three distinct epithelia: Intestinal, ductal pancreatic and biliary. Adenocarcinomas arising in this area represent an opportunity to understand the comparative biology of all periampullary malignancies. These neoplasms can exhibit intestinal, pancreaticobiliary or mixed features, whereas the subclassification based on morphology and immunohistochemical features failed in demonstrating a robust prognostic reliability. In the last few years, the molecular landscape of this tumor entity has been uncovered, identifying alterations that may serve as prognostic and predictive biomarkers. In this review, the histological and genetic characteristics of ampullary carcinomas are discussed, taking into account the main clinical and therapeutic implications related to this tumor type as well.

Keywords: Pancreatobiliary, Intestinal, Mixed, ELF3, TP53, KRAS, Ampullary, Vater, Histotype

Core tip: Ampulla of Vater carcinomas comprise tumors with intestinal and/or pancreaticobiliary differentiation, but such histotypical classification is of little help for their prognostic stratification. Integration of the recently reported molecular profiles with histopathological and clinical information furnishes novel keys for fostering the development of a more efficient prognostic stratification and the identification of novel therapeutic strategies.