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Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Oct 15, 2017; 9(10): 407-415
Published online Oct 15, 2017. doi: 10.4251/wjgo.v9.i10.407
Ampullary cancer of intestinal origin and duodenal cancer - A logical clinical and therapeutic subgroup in periampullary cancer
Manju D Chandrasegaram, Anthony J Gill, Jas Samra, Tim Price, John Chen, Jonathan Fawcett, Neil D Merrett
Manju D Chandrasegaram, the Prince Charles Hospital, Brisbane, Queensland 4032, Australia
Manju D Chandrasegaram, Jonathan Fawcett, School of Medicine, University of Queensland, Queensland 4006, Australia
Anthony J Gill, Jas Samra, Sydney Medical School, University of Sydney, New South Wales 2006, Australia
Anthony J Gill, Cancer Diagnosis and Pathology Group, Kolling Institute of Medical Research, Royal North Shore Hospital, St Leonards, New South Wales 2065, Australia
Jas Samra, Department of Surgery, Royal North Shore Hospital, Sydney, New South Wales 2065, Australia
Tim Price, Queen Elizabeth Hospital, Adelaide, South Australia 5011, Australia
Tim Price, University of Adelaide, South Australia 5005, Australia
John Chen, Flinders Medical Centre, Adelaide, South Australia 5042, Australia
John Chen, Royal Adelaide Hospital, Adelaide, South Australia 5000, Australia
Jonathan Fawcett, Princess Alexandra Hospital, Brisbane, Queensland 4102, Australia
Neil D Merrett, Department of Upper GI Surgery, Bankstown Hospital, Sydney, New South Wales 2200, Australia
Neil D Merrett, Discipline of Surgery, Western Sydney University, Sydney, New South Wales 2560, Australia
Author contributions: All the authors made substantial contributions to (1) conception, design, analysis and interpretation of data; (2) making critical revisions related to important intellectual content of the manuscript; and (3) final approval of the version of the article to be published.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
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: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Dr. Manju D Chandrasegaram, the Prince Charles Hospital, Rode Road, Chermside, Brisbane, Queensland 4032, Australia. m.chandrasegaram@uq.edu.au
Telephone: +61-7-31930800 Fax: +61-7-33196761
Received: January 31, 2017
Peer-review started: February 8, 2017
First decision: May 8, 2017
Revised: June 26, 2017
Accepted: August 16, 2017
Article in press: August 17, 2017
Published online: October 15, 2017
Abstract

Periampullary cancers include pancreatic, ampullary, biliary and duodenal cancers. At presentation, the majority of periampullary tumours have grown to involve the pancreas, bile duct, ampulla and duodenum. This can result in difficulty in defining the primary site of origin in all but the smallest tumors due to anatomical proximity and architectural distortion. This has led to variation in the reported proportions of resected periampullary cancers. Pancreatic cancer is the most common cancer resected with a pancreaticoduodenectomy followed by ampullary (16%-50%), bile duct (5%-39%), and duodenal cancer (3%-17%). Patients with resected duodenal and ampullary cancers have a better reported median survival (29-47 mo and 22-54 mo) compared to pancreatic cancer (13-19 mo). The poorer survival with pancreatic cancer relates to differences in tumour characteristics such as a higher incidence of nodal, neural and vascular invasion. While small ampullary cancers can present early with biliary obstruction, pancreatic cancers need to reach a certain size before biliary obstruction ensues. This larger size at presentation contributes to a higher incidence of resection margin involvement in pancreatic cancer. Ampullary cancers can be subdivided into intestinal or pancreatobiliary subtype cancers with histomolecular staining. This avoids relying on histomorphology alone, as even some poorly differentiated cancers preserve the histomolecular profile of their mucosa of origin. Histomolecular profiling is superior to anatomic location in prognosticating survival. Ampullary cancers of intestinal subtype and duodenal cancers are similar in their intestinal origin and form a logical clinical and therapeutic subgroup of periampullary cancers. They respond to 5-FU based chemotherapeutic regimens such as capecitabine-oxaliplatin. Unlike pancreatic cancers, KRAS mutation occurs in only approximately a third of ampullary and duodenal cancers. Future clinical trials should group ampullary cancers of intestinal origin and duodenal cancers together given their similarities and their response to fluoropyrimidine therapy in combination with oxaliplatin. The addition of anti-epidermal growth factor receptor therapy in this group warrants study.

Keywords: Periampullary cancer, Pancreatobiliary subtype, Intestinal subtype, Ampullary cancer, Duodenal cancer, Epidermal growth factor receptor, Pancreatic cancer, Chemotherapy, Pancreaticoduodenectomy, KRAS

Core tip: Periampullary cancers include pancreatic, ampullary, bile duct and duodenal cancers. Pancreatic cancer is the most common cancer resected with a pancreaticoduodenectomy followed by ampullary, bile duct and duodenal cancer. Patients with resected duodenal and ampullary cancers have better prognosis compared to pancreatic cancer. Ampullary cancers can be subdivided into intestinal or pancreatobiliary subtype cancers with histomolecular staining. Histomolecular profiling is superior to anatomic location in prognosticating survival. Ampullary cancers of intestinal subtype and duodenal cancers are similar in their intestinal origin and form a logical clinical and therapeutic subgroup. They respond to 5-FU based chemotherapeutic regimens such as capecitabine-oxaliplatin.