Back to Archived Journals » Gastrointestinal Cancer: Targets and Therapy » Volume 3

Neoplastic lesions of endocrine cells in the gastrointestinal tract: ten evolving principles as a basis for clinical understanding

Authors Lawrence B, Anderson, Schimmack, Findlay, Kidd, Modlin

Received 4 July 2012

Accepted for publication 4 October 2012

Published 28 December 2012 Volume 2013:3 Pages 1—17

DOI https://doi.org/10.2147/GICTT.S21939

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 6


Ben Lawrence,1,2 Malcolm Anderson,3 Simon Schimmack,1 Michael Findlay,2,3 Mark Kidd,1 Irvin Modlin1

1Department of Surgery, Yale University School of Medicine, New Haven, CT, USA; 2School of Medical Sciences, Faculty of Medical and Health Sciences, University of Auckland, 3Department of Oncology, Auckland District Health Board, Auckland, New Zealand

Abstract: Timely and appropriate diagnosis and treatment of patients with gastroenteropancreatic neuroendocrine neoplasms is a difficult clinical endeavor. The field is particularly dynamic, not only in terms of expanding therapeutic options, but in the classifications and biological principles that underpin good decision-making. Acknowledging the confusion created by past changes and the inevitability of future development, we combine our clinical experience with a review of the literature to frame the current understanding of gastroenteropancreatic neuroendocrine neoplasms in terms of a set of principles that have stabilized in the midst of this change. Firstly, we present five principles that guide classification of neuroendocrine neoplasms; specifically principles of prognostic classification, mechanisms of tumorigenesis, undiagnosed disease burden, clues regarding genetic etiology, and typical clinical presentation. Secondly, we offer five clinical principles upon which to build a therapeutic strategy. Specifically, these treatment principles include the separation of options by tumor cell differentiation, and the site of the primary lesion in well differentiated tumors. Chromogranin A is a moderately useful biomarker. Treatment should only be considered by clinicians in a multidisciplinary team, and in the face of multiple potential therapeutic options without a supporting evidence base, clinical trial enrolment remains imperative. Therefore, we provide a current synopsis of classification of gastroenteropancreatic neuroendocrine neoplasms, and their etiology, clinical presentation, and management in a novel framework of ten relatively stable principles.

Keywords: gastroenteropancreatic neuroendocrine neoplasms, neuroendocrine tumors, epidemiology, molecular targeted therapy, histopathology, grading, staging, carcinoid, tumorigenesis

Creative Commons License This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.

Download Article [PDF]