Review
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 28, 2015; 21(8): 2303-2314
Published online Feb 28, 2015. doi: 10.3748/wjg.v21.i8.2303
Succinate dehydrogenase-deficient gastrointestinal stromal tumors
Ya-Mei Wang, Meng-Li Gu, Feng Ji
Ya-Mei Wang, Meng-Li Gu, Feng Ji, Department of Gastroenterology, The First Affiliated Hospital of Zhejiang University, Hangzhou 310009, Zhejiang Province, China
Author contributions: All of the authors generated the ideas. Wang YM was responsible for collating the data and writing the manuscript, which were verified by Gu ML and Ji F, who also acts as a guarantor for the manuscript.
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: Feng Ji, MD, PhD, Professor, Department of Gastroenterology, The First Affiliated Hospital, College of Medicine, Zhejiang University, No. 79 Qing Chun Road, Hangzhou 310003, Zhejiang Province, China. wym910319@126.com
Telephone: +86-571-87236586 Fax: +86-571-87236611
Received: July 24, 2014
Peer-review started: July 25, 2014
First decision: August 27, 2014
Revised: October 29, 2014
Accepted: December 16, 2014
Article in press: December 16, 2014
Published online: February 28, 2015
Core Tip

Core tip: Succinate dehydrogenase (SDH) deficiency occurs in about 5%-7.5% of gastrointestinal stromal tumors (GISTs). These so-called SDH-deficient GISTs lack KIT and PDGFRA mutations. Such type of GISTs has its own clinical, morphological and molecular characteristics. The accumulation of hypoxia-inducible factor 1α and the upregulation of its downstream molecules, such as insulin-like growth factor 1 and vascular endothelial growth factor receptor, may play important roles in the tumorigenesis of SDH-deficient GISTs. They are promising to be the novel therapeutic targets of GISTs.