Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Pathophysiol. Jan 22, 2022; 13(1): 15-33
Published online Jan 22, 2022. doi: 10.4291/wjgp.v13.i1.15
Current treatment strategies and future perspectives for gastrointestinal stromal tumors
Yoichi Sugiyama, Masaru Sasaki, Mohei Kouyama, Tatsuya Tazaki, Shinya Takahashi, Atsushi Nakamitsu
Yoichi Sugiyama, Masaru Sasaki, Mohei Kouyama, Tatsuya Tazaki, Atsushi Nakamitsu, Department of Gastrointestinal Surgery, JA Hiroshima General Hospital, Hatsukaichi 738-8503, Hiroshima, Japan
Shinya Takahashi, Department of Surgery, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima 734-8551, Japan
Author contributions: Sugiyama Y wrote the report; Sasaki M, Kouyama M, Tazaki T, and Nakamitsu A reviewed the literature and contributed to manuscript drafting; Takahashi S was responsible for the revision of the manuscript for important intellectual content; all authors have read and approve the final manuscript.
Conflict-of-interest statement: The author has no conflict of interests.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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:
Corresponding author: Yoichi Sugiyama, MD, PhD, Doctor, Surgeon, Surgical Oncologist, Department of Gastrointestinal Surgery, JA Hiroshima General Hospital, Jigozen 1-3-3, Hatsukaichi 738-8503, Hiroshima, Japan.
Received: March 8, 2021
Peer-review started: March 8, 2021
First decision: May 3, 2021
Revised: May 23, 2021
Accepted: November 13, 2021
Article in press: November 13, 2021
Published online: January 22, 2022

Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that originate from the gastrointestinal tract, mostly from the stomach. GISTs are derived from the myenteric interstitial cells of Cajal and are caused by several mutations in the c-kit and platelet-derived growth factor receptor genes. Clinically, GISTs are detected by endoscopic and imaging findings and are diagnosed by immunostaining. Surgery is the first line of treatment, and if the tumor is relatively small, minimally invasive surgery such as laparoscopy is performed. In recent years, neoadjuvant therapy has been administered to patients with GISTs that are suspected of having a large size or infiltration to other organs. Postoperative adjuvant imatinib is the standard therapy for high-risk GISTs. It is important to assess the risk of recurrence after GIST resection. However, the effect of tyrosine kinase inhibitor use will vary by the mutation of c-kit genes and the site of mutation. Furthermore, information regarding gene mutation is indispensable when considering the treatment policy for recurrent GISTs. This article reviews the clinicopathological characteristics of GISTs along with the minimally invasive and multidisciplinary treatment options available for these tumors. The future perspectives for diagnostic and treatment approaches for these tumors have also been discussed.

Keywords: Gastrointestinal stromal tumor, Minimally invasive surgery, Laparoscopic surgery, Imatinib, Neoadjuvant therapy, Risk assessment

Core Tip: Radical resection is the most effective treatment for gastrointestinal stromal tumors, but there are other options including minimally invasive surgery and multidisciplinary treatment, which involves the use of neoadjuvant therapy in consideration of tumor size and location. Combination with tyrosine kinase inhibitors is important for maximizing the therapeutic effect of surgery. To predict the effect, it is important to examine the presence of tumor mutations, including type, location of the mutation, and molecular subtype. We herein discuss the current treatment strategies for gastrointestinal stromal tumors and promising treatments based on clinical trials.