Published online Jan 22, 2022. doi: 10.4291/wjgp.v13.i1.15
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.
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.