Published online Dec 21, 2020. doi: 10.3748/wjg.v26.i47.7550
Peer-review started: September 12, 2020
First decision: October 27, 2020
Revised: November 9, 2020
Accepted: November 21, 2020
Article in press: November 21, 2020
Published online: December 21, 2020
Multiple gastrointestinal stromal tumors (MGISTs) is a very rare type of gastrointestinal stromal tumor (GIST) and is usually misdiagnosed as metastatic tumors.
As physicians become more aware of MGISTs, researchers believed that it was imperative to describe MGISTs to help surgeons make appropriate diagnosis and treatment.
The study aimed to describe the clinical and oncological features of MGISTs and to offer evidence for MGISTs diagnosis and treatment.
Data of consecutive patients with MGISTs who were diagnosed at Peking University People’s Hospital (PKUPH) from 2008 to 2019 were retrospectively evaluated. Further, a literature search was conducted by retrieving data from PubMed, EMBASE, and the Cochrane library databases from inception up to November 30, 2019.
In all, 12 patients were diagnosed with MGISTs at PKUPH, and 43 published records were ultimately included following literature review. Combined analysis of all the individual patient data showed that female (59.30%), young (14.45%), and syndromic GIST (63.95%) patients comprised a large proportion of the total patient population. Tumors were mainly located in the small intestine (58.92%), and both CD117 and CD34 were generally positive. After a mean 78.32-mo follow-up, the estimated median overall survival duration (11.5 years) was similar to single GISTs, but recurrence-free survival was relatively poorer.
The clinical and oncological features are potentially different between MGISTs and single GIST, such as lower morbidity, female predominance, young age, multiple organ involvement, and possible syndromic GIST. Although overall survival was similar between single GISTs and MGISTs, a high rate of metastasis in MGIST patients resulted in a poorer long-time RF survival rate.
In further studies, focusing on gene detection and molecular biological analysis can contribute to the understanding of the mechanism underlying this special type of GIST in future studies. Moreover, an appropriate surgical approach and auxiliary therapy are urgently need to be determined by prospective, multicenter, and large-scale studies.