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) are a very rare type of gastrointestinal stromal tumor (GIST) and are usually observed in syndrome.
The paper aimed to describe the clinical and oncological features of MGISTs and to offer evidence for the 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 the literature review. Combined analysis of the whole 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. Further studies are needed to explore appropriate surgical approach and adjuvant therapy.
Core Tip: The study retrospectively collected 12 patients of Peking University People’s Hospital and 161 patients of literature research to illustrate the demographic, oncological, and surgical features of patients with multiple gastrointestinal stromal tumors (MGISTs). After analysis, MGISTs might have unique characteristics, such as lower morbidity, female predominance, young age, multiple organ involvement, and more likely to occur in syndrome. Although overall survival was similar to single gastrointestinal stromal tumor, the high rate of metastasis resulted in a poor recurrence free survival in MGISTs. Based upon evidence and gap map, gene detection and molecular biological analysis are necessary to explore the mechanism and provide appropriate therapy.