“Wild-type” gastrointestinal stromal tumors: a problem to define

Abstract

Introduction: Due to progress in immunohistochemistry and the growing understanding of gastrointestinal stromal tumors (GISTs), it has been possible to elucidate certain aspects related to this group of neoplasms, but, given its unusual characteristics, new questions arose. Such is the case of the subtype called "wild-type GIST" (WT-GIST) (GIST, which lacks the mutations (KIT or PDGFRA). They represent a therapeutic challenge due to their resistance to agents that act as a molecular target for the disease. The aim of this study was to make a brief analysis of the WT-GIST. Material and methods: A bibliographic review of the PubMed and ResearchGate database was conducted. The MeSH terms "GIST" AND "Wild-type" OR "pediatric" were used. Studies published between 2005 and 2018 were cataloged, and originals, reviews, and editorials in English were selected. Given that the aim of the study was bibliographical, there was no need to perform a formal statistical analysis. Results: When comparing WT-GISTs with the classic phenotype, wild tumors have a set of non-specific heterogeneous mutations such as BRAF, NF-1, KRAS, CBL, PI3K3CA, or protein succinate dehydrogenase (subunits A, B, C, and D), this last one being the most common. Conclusions: Nowadays, given the unique and indolent characteristics of this group of neoplasms, there is a need for the correct diagnosis and limitation of the subgroup to which they belong, to reduce the risk of failure for the various existing therapeutic pathways.