The “Wild”-type gastrointestinal stromal tumors: Heterogeneity on molecule characteristics and clinical features

KIT and platelet-derived growth factor receptor alpha (PDGFRA) are known as the most driven genes in gastrointestinal stromal tumors (GISTs). However, about 10%–15% of GISTs without KIT and PDGFRA mutation are named “Wild-type” (WT) GISTs. Gene abnormalities and clinical features of WT GISTs are significantly different from KIT/PDGFRA-mutated GISTs. Recently, based on the findings of next-generation sequencing, WT GISTs have been shown to be not a single disease entity, but instead a set of various pathologic and clinical diseases. Nevertheless, although several genetic alterations have been identified in WT GISTs, the exact roles of these molecules have not yet been well defined. We herein summarize the recent progression on KIT/PDGFRA WT GISTs.