Gene expression profiling analyses on HG-ESS harboring YWHAE and BCOR fusions or ITD have demonstrated increased levels of NTRK3, FGFR3, and RET, implying a potential role of tyrosine kinase inhibitors, such as pazopanib and imatinib, in the treatment of high-grade ESS subtypes. Here, BCOR is linked to endometrioid stromal sarcoma.