Seven patients were identified with YWHAE-rearranged HG-ESS...Median follow-up for the cohort was 27 months (range 6–123)...One patient (Case-5) presenting with FIGO Stage IVB disease received chemotherapy with doxorubicin, ifosfamide and mesna (AIM) followed by liposomal doxorubicin resulting in a radiographic CR, with subsequent hysterectomy and salpingo-oophorectomy that revealed no viable tumor.