Seven cases diagnosed as LG-ESS were reclassified either as HG-ESS with BCOR rearrangement (n = 2), malignant epithelioid neoplasm with GLI1 rearrangement (n = 2), NCOA fusion-positive uterine tumors (n = 2) or uterine leiomyoma (n = 1). The gene discussed is BCOR; the disease is endometrioid stromal sarcoma.