However, endometrial stromal sarcomas show morphological evidence of endometrial involvement and endometrial stromal differentiation; furthermore, their immunoistochemical profile, CD10+ /HMB-45, Melan-A-; MiTF, should help in the distinction from PEComas (Folpe et al., 2005, Conlon et al., 2015, Bennett et al., 2018). Here, PMEL is linked to neoplasm with perivascular epithelioid cell differentiation.