Additionally, 31% of PEComas showed at least focal desmin staining in a recent review of all reported cases up to 2005 (61 cases) [8], and therefore desmin staining of uterine epithelioid tumors with clear cell areas and HMB-45 positivity does not exclude the diagnosis of PEComa. Here, DES is linked to neoplasm with perivascular epithelioid cell differentiation.