PGR and neoplasm with perivascular epithelioid cell differentiation: Epidemiologically, PEComas are rare and predominantly affect middle-aged women (male-to-female ratio of 1:5, average age: 45 years).7) The higher prevalence in women is suspected to be linked to female hormones, as most PEComas express progesterone receptors.8) While PEComas can occur anywhere in the body, the most common locations are the kidneys, urogenital organs, and uterus.9–12)