Case Report
Copyright ©The Author(s) 2019.
World J Clin Cases. Mar 6, 2019; 7(5): 684-690
Published online Mar 6, 2019. doi: 10.12998/wjcc.v7.i5.684
Table 1 Comparative features of melanotic Xp11 tumors and perivascular epithelioid cell tumors
Melanotic Xp11 tumorsPEComas
Sex predilectionFemales > malesFemales > males
AgeChildren and young adults, but may affect older adultsMiddle age, but may affect children and older adults
Family historyTuberous sclerosis
SiteKidneys, uterus, ovaries, cervix, colon, pancreas, bladder, and pelvisGenitourinary tract, viscera, skin, soft tissue, and bone
HistologyEpithelioid with clear cytoplasmSpindled, epithelioid, or sclerosing
Melanin pigmentUsually presentUsually absent
Muscle marker expressionUsually absentUsually present
Melanocytic marker expressionPresentPresent
Epithelial marker expressionAbsentAbsent
Molecular genetic alterationXp11 translocation; PSF-TFE3 fusionLoss of TSC1 (9q34) or TSC2 (16p13.3) gene