Clinicopathological observation of neoplasms with perivascular epithelioid cell differention

Objective To investigate the common pathological features,diagnosis,and differential diagnosis of angiomyolipoma(AML) of the kidney and liver,clear cell sugar tumor(CCST) of the lung,and perivascular epithelioid cell tumor(PEComa) of the uterus.Methods Each case of AML of the kidney and liver,CCST of the lung,and PEComa of the uterus was analyzed by light microscopy and immunohistochemistry.Results Four cases tumors of three different terms had the same morphology,mainly presented epithelioid neoplastic cell around thin-walled blood vessels.Immunohistochemically,HMB45 was positive in four cases(4/4),and SMA was positive in three of four cases(3/4).Conclusion Though the above mentioned tumors occur in different sites and have different terms.,they share common morphological and immunophenotypic features including epithelioid neoplastic cell around thin-walled blood vessels,positive melanocytic marker and smooth muscle marker.Our results support the view that these tumors belong to a larger family descriptively termed PEComas.