Clinicopathological features of uterine perivascular epithelioid cell tumor (PEComa)

Aim To report a case of perivascular epithelioid cell tumor(PEComa) of uterus and to study its clinicopathological features,diagnosis and differrential diagnosis.Methods One case of uterine PEComa was studied by HE staining and immunohistochemistry,with review of the relevant literatures.Results The patient was a 51-year-old female.The tumor occurred in the subserosa of the uterus.The tumor was partly composed of round to polyonal epithelial cells with abundant clear to slightly eosinphilic granular cytoplasm in a perivascular location.The tumor cells showed no atypia and mitotic figures,and they were strongly immunoactive for HMB45 and Vimentin.The tumor still had areas of spindle-shaped cells.They were positive for SMA,Actin(HHF35)and desmin.The tumor cells were negative for CK(AE1/AE3),EMA,CD10,CD34,CD117 and S-100.The patient had no evidence of disease recurrence and metastasis 26 months postoperatively.Conclusions Uterine PEComa is a novel member of the family of PEComa.It should be regarded as tumor with an uncertain malignant potential.