Abstract：【Objective】To investigate the clinical characteristics and the latest treatment of renal perivascular epithelioid cell tumor. 【Methods】The clinical data of 3 patients with perivascular epithelioid cell tumor in our department from January 2019 to September 2019 were analyzed retrospectively, and the latest diagnosis and treatment of this disease were summarized by referring to relevant literature. 【Results】All the patients were female, with an average age of 51.3 years. Before operation, B-ultrasonography and CT were performed in all the patients. Two cases were diagnosed as renal angiomyolipoma before operation, including one case of bilateral renal angiomyolipoma, one case of tuberous sclerosis to be excluded and one case of renal malignant tumor. All of the 3 patients underwent partial nephrectomy, and all of them were diagnosed as perivascular epithelioma. One of them could not completely exclude certain malignant potential. TSC2 mutation was found by gene detection, with a mutation rate of 28.96%. It was sensitive to targeted drugs of everolimus and sirolimus. The patient requested temporary follow-up, but did not receive drug treatment. There was no obvious recurrence of tumor and enlarged lymph nodes in 1~3 months follow-up. 【Conclusion】It is difficult to diagnose the perivascular epithelioid cell tumor of kidney before operation. The treatment plan is still to resect the tumor completely. For the recurrence or metastasis of the tumor, targeted therapy and immunotherapy are expected to be the effective treatment.
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2020, Vol. 37 
