The Research Progress On Renal Epithelioid Angiomyolipoma

Renal epithelioid angiomyolipoma(REAML)is a rare kind of renal solid tumors in clinic,with malignant potential characteristics,which belongs to the family of perivascular epithelioid cell tumor(PEComa),which is histologically mainly composed of epithelial cells.Mutations in the P53 gene can be found in malignant REAML tissues and may play a key role in the malignant transformation of REAML.Because clinicians understanding of it is still shallow,no specific clinical manifestations,conventional imaging can not confirm the diagnosis,so the clinical diagnosis is relatively difficult,easily lead to misdiagnosed as renal cell carcinoma,pathological results and immunohistochemical phenotypic results are of effective evidence to diagnostic and differential diagnosis,and the preferred are melanin-associated protein HMB-45,Melan-A.There is no effective treatment for REAML,but it is still the first choice for surgical treatment.It is still possible to relapse and metastasis after operation.Therefore,the prognosis is poor,the survival time is short,the mortality rate is high,and long-term follow-up observation is needed.The patients who can not be treated surgically,can try chemotherapy,m TOR inhibitors or Imatinib and other new targeted drug therapy,but the clinical efficacy remains to be further verified.