| Objective: As a potentially malignant and clinically rare perivascular epithelioid cell tumor,renal epithelial angiomyolipoma has a very high misdiagnosis rate.At present,there is still insufficient experience in the diagnosis and treatment of this disease.This article aims to explore the clinical features,diagnosis,treatment and prognosis of eAMLs,in order to improve the understanding of the disease and the level of clinical diagnosis and treatment.Methods: Retrospectively analyze the clinical data of 12 eAML patients admitted to our hospital from August 2010 to May 2020,and analyze the clinical characteristics of eAML patients,including clinical manifestations,imaging examinations,medical history characteristics,treatment and follow-up,combined with review of relevant domestic and foreign literature,summarized the diagnosis,identification,prognosis,treatment and prospects of the disease.Results: Among the 12 eAML patients,3 were males and 9 were females,with an average age of 43.1 years,all of which were solitary masses;8 had symptoms of waist and abdomen pain and discomfort,and the remaining 4 had no obvious symptoms,which were found during physical examination.One case was admitted to the emergency department due to tumor rupture and bleeding.Only 1 of the 12 patients considered cystic renal cell carcinoma/eAML for preoperative diagnosis,5 patients were diagnosed as renal cancer,5 patients were diagnosed as hamartoma,and 1 patient was considered retroperitoneal liposarcoma.Doppler ultrasound in the urinary system showed hyperechoic/isoechoic/hypoechoic masses with clear boundaries;enhanced CT showed that the masses were round or elliptical,most of which were slightly high-density,mixeddensity shadows,with obvious enhancement,and some showed grid-like enhancement,lesions Malformed or thick vascular shadows are seen inside;MRI shows short T1 and T2 signals,low signal after fat suppression,and enhanced vascular shadows in the lesions after enhancement.ECT renal dynamic imaging showed that the kidneys in the affected area had varying degrees of glomerular filtration function impairment.The maximum diameter of the tumor is 2.4～21.5cm.The average tumor diameter was 8.2cm.6 patients underwent retroperitoneal laparoscopic radical nephrectomy,4 patients underwent retroperitoneal partial nephrectomy,and 2 patients underwent open nephrectomy due to emergency surgery and huge tumor size.Seven-sixths of total kidney specimens,showed that aggressive growth of tumors,which invaded the collecting system,renal capsule,renal cortex or renal medulla.Under the light microscope,the tumor cells were fusiform or epithelioid(polygonal),among which a large number of thick-walled blood vessels and varying amounts of adipose tissue were staggered,showing focal necrosis and hemorrhage.Immunohistochemistry showed that HMA-45,Melan-A,SMA,and Vim were positive,CK was negative,and Ki67 was less than 5%.The follow-up time ranged from 3 to 56 months,and no recurrence or distant metastasis was found.Conclusion: EAML is rarely seen.The prevalence of female patients is high,and the symptoms are closely related to the size of the tumor.The imaging manifestations are diverse,which can be helpful to the initial diagnosis of the disease.The final diagnosis depends on histopathology and immunohistochemistry.Characteristic epithelioid cells or spindle cells appear under the light microscope,with large nuclei,rich cytoplasm,eosinophilia,and pathological mitotic figures.Tumors specifically express HMA-45,Melan-A and SMA.Genetic testing has potential value for the identification of eAML,tumor typing,and the use of targeted chemotherapeutics.It is confirmed that surgical treatment can bring a good prognosis for eAML.However,relevant case reports suggest that eAML can have metastasis and recurrence,and metastatic eAML can benefit from chemotherapy,immunotherapy,TAE and many other treatment methods.Considering the potential malignancy of eAML,long-term close follow-up is still recommended. |
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