Epithelioid angiomyolipoma of the kidney / 中华泌尿外科杂志

ABSTRACT

Objective To investigate the clinicopathologic features of epithelioid angiomyolipoma of renal(REAML). Methods Six cases of REAML were analyzed by histopathological and clinical characteristics.Of the 6 cases,4 cases were solitary tumors and 2 cases were multiple lesions.The diameter of tumors was about 9 cm in average.One case had a family history of nodular sclerosis.Three cases were found adipose tissue in CT scan and diagnosed for RAML,the other 3 cases were diagnosed for renal cancer.All cases were undergone surgical approach,3 cases were undergone resection of tumors only and the other 3 were performed nephrectomy. Results Pathological characterstumor was mainly composed of epithelioid cells presented with invasive hyperplasia of atypical pleomorphism,hyperchromatic nuclei with frequent mitotic figures,giant neoplasic cells and extensive hemorrhagic necrosis immunohistochemcial staining showing a positive HMB45 and negative of EMA,CK in most neoplasic cells.All cases were followed up for 10 to 44 months,5 cases did not recurrence and metastases;the other had lung metastasis after operation 18 months later,without any treatment the patient died 10 months later. Conclusions Most of REAMLs are benign and often misdiagnosed for renal cancer by CT scan.HMB45 is positive in immunohistochemcial staining available for diagnose.The minority of REMAL is malignant potentially and should be followed up closely.Operation is major method.