Clinical analysis on 12 cases of renal epithelioid angiomyolipomas
Liu Yongguo
Huang Qingbo
Zhang Lei
Zhang Zhiyong
Chen Wenzheng
Guo Gang
Abstract:Objective:To investigate the clinical characteristics of renal epithelioid angiomyolipomas(RE-AML)and evaluate the clinical outcomes.Methods:The clinical data of 12 patients with pathologically confirmed REAML from March 2012 to October 2017 were collected,and clinical features,pathological characteristics,im-age findings and treatment effect were retrospectively analyZed.Results:In the 12 cases,there were 5 males and 7 of females.The median age was 39.5 years(range from 26-61 years).All cases were examined by ultrasound,CT or MRI.There were 4 cases of renal tumors,1 case of adrenal tumor,2 cases of renal cell carcinoma with thrombus and 5 cases of renal angiomyolipoma,which were preoperatively diagnosed.Four cases were subjected to TSC gene detection in this group:1 was negative,and the rest 3 were positive.The one case positive for TSC gene detection was diagnosed as having nodular sclerosis before surgery,and the tumor was significantly reduced and operated successfully after 3 months of treatment with ivermox.All the 12 patients underwent operation,including 3 cases given radical nephrectomy,2 cases given radical nephrectomy and vena venous carcinoma,and 7 cases giv-en partial nephrectomy.The median follow-up period was 20 months(range one month to 70 months).Eight cases obtained disease-free survival,and one case developed contralateral tumor after radical operation.The two cases of tumor with IVC thrombus have not yet reviewed images in the short term follow-up.One case had recurrence and multiple metastases in five years after surgery,and died one year later after the second operation.Conclusions:The clinical symptoms of REAML have no specificity.It is easily confused with renal carcinoma or classic renal angio-myoma in preoperative image,and is confirmed pathologically.Surgical resection is primary treatment,and com-plete resection may improve the survival.For the patients with un-complete resection,un-operation or merge dis-tant metastases,adjuvant chemotherapy and molecular targeted therapy may have certain curative effect.
Keywords:renal neoplasmrenal angiomyolipomarenal epithelioid angiomyolipomas
Publication Date:2018-01-01
Online Publishing Date:2025-08-15(First online date of this platform, not the publication date of the document)
Pages:5( 122-126 )
