|Table of Contents|

A clinicopathological study on renal epithelioid angiomyolipoma

Journal Of Modern Oncology[ISSN:1672-4992/CN:61-1415/R]

Issue:
2023 17
Page:
3266-3269
Research Field:
Publishing date:

Info

Title:
A clinicopathological study on renal epithelioid angiomyolipoma
Author(s):
PEI Yuanyuan1; WANG Long2; PU Zhenhong1; LU Qinli1; ZHANG Jun1
1.Department of Pathology,the Affiliated Hospital of Guizhou Medical University,Guizhou Guiyang 550004,China;2.Department of Pathology,Guiqian International General Hospital,Guizhou Guiyang 550004,China.
Keywords:
epithelioid angiomyolipoma; malignant; differential diagnosis
PACS:
R737.11
DOI:
10.3969/j.issn.1672-4992.2023.17.022
Abstract:
Objective:To investigate the clinicopathologic features,diagnosis,differential diagnosis and prognosis of renal epithelioid angiomyolipoma(EAML).Methods:The clinical and pathological data of 8 cases of renal EAML were analyzed retrospectively,and the related literatures were reviewed.The diagnosis and differential diagnosis were analyzed,and the prognosis and judgement indexes were discussed.Results:Among the 8 patients,there were 3 males and 5 females with an average age of 45.1 years(34~58 years).None of them had a history of tuberous sclerosis.5 cases were considered as renal cell carcinoma by imaging.Postoperative pathology showed that the tumor was composed of epithelioid cells,dysmorphic blood vessels and scattered mature adipocytes.Mitoses were identified in 2 cases and atypical mitosis were seen in 1 case.All 8 cases were diagnosed as renal EMAL,of which 1 case was associated with AML.One of the eight cases had high malignant potential.No recurrence or distant metastatic disease was found in all patients during the follow up period.Conclusion:Renal EAML is a tumor with malignant potential,and its diagnosis mainly depends on histomorphology and immunohistochemistry.The prognosis and pathological criteria of malignant biological behavior need to be accumulated and summarized in more cases.

References:

［1］HE WL，CHEVILLE JC，SADOW PM，et al.Epithelioid angiomyolipoma of the kidney:Pathological features and clinical outcome in a series of consecutively resected tumors［J］.Modern Pathology,2013,26:1355-1364.
［2］SAOUD R,KRISTOF TW,JUDGE C,et al.Clinical and pathological features of renal epithelioid angiomyolipoma(PEComa):A single institution series［J］.Urologic Oncology:Seminars and Original Investigations,2022,40:18-24.
［3］LEE WC，CHOI SY,LEE CW,et al.Does epithelioid angiomyolipoma have poorer prognosis,compared with classic angiomyolipoma［J］.Investigative and Clinical Urology,2018,59:357-362.
［4］LEE KH,TSAI HY,KAO YT,et al.Clinical behavior and management of three types of renal angiomyolipomas［J］.Journal of Formosan Medical Association,2019,118:162-169.
［5］MARTIGNONI G,BONETTI F,PEA M,et al.Renal disease in adults with TSC2/PKD1 contiguous gene syndrome［J］.The American Journal of Surgical Pathology,2002,26(2):198-205.
［6］姜国锦，王亮，方松华.肾上皮样血管平滑肌脂肪瘤的MSCT表现及误诊原因分析［J］.医学影像学杂志，2021，31（8）：1363:-1366. JIANG GJ,WANG L,FANG SH.Analysis of MSCT in manifestation and misdiagnosis of renal epithelioid angiomyolipoma ［J］.J Med Imaging,2021,31(8)：1363-1366.
［7］LUO C，LIU ZW，GAO MY，et al.Renal epithelioid angiomyolipoma:computed tomography manifestation and radiologic-pathologic correlation depending on different epithelioid component percentages［J］.Abdominal Radiology,2022,47:310-319.
［8］刘从涛，孙昊，金征宇.肾脏上皮样血管平滑肌脂肪瘤的影像学诊断［J］.医学影像学杂志，2017,27（9）：1800-1801. LIU CT,SUN H,JIN ZY.Imaging diagnosis of renal epithelioid angiomyolipoma［J］.J Med Imaging,2017,27（9）：1800-1801.
［9］郭锐，康素海，李达，等.肾上皮样血管平滑肌脂肪瘤与透明细胞癌MRI鉴别诊断［J］.中国医学计算机成像杂志，2018,24（3）：229-233. GUO R,KANG SH,LI D,et al.Can routine MRI differentiate renal epithelioid angiomyolipoma from clear cell renal cell carcinoma［J］.Chin Comput Med Imag,2018,24（3）：229-233.
［10］NESE N,MARTIGNONI G,FLERCHER CD,et al.Pure epithelioid PEComas(so-called epithelioid angiomyolipoma) of the kidney:a clinicopathologic study of 41 cases.Detailed assessment of morphology and riskstratification［J］.Am J Surg Pathol,2011,35(2):161-176.
［11］BRIMO F,ROBINSON B,GUO C,et al.Renal epithelioid angiomyolipoma with atypia:a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy［J］.Am J Surg Pathl,2010,34(5):715-722.
［12］ANWAIER A,XU WH,TIAN X,et al.Evaluation of clinicopathological profiles and development of a risk model in renal epithelioid angiomyolipoma patients:a large-scale retrospective cohort study［J］.BMC Urology,2022,22:148.
［13］SHEN Q,RAO Q,XIA QY,et al.Perivascular epithelioid cell tumor(PEComa) with TFE3 gene rearrangement:Clinicopathological,immunohistochemical,and molecular features［J］.Virchows Arch,2014,465:607-613.
［14］WANG HL,ZHAN HY,YAO ZG,et al.Malignant renal epithelioid angiomyolipoma with TFE3 gene amplification mimicking renal carcinoma［J］.Clinical Nephrology Case Studies,2018,6:11-15.
［15］ZHANG YN,WEI XJ,TENG XJ et al.p53 aberration and TFE3 gene amplification may be predictors of adverse prognosis in epithelioid angiomyolipoma of the kidney［J］.Diagnostic Pathology,2023,18:14.
［16］RAMMAL R，KORENTZELOS D，SKAUGEN JM，et al.Molecular characterization of malignant renal epithelioid angiomyolipoma［J］.Am J Clin Pathol,2023,159:211-220.
［17］MCCARTHY MR,NICHOLS PE,SHARMA V,et al.Molecular and immunophenotypic correlates of metastatic epithelioid angiomyolipoma include alteration of TP53,RB1,and ATRX ［J/OL］.Arch Pathol Lab Med,2023,147:817-825［2023-5-25］.Https://pubmed.ncbi.nlm.nih.gov/36308711.DOI:10.5858/arpa.
［18］苏世强，刘丽哲，苏晓哲，等.肾上皮样血管平滑肌脂肪瘤17例临床病理特征及诊治分析［J］.大连医科大学学报，2020,42（1）：16-20. SU SQ,LIU LZ,SU XZ,et al.Renal epithelioid angiomyolipoma:clinicopathological features,diagnosis and treatment analysis of 17 cases［J］.Journal of Dalian Medical University,2020,42（1）：16-20.
［19］郭刚，陈文政，张帆,等.依维莫司在侵袭性肾脏上皮样血管平滑肌脂肪瘤治疗中的应用［J］.微创泌尿外科杂志,2019,8(6):371-376. GUO G,CHEN WZ,ZHANG F,et al.Application of everolimus in patients with invasive renal epithelioid angiomyolipoma［J］.Journal of Minimally Invasive Urology,2019,8(6):371-376.

Memo

Memo:
National Natural Science Foundation of China(No.82060565）；国家自然科学基金资助项目（编号：82060565）
Last Update: 2023-07-31