肾上皮样血管平滑肌脂肪瘤临床病理分析  

A clinicopathological study on renal epithelioid angiomyolipoma

作　　者：裴媛媛 王龙 濮珍红 陆钦丽 章俊 PEI Yuanyuan;WANG Long;PU Zhenhong;LU Qinli;ZHANG Jun(Department of Pathology,the Affiliated Hospital of Guizhou Medical University,Guizhou Guiyang 550004,China;Department of Pathology,Guiqian International General Hospital,Guizhou Guiyang 550004,China.)

机构地区：[1]贵州医科大学附属医院病理科,贵州贵阳550004 [2]贵黔国际总医院病理科,贵州贵阳550004

出　　处：《现代肿瘤医学》2023年第17期3266-3269,共4页Journal of Modern Oncology

基　　金：国家自然科学基金资助项目(编号:82060565)。

摘　　要：目的:探讨肾上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)的临床病理学特征、诊断、鉴别诊断及预后。方法:回顾性分析8例肾上皮样血管平滑肌脂肪瘤临床病理资料及复习有关文献,对其诊断及鉴别诊断进行分析,探讨其预后及判断指标。结果:8例患者中男性3例,女性5例,平均年龄45.1岁(34~58岁),均无结节性硬化症病史。其中5例影像学考虑肾癌。术后病理显示肿瘤由上皮样肿瘤细胞、畸形血管及散在成熟脂肪细胞组成。2例病灶内可见核分裂象,其中1例可见病理性核分裂象。8例均诊断为肾EAML,其中1例伴血管平滑肌脂肪瘤(angiomyolipoma,AML)。8例肾EAML中1例为高度恶性潜能。所有患者在随访期间均未见复发或转移。结论:肾EAML是具有恶性潜能的肿瘤,诊断主要依靠组织形态学及免疫组化,其预后及恶性生物学行为的病理判断标准尚需更多的病例积累、总结。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.

关 键 词：上皮样血管平滑肌脂肪瘤 恶性 鉴别诊断 

分 类 号：R737.11[医药卫生—肿瘤]