肾周血管平滑肌脂肪瘤与脂肪肉瘤的临床病理对比分析

Clinicopathologically comparative analysis of perirenal angiomyolipoma and liposarcoma

目的对比分析肾周血管平滑肌脂肪瘤(AML)与脂肪肉瘤的临床病理特征及鉴别、诊治要点。方法回顾性分析2015年1月-2020年12月北京大学国际医院诊治的11例肾周AML及39例肾周脂肪肉瘤的临床表现、影像学检查、病理学形态、免疫表型及治疗随访情况。结果两组患者肿瘤均紧邻肾脏,影像学肾缺损征不明显,易误诊。肾周AML女性患者占比较脂肪肉瘤高(P=0.003),发病年龄较脂肪肉瘤小(P<0.001)。肾周AML绝大多数单发(90.9%,10/11)、原发(100.0%,11/11),组织形态多伴有血管玻璃样变性(63.6%,7/11),免疫组化表达Melan-A和HMB45;而脂肪肉瘤常多发(51.3%,20/39)、复发(79.5%,31/39),免疫组化Ki-67、P16、CDK4、MDM2较AML高表达(P<0.05),MDM2基因扩增。两组患者肿瘤体积最大径及坏死占比差异无统计学意义(P>0.05)。结论腹膜后肾周AML与脂肪肉瘤具有相似的影像学特征,性别、年龄、肿瘤数目、原发/复发、组织形态、蛋白表达及基因扩增检测有助于鉴别诊断。脂肪肉瘤需要更广泛的切除、积极治疗并随访。

Objective To compare the clinicopathological features,differential diagnosis and treatment between perirenal angiomyolipoma(AML)and liposarcoma.Methods Eleven cases of AML and thirty-nine cases of liposarcoma around the kidney from Peking University International Hospital from January 2015 to December 2020 were reviewed in terms of clinical manifestations,imaging examination,pathological morphology,immunophenotype and treatment follow-up.Results The tumors in both groups were close to the kidney,and the imaging signs of renal defect were not distinctive enough to diagnose correctly.Perirenal AML was mainly female(P=0.003),the age of onset was younger than liposarcoma(P<0.001).Most of perirenal AML were single(90.9%,10/11)and primary(100.0%,11/11),the histomorphology was mostly accompanied by vascular hyaline degeneration(63.6%,7/11),and Melan-A and HMB45 were positive by immunohistochemistry.While liposarcoma was often multiple(51.3%,20/39)and recurrent(79.5%,31/39),Ki-67,P16,CDK4,MDM2 were more highly expressed than AML(P<0.05),and MDM2 gene was amplified.There was no significant difference in the maximum diameter and necrosis between the two groups(P>0.05).Conclusions Retroperitoneal perirenal AML and liposarcoma have similar imaging features,and gender,age,tumor number,primary/recurrent character,histomorphology,protein expression and gene amplification detection are helpful to differentiate them.Liposarcoma needs more extensive resection,active treatment and follow-up.