肾血管平滑肌脂肪瘤临床病理分析

The clinical pathology analysis of renal angioleiomyolipoma

目的:观察肾血管平滑肌脂肪瘤(AML)的临床病理特点并复习相关文献,探讨肾AML的诊断及鉴别诊断。方法:对18例肾AML进行临床病理学及免疫组化观察、随访,并复习相关文献。结果:6例属经典型,5例属肌瘤型,3例属脂肪瘤样型,4例为上皮样型。经典型中1例为多发性。镜检:肿瘤由不同比例的厚壁血管、平滑肌和脂肪组织组成,平滑肌细胞呈梭形/上皮样,可有一定异型性;上皮样细胞胞质丰富,透明/微嗜酸性,核大深染,核仁大而明显,可见多核瘤细胞/神经节样细胞。免疫组化:HMB45、Mart-1、Vimentin、SMA均阳性,CD34血管阳性,p-CK、EMA阴性,Desmin、S-100、Ki-67不同程度阳性。结论:肾AML是良性肿瘤;然而,当肿瘤体积巨大、出现上皮样细胞成分并伴有坏死、核异型性明显、核增殖活性高及核分裂象多时,应考虑为恶性,需长期密切随访。

Objective： To observe the clinicopathologic characteristics of renal angioleiomyolipoma and review related articles,then discuss its diagnosis and differential diagnosis. Methods： Observing the clinicopathology and immunohistochemical staining of 18 renal angioleiomyolipoma cases,following up them and reviewing related articles.Results： Six cases were classified as classical type,5 cases as liomyoma type,3 cases lipomyoma type,4 cases epithelioid type. One of the classical type case was multiple angioleiomyolipoma. Microscopic examination： Angioleiomyolipoma was composed of thick-walled vessels,smooth muscles and adipose tissues by different proportions. The smooth muscle cells showed fusiform shape/epithelium structure and heteromorphism invariably. The epithelioid cells have plenty transparence/acidophilia cytoplasm,big anachromasis nucleus and large manifest nucleolus,polynuclear tumor cells/ganglion-like cells can also be seen. IHC results： The positive markers were HMB45,Mart-1,Vimentin,SMA,Desmin,S-100,Ki-67 were positive in different degree,the vessels showed CD34 positive,and p-CK,EMA were negative. Conclusion： Renal AML is benign tumor,while giga-tumor volume,epithelioid cell appearance,accompany with necrosis,nuclear atypia,high nuclear proliferation and plenty mitosis,may consider its malignancy,thus,should be closely followed up.