背景:尽管血管平滑肌脂肪瘤(AML)是一种相对罕见的实体,它是肾脏最常见的良性间充质肿瘤。
目的:为了强调AML的临床病理特征,并评估人黑色素瘤Black-45(HMB-45)的作用,Melan-A,平滑肌肌动蛋白(SMA),S-100和细胞角蛋白在其诊断中的作用。
方法:本研究纳入15例AML患者。从档案文件中检索临床和放射学数据,并对所有病例进行组织病理学评估以及HMB-45,Melan-A的免疫组织化学染色,SMA,S-100和细胞角蛋白。
结果:AML多见于女性(女性:男性=4:1),平均年龄为53.9±6.45岁.60%的患者有症状,其余40%无症状。发现肿瘤大小与症状之间存在统计学上的显着关系(P=0.02)。肿瘤大小小于4厘米的患者无症状,而肿瘤大小大于4厘米的患者则有不同的症状。13例是典型的AML,2例为上皮样AML。经典AML表现为脂肪组织的混合物,厚壁血管,和平滑肌,而上皮样AML主要由上皮样细胞组成,不含脂肪。HMB-45在所有AML病例中均呈阳性(100%),Melan-A在13/15(87%)中呈阳性,而SMA在11/15(73%)的AML中呈阳性,染色强度可变。所有AML病例的S-100和细胞角蛋白均为阴性。
结论:AMLs具有特征性的临床病理和免疫组织化学特征,其识别对于正确的诊断和治疗至关重要。
BACKGROUND: Although angiomyolipoma (AML) is a relatively rare entity, it is the most common benign mesenchymal neoplasm of the kidney.
OBJECTIVE: To highlight the clinicopathological characteristics of AML and to assess the role of Human Melanoma Black-45 (HMB-45), Melan-A, smooth muscle actin (SMA), S-100 and cytokeratin in its diagnosis.
METHODS: The
study included 15 cases of AML. Clinical and radiological data were retrieved from the archival files and all cases were subjected to a histopathological evaluation as well as immunohistochemical staining for HMB-45, Melan-A, SMA, S-100, and cytokeratin.
RESULTS: AML was more common in females (female:male = 4:1), the mean age was 53.9 ± 6.45 years. 60% of patients were symptomatic while the remaining 40% were asymptomatic. A statistically significant relationship was found between size of the tumor and the presence of the symptoms (P = 0.02). Patients with tumor size less than 4 cm were asymptomatic, while those with tumor size larger than 4 cm had different symptoms. Thirteen cases were classic AML, while 2 cases were epithelioid AML. Classic AML demonstrated admixture of fatty tissue, thick-walled blood vessels, and smooth muscle, while epithelioid AML was composed mainly of epithelioid cells and contained no fat. HMB-45 was positive in all cases of AML (100%), Melan-A was positive in 13/15 (87%) while SMA was positive in 11/15 (73%) of AML with variable staining intensity. All cases of AML were negative for S-100 and cytokeratin.
CONCLUSIONS: AMLs have characteristic clinicopathological and immunohistochemical features and their recognition is crucial for proper diagnosis and treatment.