Angiomyoliploma with epithelial cysts (AMLEC) - A distinct cystic variant of angiomyolipoma
Angiomyoliploma with epithelial cysts (AMLEC) - A distinct cystic variant of angiomyolipoma
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DOI：
10.1097/01.pas.0000194298.19839.b4
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发表时间：
2006-05-01
影响因子：
5.6
通讯作者：
Argani, P
中科院分区：
文献类型：
--
作者：
Fine, SW;Reuter, VE;Argani, P
肾血管平滑肌脂肪瘤（AML）是典型的实性病变，由不同数量的脂肪、血管和肌肉组织组成，缺乏上皮成分。虽然已知在急性髓性白血病中可观察到被包裹的肾小管，但以囊性肿块的表现尚未见报道。我们报告4例囊性肾性急性髓性白血病的临床病理和免疫组织化学特征。患者男2例，女2例，年龄37 ~ 76岁。都没有激素治疗史。其中一个病人患有结节性硬化症，这个病人和我都有双侧囊性肾损伤。肉眼可见，病变最大尺寸在1.3 - 4.5cm之间。组织学上，病变由三部分组成：1)上皮囊肿排列成立方体到鞋钉状细胞；2)致密的上皮下“形成层样”细胞层，骨髓样急性髓性白血病伴有明显的混合慢性炎症；3)肌肉为主的AML伴细胞上皮下基质外血管畸形。免疫组织化学，所有四个病变的基质成分标记有HMB-45和melana。在细胞上皮下基质中最强烈，而平滑肌肌动蛋白和desmin表现出相反的模式，在肌肉为主的AML区域中强度最大。雌激素和孕激素受体（ER， PR）和CD10的免疫反应性在所有病例中都存在，在上皮下AML细胞中染色最强且最弥漫性。囊肿内膜细胞呈泛细胞角蛋白、豆凝集素（远端肾细胞上皮的后一种特征）阳性，但未标记ER、PR或黑素细胞标志物。尽管这些独特的病变可能与混合性上皮-间质瘤混淆。它们缺乏女性优势或与激素治疗有关，它们独特的血管发育不良和肌肉外层紊乱，以及它们的免疫组织化学特征，支持它们的AML伴上皮囊肿分类。尽管囊肿似乎主要来自于被包裹的收集管，但AML间质细胞的特殊上皮下凝聚，其形态学和免疫组织化学特征表明向子宫内膜基质分化，可能代表了以前未报道的AML上皮诱导的苗勒管分化。
Renal angiomyolipoma (AML) is typically a solid lesion, composed of varying amounts of adipose, vascular, and muscular tissue, lacking an epithelial component. Although it is known that entrapped renal tubules may be observed in AML, presentation as a cystic mass has not been previously reported. We report the clinicopathologic and immunohistochemical features of four cystic renal AML. The lesions were found in 2 male and 2 female patients, ranging in age from 37 to 76 years.. none with a history of hormonal therapy. One of the four patients had known tuberous sclerosis, and this patient and I other presented with bilateral cystic renal lesions. Grossly, the lesions measured between 1.3 and 4.5cm in greatest dimension. Histologically, the lesions were each composed of three components: 1) epithelial cysts lined by cuboidal to hobnail cells; 2) a compact subepithelial "cambiurn-like" layer of cellular, mijilerian-like AML strorna with prominent admixed chronic inflammation; and 3) muscle-predominant AML with associated dysmorphic blood vessels exterior to the cellular subepithelial strorna. Immunohistochemically, the stromal components of all four lesions labeled with HMB-45 and Melan-A. most intensely in the cellular subepithelial strorna, whereas smooth muscle actin and desmin demonstrated the opposite pattern, with greatest intensity in the muscle-predominant AML areas. Immunoreactivity for estrogen and progesterone receptors (ER, PR) and CD10 was present in all cases, with strongest and most diffuse staining in the subepithelial AML cells. The cyst lining cells were positive for pancytokeratin and so), bean agglutinin (the latter characteristic of distal nephron epithelium) but did not label for ER, PR, or melanocytic markers. Although these distinctive lesions may be confused with mixed epithelial-stromal tumor. their lack of female predominance or association with hormone therapy, their distinctive dysplastic blood vessels and disorganized exterior muscular layer, along with their immunohistochemical profile, support their classification as AML with epithelial cysts. Whereas the cysts appear to arise primarily from entrapped collecting ducts, the peculiar subepithelial condensation of AML stromal cells, with morphologic and immunohistochemical features Suggesting differentiation toward endometrial strorna, may represent epithelial-induced mullerian differentiation not previously reported in AML.