肾透明细胞肉瘤的临床病理及免疫表型特征

目的 探讨肾透明细胞肉瘤（clear cell sarcoma of the kidney，CCSK）的临床病理特点、免疫表型特征及鉴别诊断。方法 应用HE和免疫组化vimentin、bcl-2、desmin、S-100蛋白、CD99、CD34、CDll7、CK、EMA染色，观察2例CCSK的病理组织学形态，并复习文献。结果 镜下见瘤细胞为上皮样或短梭形，被分枝状纤维血管间质分隔成巢团状，部分区域见黏液样变性微囊肿和细胞外胶原玻璃样变类似骨样组织的硬化型等形态变异。免疫组化示：瘤细胞vimentin和bcl-2弥漫阳性，余为阴性。结论 CCSK是一种罕见的儿童期恶性肾肿瘤，诊断主要依靠组织病理学和免疫组化，熟悉其形态学变异有利于与其它类似病变如肾母细胞瘤、先天性中胚叶肾瘤、肾恶性横纹肌样瘤、原始神经外胚叶肿瘤等鉴别。

Clear cell sarcoma of kidney: two cases report and review of the literature

Purpose To study the clinicopathological and immunohistochemical features,and differential diagnosis of clear cell sarcoma of kidney(CCSK).Methods Two cases of CCSK were investigated by means of light microscopy,HE stain,immunohistochemical stain for vimentin,bcl-2,desmin,S-100 protein,CD99,CD34,CD117,CK and EMA,together with review of the related literature.Results Microscopically,the tumor cells were epithelioid or short spindled and special nests of tumor cells separated by arboring fibrovascular septa.Such morphological variants of CCSK as myxoid degeneration,microcysts formation and sclerosing pattern of extracellular hyalined collagen simulating osteoid were present in some regions.Immunohistochemical stains showed that tumor cells were diffusely positive for vimentin and bcl-2 antigen,and others negative.Conclusions CCSK is a rare type of renal neoplasm in childhood and its diagnosis relies mainly on histopathology and immunohistochemistry.It is important to be familiar with the morphological variants of CCSK to avoid confusion with other similar lesions,such as Wilm's tumor,congenital mesoblastic nephroma,malignant rhabdoid tumor of kidney,primitive neuroectodermal tumor.