儿童肾透明细胞肉瘤临床病理分析及文献复习

Clinicopathologic analysis and literature review of clear cell sarcoma of the kidney in children

目的:探讨儿童肾透明细胞肉瘤(clear cell sarcoma of the kidney,CCSK)的临床病理特点与鉴别诊断。方法:观察1例儿童肾透明细胞肉瘤的病理学形态、特殊染色及免疫表型特征并复习有关文献。结果:片内显示为小细胞肿瘤,呈弥漫性分布,部分细胞丰富,密集,部分区域细胞排列较松散,以分枝状纤维血管间质分隔肿瘤细胞呈不规则索状和小团状为结构特征,细胞呈圆形、多角形或梭形,细胞核呈圆形、卵圆形或棒状,核染色质呈细颗粒状,核膜较薄,核仁不明显。网状纤维染色:网状纤维密布于单个或小团肿瘤细胞之间。Masson三色:肿瘤间质小血管周纤维组织呈绿色。免疫组化染色:Vim、Bcl2和CD99(+),其他(-)。结论:CCSK是少见的小儿恶性肾肿瘤,诊断主要依赖于病理学,熟悉CCSK典型形态学特征以及合理应用特殊染色和免疫组化有助于同肾母细胞瘤、PNET和先天性中胚叶肾瘤等鉴别。

Objective：To investigate the clinicopathologic features and differential diagnosis of clear cell sarcoma of the kidney in children. Methods：Features of pathology,histochemical and immunohistochemical stains of renal clear cell sarcoma were described in 1 child,with review of the literatures. Results：The cells are small with diffuse distribution and densely spaced in some area while loosely spaced in others. The pattern of CCSK was characterized by irregular nests or cords of cells separated by regularly spaced,arborizing fibrovascular septa. The tumor cells had round,polygonal or spindle shape and characteristically possessed round,oval or rod like nuclei with fine,granular texture and thin nuclear membrane without prominent nucleoli. The reticular fiber staining showed dense reticular fi-bers enveloping individual tumor cells or clusters. The Masson staining showed collagen formation around small ves-sels. Tumor cells were immunoreactive for Vim,Bcl2 and CD99 and not immunoreactive for others. Conclusion：CCSK is an uncommon malignant renal neoplasm of childhood and its diagnosis relies mainly on histopathology. Familiar with classical pattern of CCSK and reasonable use of special stains and IHC helps to distinguish from other tumors such as Wilmˊs tumor,PNET and congenital mesoblastic nephroma.