肾透明细胞肉瘤的临床病理学研究

目的：探讨肾透明细胞肉瘤的临床病理,免疫组织化学,电镜特点和组织起源及预后,方法：对45例患儿（男31例,女14例,年龄3个月至12岁,平均3．2岁）肿瘤标本,用HE,免疫组织化学（LSAB法）和电镜技术进行观察。结果：45例患儿中,35例随访6－192个月,骨转移15例,,肺,肝等转移6例,8例复发,20例死亡。年龄和临床分期与存活率有关。典型的组织学形态为胞质浅染,核染色质细腻,核仁不清楚的片状瘤树枝状分布的纤维血管间质分隔,此外还可见粘液型,梭形细胞型,栅栏状型,上皮样型,硬化型,富于细胞型,囊肿型和血管扩张型等组织亚型结构,免疫组织化学;45例均表达波形蛋白,上皮膜抗原,细胞角蛋白,结蛋白,肌动蛋白,S－100蛋白,神经元特异性烯醇化酶,CD99,CD34和白细胞共抗原均为阴性,电镜下9例均显示瘤细胞细胞连接很原始,细胞器相对较少。结论：肾透明细胞肉瘤好发于儿童,具特殊的病理形态,可能起源于肾原始间质细胞,易发生骨;转移,死亡率高,为预后差的儿童肾肿瘤。

A clinicopathological study of clear cell sarcoma of the kidney

Objective To study the clinicopathological, immunohistochemical features and the histogenesis of clear cell sarcoma of the kidney (CCSK). Methods CCSK specimens from 45 pediatric cases, including 31 male and 14 female with an age range from 3 months to 12 years(mean of 3.2 years), were retrieved. Routine pathological, immunohistochemical and electron microscopic methods were utilized to analyze the CCSK specimens. Results 35 of the 45 cases were followed from 6 to 192 months. 15 patients presented with bone metastases, 6 had lung or liver metastases, 8 recurred and 20 died. Age and clinical stage at diagnosis correlated with the rate of survival. Histologically, the classic pattern of CCSK consisted of cells with pale cytoplasm,fine nuclear chromatin and indistinct nucleoli separated by an arborizing fibrovascular stroma. Other patterns were identified, including myxoid, spindle, palisading, epithelioid, sclerosing, cellular, cystic, and angiectatic. All tumors contained multiple patterns. Immunohistochemically, all cases were positive for vimentin , but negative for EMA, CK, desmin, actin, S-100, NSE, CD99, CD34 and LCA. Electron microscopy of 9 cases showed features of primitive cell conjunction and few organelles. Conclusion CCSK is a common renal neoplasm of childhood. CCSK may arise from renal mesenchymal cells and has the propensity to metastasize to the bone with poor clinical outcome.