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目的探讨肾透明细胞肉瘤的临床病理特点和鉴别诊断要点。方法结合文献对2例肾透明细胞肉瘤的临床资料、病理特征、免疫表型及治疗预后进行分析。结果典型的组织学特征表现为温和的卵圆形上皮样或梭形透明细胞排列成巢状或条索状,可见多量纤维间质分隔,并伴有特征性的“鸡爪样”小血管。免疫组化:肿瘤细胞vimentin、bcl-2、EGFR、CD117、CD99和CD10(+),S-100、CK、EMA、desmin、SMA、CD34和P504S(-)。结论肾透明细胞肉瘤是一种罕见的儿童肾恶性肿瘤,病理诊断尤其应注意与肾母细胞瘤和中胚层肾瘤鉴别。肿瘤细胞免疫组化染色有助于与其他肾脏肿瘤鉴别。
Objective To investigate the clinicopathological characteristics and differential diagnosis of renal clear cell sarcoma. Methods The clinical data, pathological features, immunophenotypes and prognosis of 2 cases of renal clear cell sarcoma were analyzed with the literature. Results Typical histological features were mild oval epithelial-like or spindle-shaped transparent cells arranged in nests or cords, showing numerous interstitial fibroids with characteristic “chicken-like” small Blood vessels. Immunohistochemistry: tumor cells vimentin, bcl-2, EGFR, CD117, CD99 and CD10 (+), S-100, CK, EMA, desmin, SMA, CD34 and P504S (-). Conclusions Renal clear cell sarcoma is a rare childhood malignant neoplasm. The pathological diagnosis should be distinguished from nephroblastoma and mesodermal nephroma. Immunohistochemical staining of tumor cells can help identify other renal tumors.