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目的探讨散发性肾血管母细胞瘤的临床病理特征、免疫表型、诊断与鉴别诊断。方法对1例原发性肾血管母细胞瘤进行组织学观察、免疫组化染色检测和临床随访,并复习相关文献。结果组织学形态显示肿瘤由2种成分构成:即不同成熟阶段的毛细血管和毛细血管网之间丰富的间质细胞。免疫组化:肿瘤间质细胞特征性地呈现α-Inhibin、vimentin、NSE和S-100(+),毛细血管壁CD34(+)。特殊染色显示苏丹Ⅲ(-)。结论散发性肾血管母细胞瘤是一种极少见的肿瘤,属于肾良性间叶性肿瘤,组织病理学特点结合免疫组化标记有助于该瘤的确诊。
Objective To investigate the clinicopathological characteristics, immunophenotype, diagnosis and differential diagnosis of sporadic renal hemangioblastoma. Methods One case of primary renal hemangioblastoma was observed by histology, immunohistochemical staining and clinical follow-up, and the related literatures were reviewed. Results Histology showed that the tumor consisted of two components: the abundant interstitial cells between the capillaries and the capillary network at different stages of maturation. Immunohistochemistry: Tumor stromal cells characteristic α-Inhibin, vimentin, NSE and S-100 (+), capillary wall CD34 (+). Special staining shows Sudan Ⅲ (-). Conclusions Sporadic renal hemangioblastoma is a very rare tumor belonging to benign nephropathy. The histopathological features combined with immunohistochemical staining are helpful for the diagnosis of this tumor.