成人肾原发性神经母细胞瘤临床病理观察

目的探讨成人肾原发性神经母细胞瘤的临床病理特征。方法对1例肾原发性神经母细胞瘤的临床表现、影像学、组织形态、免疫组化等进行分析并复习相关文献。结果患者女性，54岁。间褐性肉眼血尿半年伴左腰痛2个月。CT示左肾癌伴左肾静脉癌栓，腹膜后淋巴结转移。术中见肿瘤位于左肾中下极。组织学上，瘤细胞弥漫性或片状分布，主要为不同分化阶段的神经母细胞，核分裂多见，有菊形团、神经管和神经毡结构；灶性分布未分化神经母细胞，成熟的神经节细胞、胶质细胞和Schwannian基质。免疫组化显示肿瘤细胞表达Vim，Synaptophysin，ChromograninA，GFAP，NSE，S100，NF，而CK，CD99，EMA等阴性。结论成人肾原发性神经母细胞瘤临床上罕见，与肾癌难鉴别，CT和患者尿中儿茶酚胺代谢产物的检查，对诊断有指导意义，确诊需病理检查。

Objective To explore the clinicopathological characteristics of adult primary intrarenal neuroblastoma. Methods The clinical manifestation, images, histopathological features, and immunohistochemical results were analyzed in one case of adult primary intrarenal neurlblastoma, and the related literatures were reviewed. Results A 54-year-old woman presented with intermittent gross hematuria and left flank pain for 2 mouths. CT revealed left renal carcinoma, left renal vein cancer embolus, and retropefitoneal lymph node metastasis. The tumor was located at the lower left kidney. Histologically, the tumor cells distributed diffusely or flakily, mainly neuroblasts at different developmental stages, with nuclear division, chrysanthemum-shaped mass, neural tube, and neuropil. Immunohistochemical assay showed Vim, GFAP, chromogranin A, NF,S100, and synaptophysin were positively expressed in the tumor cells but CK, CD99, and EMA were not.Conclusions Adult primary renal neuroblastoma is rare and is difficult to be distinguished from renal carcinoma clinically. CT scans and detection of urinary catecholamine metabolites in patients has guiding significance in the diagnosis. Pathological examination is needed to confirm the diagnosis.