【摘要】 目的探讨中枢神经系统原发性黑色素细胞瘤的临床病理特点、免疫表型、诊断及鉴别诊断要点。方法收集2011年收治的2例中枢神经系统原发性黑色素细胞瘤患者的临床资料,对病理组织学及免疫表型进行分析。结果 2例患者临床上均为单发肿块,无皮肤色素性肿瘤病变。1例发生于鞍区,1例发生于椎管内,均表现为局部缓慢生长的软组织肿块,术前病程分别为3年及10年。手术时肿瘤大体检查肿块呈褐色、质实。病理检查镜下表现为梭形及卵圆形肿瘤细胞漩涡状、巢状分布,细胞胞质内见不同程度的黑色素沉着,核分裂罕见,平均计数<1个/10 HPFs。免疫组化学检测示肿瘤细胞S-100蛋白、HMB45蛋白、波形蛋白均阳性;细胞角蛋白、上皮细胞膜抗原、胶质纤维酸性蛋白均阴性;1例神经元特异性烯醇化酶局部阳性表达。结论发生于鞍区及脊髓腰段的中枢神经系统原发性黑色素细胞瘤较少见,具有一定的组织病理学特点。肿瘤虽然生长缓慢,但可局部复发,甚至可恶变,需长期随访。更多还原

【Abstract】 Aim To analyze the clinicopathologic and immunohistochemical features, diagnosis, differential diagnosis of primary melanocytoma of central nervous system. Methods Two cases of primary melanocytoma of CNS in 2007 and 2014 were collected and the clinicopathologic and immunohistochemical data were analyzed. Results Two patients presented without melanocytic tumour of the skin. The tumours were solitary and located in sella area and intraspinal canal with slow-growing. The duration of disease onset was 3 years and 10 years respectively. Generally the tumours were brown and solid, histologically slightly spindled or oval tumour cells containing variable melanin arranged in whorls, nests. Cytologic mitoses were absent(on average less than 1/10 HPFs). Immunohistochemical study showed that the two cases were positive for s-100, HMB45, vimentin and negative for cytokeratin, epithelial membrane antigen, GFAP. The staining for NSE were partly positive in one case. Conclusion Primary melanocytoma of CNS located in sella and lumbar spinal cord is rarer. It presents some pathological characteristics. Although slow-growing, itcarries a recurrence rate and even has manignant change, warranting long-term follow up needed.更多还原