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目的:探讨中枢神经系统黑素细胞病变的临床病理特点,并分析其诊断与鉴别诊断的方法及治疗和预后。方法:对1例新生儿神经皮肤黑变病尸解标本、10例脑脊膜黑素细胞瘤及2例脑膜原发恶性黑素瘤患者的病理组织标本进行光镜、免疫组化及电镜检查,并结合文献进行分析。结果:新生儿神经皮肤黑变病患儿全身多个部位可见黑素痣,大脑顶叶、颞叶、小脑等处软脑膜出现广泛黑色素沉着及脑实质内多灶性黑素沉着。黑素细胞瘤细胞呈梭形或上皮样,排列成片状或束状,核圆形或卵圆形,异型不明显;免疫组化提示瘤细胞HMB-45、S-100阳性,部分波形蛋白阳性(7例),神经胶质纤维酸性蛋白(GFAP)、上皮细胞膜抗原(EMA)、神经元特异性烯醇化酶(NSE)、Syn及Leu-7阴性;电镜下胞质内可见黑素颗粒。脑膜原发恶性黑素瘤均为孤立肿块伴有浸润性边缘,与脑膜关系密切,瘤细胞异型性明显,病理性核分裂象多见,伴出血坏死;免疫组化提示HMB-45和S-100阳性,不表达波形蛋白等其他标志物。结论:中枢神经系统黑素细胞增生形成的3种病变均较少见,且三者间的生物学行为差异较大,预后不同。
Objective: To investigate the clinical and pathological features of central nervous system melanocytic lesions and to analyze the methods of diagnosis and differential diagnosis, treatment and prognosis. Methods: The histopathological specimens of one patient with melasma of autonomic skin degeneration, 10 meningioma of brain and 2 patients with primary meningioma of meningioma were examined by light microscopy, immunohistochemistry and electron microscopy , And combined with the literature for analysis. Results: Neonatal melanocytic melanoma in children with multiple parts of the body can be seen melanocytic nevus, parietal lobe, temporal lobe, cerebellum and other pachypletes widespread melanoma and intraparenchymal multifocal melanin deposition. Melanoma cells were fusiform or epithelioid, arranged in a sheet or bundle, nuclear or oval, irregular shape; immunohistochemistry prompted tumor cells HMB-45, S-100 positive, part of the vimentin (7 cases), glial fibrillary acidic protein (GFAP), epithelial cell membrane antigen (EMA), neuron specific enolase (NSE), syn and Leu-7 negative; . Meningeal malignant melanoma were isolated lumps associated with infiltrative edge, closely related with the meninges, tumor atypia obvious pathological mitosis more common with hemorrhage necrosis; immunohistochemistry prompted HMB-45 and S-100 Positive, do not express other markers such as vimentin. CONCLUSION: All three lesions of melanocyte proliferation in the central nervous system are rare, and the biological behavior among the three is quite different with different prognosis.