中枢神经系统黑素细胞病变的临床病理观察及文献复习

Clinicopathologic study of melanocytic lesions in central nervous system

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 appraise the clinicopathologic feature,diagnosis and differential diagnosis,and treatment and prognosis of melanocytic lesions in central nervous system（CNS）.Methods An infant autopsy case of neurocutaneous diffuse melanosis,10 cases of meningeal melanocytoma and 2 cases of primary meningeal malignant melanoma were studied by light microscopy,immunohistochemistry and electron microscopy,and literatures were reviewed.Results The full-term male infant autopsy case was seven days old,with several melanocyte nevi in occiput,left face and neck.Melanin deposition was found in leptomeninges of cerebrum,temporal and cerebellum and also in brain parenchyma.Of the ten meningeal melanocytoma patients,six were female and four were male,aged 14-69 years（mean 37 years）.Microscopically,the tumor consisted of spindle cells and polygonal cells arranged in patchy and fascicular form.The nuclei were oval with no obvious abnormality.Immunohistochemistry showed that the tumor cells were positive for HMB-45,S-100 and vimentin,but were negative for GFAP,EMA,NSE,Syn and Leu-7.Many melanosomes were found in cytoplast under electron microscope.Primary meningeal malignant melanoma appeared as isolated tumor mass with invasive brim.The tumor cells were pleomorphic with mitoses and necrosis was easy to find.Conclusions The three types of pathological melanocytosis of CNS are rare,and the biological behaviour and prognosis of these three types of lesion were different.