中枢神经系统黑色素细胞病变的临床与病理特征

Clinical and pathologic features of melanocytic lesion of the central nervous system

目的:探讨中枢神经系统黑色素细胞病变的临床与病理特征。方法:分析2例神经皮肤黑色素沉着症(NCM)和4例原发性脑膜黑色素瘤(PLM)患者的临床表现、影像学资料及术中与病理所见。结果:6例患者均以颅高压、癫痫为主要临床特征,CT或MRI有不同程度异常发现;2例NCM自出生后即有全身皮肤多处大片黑色素痣;4例PLM则无皮肤色素沉着及黑色斑块,其脑组织病理报告均发现脑膜黑色素瘤细胞。结论:颅高压、癫痫性发作是中枢神经系统黑色素细胞病变主要临床表现,皮肤损害、影像学改变对诊断有重要价值。

Objective To investigate of the central nervous system. Methods the clinical and pathologic features of melanocytic lesion We analyzed the clinical features, neuroimagings, and operational and neuropathological findings of 2 patients of neurocutaneous melanosis and 4 patients of primary leptomeningeal melanoma. Results All the 6 patients had the common clinical features of intracranial hypertension and epilepsy. Brain CT and MRI showed abnormal signals. More melanin pigment nevi were found on the skin of the 2 patients subjected to neurocutaneous melanosis. The other 4 patients subjected to primary leptomeningeal melanoma had no melanin pigment nevus on the skin, but the brain section displayed positive meningeal melanoma. Conclusion Intracranial hypertension and epilepsy are the main clinical manifestations of melanocytic lesion of the central nervous system, and cutaneous lesion and radiological findings are very important for the diagnosis.