中枢神经系统原发性恶性黑色素瘤少见,约占黑色素瘤总发病率的1％。而原发性脊髓黑色素瘤更为罕见,1906年Hirschberg首先报告1例,迄今为止,文献报告仅有37例(含本例),肿瘤多位于中或低胸段脊髓。Hayward提出,诊断原发性脊髓黑色素瘤应具备以下条件:①除中枢神经系统外无其它任何部位的原发或转移性黑色素瘤;②病变未累及神经根及硬膜;③病变应经病理证实。 Central nervous system rare primary malignant melanoma, accounting for about 1% of the total incidence of melanoma. The primary spinal melanoma is more rare, Hirschberg first reported in 1906, so far, the literature reported only 37 cases (including this case), the tumor is located in the middle or low thoracic spinal cord. Hayward suggested that the diagnosis of primary spinal cord melanoma should meet the following conditions: ① in addition to the central nervous system without any other part of the primary or metastatic melanoma; ② lesions do not affect the nerve roots and dura; ③ lesions should be pathologically confirmed .