原发性脊髓恶性黑色素瘤1例报告及文献复习

目的 探讨原发性脊髓恶性黑色素瘤的临床特点。方法 报告1例原发性脊髓恶性黑色素瘤的详细临床及病理资料，并结合献，对该罕见疾病的临床诊断、影像学、组织病理学及治疗等做一复习。结果 MRI显示肿瘤位于于髓外、硬脊膜下，以胸段为甚，波及全脊髓。T1加权稍高信号，T2加权呈高信号，增强后有明显强化。随着病程发展，肿瘤扩散至颅内。手术证实肿瘤位于软脊膜下并侵犯脊髓。组织病理、免疫组织化学电镜均表现为典型的恶性黑色素瘤特征。结论 该病例诊断为原发性脊髓恶性黑色素瘤，因肿瘤广泛扩散，术后化疗及免疫治疗效果不佳，发病约1年后死亡。

Primary intramedullary malignant melanoma: one case report and litertures review

Objective To study the clinical characteristics of primary intramedullary malignant melanoma.Methods The detail clinicaland pathological data of a case withprimary intramedullary malignant melanoma werereported.The literatures about primary intramedullary melanoma are reviewed in discussion.Results The MRI appeared that the extramedullary lesion occurred in nearly the whole spinal canal.The lesion showedslightly increased signal intensity on T1-weighted images and hyperintensity on T2-weighted images.Contrast-enhanced MRI showed enhancement in the lesion.Observed in operation,the jet black tumor was located in leptomeninges and invaded the cord.Histopathological characteristicsof malignant melanoma were confirmed in samples,and so as immunohistochemistory and electron micrograph.Conclusion The final diagnosisof primaryintramedullarymalignantmelanomawasestablished,whichwasbasedon MRI,surgicalobtainsandtheresultsof histopathologicalexamination.Theprognosiswasbadandthepatientdied1yearlater.