中枢神经系统原发性黑色素细胞瘤的治疗体会

Diagnosis and treatment of primary melanocytoma in central nervous system

目的：中枢神经系统原发性黑色素细胞瘤罕见。文中探讨其治疗经验。方法回顾性分析南京军区南京总医院神经外科1999年1月至2012年12月收治的14例中枢神经系统原发性黑色素细胞瘤患者的临床资料，其中男5例，女9例，发病年龄14～52岁，平均年龄（32．7±10．7）岁。病程15 d至19年，中位值7个月。复发5例，首发9例。发生于颅内10例，发生于椎管内4例。 MRI检查结果示病灶T1WI相呈等、高信号，T2WI相呈等、低信号。病理免疫组化结果：所有患者S-100（＋）、HMB45（＋）、GFAP（-）、EMA（-），8例vimentin（＋），5例MelanA（＋）。14例患者均行手术治疗，其中全切除7例，近全切除3例，大部分切除4例。术后仅4例行放疗，2例行普通放疗，2例行γ刀放疗，共1～3个疗程。结果术后除出现1例高位截瘫、1例面瘫外，其余12例均恢复良好出院。术后随访时间1个月至13年，死亡2例均为非全切除术患者。结论中枢神经系统原发性黑色素细胞瘤的治疗首选手术，提倡早期彻底全切。

Objective There are differences in the diagnosis and treatment of primary melanocytoma in central nervous sys -tem.The article was to investigate the experience of its diagnosis and treatment . Methods Retrospective analysis were made on the clinical data of 14 cases with primary melanocytoma in central nervous system （ CNS） from January 1999 to December 2012, among which were 5 males and 9 females.The incidence ages were 14-52, average 32.7.The course of disease ranged from half a month to 19 years, geometric average 7.9 months.5 cases recurred and 9 cases occurred first.10 cases were intracranial and 4 were intraspinal. Results 14 patients underwent surgery and had pathologic diagnosis of melanocytoma .Total resection was performed in 7 patients, subtotal resection in 3, and partial resection in 1.Immunohistochemical study showed , in all cases, S-100 and HMB-45 were positive, GFAP and EMA were negative .Vimentin was positive in 8 cases and MelanA positive in 5 cases.12 cases recovered well and dis-charged except for paraplegia and facial paralysis in 1 case each. Conclusion Primary melanocytoma in CNS is very rare .Diagnosis is based on intraoperative findings , surgical pathology and immunohistochemistry results .Surgery is the primary therapy and early total resection is advocated .Adjuvant radiotherapy can reduce the recurrence rate .