中枢神经系统原发性黑色素瘤10例临床分析

Diagnosis and treatment of primary melanoma of the central nervous system: a clinical analysis of 10 cases

摘要:

目的 探讨中枢神经系统原发性黑色素瘤的诊治与随访结果。方法 回顾性分析自2002年1 月至2013年1月收治的10例中枢神经系统原发性黑色素瘤患者的临床表现、影像学特点、病理结果、手术治疗与预后情况。结果 10例黑色素瘤患者中发生于颅内者8例， 椎管内者1例，颅颈交界区1例。手术全切除8例，大部切除2例。病理检查黑色素瘤8例， 脑脊膜黑色素细胞瘤2例。随访3月至11年，6例死亡，其中1例术后综合治疗后存活了7年，后因并发症死亡，术后中位生存期1.1年。4例术后放疗，恢复正常生活。结论 中枢神经系统原发性黑色素瘤发病率低，但术前诊断困难，误诊率较高。手术切除为首选，术后结合放、化疗，可延长生存时间，但总的预后差。

Abstract:

Objective To investigate the diagnosis and treatment and follow-up results of primary melanoma of the central nervous system (CNS). Methods A retrospective analysis was performed on the clinical manifestations, radiological features, pathological findings, surgical treatment, and prognosis of 10 patients with primary melanoma of the CNS who were hospitalized from January 2002 to January 2013. Results Of the 10 cases, 8 had intracranial melanoma, 1 had intraspinal melanoma, and 1 had melanoma at the craniocervical junction. Total resection was achieved in 8 cases, and subtotal resection in 2 cases. The pathological examination showed that melanoma was found in 8 cases, and meningeal melanocytoma in 2 cases. During follow-up (3 months to 11 years), 6 cases died, and one of them survived for 7 years after operation with comprehensive treatment and died of complications. The median survival time was 1.1 years. 4 cases showed a significant recovery after surgery and a full course of radiotherapy. Conclusions Primary melanoma of the CNS is an extremely rare tumor and radiological presentation and preoperative diagnosis could be difficult and confusing, leading to a high misdiagnosis rate. The optimal treatment for this disease is surgical resection followed by radiotherapy or chemotherapy for patients whose tumors are not totally removed. Comprehensive treatment for primary melanoma may prolong the survival period. However, the ultimate prognosis remains poor.