中枢神经系统黑色素细胞肿瘤的临床分析  

Clinical analysis of melanocytic tumors of the central nervous system l

作　　者：甄英伟[1] 吴震[2] 南阳[1] 郭红宝 王永志[2] 付胜伟[3] 黄强[1] 俞凯[1] 岳树源[1] 钟跃[1] 

机构地区：[1]天津医科大学总医院神经外科,300052 [2]首都医科大学附属北京天坛医院神经外科 [3]天津医科大学总医院滨海医院神经外科

出　　处：《中华神经外科杂志》2015年第10期1030-1033,共4页Chinese Journal of Neurosurgery

基　　金：天津市应用基础与前沿技术研究计划（13JCQNJc10800、13JCZDJC31000）

摘　　要：目的探讨中枢神经系统（CNS）黑色素细胞肿瘤的临床特征、影像学特点、治疗措施及其预后。方法回顾性分析14例经手术及病理证实为CNS黑色素细胞肿瘤患者的临床资料，并结合文献进行分析。结果CNS黑色素细胞肿瘤临床表现多样，以头痛、颅内压增高、癫痫、局灶性神经功能缺失为主要症状。其影像学表现多样，13例行MRI检查的患者中，呈短T1、短T2信号7例，等T1、稍长T2信号3例，混杂信号3例。免疫组化检查结果显示，14例HMB-45（＋），13例S-100（＋），8例波形蛋白（＋）。14例中，初次手术全切除7例，近全切除5例，部分切除2例。术后4例因复发再次行手术治疗。术后随访时间2—60个月。11例获得随访，死亡9例，存活2例，有7例行辅助治疗；死亡患者中位生存期为14个月。结论CNS黑色素细胞肿瘤临床表现无特异性，MRI上以短T1、短T2信号为其相对特征性的影像学表现。免疫组化结果有助于确诊。由于肿瘤恶性程度高，总体预后较差。手术应作为首选的治疗措施，术后可采取放、化疗等辅助治疗措施。Objective To investigate the clinical features,imaging characteristics, treatment measures, and prognosis of melanocytic tumors of the central nervous system （ CNS）. Methods The clinical data of 14 patients with melanocyfic tumors of CNS confirmed by surgery and pathology were analyzed retrospectively, and the disease was analyzed according to the literature. Results The clinical manifestations of melanoeytic tumors of CNS were diverse ; the main symptoms were headache, increased intracranial pressure, seizures, and focal neurological deficits. The imaging features were diversified, with 7 of short T1 and short T2 signal, 3 of identical T1 and long T2 signal, and 3 of heterogenous signal among the 13 patients who had the MRI examination. Immunohistochemistry showed HMB45 （＋） in 14 cases, S - 100 （ ＋ ） in 13 cases, and VIM （ ＋ ） in 8 cases. In the initial operations,the total, subtotal and partial removals of the tumors were achieved in 7, 5, and 2 cases, respectively. Four patients were operated again for recurrence of tumors after procedure. The postoperative follow- up time was 2 to 60 months. Eleven patients were followed up, and 7 of them received adjuvant therapy. Nine cases died and 2 cases survived; the median survival time of the died cases was 14 months. Conclusions The clinical manifestation of melanoeytie tumors of CNS do not have any specificity, and short T1 and short T2 signal on the MRI is the relatively characteristic imaging display. The immunohistochemistry test is help to get the definite diagnosis. The overall malignant degree is high and the prognosis is poor. Operation should be the preferred therapeutic measure. Postoperative adjuvant treatment measures can be taken, such as radiotherapy and chemotherapy.

关 键 词：中枢神经系统 黑色素瘤 神经外科手术 放射疗法 化学疗法 

分 类 号：R739.4[医药卫生—肿瘤]