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原发中枢神经系统恶性黑色素瘤的临床特点
引用本文:原发中枢神经系统恶性黑色素瘤的临床特点.原发中枢神经系统恶性黑色素瘤的临床特点[J].首都医学院学报,2023,44(2):335-342.
作者姓名:原发中枢神经系统恶性黑色素瘤的临床特点
作者单位:首都医科大学三博脑科医院神经外科,北京100093
摘    要:目的 分析总结原发中枢神经系统(central nervous system,CNS)恶性黑色素瘤的临床、影像、治疗及预后特点。方法 对首都医科大学三博脑科医院神经外科2011年5月至2021年5月收治并经病理证实的17例原发性CNS恶性黑色素瘤患者的临床资料进行回顾性分析,将其分为4种类型,分析不同类型患者的年龄、影像特点及治疗和预后情况。结果 17例患者中6例为A型(脑表型),5例为B型(脑池型),4例为C型(深部孤立型),2例为D型(椎管及其他型)。表现为颅高压症状者11例、局部神经功能障碍者9例、癫痫者3例。磁共振(magnetic resonance imaging,MRI)平扫出现T1高信号者12例。播散转移13例,其中11例呈线片状。手术切除14例、活检3例,6例术后进行了抗肿瘤治疗。总体中位生存期7个月,综合治疗的患者较单纯手术治疗的患者生存期明显延长[(20.6±7.9)个月vs (3.3±0.9)个月]。各分型间症状、影像及治疗方式均有明显差别。结论 原发CNS恶性黑色素瘤发病率低,多具有较明显的影像特点,临床可分为4种类型。该病需手术联合放射治疗等进行综合治疗,但其恶性程度高,预后差。

关 键 词:中枢神经系统  恶性黑色素瘤  临床分型  诊断  治疗  
收稿时间:2022-06-01

Clinical features of primary central nervous system malignant melanoma
Wang Haoran,Gu Chunyu,Qu Yanming,Liu Ning,Ning Weihai,Ji Junpeng,Zhang Mingshan.Clinical features of primary central nervous system malignant melanoma[J].Journal of Capital University of Medical Sciences,2023,44(2):335-342.
Authors:Wang Haoran  Gu Chunyu  Qu Yanming  Liu Ning  Ning Weihai  Ji Junpeng  Zhang Mingshan
Affiliation:Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, China
Abstract:Objective To summarize the clinical, imaging, therapeutic and prognostic features of primary central nervous system (CNS) malignant melanoma(MM). MethodsThe clinical data of 17 patients with primary CNS MM admitted to the Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, during 2011-2021 were divided into four clinical types for retrospective analysis. Results The male to female ratio was 11∶6, and the mean age was (39.9±16.6) years. Among them, 6 cases were type A, 5 type B, 4 type C, and 2 type D. There were 11 cases of cranial hypertension, 9 cases of local nerve dysfunction and 3 cases of epilepsy. High T1 signal was observed in 12 cases on magnetic resonance imaging(MRI) scan. There were 13 cases had seeding metastasis, 11 of which were linear. The fourteen cases underwent surgical resection, 3 cases underwent biopsy, and 6 cases underwent anti-tumor therapy after surgery. Overall, the median overall survival (mOS) was 7 months, and the survival of patients with combined treatment was significantly longer than that with surgery alone [(20.6±7.9) months vs (3.3±0.9) months, P=0.001]. There were significant differences in symptoms, imaging, and treatment among the 4 clinical types. Conclusion The incidence of primary CNS malignant melanoma is low, and most of them have obvious imaging features, which can be divided into four clinical types. The disease requires surgery combined with radiotherapy and other comprehensive treatment, yet now the prognosis is stile poor.
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