摘要
目的探讨中枢神经系统黑色素细胞肿瘤的临床和病理学特征。方法回顾性分析2018年6月至2021年11月首都医科大学附属北京地坛医院神经外科收治的6例中枢神经系统黑色素细胞肿瘤患者的临床资料。收集患者的一般资料、既往病史、临床表现、影像学资料及术后病理学结果等。采用门诊、电话及影像学随访,随访内容包括术后辅助治疗情况、症状改善情况及有无肿瘤复发、转移等。结果临床表现为头痛4例、下肢疼痛及麻木1例、面部疼痛1例、记忆力下降1例。影像学检查显示6例均为单发肿瘤,肿瘤位于L2椎体水平椎管内1例,颅内5例。肿瘤达全切除4例,次全切除1例,部分切除1例。病理学诊断为脑膜和脊膜黑色素细胞瘤各1例(影像学分型为黑色素型),原发性黑色素瘤2例(影像学分型为非黑色素型),转移性黑色素瘤2例(影像学分型为血肿型)。HE染色结果显示,6例肿瘤组织均可见不同含量的黑色素颗粒。免疫组织化学染色结果显示,抗黑色素瘤特异性单抗(HMB-45)(+)6例,S-100(+)4例,黑色素细胞抗原(Melan-A)(+)6例,波形蛋白(VIM)(+)占比为3/3,上皮膜抗原(-)占比为3/3。2例黑色素细胞瘤患者的Ki-67增殖指数≤30%,另4例黑色素瘤患者的Ki-67增殖指数为40%~80%。6例患者的随访时间为2.9~44.2个月。术后未接受辅助治疗2例,接受放化疗2例,单纯化疗1例,免疫治疗1例。至末次随访,复发2例,转移2例,有2例黑色素瘤患者死亡(术后分别生存2.9、21.4个月)。结论初步观察发现,影像学分型为黑色素型的肿瘤更倾向于良性黑色素细胞瘤。手术切除联合术后辅助治疗可相对改善预后,但黑色素瘤总体预后不佳。
Objective To investigate the clinical and pathological features of central nervous system(CNS)melanocytic tumors.Methods A retrospective analysis was conducted on the clinical data of 6 patients with CNS melanocytic tumors admitted to the Department of Neurosurgery of Beijing Ditan Hospital,Capital Medical University from June 2018 to November 2021.The general information,past medical history,clinical manifestations,imaging data and postoperative pathological results of the patients were collected.Outpatient,telephone and imaging were used for follow-up,and the content of follow-up included postoperative adjuvant treatment,improvement of symptoms,tumor recurrence and metastasis,etc.Results The clinical manifestations were headache in 4 cases,lower limbs pain and numbness in 1 case,facial pain in 1 case and memory decline in 1 case.Imaging examination showed that all 6 cases had single tumors,including 1 case in the spinal canal at L2 vertebral level and 5 cases located at the brain.Total resection was performed in 4 cases,subtotal resection in 1 case,and partial resection in 1 case.Based on the medical history and pathological examination results,the patients were diagnosed as meningeal melanoma cell tumor in 2 cases(melanotic pattern),primary melanoma in 2 cases(amelanotic pattern)and metastatic melanoma in 2 cases(hematoma pattern).HE staining results showed that different amounts of melanin particles were observed in the tumor tissues of all 6 cases.Immunohistochemical staining showed HMB-45(+)in 6 cases,S-100(+)in 4 cases,Melan-A(+)in 6 cases,VIM(+)in 3/3 and EMA(-)in 3/3 cases.The Ki-67 proliferation index was≤30%in the 2 melanoma cell tumors and 40%-80%in the other 4 melanoma tissues.Follow-up time ranged from 2.9 to 44.2 months.Postoperative adjuvant therapy was not received in 2 cases,radiotherapy and chemotherapy were received in 2 cases,chemotherapy alone in 1 case,and immunotherapy in 1 case.At the last follow-up,there were 2 cases of relapse,2 cases of metastasis and 2 cases of death(diagnosed as melanoma,survival time was 2.9 and 21.4 months,respectively).Conclusions Preliminary findings have shown that the melanotic imaging pattern is more likely to be benign melanoma.Surgical excision combined with postoperative adjuvant therapy can improve the prognosis,while the overall prognosis seems poor.
作者
丁兴欢
梁博
李晶晶
陈佳敏
韩晓艺
王芳
冯恩山
Ding Xinghuan;Liang Bo;Li Jingjing;Chen Jiamin;Han Xiaoyi;Wang Fang;Feng Enshan(Department of Neurosurgery,Beijing Ditan Hospital,Capital Medical University,Beijing 100015,China;Department of Radiology,Beijing Ditan Hospital,Capital Medical University,Beijing 100015,China;Department of Pathology,Beijing Ditan Hospital,Capital Medical University,Beijing 100015,China)
出处
《中华神经外科杂志》
CSCD
北大核心
2023年第4期374-378,共5页
Chinese Journal of Neurosurgery
基金
首都医科大学附属北京地坛医院育苗计划(DTYM-202106)。
关键词
黑色素瘤
中枢神经系统肿瘤
疾病特征
病理学
Melanoma
Central nervous system neoplasms
Disease attributes
Pathology
