脑膜黑色素瘤病五例临床、脑脊液及影像学特征  

Clinical,cerebrospinal fluid and imaging characteristics of diffuse meningeal melanomatosis:a report of 5 cases

作　　者：武力勇[1] 王向波[1] 贾建平[1] 李存江[1] 王锁彬[1] 赵利杰[1] 

机构地区：[1]首都医科大学北京宣武医院神经内科,100053

出　　处：《中华神经科杂志》2007年第12期818-821,共4页Chinese Journal of Neurology

摘　　要：目的探讨脑膜黑色素瘤病的临床、脑脊液以及影像学特征。方法回顾性分析5例经病理诊断的脑膜黑色素瘤病患者的临床表现、影像学特征及腰穿检查结果。结果 5例患者临床上以颅内压增高、脑膜刺激征、脑神经损害以及脊神经根性疼痛为主要表现。1例有左眼视乳头上方视网膜隆起伴色素沉着,4例皮肤有先天性色痣,其中2例为先天性巨大色痣。5例腰穿压力均≥180 mm H_2O(1 mm H_2O=0.0098 kPa),3例为血性或淡黄色脑脊液,4例蛋白增高,3例葡萄糖降低。2例头颅 MRI 增强检查显示软脑膜异常强化。病理学检查:3例腰穿可见恶性黑色素瘤细胞;1例腋窝淋巴结活体组织检查可见恶性黑色素细胞浸润;1例尸体解剖可见脑底部软脑膜和蛛网膜下腔呈弥散性黑色素瘤浸润。2例诊断为转移性脑膜黑色素瘤病,3例可能为原发性脑膜黑色素瘤病。结论脑膜黑色素瘤病临床上表现为脑脊膜、神经根受累,常合并存在皮肤黑色素痣,腰穿检查常提示蛛网膜下腔出血,腰穿检查脑脊液发现黑色素瘤细胞能够确定诊断。Objective To investigate the clinical, cerebrospinal fluid and imaging characteristics of 5 cases of diffuse meningeal melanomatosis. Methods The clinical manifestation, features of cerebrospinal fluid and image of 5 patients with meningeal melanomatosis diagnosed by biopsy or autopsy were retrospectively summarized. Results Clinical manifestations of these 5 cases included intracranial hyperpressure, meningeal irritation sign, intracrarial nerves impairment, root pain of spinal nerve. In all of these 5 cases, retina hyperpigmentation above left discus opticus was found by funduscope in one case, and congenital melanocytic nevi were found in 4 patients, in which 2 cases were giant congenital melanocytic nevi. Increased lumbar puncture cerebrospinal fluid （CSF） pressure occurred in all cases. Subarachnoid hemorrhage was found in 3 cases. Analysis of CSF revealed increased protein in 4 cases and decreased glucose in 3 cases. Cranial MRI obtained after the intravenous administration of Gd-DTPA showed leptomeningeal enhancement. Malignant melanoma cells were found in CSF of 3 cases. Metastatic malignant melanoma cells were found by biopsy of axillary fossa lymph node in one case. Autopsy of one case revealed diffuse black pigmentation of the leptomeninges, especially in base of skull. Two cases were diagnosed as metastatic meningeal melanomatosis and 3 cases were possible primary meningeal melanomatosis. Conclusions Menings, root of cranial nerve and spinal nerve are impaired in meningeal melanomatosis, which is usually accompanied by congenital melanocytic nevi. Subarachnoid hemorrhage implies meningeal melanomatosis. Diagnosis can be identified when malignant melanoma cells are found in CSF.

关 键 词：脑膜肿瘤 黑色素瘤 痣 色素 脑脊髓液 磁共振成像 

分 类 号：R739.4[医药卫生—肿瘤]