Sun Chenjing, Liu Jianguo, Gui Qiuping, Lu Dehong, Qi Xiaokun
Department of Neurology, Navy Clinical College, Second Military Medical University, Beijing 100048, China.
Department of Neurology, Navy General Hospital, Beijing 100048, China. Email:
Zhonghua Yi Xue Za Zhi. 2014 Dec 9;94(45):3557-61.
To summarize the clinical features, neuroimaging findings and pathological characteristics of pathologically confirmed tumefactive demyelinating lesions (TDL).
The clinical features, neuroimaging findings and pathological characteristics were retrospectively collected and analyzed for 58 patients with pathologically confirmed TDLs.For pathological studies, a combination of hematoxylin and eosin staining, myelin staining (Luxol fast blue/periodic acid-Schiff or immunohistochemistry for myelin basic protein), macrophage-specific marker (immunohistochemistry for KiM1P or CD68) and staining for axons (Bielschowski silver impregnation or immunohistochemistry for neurofilament protein) were employed.
The mean age of onset was 6-56 (36 ± 13) years. The onsets were acute (n = 21, 36%), subacute (n = 27, 46.5%) and chronic (n = 10, 17.5%). The diagnoses of TDL were confirmed by repeat biopsy and pathological examinations (n = 2, 3.4%).In acute phase, the plaques of lesions were characterized by massive demyelination with relatively axonal preservation associated with prominent reactive astrocytosis and profound infiltrates of macrophages.In plaques of chronic lesions, demyelinated lesions with relative axonal preservation and sharply defined margins were major findings. And myelin-laden macrophages accumulated at the edges of plaques and stayed relatively inactive with densely gliotic center and processbearing astrocytosis.
TDL is a distinct entity of demyelinating disease.Even though it is often misdiagnosed as neoplasm in brain, bilateral brain involvements and multiple lesions are more common in TDL. The pathological features of TDL are important for the early diagnosis of this disease and helpful for differentiating with brain tumors and central nervous system vasculitis.
总结经病理证实的瘤样脱髓鞘病变（TDL）的临床特征、神经影像学表现及病理特征。
回顾性收集并分析58例经病理证实的TDL患者的临床特征、神经影像学表现及病理特征。病理研究采用苏木精-伊红染色、髓鞘染色（Luxol固蓝/过碘酸-希夫染色或髓鞘碱性蛋白免疫组化）、巨噬细胞特异性标志物（KiM1P或CD68免疫组化）及轴突染色（ Bielschowski银浸染或神经丝蛋白免疫组化）相结合的方法。
发病年龄中位数为6 - 56岁（36±13岁）。起病方式为急性（n = 21，36%）、亚急性（n = 27，46.5%）和慢性（n = 10，17.5%）。TDL的诊断通过重复活检和病理检查得以证实（n = 2，3.4%）。急性期，病变斑块的特征为大量脱髓鞘，轴突相对保留，伴有显著的反应性星形细胞增生和巨噬细胞的大量浸润。慢性病变斑块中，主要表现为轴突相对保留且边界清晰的脱髓鞘病变。富含髓鞘的巨噬细胞聚集在斑块边缘，相对不活跃，中心为致密的胶质增生及有突起的星形细胞增生。
TDL是一种独特的脱髓鞘疾病。尽管在脑部常被误诊为肿瘤，但双侧脑受累及多发病变在TDL中更为常见。TDL的病理特征对该病的早期诊断很重要，有助于与脑肿瘤和中枢神经系统血管炎相鉴别。
