Department of Neurological Surgery, Houston Methodist Hospital, 6560 Fannin Street, Houston, TX, USA.
Neurol India. 2020 Jan-Feb;68(1):35-41. doi: 10.4103/0028-3886.279688.
Tumefactive demyelination is a phenomenon involving the radiographic resemblance of an acute demyelinating process in the central nervous system to neoplasia. Although this has been described and characterized for multiple sclerosis, it has been reported in a few cases in patients with acute disseminated encephalomyelitis (ADEM) within the past decade. While it may be challenging to establish a diagnosis of tumefactive ADEM according to clinical and radiological data alone, a thorough review of the clinical history and following the patient over time can be supportive of the same. The principal diagnostic confounds include neoplastic disease and a first attack of multiple sclerosis. A definitive diagnosis can be made by biopsy, which reveals perivenular demyelination and mononuclear cell infiltration in ADEM, in contrast to confluent plaque-like areas of demyelination in patients with multiple sclerosis. Histopathologic evidence of neoplastic disease includes characteristic features, including nuclear atypia and polymorphism, cellular hyperproliferation, mitoses, necrosis, endothelial proliferation, rosettes, and/or pseudorosettes. ADEM responds excellently to treatment with corticosteroids and is monophasic, with recurrence occurring infrequently. We review the literature on tumefactive ADEM and discuss the clinical manifestations, imaging characteristics, and histopathologic findings used to distinguish it from other conditions.
肿块样脱髓鞘是一种现象，涉及中枢神经系统急性脱髓鞘过程的影像学类似于肿瘤。虽然这种现象已经在多发性硬化症中得到了描述和特征化，但在过去十年中，在少数急性播散性脑脊髓炎 (ADEM) 患者中也有报道。虽然仅根据临床和影像学数据可能难以确定肿块样 ADEM 的诊断，但对临床病史的全面回顾和随时间推移对患者的随访可以支持相同的诊断。主要的诊断混淆因素包括肿瘤性疾病和多发性硬化症的首次发作。通过活检可以做出明确的诊断，与多发性硬化症患者融合斑块样脱髓鞘区域相比，活检显示 ADEM 存在围绕小静脉的脱髓鞘和单核细胞浸润。肿瘤性疾病的组织病理学证据包括特征性特征，包括核异型性和多形性、细胞过度增殖、有丝分裂、坏死、内皮细胞增殖、玫瑰花结和/或假玫瑰花结。皮质类固醇治疗 ADEM 效果极好，呈单相病程，复发罕见。我们回顾了有关肿块样 ADEM 的文献，并讨论了用于将其与其他疾病区分开来的临床表现、影像学特征和组织病理学发现。
