Department of Neurology and Neurological Sciences, Stanford Medicine, Palo Alto, CA, USA.
Department of Pathology, Stanford Medicine, Palo Alto, CA, USA.
Mult Scler. 2022 Sep;28(10):1651-1654. doi: 10.1177/13524585221097564.
Primary central nervous system (CNS) histiocytic sarcoma is a rare hematolymphoid malignancy with features of mature histiocytes and carries a poor prognosis. We describe a unique case in which a 50-year-old woman presented with recurrent acute brainstem syndrome, area postrema syndrome, and myelitis with corresponding magnetic resonance imaging (MRI) lesions meeting diagnostic criteria for seronegative neuromyelitis optica spectrum disorder (NMOSD). Despite initial improvement with steroids and plasma exchange, she experienced recurrent symptoms over 10 months referable to new and persistently enhancing lesions. At autopsy, neuropathology revealed a diffusely infiltrative primary CNS histiocytic sarcoma. This case represents a rare clinicoradiologic mimic of NMOSD, underscoring the importance of evaluation for infiltrative diseases in cases of atypical seronegative NMOSD.
原发性中枢神经系统（CNS）组织细胞肉瘤是一种罕见的血液淋巴恶性肿瘤，具有成熟组织细胞的特征，预后不良。我们描述了一个独特的病例，一名 50 岁女性出现复发性急性脑干综合征、孤束核后区综合征和脊髓炎，相应的磁共振成像（MRI）病变符合血清阴性视神经脊髓炎谱系疾病（NMOSD）的诊断标准。尽管最初使用类固醇和血浆置换有所改善，但她在 10 个月内出现了与新的和持续增强病变相关的复发性症状。尸检时，神经病理学显示弥漫浸润性原发性 CNS 组织细胞肉瘤。该病例代表了 NMOSD 的一种罕见临床影像学模拟，强调了在不典型血清阴性 NMOSD 病例中评估浸润性疾病的重要性。
