Neurosurgical Department, Wollongong Hospital, University of Wollongong, Wollongong, New South Wales, Australia.
Anatomical Pathology Department, Liverpool Hospital, Liverpool, New South Wales, Australia.
World Neurosurg. 2014 Feb;81(2):442.e7-9. doi: 10.1016/j.wneu.2013.01.002. Epub 2013 Jan 4.
Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare, benign, lymphoproliferative disorder that commonly manifests as a massive painless cervical lymphadenopathy with associated fever and weight loss. Central nervous system involvement is extremely rare, and cervical spinal cord manifestation has not been reported.
A 76-year-old man presented with a 10-week history of right hemiparesis. Magnetic resonance imaging identified an enhancing cervical intramedullary lesion consistent with a primary spinal cord tumor.
Histopathology revealed intramedullary histiocytosis.
This cervical presentation of Rosai-Dorfman disease with central nervous system involvement suggests variation in the clinical manifestations of the disease, necessitating greater surgical awareness.
罗萨达-多夫曼病，又称窦组织细胞增生伴巨大淋巴结病，是一种罕见的良性淋巴组织增生性疾病，通常表现为无痛性颈淋巴结病伴发热和体重减轻。中枢神经系统受累极为罕见，且尚未有颈脊髓表现的报道。
一名 76 岁男性因右侧偏瘫就诊，病史 10 周。磁共振成像（MRI）发现颈髓内有增强病灶，符合原发性脊髓肿瘤。
组织病理学显示髓内组织细胞增生。
本例罗萨达-多夫曼病伴中枢神经系统受累的颈髓表现提示疾病临床表现存在差异，需要更高的手术意识。
