Catalucci A, Lanni G, Ventura L, Ricci A, Galzio R J, Gallucci M
Department of Neuroradiology, S. Salvatore Hospital, University of L'Aquila, Italy -
Neuroradiol J. 2012 Nov;25(5):569-74. doi: 10.1177/197140091202500510. Epub 2012 Nov 9.
Rosai-Dorfman disease (RDD) was firstly described in 1969 as a benign proliferative disorder of histiocytes with systemic symptoms and lymphadenopathy. This disease is of uncertain pathogenesis and mostly occurs in children and young adults. The typical clinical features of RDD include bilateral painless cervical lymphadenopathy, but extranodal involvement may also be present. The most common extranodal sites include organs such as the respiratory tract, skin, nasal cavity, orbit and bone. Isolated central nervous system (CNS) manifestations are extremely rare. In case of CNS involvement, the commonest imaging findings are dural-based extra-axial enhancing masses. We describe a case of intracranial RDD mimicking multiple meningiomas both clinically and radiologically in a 57-year-old man presenting with a six-year history of progressive right visual and hearing loss and tinnitus. In cases of multiple extra-axial lesions it is worth bearing in mind the possible differential diagnosis for intracranial RDD and eventually propose to the patient further investigations.
罗萨伊-多夫曼病（RDD）于1969年首次被描述为一种具有全身症状和淋巴结病的组织细胞良性增生性疾病。该病的发病机制尚不清楚，主要发生于儿童和年轻人。RDD的典型临床特征包括双侧无痛性颈部淋巴结病，但也可能出现结外受累。最常见的结外部位包括呼吸道、皮肤、鼻腔、眼眶和骨骼等器官。孤立的中枢神经系统（CNS）表现极为罕见。若CNS受累，最常见的影像学表现是硬脑膜外轴外强化肿块。我们描述了一例57岁男性颅内RDD病例，该病例在临床和放射学上均酷似多发脑膜瘤，患者有6年进行性右视力和听力丧失及耳鸣病史。对于多发轴外病变病例，值得牢记颅内RDD的可能鉴别诊断，并最终建议患者进行进一步检查。
