title: Rosai-Dorfman disease involving the central nervous system: seven cases from one institute.
abstract: BACKGROUND
  Rosai-Dorfman disease (RDD) is a rare, idiopathic, non-neoplastic histioproliferative disease. Central nervous system (CNS) manifestations are extremely rare. Its low incidence and unknown etiology restrict early diagnosis and optimal therapy.
  
  
  METHODS
  In the 1995-2013 period, seven CNS-RDD patients with intracranial and/or spinal lesions were retrospectively analyzed, including the clinical data, laboratory and imaging results, treatment applied and outcome. All seven case samples were screened for the SLC29A3 gene mutation, and the literature was reviewed.
  
  
  RESULTS
  Seven RDD patients (6 male/1 female, aged between 7 and 68) with CNS manifestations are reported. Five of the seven patients (71.4 %) had intracranial lesions (1 with skull erosion and 1 with multiple lesions mimicking meningiomas), and two (28.6 %) had spinal subdural lesions. The patients' symptoms included headaches, seizures, visual loss, epileptoid convulsions in the lower legs, fever, spastic paraparesis and paraplegia. An elevated erythrocyte sedimentation rate (ESR) was detected in five of the seven cases. The diagnosis was confirmed by immunohistochemical staining revealing that the characteristic histiocytes were positive for the S100 protein and CD68 and negative for CD1a. All patients were operated on: three recovered completely, two were partially rehabilitated, and two died. No SLC29A3 gene mutations were found in any of the seven samples.
  
  
  CONCLUSIONS
  This short series suggests the following: (1) RDD should be included in the differential diagnosis of lesions mimicking intracranial/spinal meningiomas or inflammatory lesions, especially in children; (2) the definitive diagnosis is based on histopathology and immunocytochemistry; (3) surgical resection seems to be the most effective therapy; (4) the exact etiology and adjuvant therapy for relapsing/incompletely resected lesions remain to be established.
authors.affiliation: Department of Neurosurgery, Beijing TianTanHospital, Capital Medical University, Beijing, People's Republic of China.
authors.forename: Yongji
authors.initials: Y
authors.lastname: Tian
authors.forename: Junmei
authors.initials: J
authors.lastname: Wang
authors.forename: Mingtao
authors.initials: M
authors.lastname: Li
authors.forename: Song
authors.initials: S
authors.lastname: Lin
authors.forename: Guihuai
authors.initials: G
authors.lastname: Wang
authors.forename: Zhen
authors.initials: Z
authors.lastname: Wu
authors.forename: Ming
authors.initials: M
authors.lastname: Ge
authors.forename: Benoit J M
authors.initials: BJ
authors.lastname: Pirotte
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002490:Central Nervous System / Q000473:pathology*; D002648:Child; D005260:Female; D015618:Histiocytosis, Sinus / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D008297:Male; D008577:Meningeal Neoplasms / Q000175:diagnosis*; D008579:Meningioma / Q000175:diagnosis*; D008875:Middle Aged; D033703:Nucleoside Transport Proteins / Q000235:genetics
chemical_list: D033703:Nucleoside Transport Proteins; C501424:SLC29A3 protein, human