title: Isolated Intramedullary Spinal Rosai-Dorfman Disease: A Case Report and Literature Review.
abstract: BACKGROUND
  Rosai-Dorfman disease (RDD) is a rare histioproliferative disorder that only occasionally involves the central nervous system.
  
  
  METHODS
  We present the diagnosis and treatment of an exceedingly rare case of isolated intramedullary spinal RDD that has been reported only 3 times previously. Moreover, it is the first time that intramedullary spinal RDD has been described in a child. The patient was treated by total surgical resection and experienced no recurrence during the 12-month follow-up. Histopathologic examination showed a characteristic emperipolesis; the lymphocytes were engulfed in the S-100-protein-positive histiocytes with negative expression of CD1a.
  
  
  CONCLUSIONS
  Preoperative diagnosis of spinal RDD is still challenging because the lesion usually is a dura-based lesion that mimics a meningioma. Surgical resection is an effective treatment and radiotherapy; and steroid and chemotherapy have not demonstrated reliable therapeutic efficiency.
authors.affiliation: Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing, China.
authors.forename: Bo Yuan
authors.initials: BY
authors.lastname: Huang
authors.affiliation: Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing, China.
authors.forename: Hai Long
authors.initials: HL
authors.lastname: Liu
authors.affiliation: Department of Neurosurgery, San Bo Brain Hospital, Capital Medical University, Beijing, China. Electronic address: 13436561446@163.com.
authors.forename: Chun Jiang
authors.initials: CJ
authors.lastname: Yu
mesh_terms: D002648:Child; D005260:Female; D015618:Histiocytosis, Sinus / Q000473:pathology* / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D013122:Spinal Diseases / Q000473:pathology* / Q000601:surgery*; D016896:Treatment Outcome
keywords: Intramedullary; Spinal Rosai-Dorfman disease