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Rosai-Dorfman disease of the central nervous system

P Purav1, K Ganapathy, V S Mallikarjuna

  • 1Department of Neurosurgery, Apollo Specialty Hospital, Chennai, India.

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
|August 16, 2005
PubMed

Insights

Rosai-Dorfman disease (RDD) is a rare non-cancerous condition. Central nervous system RDD can mimic meningioma, requiring histological and immunohistochemical diagnosis for effective management.

Area of Science:

  • Neuropathology
  • Oncology

Background:

  • Rosai-Dorfman disease (RDD) is an idiopathic, non-neoplastic lymphoproliferative disorder.
  • Central nervous system (CNS) involvement by RDD can present as a meningioma-like lesion.

Observation:

  • This study reports ten cases of RDD exclusively affecting the CNS.
  • Nine cases involved the cranial cavity, with one also affecting the cervical extradural region.

Findings:

  • Histological and immunohistochemical analysis confirmed a mixed cell population with emperipolesis and S100 protein positivity in all CNS RDD cases.
  • Surgical excision or biopsy was the primary treatment modality.
  • No recurrences were observed during follow-up periods ranging from three months to eight years.

Implications:

  • Accurate diagnosis of CNS RDD is crucial, distinguishing it from neoplastic lesions like meningioma.
  • The findings support the efficacy of surgical management and highlight the benign, non-recurrent nature of CNS RDD.
  • Further research into the pathogenesis and optimal management strategies for CNS RDD is warranted.

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