Diagnosis and management of rosai-dorfman disease involving the central nervous system

Amos O. Adeleye, Gail Amir, Shifra Fraifeld, Yigal Shoshan, Felix Umansky, Sergey Spektor

Research output: Contribution to journalReview articlepeer-review

73 Scopus citations

Abstract

Introduction: Rosai-Dorfman disease is a benign non-neoplastic proliferative disorder of histiocytes originally described in the cervical lymph nodes. Extranodal sites were later recognized, and by 1990, they were shown to represent over 40% of cases; however, central nervous system involvement is still considered rare. We review the literature, which shows a steady increase in reports of Rosai-Dorfman disease involving the brain and/or spine. Methods: A literature search was performed for the period 1969-2008, using multiple search engines, with keywords Rosai-Dorfman disease, central nervous system Rosai-Dorfman disease and sinus histiocytosis with massive lymphadenopathy. Results: By December 2008, 111 cases of Rosai-Dorfman disease involving the central nervous system had been reported in the literature, including our cases. In the central nervous system, Rosai-Dorfman disease is ubiquitous. Although it is characterized by unique, indeed pathognomonic, histopathological cytoarchitecture, it may be mistaken for many other neoplastic and inflammatory histioproliferative diseases. Surgical resection with post-operative corticosteroids remains the treatment of choice. Conclusions: Rosai-Dorfman disease involving the central nervous system appears to be more common than previously thought and may well assume a more prominent place in the differential diagnoses of dural-based lesions. Expert awareness of the characteristic histopathology and immunohistochemistry of the disease is required for accurate diagnosis.

Original languageEnglish
Pages (from-to)572-578
Number of pages7
JournalNeurological Research
Volume32
Issue number6
DOIs
StatePublished - 1 Jul 2010
Externally publishedYes

Keywords

  • CENTRAL NERVOUS SYSTEM
  • HISTIOCYTOSIS
  • LYMPHADENOPATHY
  • RISING INCIDENCE
  • ROSAI-DORFMAN DISEASE
  • SINUS HISTIOCYTOSIS WITH MASSIVE LYMPHADENOPATHY

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