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Case of the Week

Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada

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January 26, 2009

Rosai-Dorfman Disease

  • Aka: sinus histiocytosis with massive adenopathy (due to infiltrates of histiocytes and lymphoplasmacytic cells, CD1a-, a marker for histiocytosis X-, is absent).
  • Young men and children.
  • Most frequent intracranial locations: convexities, parasagittal, suprasellar, cavernous sinuses, and clivus (lesions may simulate meningioma, sarcoid, lymphoma, or metastases to dura)
  • Surgical resection is the best treatment.

Suggested Reading

Konishi E, Ibayashi N, Yamamoto S, et al.Isolated Intracranial Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy). AJNR Am J Neuroradiol 2003;24:515-18.

Wang E, Anzai Y, Paulino A, et al. Rosai-Dorfman Disease Presenting with Isolated Bilateral Orbital Masses: Report of Two Cases. AJNR Am J Neuroradiol 2001;22:1386-88.

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