Diagnosis and management of Rosai–Dorfman disease involving the central nervous system

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.