Extranodal Rosai-Dorfman Disease involving the meninges in a 79-year-old man

Rosai-Dorfman disease was first described in 1969 as an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Cardinal features include massive painless cervical adenopathy, fever and polyclonal hypergammaglobulinemia 1,2 Extranodal involvement occurs in 25% to 43% of cases affecting the skin (12%), paranasal sinuses (11%), soft tissue (9%) bone (9%), salivary gland (5%), central nervous system (5%), oral cavity (3%), kidney (2%), lower respiratory tract (2%), larynx (1%), and rarely other locations such as the orbit.2-6 More than 650 cases of Rosai-Dorfman disease have been reported since 1969.7 To our knowledge, there have been 54 cases with central nervous system involvement reported in the literature.6-15 Rosai-Dorfman disease occurs most frequently in the first two decades of life.16 The age of patients with Rosai-Dorfman disease of the CNS ranges between 2 to 78 years and occurs predominantly in males.14