Intracranial Rosai–Dorfman disease: Case report and literature review

Abstract

Rosai–Dorfman disease (RDD) is an idiopathic, non-neoplastic, lympho-histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Intracranial RDD is mainly a disease characterized by dura-based masses and/or diffuse meningeal thickening and included a broad differential diagnosis radiologically. The authors report an unusual case of a 38-year-old male patient presenting with bilateral multiple intracranial dura-based nodules and diffuse meningeal thickening as well as intra-spinal canal involvement. The MR imaging and histo-pathological features of the disease are discussed. Histology with immunohistochemical analysis, in which it demonstrates emperipolesis (lymphophagocytosis) feature and that most of the histiocytes stained with strong positivity for S100 and CD68 proteins, is essential for a definitive diagnosis.