Intraventricular presentation of Rosai–Dorfman disease: a case report with review of literature

Abstract Background Rosai–Dorfman disease (RDD)/sinus lymphohistiocytosis is a rare benign lymphoproliferative disorder. Only 25% show extra-nodal manifestation, only 5% are seen involving the CNS, with intraventricular manifestation rarely reported. Our aim was to highlight important imaging features which would be useful in considering this entity as one of the differentials while encountering this rare entity. Case description We present a case of a 34-year-old female with complaints of headache, altered behavior and visual disturbances. MRI brain showed T2 hypointense lesion arising from the left choroid plexus with dense homogenous enhancement, with multiple additional extra-axial dural-based lesions and a small lesion involving right choroid plexus. Left parietal craniotomy was done, and the lesion was excised. Histopathology showed large foamy macrophages in eosinophilic background, with lymphophagocytosis (emperipolesis), confirming the diagnosis of Rosai–Dorfman disease. Conclusions Intraventricular Rosai–Dorfman disease is a rare entity. Imaging features of T2 hypointense homogenously enhancing lesion with blooming on GRE, without features of calcification or hemorrhage, may be helpful in prompting adequate histopathologic evaluation.