Cerebral Rosai–Dorfman disease

Background Sinus histiocytosis (Rosai–Dorfman disease) with massive lymphadenopathy is a rare nonneoplastic and nonlangerhans cell proliferation disorder of the histiocytes. Extranodal location with or without lymphadenopathy occurs in about 40 % of the cases. Intracranial location is rare in children often mimicking meningiomas. The parasphenoidal region is more frequently involved though intraxial or intraventricular locations were described as well. Rarely, the surgical treatment allows the complete excision of the lesion; however, in symptomatic cases, partial resections of the tumor allow to counteract its mass effect. Long survivals are possible, even without radiotherapy or chemotherapy, due to the frequent spontaneous benign evolution of the lesions.