Isolated intracranial Rosai–Dorfman disease presenting as compressive optic neuropathy

Abstract

A 56-year-old female presented with bilateral progressive painless loss of vision. Examination showed a relative afferent pupillary defect in the left eye with temporal disc pallor and visual field loss. MRI (magnetic resonance imaging) brain revealed a suprasellar mass for which mass excision via frontotemporal craniotomy was done. Histopathology examination of the mass revealed foamy histiocytes with emperipolesis, S-100 marker was positive. Thus, a diagnosis of Rosai–Dorfman disease (RDD) was made. Our case describes a rarely found isolated intracranial RDD presenting as compressive optic neuropathy and vision loss in an elderly female.