Papillary tumor of the pineal region presenting with Foster Kennedy sign

Foster Kennedy syndrome is defined as unilateral optic nerve atrophy, contralateral disc edema and anosmia. It was originally thought to be pathognomonic of space occupying lesions in the anterior fossa causing direct compression of the optic and olfactory nerves and later intracranial hypertension and contralateral disc edema. Subsequent reports of non-tumor and non-anterior fossa causes, most commonly bilateral sequential anterior ischemic optic neuropathy, have been described. We report a 29 year old man who presented with unsteady gait and diminution of vision over 2 months. He displayed findings typical of Parinaud’s syndrome, right disc edema and left optic atrophy. MRI revealed a large pineal tumor associated with hydrocephalus and evidence of increased intracranial pressure. Papillary tumor of the pineal region (PTPR) was diagnosed on histopathology. To our knowledge, this is the first report of this very rare tumor, occurring in association with the Foster Kennedy sign.