Background: The authors report the third case of a rare papillary glioneuronal tumor of the pineal gland and only the second case reported with anaplastic features in this particular location. The authors also review the literature of papillary glioneuronal tumors.
Case Report: Our patient is a 58-year-old Caucasian male who presented with diffuse headaches and loss of short-term memory. There were no deficits on physical exam. MRI Brain was performed demonstrating a large heterogeneously enhancing mass within the pineal gland causing obstructive hydrocephalus. He was taken to the operating room for supracerebellar-infratentorial approach for biopsy of the mass using neuronavigation. He required an endoscopic third ventriculostomy post-operatively for worsening hydrocephalus. Pathology demonstrated a rare malignant papillary glioneuronal tumor. It was recommended that patient undergo chemotherapy and radiation, however he refused treatment. He died six months after his initial diagnosis due to a condition unrelated to his intracranial tumor.
Conclusions: This is an unusually rare tumor of the pineal gland, with only one other malignant case noted in this location. We review the literature of this rare entity that should be considered on the differential diagnosis of a pineal gland mass.

Keywords: Pinealoma, Synaptophysin, immunostaining, Ganglioneuroblastoma