Papillary tumor of the pineal region

Tumors of the pineal region are rare in adulthood, accounting for approximately 1% of intracranial neoplasms in this age range. Papillary tumor of the pineal region (PTPR) was first described by Jouvet et al. in 2003. In 2007, PTPR was included by the World Health Organization as a distinct entity in their new classification of central nervous system tumors. We report a case of PTPR, and undertake a literature review of this rare neuroepithelial neoplasm. Patients with PTPR need long-term follow up, and new cases should be well documented so that we can gain a better understanding of this neoplasm.