Papillary Tumor of the Pineal Region

Abstract

Tumors of the central nervous system (CNS) demonstrating papillary features, and particularly in the pineal region, are rare. Papillary tumor of the pineal region (PTPR) is a recently described rare neuroepithelial tumour included in the fourth edition of the World Health Organization (WHO) classification of tumours of the CNS, published in 2007. This new histopathological entity manifests in children and adults, with magnetic resonance (MR) imaging showing variable degree of T1 hyperintensity signal as well as contrast enhancement. In addition to the initial report of six cases by Jouvet et al. (Am J Surg Pathol 27:505–512, 2003) several case reports have been published in the literature to date. PTPR is characterized by presenting an epithelial-like cytology and a papillary architecture with immunoreactivity for broad-spectrum cytokeratins. Ultrastructurally observed features suggest ependymal differentiation and a possible origin from specialized ependymal cells of the subcommissural organ (SCO) has been postulated. The differential diagnosis of PTPR include pineal parenchymal tumors, choroid plexus tumors, papillary ependymoma, papillary meningioma, and metastasis. As a result of the rarity of these tumors, the biological behaviour of PTPR is not yet clear. It may correspond to WHO grades II or III. The high risk of local recurrence makes accurate surgery followed by radiotherapy necessary to achieve optimal treatment.