An Unusual Finding: Papillary Tumor of the Pineal Region

Abstract

A papillary tumor of the pineal region (PTPR) is a rare tumor of neuroepithelial origin formed from specialized ependymocytes of the subcommissural organ located in the lining of the posterior commissure, not the pineal gland itself. Patients with this type of tumor generally present with nonspecific symptoms secondary to obstructive hydrocephalus such as headache and vision changes. The mean age of patient presentation is 31, with a slight predominance in females. This type of tumor has a high rate of recurrence (56%) following surgical resection. This case study describes the presentation of this uncommon tumor in a 61-year-old woman, including presentation, imaging, surgery, and pathology findings.

Papillary Tumor of the Pineal Gland: Series of Four Clinical Cases

“…PTPRs are composed of cells that are typically large, columnar, and frequently exhibit prominent nuclei. These cells form papillary structures, rosettes, and pseudo-rosettes, often enveloping vessels with a layer of these cells [ 6 , 7 ]. Elevated mitotic and proliferative activity is correlated with a greater risk of recurrence and poorer outcomes in patients with PTPR [ 3 ].…”

“…Immunohistochemically, positive staining is commonly observed for cytokeratin, Nestin, S-100 protein, neuron-specific enolase (NSE), and vimentin in PTPR. However, staining for epithelial membrane antigen (EMA) and glial fibrillary acidic protein (GFAP) is typically weak or negative [ 6 , 7 , 16 ]. The Ki-67 labeling index was found to be relatively low in all cases.…”

“…The first description of PTPR was conducted by Jouvet et al in 2003 [ 6 ]. Since then, approximately more than 100 cases of PTPR have been reported to date [ 7 , 8 ]. The fourth edition of the World Health Organization's (WHO) classification of central nervous system tumors, published in 2007, introduced several new entities, including the papillary tumor of the pineal region.…”