Abstract
BackgroundPapillary tumor of the pineal region is an unusual, recently described entity with potential for misinterpretation as either an ependymoma or choroid plexus papilloma. With few reported cases, there is little data regarding the biologic behavior, natural course and optimal treatment for these patients. Further accumulation of reports will assist with defining this entity and may provide further insight into management of such patients.Case presentationThis patient was a 24-year-old male with new onset seizures and a decreased level of consciousness. MRI scan showed a large heterogeneously enhancing solid-cystic mass in the pineal region extending to the third ventricle.DiscussionPapillary tumor of the pineal region is a neuroepithelial tumor with distinct morphological and immunohistochemical features that can be utilized to help differentiate it from other primary and metastatic tumors occurring within this region. Although there is currently no clear consensus, the potential for disease progression and CSF dissemination supports early aggressive therapy in these patients.
Highlights
Papillary tumor of the pineal region is an unusual, recently described entity with potential for misinterpretation as either an ependymoma or choroid plexus papilloma
Papillary tumor of the pineal region is a neuroepithelial tumor with distinct morphological and immunohistochemical features that can be utilized to help differentiate it from other primary and metastatic tumors occurring within this region
With only 181 described cases according to the 2016 edition of the World Health Organization (WHO) Classification of Tumors of the central nervous system (CNS), papillary tumor of the pineal region is a recently described entity that is important to recognize due to the potential for misinterpretation as either an ependymoma or choroid plexus papilloma [2]
Summary
As defined by the WHO, papillary tumor of the pineal region is “a neuroepithelial tumor localized in the pineal region and characterized by a combination of papillary and solid areas with epithelial-like cells and immunoreactivity for cytokeratins” [2]. The theory that papillary tumor of the pineal region arises from this region is confirmed by Shibahara and colleagues who showed expression of proteins presumed to be secreted by cells of the human subcommissural organ [5]. Santoro and colleagues provided some information regarding the natural history of papillary tumor of the pineal region In their case report, neuro-radiological follow-up for a period of four years showed an average growth rate of 3.4 mm per year [10]. Further accumulation of case reports will assist with defining this entity and may provide further insight into management of such patients
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