Papillary tumor of the pineal region in pediatric populations: An additional case and systematic review of a rare tumor entity

Mansour Mathkour; Joshua Hanna; Naser Ibrahim; Tyler Scullen; Mitchell D Kilgore; Cassidy Werner; Ian Cormier; Pierce Spencer; Joseph R Keen; Cuong J Bui

doi:10.1016/j.clineuro.2020.106404

Papillary tumor of the pineal region in pediatric populations: An additional case and systematic review of a rare tumor entity

Clin Neurol Neurosurg. 2021 Feb:201:106404. doi: 10.1016/j.clineuro.2020.106404. Epub 2020 Dec 8.

Authors

Affiliations

¹ Ochsner Health System, Department of Neurosurgery, New Orleans, LA, USA; Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA; Neurosurgery Division, Surgery Department, Jazan University, Jazan, Saudi Arabia. Electronic address: mmathkou@tulane.edu.
² Ochsner Health System, Department of Neurosurgery, New Orleans, LA, USA; Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA. Electronic address: jhanna4@tulane.edu.
³ Ochsner Health System, Department of Neurosurgery, New Orleans, LA, USA; Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA. Electronic address: nibrahim@tulane.edu.
⁴ Ochsner Health System, Department of Neurosurgery, New Orleans, LA, USA; Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA. Electronic address: tscullen@tulane.edu.
⁵ Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA. Electronic address: mkilgore@tulane.edu.
⁶ Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA. Electronic address: cwerner1@tulane.edu.
⁷ Ochsner Health System, Department of Neurosurgery, New Orleans, LA, USA; Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA. Electronic address: v-icormier@ochsner.org.
⁸ Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA. Electronic address: pspencer1@tulane.edu.
⁹ Ochsner Health System, Department of Neurosurgery, New Orleans, LA, USA; Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA. Electronic address: joseph.keen@ochsner.org.
¹⁰ Ochsner Health System, Department of Neurosurgery, New Orleans, LA, USA; Tulane Medical Center, Department of Neurosurgery, New Orleans, LA, USA. Electronic address: cjbui@ochsner.org.

PMID: 33360024
DOI: 10.1016/j.clineuro.2020.106404

Abstract

Papillary tumors of the pineal region (PTPR) are a rare tumor entity first described in 2003, later codified in the 2007 WHO tumor classification system. PTPRs most commonly occur in the third and fourth decades, with exceedingly rare presentations in pediatric populations. Herein, we present an additional case of a 10-year-old female found to have PTPR in conjunction with Trisomy 21 managed successfully with cerebrospinal fluid diversion and gross total resection (GTR). Three years after resection she has returned to baseline without recurrence. We also performed a comprehensive review of the current literature discussing the diagnosis, treatment, and pathophysiologic correlations in children. Diagnosis and management of PTPRs is a topic that is increasingly garnering attention in the literature given the recent characterization of this tumor entity. However, relatively little is known about the presentation of PTPRs in pediatric populations. In adults, PTPRs have been linked with several chromosomal and genetic abnormalities; however this correlation is limited in pediatric literature. Although GTR is the mainstay for treatment, the application of adult treatment protocols may not be advisable due to age and the developmental changes of the CNS in children.

Keywords: Child; Gross total resection; Papillary pineal region tumor; Pediatric.

Publication types

Systematic Review

MeSH terms

Adolescent
Brain Neoplasms / pathology
Brain Neoplasms / surgery*
Child
Female
Humans
Male
Neoplasm Recurrence, Local / pathology
Neoplasm Recurrence, Local / surgery*
Pediatrics
Pineal Gland / pathology
Pineal Gland / surgery*
Pinealoma / diagnosis
Pinealoma / surgery*