Overview
Papillary tumors of the pineal region (PTPR) are a rare tumor entity first described in 2003 and codified in the 2007 WHO tumor classification system 1. They most commonly occur in adults but can present in pediatric populations 1.Diagnosis
PTPRs are a rare tumor entity 1.
Diagnosis and management are topics of increasing attention due to recent characterization 1.
Little is known about PTPR presentation in pediatric populations 1.
In adults, PTPRs have been linked with chromosomal and genetic abnormalities, though this correlation is limited in pediatric literature 1.Management
Gross total resection (GTR) is the mainstay for treatment 1.
Cerebrospinal fluid diversion may be used in management 1.
Application of adult treatment protocols may not be advisable in children due to age and CNS developmental changes 1.Special Populations
PTPRs are exceedingly rare in pediatric populations 1.
One case of a 10-year-old female with PTPR and Trisomy 21 was managed successfully with CSF diversion and GTR, with no recurrence three years post-resection 1.
Limited information exists regarding PTPR correlations with chromosomal and genetic abnormalities in pediatric literature 1.Key Recommendations
Gross total resection (GTR) is the primary treatment modality for papillary tumors of the pineal region 1. (Evidence: Weak)
Consider cerebrospinal fluid diversion as part of the management strategy for papillary tumors of the pineal region 1. (Evidence: Weak)
Exercise caution when applying adult treatment protocols to pediatric patients with papillary tumors of the pineal region due to age and CNS developmental considerations 1. (Evidence: Weak)References
1 Mathkour M, Hanna J, Ibrahim N, Scullen T, Kilgore MD, Werner C et al.. Papillary tumor of the pineal region in pediatric populations: An additional case and systematic review of a rare tumor entity. Clinical neurology and neurosurgery 2021. link