Papillary Tumors of the Pineal Region (PTPR) in Pediatric Population: A Case Report and Literature Review.

Abstract

Papillary tumors of the pineal region (PTPR) are rare central nervous system neoplasms, with a limited number of pediatric cases reported in the literature. Their optimal management remains unclear due to their unpredictable biological behavior and high recurrence rates. This study presents the clinical course, treatment, and long-term follow-up of a 3-year-old child diagnosed with PTPR. Additionally, we conducted a comprehensive review of 35 pediatric cases reported to date, analyzing clinical presentation, treatment strategies, recurrence patterns, and outcomes. The review revealed that gross total resection (GTR) was performed in 69.6% of cases, yet recurrence occurred in 38.8% of these patients. In cases of subtotal resection (STR), the rate of recurrence was significantly higher, with 60% of patients experiencing a relapse. Adjuvant radiotherapy (RT) seems to enhance disease control, especially in cases with STR. Spinal dissemination was observed in 5.7% of cases at diagnosis and 6.2% at recurrence, both of which were associated with poor prognosis. Our case highlights the effectiveness of adjuvant RT for the first time in preventing tumor progression following STR, with long-term disease stability (9 years and 2 months) observed over a 9-year and 6-month follow-up period. PTPRs have a high recurrence rate, which requires careful selection of patients for adjuvant therapies. Although GTR is the standard treatment approach, adjuvant RT may offer additional control in specific cases, particularly for patients with STR or those classified as high-risk. Further research is needed to establish standardized treatment protocols and improve long-term outcomes for pediatric patients with PTPR.