Post-Surgical Prognosis of Patients with Pineoblastoma: A Systematic Review and Individual Patient Data Analysis with Trends over Time

SIMPLE SUMMARY: Pineoblastoma tumors are rare and aggressive tumors of the pineal gland. Due to their rarity, most of the literature on pineoblastoma consists of case reports and single-institution series. To better understand patient and clinical characteristics that influence survival in pineoblastoma patients, a systematic review and individual patient data meta-analysis were conducted. Another goal of this study was to determine whether or not patient outcomes had changed since the last systematic review on this topic was published in 2012. Patient survival was analyzed based on factors such as age, metastatic presentation, extent of surgical resection, adjuvant therapy, and publication year. Our study demonstrates that less-than-gross total resection, metastatic presentation, adjuvant chemotherapy without radiation, and an age of less than three years are associated with poorer survival. Furthermore, we found that the 5-year overall survival improved from 32.8% to 56.1% since 2012. ABSTRACT: Most of the literature on pineoblastoma consists of case reports and single-institution series. The goal of this systematic review and individual patient data (IPD) analysis was to summarize the existing literature, identify factors associated with overall survival (OS), and provide a contemporary update on prognosis for patients with pineoblastoma. Forty-four studies were identified with 298 patients having IPD. Kaplan–Meier analyses were used to report survival outcomes based on age, tumor metastases, extent of resection (EOR), adjuvant therapy, and publication year. Cox regression was performed to identify independent predictors of time to mortality. Multivariable recursive partitioning analysis was used to identify the most important subgroups associated with mortality. Patients were classified based on publication year before and after the last systematic review on this topic (pre-2012 and 2012 onwards) and compared using univariate and multivariable analyses. This study demonstrates that EOR less-than-gross total resection, metastatic presentation, adjuvant chemotherapy without radiation, and tumor presentation in children less than three years old are associated with poorer prognosis. Since 2012, the 5-year actuarial OS has improved from 32.8% to 56.1%, which remained significant even after accounting for EOR, age, and adjuvant therapy. Pineoblastoma remains a severe rare disease, but survival outcomes are improving.