Prognosis of Pediatric Patients with Pineoblastoma: A SEER Analysis 1990-2013

BACKGROUND: Pineoblastomas are rare, malignant embryonal tumors that have a relatively higher incidence and a poorer prognosis in children. Owing to the rarity of these tumors, there is a paucity of data on associated prognostic factors. We used the Surveillance, Epidemiology, and End Results (SEER) database to evaluate prognostic factors for pineoblastomas with the aim of improving tumor management. METHODS: Data from all pediatric patients (age <= 17 years) diagnosed with pineoblastoma between 1990 and 2013 were extracted from the SEER-18 registry database. Survival was described with Kaplan-Meier curves. The Cox proportional hazards model was used for both univariate and multivariate analyses. A nomogram was established for predicting 1-, 3-, and 5-year overall survival (OS) in patients with pineoblastoma. RESULTS: Age >5 years (P = 0.004) and radiotherapy treatment (P = 0.000) were associated with better rates of survival. Gross total resection (P = 0.054) also was correlated with better prognosis, whereas tumor size >30 mm in maximum diameter (P = 0.025) was associated with poorer outcome. A nomogram was established based on the results of the Cox model and was validated by a concordance index (C-index) of 0.767 (95% confidence interval, 0.698-0.836) and calibration plots. CONCLUSIONS: Our results show that the impact of tumor extension is not defined. OS is better in older children treated by radiotherapy, and gross total resection also appears to result in increased survival. A nomogram was built to predict 1-, 3-, and 5-year OS for these patients.