Long-term outcome of 4,040 children diagnosed with pediatric low-grade gliomas: an analysis of the Surveillance Epidemiology and End Results (SEER) database

Abstract

Background: Children with pediatric low-grade gliomas (PLGG) are known to have excellent 10-year survival rates; however the outcomes of adult survivors of PLGG are unknown. We identified patients diagnosed with PLGG diagnosed between 1973 and 2008 through the Surveillance Epidemiology and End Results (SEER) database to examine outcomes of adult survivors of PLGG.

Procedure: Four thousand and forty patients with either WHO grade I or II PLGG were identified and outcome data retrieved. Two analyses were performed to assess survival and risk of death from tumor. Competing risks analysis was conducted and cumulative incidence curves of death due to disease were generated. Cox proportional hazards regression was performed, with adjustment for non-disease death. Kaplan-Meier curves for overall cancer specific survival (OS) were also generated.

Results: The 20-year OS was 87% ± 0.8% and the 20-year cumulative incidence of death due to glioma was 12% ± 0.8%. The incidence of death after transition to adulthood (age greater than 22 years) was slightly lower, with 20-year cumulative incidence of disease death of 7% ± 1.8%. Year of diagnosis, age of diagnosis, histology, WHO grade, primary site, radiation, and degree of initial resection were prognostic in univariate analysis, while the administration of radiation was the greatest risk of death in multivariate analysis of OS (hazard ratio = 3.9).

Conclusions: PLGGs are associated with an excellent long-term survival, with a low likelihood of PLGG related death in adult survivors. Treatment strategies for pediatric tumors should therefore aim for disease control during childhood and adolescence with an emphasis on minimizing long-term treatment induced toxicities.

Keywords: SEER; outcome; pediatric low-grade glioma.

Fig. 1

Adult survivors of pediatric low-grade gliomas have excellent overall survival with low rates of mortality after patients transition into adulthood. A: Kaplan–Meier overall survival curve of patients with PLGG including only tumor related deaths. B: Kaplan–Meier overall survival curve of patients for which there is at least 15 years of follow-up. C: Pepe–Mori cumulative incidence of tumor specific death curve of patients diagnosed with PLGG. D, A: Kaplan–Meier overall survival curve of patients with PLGG showing survival starting from the patient's 22nd birthday. E: Pepe–Mori cumulative incidence of tumor specific death curves of patients starting from patient's 22nd birthday.

Fig. 2

Pepe–Mori cumulative incidence of death curves depicting univariate analysis including A, age at diagnosis B, year of diagnosis C, histology D, location of primary tumor E, grade and F, extent of resection. Number of patients at each time point are shown below.

Fig. 3

Patients with all histological subtypes of PLGG have excellent very long-term overall survival. Kaplan–Meyer curves of outcomes of patients diagnosed with different histological subtypes of PLGG.

Fig. 4

Patients with PLGG who received radiation therapy have inferior overall survival compared to patients that did not receive radiation therapy. Kaplan–Meier overall survival curve of patients with PLGG including only tumor related deaths showing outcomes of children who received radiation therapy and extent of surgical resection. Patients with a STR who did not receive radiation (curve shown by black arrow) had a superior outcome than patients with a GTR who did receive radiation (curve shown by red arrow).