Pediatric low-grade ganglioglioma: epidemiology, treatments, and outcome analysis on 348 children from the surveillance, epidemiology, and end results database

Abstract

Background: Low-grade gangliogliomas/gangliocytomas (GGs) are rare tumors of the central nervous system that occur mostly in young people. Because of their rarity, large-scale, population-based studies focusing on epidemiology and outcomes are lacking.

Objective: To use the Surveillance, Epidemiology, and End Results (SEER) data sets of the National Cancer Institute to study demographics, tumor location, initial treatment, and outcome data on low-grade GGs in children.

Methods: SEER-STAT v8.1.2 identified all patients aged 0 to 19 years in the SEER data sets with low-grade GGs. Using the Kaplan-Meier method and Cox proportional hazard regression, we examined associations between these characteristics and survival.

Results: There were 348 children with low-grade GGs diagnosed from 2004 to 2010, with a median follow-up of 37 months. Tumors were more prevalent in males (n = 208, 59.8%) than females (n = 140, 40.2%) (P < .001). Almost 63% occurred in children >10 years, whereas only 3.5% were found in those <1 year old. Approximately 50% were located in the temporal lobes, and only 3.7% and 3.5% were located in the brainstem and spinal cord, respectively. Surgery was performed on 91.6% of cases, with gross total resection achieved in 68.3%. Radiation was used in 3.2%. Young age (<1 year) and brainstem location were associated with worse overall survival.

Conclusion: This study shows that low-grade GGs occur in older children with a male preference. Gross total resection is achieved in the majority of cases, and radiation is rarely used. Although the majority of patients have an excellent prognosis, infants and patients with brainstem tumors have worse survival rates.

Figure 1

Age distribution of 348 children with low-grade gangliogliomas/gangliocytomas from the SEER datasets (2004-2010).

Figure 2

5-year relative survival analysis of different age groups of patients with low-grade gangliogliomas/gangliocytomas from the SEER datasets (2004-2010).

Figure 3

5-year relative survival analysis of patients with gangliogliomas/gangliocytomas of the brainstem, spinal cord, and other locations, from the SEER datasets (2004-2010).