Background
Low-grade gangliogliomas/gangliocytomas (GG) are rare tumors of the CNS, which occur mostly in young people. Due to their rarity, large-scale, population-based studies focusing on epidemiology and outcomes are lacking.
Objective
To use the Surveillance, Epidemiology, and End Results (SEER) datasets of the National Cancer Institute to study demographics, tumor location, initial treatment, and outcome data on low-grade GG in children.
Methods
SEER-STAT v8.1.2 identified all patients aged 0-19 years in the SEER datasets 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 GG diagnosed from 2004-2010, 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<0.001). Almost 63% percent occurred in children >10 years, while only 3.5% were found in those <1 year old. Approximately 50% were located in the temporal lobes, while 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 (GTR) achieved in 68.3%. Radiation was used in 3.2%. Young age (<1yrs) and brainstem location were associated with worse overall survival (OS).
Conclusion
This study shows that low-grade GG occur in older children with a male preference. GTR is achieved in the majority of cases, and radiation is rarely used. While the majority of patients have an excellent prognosis, infants and patients with brainstem tumors have worse survival rates.