While pediatric low-grade glioma/glioneuronal tumors (LGG/LGGNTs) are considered slow-growing, indolent tumors with excellent long-term prognosis, mortality due to the disease is not unknown. Few studies have addressed the cause of death in this population.Methods:
Retrospective review of clinicopathologic and radiologic data for children 21 years or younger with LGG/LGGNT who died at St. Jude Children's Research Hospital between April 1985 and June 2015. Our primary objective was to determine the causes and timing of mortality in affected children.Results:
For the 87 eligible patients, median age at diagnosis was 7.7 years (range, 0.21–21 years), median age at death was 14.26 years (range, 0.58–32 years), and median time to death from diagnosis was 4.02 years (range, 0.21–24 years). Midbrain/thalamus was the most common tumor location (n = 34), followed by suprasellar/hypothalamic (n = 18) and cerebrocortical (n = 13). Astrocytoma not otherwise specified (n = 24), pilocytic astrocytoma (n = 23), and fibrillary astrocytoma (n = 11) were the predominant histologic diagnoses. Causes of death included progressive primary disease (PD) (n = 43), progression of PD with histological features of a high-grade glioma at progression or at autopsy (PD-HGG) (n = 15), second cancer (n = 3), suicide (n = 4), and vehicular accident (n = 3). Among the 15 patients with PD-HGG, 12 received radiation therapy before histologic confirmation of progression.Conclusions:
PD and PD-HGG contributed to 66% of the mortality in our patient cohort. Early psychological intervention should be included as part of the multidisciplinary management approach of children with LGG/LGGNT to reduce the risk of suicide in vulnerable subjects.