A role for early surgical intervention in childhood low-grade glioma? Illustrative case

IDH-mutant diffuse gliomas are considered low grade, albeit with a propensity for malignant behavior over time, distinguishing them from pediatric-type low-grade gliomas. IDH-mutant tumors have astrocytic or oligodendrocytic phenotypes associated with TP53 and ATRX mutations or 1p/19q deletions, respectively. TP53 mutations are associated with a tumor that acquires malignant properties characteristic of glioblastoma with subsequent very poor survival.

The authors present the case of a 12-year-old female who presented with an incidental T2-FLAIR bright lesion in the right frontal lobe. Although clinically asymptomatic, there was interval minimal but definite growth on serial imaging over 5 years, and she underwent resection. Molecular pathology indicated an isolated IDH1 mutation, without cooperating molecular changes.

IDH-mutant gliomas, although typically an adult disease, may be diagnosed in childhood or adolescence. This early diagnosis of a tumor before it has acquired cooperating events suggests childhood origin and long latency for this more typical adult tumor. Early recognition and treatment of these tumors, before they reach their full malignant potential, may yield therapeutic benefit. Surgery may also play a key role to eliminate neoplastic cells and the acquisition of more fully malignant clones that may become more aggressive and drive disease progression many years later. https://thejns.org/doi/10.3171/CASE2584.