Treatment of Progressive/Recurrent Circumscribed Astrocytic Gliomas, Pediatric-Type Diffuse Low-Grade Gliomas, and Glioneuronal/Neuronal Tumors

There is no single standard treatment option for progressive/recurrent circumscribed astrocytic gliomas, pediatric-type diffuse low-grade gliomas, glioneuronal tumors, and neuronal tumors. To determine and implement optimal management, treatment is best guided by a multidisciplinary team of specialists with experience treating pediatric patients with brain tumors.

An individual plan needs to be tailored based on the following:

Recurrent disease is usually at the primary tumor site, although multifocal or widely disseminated disease to other intracranial sites and to the spinal leptomeninges has been documented.[1,2] Most recurrences are of the same tumor entity; however, transformation into a higher grade tumor is possible and associated with the molecular profile.[3] Surveillance imaging will frequently identify asymptomatic recurrences.[4] At the time of recurrence, a complete evaluation to determine the extent of the relapse is indicated.

Tumor sample sequencing was done in pediatric (n = 48) and young adult patients (n = 6) with recurrent or refractory low-grade gliomas who were enrolled in the National Cancer Institute (NCI)–Children's Oncology Group (COG) Pediatric MATCH trial. The test revealed genomic alterations that were considered actionable for treatment on MATCH study arms in 39 of 54 tumors (72.2%).[5] Alterations in MAPK pathway genes (most commonly BRAF and NF1) were detected in 26 of 54 tumors (48.1%). FGFR1 variants (n = 11) or fusions (n = 1) were identified in 12 of 54 tumors (22.2%).

Treatment options for progressive/recurrent circumscribed astrocytic gliomas, pediatric-type diffuse low-grade gliomas, and glioneuronal/neuronal tumors include the following: