Use of bevacizumab in pediatric low-grade glioma: Ten-year experience in a single center

Abstract

Purpose: Pediatric low-grade gliomas (PLGG) have an excellent overall survival, but frequently need non-surgical therapy at diagnosis or after progression when located in unresectable sites such as the optic pathway. Chemotherapy side effects have led to the need for better-tolerated regimens with a sustained response. Bevacizumab, a humanized anti-VEGF monoclonal antibody has been used in monotherapy and/or in combination for these entities. Here we present our experience with its use in PLGG. Methods: The authors performed a retrospective, observational, single-institution study, between 2008 and 2018, to evaluate the safety and efficacy of bevacizumab in progressive PLGG. Results: Twenty-six patients with a median age at diagnosis of 3.32 years old [0.12–14.7] and a median age at treatment of 8.11 years old [0.41–16.82] were included in the study. Nineteen had optic pathway gliomas and chiasmatic-hypothalamic gliomas (73.1%), 9 of them (47.4%) associated with neurofibromatosis type 1 (NF1). Fourteen non-NF1 tumors were molecularly studied, disclosing BRAF-KIAA1549 fusion transcript in 9, and BRAF V600E mutation in 2. Bevacizumab was administered in combination with other agent(s) in 16 of the 35 treatment courses. Responses were assessed at 3, 12 months and at the end of treatment. Progression free survival at 12 and 36 months was 91.4% and 31.4%, respectively, and no severe adverse events were observed. Conclusions: In our series, Bevacizumab in PLGG showed clinical efficacy with a favorable toxicity profile. Larger prospective studies may determine whether the response is conditional upon age, tumor location, and different histological and/or molecular characterization.

Keywords

• Bevacizumab
• Pediatric low-grade glioma
• Pilocytic astrocytoma
• NF1
• Biological therapy
• Optic pathway glioma