The Changing Landscape of Pediatric Low-Grade Gliomas: Clinical Challenges and Emerging Therapies

Neuropediatrics
Ana S Guerreiro StucklinMichael A Grotzer

Abstract

Pediatric low-grade gliomas (PLGGs) are the most common brain tumors in children. Though histologically benign and associated with excellent outcome, patients with unresectable lesions--mostly young children with midline tumors--experience multiple progressions and are at increased risk for long-term neurological sequelae. PLGGs in children with underlying genetic predisposition syndromes--especially neurofibromatosis type 1 and tuberous sclerosis--have a distinct natural history and biology with important treatment implications. Given the complexity of medical issues, optimal management requires a large network of health care providers; treatment decisions must address both tumor control and potential side effects of the therapy. Current treatment strategies often fail to induce sustained tumor regression and many children require several lines of therapy, highlighting the need for novel therapies. Here, we review the current management of PLGG and discuss how new molecular targets--in particular alterations of the Ras/MAPK pathway--are rapidly changing our approach to PLGG.

Citations

May 17, 2018·Documenta Ophthalmologica. Advances in Ophthalmology·Patrícia de Freitas DottoSolange Rios Salomão
Jun 20, 2018·International Journal of Molecular Sciences·Sonia Nava-SalazarAmerica Vanoye-Carlo
Sep 24, 2016·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·María Baro Fernández, Vanesa Pérez Alonso
Dec 26, 2017·Canadian Association of Radiologists Journal = Journal L'Association Canadienne Des Radiologistes·Daddy Mata-MbembaPrakash Muthusami
Oct 20, 2017·Pediatric Blood & Cancer·Michal ZapotockyEric Bouffet
Jan 10, 2018·Current Opinion in Pediatrics·Ana S Guerreiro StucklinMichael D Taylor
Sep 11, 2020·Cancer Research·Gina M NeyQing Li
Mar 31, 2021·Neurosurgery Clinics of North America·Sophie M PeetersAnthony C Wang

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