2020
DOI: 10.1007/s00381-020-04724-1
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A systematic review of ongoing clinical trials in optic pathway gliomas

Abstract: Introduction Optic pathway gliomas (OPGs), also known as Visual Pathway Gliomas, are insidious, debilitating tumours. They are most commonly WHO grade 1 pilocytic astrocytomas and frequently occur in patients with neurofibromatosis type 1. The location of OPGs within the optic pathway typically precludes complete resection or optimal radiation dosing, hence outcomes remain poor compared to many other low-grade gliomas. The aim of this systematic review was to formulate a comprehensive list of all current ongoi… Show more

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Cited by 20 publications
(16 citation statements)
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“…Therefore, BRAF inhibitors are reserved for specific indications such as recurrent or refractory cases [ 17 ]. However, targeted therapies are under investigation in low-grade gliomas, such as an upcoming European trial of frontline MEK inhibition or studies of BRAF inhibition in V600E mutated tumours [ 18 ]. Each paediatric cancer specialist group needs to develop updated guidelines where the role, timing and duration of such novel agents is clarified [ 17 , 19 ].…”
Section: Discussionmentioning
confidence: 99%
“…Therefore, BRAF inhibitors are reserved for specific indications such as recurrent or refractory cases [ 17 ]. However, targeted therapies are under investigation in low-grade gliomas, such as an upcoming European trial of frontline MEK inhibition or studies of BRAF inhibition in V600E mutated tumours [ 18 ]. Each paediatric cancer specialist group needs to develop updated guidelines where the role, timing and duration of such novel agents is clarified [ 17 , 19 ].…”
Section: Discussionmentioning
confidence: 99%
“…In the past decade, the therapeutic trend for OPGs was toward the innovative molecular targeted therapies, as >50% of the clinical trials were based on the Ras-related pathway and angiogenesis inhibitors [12,17,21,24,26,27,[77][78][79][80][81]. Among all the MEKi, selumetinib and trametinib have proven to have excellent efficacy profiles, especially for recurrent or refractory forms, with a twoyear PFS rate of 70% [82].…”
Section: Discussionmentioning
confidence: 99%
“…Conventional treatment options historically have included chemoradiotherapy, surgical resection, and radiotherapy [13][14][15][16][17][18][19][20][21]. Nevertheless, recent advances in the field of neuro-oncology have led to a dramatically wider spectrum of cures, mainly through the refinement of epigenetics and techniques in regenerative medicine [12,[22][23][24][25][26][27][28][29][30][31].…”
Section: Introductionmentioning
confidence: 99%
“…The choice of the specific molecular inhibitor depends on the underlying biology, making biopsy essential ( 22 , 23 ) to collect biological samples to perform molecular investigations to identify the best tailored treatment ( 49 ).…”
Section: Discussionmentioning
confidence: 99%