2021
DOI: 10.1007/s00401-021-02276-5
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Integrated molecular and clinical analysis of low-grade gliomas in children with neurofibromatosis type 1 (NF1)

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Cited by 48 publications
(28 citation statements)
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“…In this monogenetic disease, the NF1 gene and consecutively the tumor suppressor neurofibromin (as regulator of RAS) is altered, leading to uncontrolled RAS-mitogen-activated protein kinase (RAS/MAPK) pathway activation upon damage of the second allele (second hit). Low grade gliomas (LGG) are the most frequent CNS malignancies associated with NF1 and are almost exclusively driven by this mutation [2]. Molecular analysis of sporadic…”
Section: Targeting the Pathway In Low Grade Glioma By Mek Inhibitionmentioning
confidence: 99%
“…In this monogenetic disease, the NF1 gene and consecutively the tumor suppressor neurofibromin (as regulator of RAS) is altered, leading to uncontrolled RAS-mitogen-activated protein kinase (RAS/MAPK) pathway activation upon damage of the second allele (second hit). Low grade gliomas (LGG) are the most frequent CNS malignancies associated with NF1 and are almost exclusively driven by this mutation [2]. Molecular analysis of sporadic…”
Section: Targeting the Pathway In Low Grade Glioma By Mek Inhibitionmentioning
confidence: 99%
“…8,9 When MRI is performed, it is occasionally difficult to distinguish atypical NF-1-related myelin vacuolization or spongiform changes from PLGG, especially at a single time point, because of overlap in imaging characteristics. [9][10][11] Nonoptic pathway PLGG in NF-1 is an understudied subset. Up to 15% of all patients with NF-1 present with cerebral tumors in the first two decades of life, most of which are low-grade pilocytic astrocytomas.…”
Section: Observationsmentioning
confidence: 99%
“…Up to 15% of all patients with NF-1 present with cerebral tumors in the first two decades of life, most of which are low-grade pilocytic astrocytomas. 10,12 NF-1 has been shown to act as a positive prognostic factor for progression-free survival in LGG. 13 Specifically, NF-1-associated brainstem gliomas have a lower frequency of progression, respond more favorably to chemotherapy treatment, and demonstrate a more favorable prognosis and event-free survival rate than their non-NF-1-associated counterparts.…”
Section: Observationsmentioning
confidence: 99%
“…A recent study associated FGFR1 mutations in low-grade glioma with increased risk for intracranial bleeding (Ishi et al 2020). In experimental murine NF1 models, a co-occurring FGFR1 hotspot mutation confers an additional growth advantage in LGG (Fisher et al 2021). These hotspot mutations are of great interest, because FGFR alterations can be targeted by inhibitors including the multi tyrosine kinase inhibitors ponatinib, lucitanib and nintedanib (Porta et al 2017).…”
Section: Introductionmentioning
confidence: 99%