2023
DOI: 10.3389/fped.2023.1038937
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A new era for optic pathway glioma: A developmental brain tumor with life-long health consequences

Abstract: Optic pathway and hypothalamic glioma (OPHG) are low-grade brain tumors that arise from any part of the visual pathways frequently involving the hypothalamus. The tumors grow slowly and present with features driven by their precise anatomical site, their age at presentation and the stage of growth and development of the host neural and orbital bony tissues. Up to 50% of optic pathway glioma arise in association with Neurofibromatosis type 1 (NF1), which affects 1 in 3,000 births and is a cancer predisposition … Show more

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Cited by 8 publications
(7 citation statements)
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References 79 publications
(67 reference statements)
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“…In a subset of patients, pilocytic astrosytomas will eventually stop growing or even regress as has been shown by several authors 42 . This phenomenon may be attributed to the effect of the tumor microenvironment that contributes to negative regulation of PA growth through the induction of oncogene-induced senescence, resulting from MAPK/ERK hyperactivation, leading to growth arrest of the tumor 43–45 . Occasionally, the patients may acutely deteriorate presenting sings of increased ICP or visual problems requiring emergency treatment (ie, surgical debulking or ventriculoperitoneal shunt insertion) 46 .…”
Section: Discussionmentioning
confidence: 86%
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“…In a subset of patients, pilocytic astrosytomas will eventually stop growing or even regress as has been shown by several authors 42 . This phenomenon may be attributed to the effect of the tumor microenvironment that contributes to negative regulation of PA growth through the induction of oncogene-induced senescence, resulting from MAPK/ERK hyperactivation, leading to growth arrest of the tumor 43–45 . Occasionally, the patients may acutely deteriorate presenting sings of increased ICP or visual problems requiring emergency treatment (ie, surgical debulking or ventriculoperitoneal shunt insertion) 46 .…”
Section: Discussionmentioning
confidence: 86%
“…42 This phenomenon may be attributed to the effect of the tumor microenvironment that contributes to negative regulation of PA growth through the induction of oncogene-induced senescence, resulting from MAPK/ERK hyperactivation, leading to growth arrest of the tumor. [43][44][45] Occasionally, the patients may acutely deteriorate presenting sings of increased ICP or visual problems requiring emergency treatment (ie, surgical debulking or ventriculoperitoneal shunt insertion). 46 This was the scenario in our patients with progressive disease, especially those 3 that died, whereas the remaining 6 received multiple interventions, localized or systemic.…”
Section: Discussionmentioning
confidence: 99%
“…Symptomatic gliomas typically manifest before age 6, with an average onset of 4.5 years [ 55 ]. The location, type, size, and number of OPGs can cause various neurological symptoms affecting visual function and resulting in a range of visual deficits ( Figure 2 ) [ 56 , 57 ]. OPGs may occur unilaterally or bilaterally, be situated anterior to, posterior to, or at the chiasm, centered or asymmetric.…”
Section: Phenotypic Manifestations Of Nf1 Affecting Visionmentioning
confidence: 99%
“…( B ) Depiction of individualized visual field deficits in patients with axonal damage in the optic pathway corresponding to their respective scheme in which red marks represent OPG size and location. Drawings based on concepts presented in [ 56 , 57 , 67 ].…”
Section: Phenotypic Manifestations Of Nf1 Affecting Visionmentioning
confidence: 99%
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