The Use of Magnetic Resonance Imaging Screening for Optic Pathway Gliomas in Children with Neurofibromatosis Type 1

Neurofibromatosis type 1 and optic pathway glioma: Molecular interplay and therapeutic insights

“…These tumors are located in the optic nerves and or chiasm (85%) or the optic tracts and radiations (15%). Of all NF1–OPGs found routinely, vision loss or other symptoms are found in 30–50% of affected children . Precious puberty is occasionally seen, where it is often secondary to tumor infiltration of the hypothalamus .…”

“…Of all NF1-OPGs found routinely, vision loss or other symptoms are found in 30-50% of affected children. [6][7][8] Precious puberty is occasionally seen, where it is often secondary to tumor infiltration of the hypothalamus. 9 Initially symptomatic children are Abbreviations: cAMP, cyclic AMP; EFS, event-free survival; FASI, focal areas of signal intensity; GEM, genetically engineered mouse; LGG, low-grade glioma; NF1, neurofibromatosis type 1; OPG, optic pathway glioma; PI3K, phosphoinositide-3-kinase; RGC, retinal ganglion cell; RNFL, retinal nerve fiber layer; VEGF, vascular endothelial growth factor more likely to require treatment than are those with incidentally identified tumors.…”

Carboplatin and vincristine neurotoxicity in the treatment of pediatric low‐grade gliomas

“…Our study included more females (59%) whereas other studies have reported a male predominance in LGGs . This might be explained by the fact that patients with optic pathway glioma are more often female than male and 86% of our cohort had optic pathway glioma …”

“…7 This might be explained by the fact that patients with optic pathway glioma are more often female than male and 86% of our cohort had optic pathway glioma. 15,16 Despite the small sample size, this retrospective study adds interesting new information concerning the neurotoxicity of CV in the con- to receive a full dose of vincristine by cycle two. Pain was also a clinical concern in more than 50 percent of patients.…”

Insights into optic pathway glioma vision loss from mouse models of neurofibromatosis type 1

“…In individuals with NF1, the majority (75-85%) of OPGs are located within the ON and chiasm (pre-chiasmal or anterior optic pathway), with a smaller proportion of tumors located in the optic tracts and radiations (post-chiasmal or posterior optic pathway). NF1-OPGs occur most frequently in young children (median age at diagnosis 5 4.5 years) (Listernick, Charrow, Greenwald, & Esterly, 1989;Listernick, Charrow, Greenwald, & Mets, 1994;Prada et al, 2015), with rare cases described in older adolescents (Chong et al, 2013;Listernick et al, 2004). Hence, OPG is a manifestation of NF1 that predominates in young children who are often preverbal with comorbid attention deficits, further complicating diagnosis and accurate visual assessment in an at-risk population (Listernick et al, 2007).…”