Disease relevance of Optic Nerve Glioma

• The peripheral CSF-intensity signal in orbital optic glioma correlates with the histopathological finding of perineural arachnoidal gliomatosis and serves as a neuroradiologic marker for neurofibromatosis [1].
• In the present study, patients with optic gliomas and Type 1 neurofibromatosis (NF-1) were compared with patients with optic gliomas without NF-1, with respect to survival, time to tumor progression, and tumor location [2].
• Although many of their complications are unfortunately still difficult or impossible to treat effectively, there may be significant benefit to the patient from prompt detection of optic glioma in NF, retinal angiomas in VHL disease, and glaucoma in SWS [3].
• The association of optic glioma and other intracranial neoplasms in patients with NF1 suggests that there are fundamental pathophysiological differences between patients with and without optic glioma [4].
• We identified a large deletion in the NF1 gene in a patient with a rhabdomyosarcoma as well as a variation in intron 22 in a patient with an optic glioma [5].

High impact information on Optic Nerve Glioma

• Inactivation of NF1 in CNS causes increased glial progenitor proliferation and optic glioma formation [6].
• CNS tumours associated with NF1 were reported in 9.4% (49) of patients, optic gliomas occurring in 25 of these, 4.8% of patients [7].
• Methylphenidate and melatonin for sleep disorder with optic glioma [8].
• CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression [9].
• Since then, all children with recurrent optic glioma and all children aged 6 years old and under with newly diagnosed optic glioma have been offered a program of initial therapy with vincristine and actinomycin D for six cycles over 18 months [10].

Chemical compound and disease context of Optic Nerve Glioma

• We have demonstrated that carboplatin can eliminate or delay radiation therapy in children and adolescents with low grade optic glioma [11].
• Precocious puberty associated with neurofibromatosis and optic gliomas. Treatment with luteinizing hormone releasing hormone analogue [12].

Biological context of Optic Nerve Glioma

• To clarify whether silencing of NF1 by promoter methylation plays a role in PA and especially in optic glioma, the authors investigated the methylation status in 30 PA, 6 of which had optic glioma [13].

Anatomical context of Optic Nerve Glioma

• A striking association has been observed between the presence of optic glioma and of other central nervous system (CNS) tumours in NF1 patients [4].
• PS VEPs were performed on 10 children with NF 1 and optic gliomas and 20 children with NF 1 and normal visual pathways (as defined on MRI) [14].

Gene context of Optic Nerve Glioma

• CASE: A 4-year-old girl with neurofibromatosis type 1 and GH excess associated with optic glioma received pegvisomant injections (10 mg subcutaneously) with increasing intervals from daily to every 4th day [15].
• Frequent eye disorders were: Lisch nodules (Iris hamartomas, IH) 78%, hyperthelorism 19.7%, bulbomotoric disorders 15.9%, disorders of the optic disc 16.7% and optic gliomas (18.9%) [16].

Analytical, diagnostic and therapeutic context of Optic Nerve Glioma

• This entity represents a differential diagnosis to optic glioma in patients with NF-1 [17].

References