PAs can arise in association with neurofibromatosis type 1 (NF1), wherein they most commonly manifest as "
optic glioma," a nonspecific term that implies the involvement of the optic nerve proper (10).
Among the neuroepithelial tumors, one case of optic meningioma grade 1 constituting 2% of benign optic tumors in males and one case of low grade
optic glioma was found to be closer to the other study in Pakistan.15
(1) Six or more pigmentations greater than 5 mm before puberty and greater than 15 mm after puberty (cafe au lait) (2) One plexiform neurofibroma or more than two neurofibromas of any type (3) Axillary or inguinal freckling (4)
Optic glioma (5) Two or more "Lisch" nodules (iris hamartoma) (6) Bone lesions (7) Presence of at least one of these clinical findings in first-degree relatives
NG2-cells are not the cell of origin for murine neurofibromatosis-1 (Nf1)
optic glioma. Oncogene.
Tumors on the optic nerve, also known as an
optic gliomaThe rare condition means that Keane, six, suffers from
optic glioma and will eventually lose his eyesight.
Optic glioma in children: surveillance, resection, or irradiation?
Increased c-Jun-NH2-kinase signaling in neurofibromatosis1 heterozygous ([Nf1.sup.+/-]) microglia was shown to promote
optic glioma proliferation [72] and glioma growth [73].
Diagnosis of the autosomal dominant syndrome--which results from a neurofibromin defect and occurs in 1 of 2,000-4,500 individuals--requires the presence of two or more of the following diagnostic criteria: six or more CALMs greater than 0.5 cm in diameter in prepubertal children, or greater than 1.5 cm in those postpubertal; two or more neurofibromas of any type, or one plexiform neurofibroma; axillary and/or inguinal freckling (Crowe's sign);
optic glioma; two or more Lisch nodules; a bony lesion (such as sphenoid dysplasia or long bone dysplasia); and a first-degree relative with NF1.