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==Overview==
==Overview==
Optic nerve gliomas may be classified into two subtypes based on anatomic location, and whether or not they are associated with neurofibromatosis type 1.
Optic nerve gliomas may be classified into two subtypes based on anatomic location, and whether or not they are associated with neurofibromatosis type 1. Optic nerve gliomas may be classified according to anatomic location into two subtypes: anterior tumors and posterior tumors. 
==Classification==
==Classification==
Optic pathway gliomas (OPGs) are classified by whether or not they are associated with neurofibromatosis type 1 (NF1, von Recklinghausen's disease) and by anatomic location.  
Optic nerve gliomas may be classified into several subtypes based on anatomic location, and whether or not they are associated with neurofibromatosis type 1. Optic nerve gliomas may be classified according to anatomic location into two subtypes: anterior tumors and posterior tumors.
According to their location along the optic pathway, the two major categories are:
According to their location along the optic pathway, the two major categories are:
Location — Low-grade gliomas may involve the anterior visual pathway of the optic nerve (25 to 35 percent of cases) or the posterior visual pathways (chiasmal and postchiasmal).
===Anterior visual pathway===
 
Anterior visual pathway — Anterior tumors can be subdivided into:
*Orbital
*Orbital
*Intracanalicular
*Intracanalicular
*Intracranial prechiasmal lesions.
*Intracranial prechiasmal lesions.
Most of anterior visual pathway tumors are classified as pilocytic astrocytomas and they occur most frequently in prepubertal children.
Most of anterior visual pathway tumors are classified as pilocytic astrocytomas and they occur most frequently in prepubertal children.<ref name="pmid7108594">{{cite journal| author=Tenny RT, Laws ER, Younge BR, Rush JA| title=The neurosurgical management of optic glioma. Results in 104 patients. | journal=J Neurosurg | year= 1982 | volume= 57 | issue= 4 | pages= 452-8 | pmid=7108594 | doi=10.3171/jns.1982.57.4.0452 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7108594  }} </ref>
Neurofibromatosis type 1 tumors are more likely to invole anterior visual pathway.
===Posterior visual pathway===
Posterior visual pathway — Posterior tumors may arise in the
*Optic chiasm
*Optic chiasm
*Hypothalamus
*Hypothalamus
*Anterior third ventricle  
*Anterior third ventricle  
Most of posterior visual pathway tumors are classified histologically as pilocytic astrocytomas, and occasionally gangliogliomas. Hypothalamic and chiasmal lesions present at a mean age of about three years. Optic nerve gliomas without an association with neurofibromatosis type 1 are more frequent in the posterior visual pathway.
Most of posterior visual pathway tumors are classified histologically as pilocytic astrocytomas, and occasionally gangliogliomas. Hypothalamic and chiasmal lesions present at a mean age of about three years.<ref name="pmid2340532">{{cite journal| author=Benes V, Julisová I, Julis I| title=Our treatment philosophy of gliomas of the anterior visual pathways. | journal=Childs Nerv Syst | year= 1990 | volume= 6 | issue= 2 | pages= 75-8 | pmid=2340532 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2340532 }} </ref>
Optic nerve gliomas can involve one or both optic nerves.   
About 10% of optic pathway tumors are located within an optic nerve, one third of the tumors involve both optic nerve and chiasm, another one third involves predominantly the chiasm itself, and one fourth involves predominantly the hypothalamus. 5 5% of optic nerve gliomas are multicentric.
About 10% of optic pathway tumors are located within an optic nerve, one third of the tumors involve both optic nerve and chiasm, another one third involves predominantly the chiasm itself, and one fourth involves predominantly the hypothalamus. 5 5% of optic nerve gliomas are multicentric.
In approximately 50% of patients, optic nerve gliomas occurs without any known chromosomal aberration. In the remaining 50% of patients, a diagnosis of neurofibromatosis type 1 is seen. NF-1 is an autosomal dominant disorder with an incidence of one in 3000-5000 births. In children diagnosed with NF-1, about 10% develop optic gliomas, and 25-40% of optic gliomas occur in children with NF-1.
===Association with NF1===
Neurofibromatosis type 1 tumors are more likely to involve anterior visual pathway. Optic nerve gliomas without an association with neurofibromatosis type 1 are more frequent in the posterior visual pathway.<ref name="pmid18796556">{{cite journal| author=Taylor T, Jaspan T, Milano G, Gregson R, Parker T, Ritzmann T et al.| title=Radiological classification of optic pathway gliomas: experience of a modified functional classification system. | journal=Br J Radiol | year= 2008 | volume= 81 | issue= 970 | pages= 761-6 | pmid=18796556 | doi=10.1259/bjr/65246351 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18796556  }} </ref> NF-1 is an autosomal dominant disorder with an incidence of one in 3000-5000 births. In children diagnosed with NF-1, approximately 15% develop optic gliomas.<ref name="pmid9029062">{{cite journal| author=Listernick R, Louis DN, Packer RJ, Gutmann DH| title=Optic pathway gliomas in children with neurofibromatosis 1: consensus statement from the NF1 Optic Pathway Glioma Task Force. | journal=Ann Neurol | year= 1997 | volume= 41 | issue= 2 | pages= 143-9 | pmid=9029062 | doi=10.1002/ana.410410204 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9029062  }} </ref> <ref name="pmid8021787">{{cite journal| author=Listernick R, Charrow J, Greenwald M, Mets M| title=Natural history of optic pathway tumors in children with neurofibromatosis type 1: a longitudinal study. | journal=J Pediatr | year= 1994 | volume= 125 | issue= 1 | pages= 63-6 | pmid=8021787 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8021787  }} </ref>  Twenty to forty percent of optic gliomas occur in children with NF-1.

Revision as of 03:45, 5 October 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Optic nerve gliomas may be classified into two subtypes based on anatomic location, and whether or not they are associated with neurofibromatosis type 1. Optic nerve gliomas may be classified according to anatomic location into two subtypes: anterior tumors and posterior tumors.

Classification

Optic nerve gliomas may be classified into several subtypes based on anatomic location, and whether or not they are associated with neurofibromatosis type 1. Optic nerve gliomas may be classified according to anatomic location into two subtypes: anterior tumors and posterior tumors. According to their location along the optic pathway, the two major categories are:

Anterior visual pathway

  • Orbital
  • Intracanalicular
  • Intracranial prechiasmal lesions.

Most of anterior visual pathway tumors are classified as pilocytic astrocytomas and they occur most frequently in prepubertal children.[1]

Posterior visual pathway

  • Optic chiasm
  • Hypothalamus
  • Anterior third ventricle

Most of posterior visual pathway tumors are classified histologically as pilocytic astrocytomas, and occasionally gangliogliomas. Hypothalamic and chiasmal lesions present at a mean age of about three years.[2] About 10% of optic pathway tumors are located within an optic nerve, one third of the tumors involve both optic nerve and chiasm, another one third involves predominantly the chiasm itself, and one fourth involves predominantly the hypothalamus. 5 5% of optic nerve gliomas are multicentric.

Association with NF1

Neurofibromatosis type 1 tumors are more likely to involve anterior visual pathway. Optic nerve gliomas without an association with neurofibromatosis type 1 are more frequent in the posterior visual pathway.[3] NF-1 is an autosomal dominant disorder with an incidence of one in 3000-5000 births. In children diagnosed with NF-1, approximately 15% develop optic gliomas.[4] [5] Twenty to forty percent of optic gliomas occur in children with NF-1.

  1. Tenny RT, Laws ER, Younge BR, Rush JA (1982). "The neurosurgical management of optic glioma. Results in 104 patients". J Neurosurg. 57 (4): 452–8. doi:10.3171/jns.1982.57.4.0452. PMID 7108594.
  2. Benes V, Julisová I, Julis I (1990). "Our treatment philosophy of gliomas of the anterior visual pathways". Childs Nerv Syst. 6 (2): 75–8. PMID 2340532.
  3. Taylor T, Jaspan T, Milano G, Gregson R, Parker T, Ritzmann T; et al. (2008). "Radiological classification of optic pathway gliomas: experience of a modified functional classification system". Br J Radiol. 81 (970): 761–6. doi:10.1259/bjr/65246351. PMID 18796556.
  4. Listernick R, Louis DN, Packer RJ, Gutmann DH (1997). "Optic pathway gliomas in children with neurofibromatosis 1: consensus statement from the NF1 Optic Pathway Glioma Task Force". Ann Neurol. 41 (2): 143–9. doi:10.1002/ana.410410204. PMID 9029062.
  5. Listernick R, Charrow J, Greenwald M, Mets M (1994). "Natural history of optic pathway tumors in children with neurofibromatosis type 1: a longitudinal study". J Pediatr. 125 (1): 63–6. PMID 8021787.