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__NOTOC__
__NOTOC__
{{Optic nerve glioma}}
{{Optic nerve glioma}}
{{CMG}}{{AE}}{{Simrat]]
{{CMG}}{{AE}}{{Simrat}}
==Overview==
==Overview==
Optic nerve glioma may be classified into several subtypes based on [[microscopic]] [[histopathology]], anatomic location, and association with [[neurofibromatosis]] type 1. Optic pathway gliomas are classified according to WHO classification into two subtypes: [[pilocytic astrocytoma]] and fibrillary astrocytoma. Optic nerve gliomas may be classified according to anatomic location into two subtypes: anterior visual pathway tumors and posterior visual pathway [[tumors]]. Based on whether or not they are associated with [[neurofibromatosis type 1]], they may be classified into two subtypes: optic nerve gliomas associated with [[neurofibromatosis type 1]] and optic nerve gliomas not associated with [[neurofibromatosis type 1]].
==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 glioma may be classified into several subtypes based on [[microscopic]] [[histopathology]], anatomic location, and association with neurofibromatosis type 1.
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).
 
{| class="wikitable"
|+
! colspan="2" |Classifications of Optic Nerve Glioma
|-
| rowspan="2" |Microscopic Histopathology
|Fibrillary astrocytoma(40%)
|-
|[[Pilocytic astrocytoma]](60%)
|-
| rowspan="2" |Association with Neurofibromatosis type 1
|Optic nerve gliomas associated with [[neurofibromatosis type 1]]
|-
|Optic nerve gliomas not associated with [[neurofibromatosis type 1]]
|-
| rowspan="2" |Anatomic location
|Anterior visual pathway
 
*Orbital
*Intracanalicular
*Intracranial prechiasmal lesions.
|-
|Posterior visual pathway
 
*[[Optic chiasm]]
*[[Hypothalamus]]
*Anterior third [[ventricle]]
|}
 
==Classification based on Microscopic Histopathology==
 
*Optic pathway gliomas are classified according to WHO classification into two subtypes:
 
:*[[Pilocytic astrocytoma]](60%)
 
:*Fibrillary astrocytoma(40%)
==Classification based on Association with Neurofibromatosis type 1==
*Based on whether or not they are associated with [[neurofibromatosis type 1]], they may be classified into two subtypes:
 
:*Optic nerve gliomas associated with [[neurofibromatosis type 1]]
 
:*Optic nerve gliomas not associated with [[neurofibromatosis type 1]]
[[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>  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.


Anterior visual pathway — Anterior tumors can be subdivided into:
==Classification based on Anatomic location==
*According to their location along the optic pathway, the two major categories are:
===Anterior visual pathway===
*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>
===Posterior visual pathway===
*[[Optic chiasm]]
*[[Hypothalamus]]
*Anterior third [[ventricle]]
 
<br />
 
* 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>


Posterior visual pathway — Posterior tumors may arise in the
* 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.
*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.
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.
In approximately 50% of patients, an OPG occurs in isolation, without any known chromosomal aberration; however, in the remaining 50% of patients, a diagnosis of von Recklinghausen disease is seen. Von Recklinghausen disease, also known as Neurofibromatosis Type I (NF1), is an autosomal dominant disorder with an incidence of one in 3000-5000 births, but approximately 50% of cases are spontaneous mutations. When associated with NF1, most OPGs are benign, and can be multifocal and bilateral in nature and usually confined to the optic nerves. If a child has bilateral tumors, then it is very likely that the child has NF-1.
OPGs are the most common central nervous system tumors associated with NF1. If all children underwent screening, estimates indicate that OPGs would be found in 15 percent of those with NF1 [7,8]. NF1 tumors more commonly involve the anterior visual pathway, while tumors in NF1 negative children are more frequent in the posterior visual pathway [9]. OPGs have also been described in patients with NF2 25-40% of optic gliomas occur in children with NF-1, and of children with NF-1, about 10% develop optic gliomas.


This classification correlates highly with:
==References==
{{reflist|2}}
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The appropriate treatment for the disease
[[Category:Neurology]]
Symptoms associated with the tumor
[[Category:Ophthalmology]]
Patient prognosis
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Ophthalmology]]
[[Category:Neurosurgery]]

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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 glioma may be classified into several subtypes based on microscopic histopathology, anatomic location, and association with neurofibromatosis type 1. Optic pathway gliomas are classified according to WHO classification into two subtypes: pilocytic astrocytoma and fibrillary astrocytoma. Optic nerve gliomas may be classified according to anatomic location into two subtypes: anterior visual pathway tumors and posterior visual pathway tumors. Based on whether or not they are associated with neurofibromatosis type 1, they may be classified into two subtypes: optic nerve gliomas associated with neurofibromatosis type 1 and optic nerve gliomas not associated with neurofibromatosis type 1.

Classification

  • Optic nerve glioma may be classified into several subtypes based on microscopic histopathology, anatomic location, and association with neurofibromatosis type 1.


Classifications of Optic Nerve Glioma
Microscopic Histopathology Fibrillary astrocytoma(40%)
Pilocytic astrocytoma(60%)
Association with Neurofibromatosis type 1 Optic nerve gliomas associated with neurofibromatosis type 1
Optic nerve gliomas not associated with neurofibromatosis type 1
Anatomic location Anterior visual pathway
  • Orbital
  • Intracanalicular
  • Intracranial prechiasmal lesions.
Posterior visual pathway

Classification based on Microscopic Histopathology

  • Optic pathway gliomas are classified according to WHO classification into two subtypes:
  • Fibrillary astrocytoma(40%)

Classification based on Association with Neurofibromatosis type 1

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.[1] In children diagnosed with NF-1, approximately 15% develop optic gliomas.[2] [3] Twenty to forty percent of optic gliomas occur in children with NF-1.

Classification based on Anatomic location

  • 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.[4]

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.[5]
  • 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.

References

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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.

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