Optic nerve glioma natural history, complications and prognosis: Difference between revisions

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==Overview==
==Overview==
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3]. Common complications of [disease name] include [complication 1], [complication 2], and [complication 3]. Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
If left untreated, less than 5% of patients with optic nerve gliomas may progress to develop [[blindness]]. The clinical course of optic nerve gliomas is unpredictable.<ref name="pmid9135269">{{cite journal| author=Shuper A, Horev G, Kornreich L, Michowiz S, Weitz R, Zaizov R et al.| title=Visual pathway glioma: an erratic tumour with therapeutic dilemmas. | journal=Arch Dis Child | year= 1997 | volume= 76 | issue= 3 | pages= 259-63 | pmid=9135269 | doi= | pmc=PMC1717103 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9135269  }} </ref>  Common complications of optic nerve glioma include decreased vision, [[blindness]], [[growth hormone deficiency]], [[precocious puberty]], and [[hydrocephalus]]. Prognosis is generally good in most patients with optic pathway gliomas. Most optic nerve gliomas are [[benign]] and produce slowly progressive visual loss associated with variable [[proptosis]] and anterior or posterior [[optic neuropathy]].


==Natural History==
*Optic nerve gliomas have an unpredictable natural history.<ref name="pmid19433276">{{cite journal| author=Schupper A, Kornreich L, Yaniv I, Cohen IJ, Shuper A| title=Optic-pathway glioma: natural history demonstrated by a new empirical score. | journal=Pediatr Neurol | year= 2009 | volume= 40 | issue= 6 | pages= 432-6 | pmid=19433276 | doi=10.1016/j.pediatrneurol.2008.12.015 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19433276  }} </ref>
*The majority of the optic nerve gliomas grow slowly in a self limited manner and some of them spontaneously regress.<ref name="pmid11296017">{{cite journal| author=Parsa CF, Hoyt CS, Lesser RL, Weinstein JM, Strother CM, Muci-Mendoza R et al.| title=Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging. | journal=Arch Ophthalmol | year= 2001 | volume= 119 | issue= 4 | pages= 516-29 | pmid=11296017 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11296017  }} </ref>
*If left untreated, less than 5 percentage of patients with optic nerve gliomas may progress to develop [[blindness]]. Significant [[visual impairment]] is  frequently present in [[neurofibromatosis type 1]] patients with optic nerve gliomas.<ref name="pmid17704361">{{cite journal| author=Dalla Via P, Opocher E, Pinello ML, Calderone M, Viscardi E, Clementi M et al.| title=Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program. | journal=Neuro Oncol | year= 2007 | volume= 9 | issue= 4 | pages= 430-7 | pmid=17704361 | doi=10.1215/15228517-2007-031 | pmc=PMC1994100 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17704361  }} </ref><ref name="pmid20400294">{{cite journal| author=Moreno L, Bautista F, Ashley S, Duncan C, Zacharoulis S| title=Does chemotherapy affect the visual outcome in children with optic pathway glioma? A systematic review of the evidence. | journal=Eur J Cancer | year= 2010 | volume= 46 | issue= 12 | pages= 2253-9 | pmid=20400294 | doi=10.1016/j.ejca.2010.03.028 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20400294  }} </ref>
*These tumors demonstrate variable clinical and radiological progression. In patients with neurofibromatosis type 1, it is not unusual for these tumors to be quiescent, with little progression demonstrated over a number of years. In others, the tumors are more aggressive with extension along the optic pathways.<ref name="radio">  Optic nerve glioma. Radiopedia(2015) http://radiopaedia.org/articles/optic-nerve-glioma Accessed on October 5 2015</ref>
*Normally, the growth of the tumor is very slow, and the condition remains stable for long periods of time. However, in adults and some children, when the optic chiasm is involved, the tumor is more aggressive.
*In adults, the clinical course of an optic nerve gliomas resembles the behavior of the tumors's histology, which is commonly a malignant glioma. [[Malignant]] optic nerve gliomas can result in rapidly progressive visual loss. Rapid  onset blindness is seen in elderly and middle-aged individuals.<ref name="pmid7469853">{{cite journal| author=Spoor TC, Kennerdell JS, Zorub D, Martinez AJ| title=Progressive visual loss due to glioblastoma: normal neuroroentgenorgraphic studies. | journal=Arch Neurol | year= 1981 | volume= 38 | issue= 3 | pages= 196-7 | pmid=7469853 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7469853  }} </ref><ref name="pmid6243868">{{cite journal| author=Spoor TC, Kennerdell JS, Martinez AJ, Zorub D| title=Malignant gliomas of the optic nerve pathways. | journal=Am J Ophthalmol | year= 1980 | volume= 89 | issue= 2 | pages= 284-92 | pmid=6243868 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6243868  }} </ref><ref name="pmid10536925">{{cite journal| author=Dario A, Iadini A, Cerati M, Marra A| title=Malignant optic glioma of adulthood. Case report and review of the literature. | journal=Acta Neurol Scand | year= 1999 | volume= 100 | issue= 5 | pages= 350-3 | pmid=10536925 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10536925  }} </ref>
*Death can occur with in few months of presentation if tumor invasion into adjacent brain occurs.<ref name="pmid2643688">{{cite journal| author=Taphoorn MJ, de Vries-Knoppert WA, Ponssen H, Wolbers JG| title=Malignant optic glioma in adults. Case report. | journal=J Neurosurg | year= 1989 | volume= 70 | issue= 2 | pages= 277-9 | pmid=2643688 | doi=10.3171/jns.1989.70.2.0277 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2643688  }} </ref>


==Natural History==
Optic nerve gliomas have an unpredictable natural history.
==Complications==
==Complications==
Complications of optic nerve gliomas include:
*Decreased vision
*Decreased vision
*Blindness
*[[Blindness]]
*Hydrocephalus
*[[Hydrocephalus]]
*Growth horme deficiency
*[[Growth hormone deficiency]]
*Precocious puberty
*[[Precocious puberty]]
Vision may be seriously impaired in patients with optic nerve gliomas.<ref name="pmid7842408">{{cite journal| author=Janss AJ, Grundy R, Cnaan A, Savino PJ, Packer RJ, Zackai EH et al.| title=Optic pathway and hypothalamic/chiasmatic gliomas in children younger than age 5 years with a 6-year follow-up. | journal=Cancer | year= 1995 | volume= 75 | issue= 4 | pages= 1051-9 | pmid=7842408 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7842408  }} </ref><ref name="pmid7674005">{{cite journal| author=Sutton LN, Molloy PT, Sernyak H, Goldwein J, Phillips PL, Rorke LB et al.| title=Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery. | journal=J Neurosurg | year= 1995 | volume= 83 | issue= 4 | pages= 583-9 | pmid=7674005 | doi=10.3171/jns.1995.83.4.0583 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7674005  }} </ref><ref name="pmid17704361">{{cite journal| author=Dalla Via P, Opocher E, Pinello ML, Calderone M, Viscardi E, Clementi M et al.| title=Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program. | journal=Neuro Oncol | year= 2007 | volume= 9 | issue= 4 | pages= 430-7 | pmid=17704361 | doi=10.1215/15228517-2007-031 | pmc=PMC1994100 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17704361  }} </ref>. Significant visual impairment is  frequently present in NF-1 patients with optic nerve gliomas.<ref name="pmid17704361">{{cite journal| author=Dalla Via P, Opocher E, Pinello ML, Calderone M, Viscardi E, Clementi M et al.| title=Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program. | journal=Neuro Oncol | year= 2007 | volume= 9 | issue= 4 | pages= 430-7 | pmid=17704361 | doi=10.1215/15228517-2007-031 | pmc=PMC1994100 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17704361  }} </ref><ref name="pmid20400294">{{cite journal| author=Moreno L, Bautista F, Ashley S, Duncan C, Zacharoulis S| title=Does chemotherapy affect the visual outcome in children with optic pathway glioma? A systematic review of the evidence. | journal=Eur J Cancer | year= 2010 | volume= 46 | issue= 12 | pages= 2253-9 | pmid=20400294 | doi=10.1016/j.ejca.2010.03.028 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20400294  }} </ref> In adults the clinical course of an optic nerve gliomas resembles the behavior of the tumors's histology, which is commonly a malignant glioma. Malignant optic nerve gliomas can result in rapidly progressive visual loss. Rapid  onset blindness is seen in elderly and middle-aged individuals.<ref name="pmid7469853">{{cite journal| author=Spoor TC, Kennerdell JS, Zorub D, Martinez AJ| title=Progressive visual loss due to glioblastoma: normal neuroroentgenorgraphic studies. | journal=Arch Neurol | year= 1981 | volume= 38 | issue= 3 | pages= 196-7 | pmid=7469853 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7469853  }} </ref><ref name="pmid6243868">{{cite journal| author=Spoor TC, Kennerdell JS, Martinez AJ, Zorub D| title=Malignant gliomas of the optic nerve pathways. | journal=Am J Ophthalmol | year= 1980 | volume= 89 | issue= 2 | pages= 284-92 | pmid=6243868 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6243868  }} </ref><ref name="pmid10536925">{{cite journal| author=Dario A, Iadini A, Cerati M, Marra A| title=Malignant optic glioma of adulthood. Case report and review of the literature. | journal=Acta Neurol Scand | year= 1999 | volume= 100 | issue= 5 | pages= 350-3 | pmid=10536925 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10536925  }} </ref> Death can occur with in few months of presentation if tumor invasion into adjacent brain occurs.<ref name="pmid2643688">{{cite journal| author=Taphoorn MJ, de Vries-Knoppert WA, Ponssen H, Wolbers JG| title=Malignant optic glioma in adults. Case report. | journal=J Neurosurg | year= 1989 | volume= 70 | issue= 2 | pages= 277-9 | pmid=2643688 | doi=10.3171/jns.1989.70.2.0277 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2643688  }} </ref>
 
==Prognosis==
==Prognosis==
These tumours demonstrate variable clinical and radiological progression. In patients with NF1 it is not unusual for these tumours to be quiescent, with little progression demonstrated over a number of years. In others, the tumours are more aggressive with extension along the optic pathways.<ref>{{Cite web | title =Radiopedia optic  nerve glioma prognosis | url =http://radiopaedia.org/articles/optic-nerve-glioma }}</ref> Normally, the growth of the tumor is very slow, and the condition remains stable for long periods of time. However, in adults and some children, when the optic chiasm is involved, the tumor is more aggressive.<ref>{{Cite web | title =NIH optic  nerve glioma prognosis | url =https://www.nlm.nih.gov/medlineplus/ency/article/001024.htm }}</ref> There is nearly 90 percent survival rate for optic pathway gliomas. Children with neurofibromatosis and older children have a better prognosis. Spontaneous remission of the optic pathway gliomas is seen in two-thirds of children with NF-1. The clinical course of optic nerve gliomas is unpredictable.<ref name="pmid9135269">{{cite journal| author=Shuper A, Horev G, Kornreich L, Michowiz S, Weitz R, Zaizov R et al.| title=Visual pathway glioma: an erratic tumour with therapeutic dilemmas. | journal=Arch Dis Child | year= 1997 | volume= 76 | issue= 3 | pages= 259-63 | pmid=9135269 | doi= | pmc=PMC1717103 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9135269 }} </ref> Metastatic dissemination through ventriculoperitoneal shunts , malignant degeneration, and spontaneous regression have all been reported. In adults the optic nerve gliomas tend to be histologically anaplastic and display aggressive clinical course.<ref name="pmid4707622">{{cite journal| author=Hamilton AM, Garner A, Tripathi RC, Sanders MD| title=Malignant optic nerve glioma. Report of a case with electron microscope study. | journal=Br J Ophthalmol | year= 1973 | volume= 57 | issue= 4 | pages= 253-64 | pmid=4707622 | doi= | pmc=PMC1214877 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4707622  }} </ref><ref name="pmid4361475">{{cite journal| author=Gibberd FB, Miller TN, Morgan AD| title=Glioblastoma of the optic chiasm. | journal=Br J Ophthalmol | year= 1973 | volume= 57 | issue= 10 | pages= 788-91 | pmid=4361475 | doi= | pmc=PMC1215165 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4361475  }} </ref><ref name="pmid4695718">{{cite journal| author=Hoyt WF, Meshel LG, Lessell S, Schatz NJ, Suckling RD| title=Malignant optic glioma of adulthood. | journal=Brain | year= 1973 | volume= 96 | issue= 1 | pages= 121-32 | pmid=4695718 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4695718  }} </ref> In children, in contrast the variable clinical course of OPGs is more a function of location than specific histology.<ref name="pmid7066671">{{cite journal| author=Borit A, Richardson EP| title=The biological and clinical behaviour of pilocytic astrocytomas of the optic pathways. | journal=Brain | year= 1982 | volume= 105 | issue= Pt 1 | pages= 161-87 | pmid=7066671 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7066671  }} </ref><ref name="pmid6818504">{{cite journal| author=Rush JA, Younge BR, Campbell RJ, MacCarty CS| title=Optic glioma. Long-term follow-up of 85 histopathologically verified cases. | journal=Ophthalmology | year= 1982 | volume= 89 | issue= 11 | pages= 1213-9 | pmid=6818504 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6818504  }} </ref>
*There is nearly 90 percent survival rate for all optic pathway gliomas whether those with or without neurofibromatosis type 1.  
Excellent prognosis with a median survival of more than 15 years is seen in children with intracranial and intraorbital tumors.<ref name="pmid3275755">{{cite journal| author=Alvord EC, Lofton S| title=Gliomas of the optic nerve or chiasm. Outcome by patients' age, tumor site, and treatment. | journal=J Neurosurg | year= 1988 | volume= 68 | issue= 1 | pages= 85-98 | pmid=3275755 | doi=10.3171/jns.1988.68.1.0085 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3275755  }} </ref> Patients with chiasmal gliomas have usually less favorable prognosis. Better prognosis is seen in patients with optic nerve gliomas associated with neurofibromatosis type 1, due to predominance of anterior lesions. Patients with neurofibromatosis type 1 have appoximately twice the recurrence rate following complete excision of an intraorbital glioma as compared with patients without NF-1. However, similar prognosis following radiation of a optic nerve gliomas has been seen in patients with NF-1, as compared to patients without NF-1.<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="pmid2497236">{{cite journal| author=Listernick R, Charrow J, Greenwald MJ, Esterly NB| title=Optic gliomas in children with neurofibromatosis type 1. | journal=J Pediatr | year= 1989 | volume= 114 | issue= 5 | pages= 788-92 | pmid=2497236 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2497236  }} </ref><ref name="pmid8727140">{{cite journal| author=Deliganis AV, Geyer JR, Berger MS| title=Prognostic significance of type 1 neurofibromatosis (von Recklinghausen Disease) in childhood optic glioma. | journal=Neurosurgery | year= 1996 | volume= 38 | issue= 6 | pages= 1114-8; discussion 1118-9 | pmid=8727140 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8727140  }} </ref>
*Children with neurofibromatosis and older children have a better prognosis.  
*In patients with optic nerve gliomas associated with neurofibromatosis type 1, beter prognosis is seen.
*Spontaneous remission of the optic pathway gliomas is seen in two-thirds of children with neurofibromatosis type 1.   
*[[Metastatic]] dissemination through ventriculoperitoneal [[shunts]], [[malignant]] degeneration, and spontaneous regression have all been reported.
*Patients with chiasmal gliomas have usually less favorable prognosis.  
*Patients with neurofibromatosis type 1 have appoximately twice the recurrence rate following complete excision of an intraorbital glioma as compared with patients without neurofibromatosis type 1.  
*Approximately sixty percentage of children treated with chemotherapy for gliomas of the hypothalamus and optic pathways had a relapse.<ref name="pmid7842408">{{cite journal| author=Janss AJ, Grundy R, Cnaan A, Savino PJ, Packer RJ, Zackai EH et al.| title=Optic pathway and hypothalamic/chiasmatic gliomas in children younger than age 5 years with a 6-year follow-up. | journal=Cancer | year= 1995 | volume= 75 | issue= 4 | pages= 1051-9 | pmid=7842408 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7842408 }} </ref>
 
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
|valign=top|
|+
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Prognosis}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Age at onset}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Clinical features}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Radiographic location}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
:Favorable prognosis
| style="padding: 5px 5px; background: #F5F5F5;" | Early childhood to adolescence
| style="padding: 5px 5px; background: #F5F5F5;" |
*Visual loss with laterality
*Slowly progressive or arrested course
*Incidental finding in child with neurofibromatosis
*No symptoms of endocrine dysfunction or hydrocephalus
*Does not have diencephalic syndrome
| style="padding: 5px 5px; background: #F5F5F5;" |
*Intrinsic optic nerve and/or chiasmal location
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
:Poor Prognosis
| style="padding: 5px 5px; background: #F5F5F5;" |Infancy to early childhood and adulthood
| style="padding: 5px 5px; background: #F5F5F5;" |
*Hypothalamic symptoms and/or signs of increased intracranial pressure
*Severely affected vision in both eyes
| style="padding: 5px 5px; background: #F5F5F5;" |
*Large exophytic chiasmal tumor with posterior extension
*Extension into third ventricle
|-
|}


==References==
==References==
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[[Category:Neurology]]
[[Category:Neurology]]
[[Category:Ophthalmology]]
[[Category:Ophthalmology]]
[[Category:Needs content]]

Latest revision as of 07:00, 10 October 2015

Optic nerve glioma Microchapters

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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

If left untreated, less than 5% of patients with optic nerve gliomas may progress to develop blindness. The clinical course of optic nerve gliomas is unpredictable.[1] Common complications of optic nerve glioma include decreased vision, blindness, growth hormone deficiency, precocious puberty, and hydrocephalus. Prognosis is generally good in most patients with optic pathway gliomas. Most optic nerve gliomas are benign and produce slowly progressive visual loss associated with variable proptosis and anterior or posterior optic neuropathy.

Natural History

  • Optic nerve gliomas have an unpredictable natural history.[2]
  • The majority of the optic nerve gliomas grow slowly in a self limited manner and some of them spontaneously regress.[3]
  • If left untreated, less than 5 percentage of patients with optic nerve gliomas may progress to develop blindness. Significant visual impairment is frequently present in neurofibromatosis type 1 patients with optic nerve gliomas.[4][5]
  • These tumors demonstrate variable clinical and radiological progression. In patients with neurofibromatosis type 1, it is not unusual for these tumors to be quiescent, with little progression demonstrated over a number of years. In others, the tumors are more aggressive with extension along the optic pathways.[6]
  • Normally, the growth of the tumor is very slow, and the condition remains stable for long periods of time. However, in adults and some children, when the optic chiasm is involved, the tumor is more aggressive.
  • In adults, the clinical course of an optic nerve gliomas resembles the behavior of the tumors's histology, which is commonly a malignant glioma. Malignant optic nerve gliomas can result in rapidly progressive visual loss. Rapid onset blindness is seen in elderly and middle-aged individuals.[7][8][9]
  • Death can occur with in few months of presentation if tumor invasion into adjacent brain occurs.[10]

Complications

Complications of optic nerve gliomas include:

Prognosis

  • There is nearly 90 percent survival rate for all optic pathway gliomas whether those with or without neurofibromatosis type 1.
  • Children with neurofibromatosis and older children have a better prognosis.
  • In patients with optic nerve gliomas associated with neurofibromatosis type 1, beter prognosis is seen.
  • Spontaneous remission of the optic pathway gliomas is seen in two-thirds of children with neurofibromatosis type 1.
  • Metastatic dissemination through ventriculoperitoneal shunts, malignant degeneration, and spontaneous regression have all been reported.
  • Patients with chiasmal gliomas have usually less favorable prognosis.
  • Patients with neurofibromatosis type 1 have appoximately twice the recurrence rate following complete excision of an intraorbital glioma as compared with patients without neurofibromatosis type 1.
  • Approximately sixty percentage of children treated with chemotherapy for gliomas of the hypothalamus and optic pathways had a relapse.[11]
Prognosis Age at onset Clinical features Radiographic location
Favorable prognosis
 Early childhood to adolescence
  • Visual loss with laterality
  • Slowly progressive or arrested course
  • Incidental finding in child with neurofibromatosis
  • No symptoms of endocrine dysfunction or hydrocephalus
  • Does not have diencephalic syndrome
  • Intrinsic optic nerve and/or chiasmal location
Poor Prognosis
 Infancy to early childhood and adulthood
  • Hypothalamic symptoms and/or signs of increased intracranial pressure
  • Severely affected vision in both eyes
  • Large exophytic chiasmal tumor with posterior extension
  • Extension into third ventricle

References

  1. ↑ Shuper A, Horev G, Kornreich L, Michowiz S, Weitz R, Zaizov R; et al. (1997). "Visual pathway glioma: an erratic tumour with therapeutic dilemmas". Arch Dis Child. 76 (3): 259–63. PMC 1717103. PMID 9135269.
  2. ↑ Schupper A, Kornreich L, Yaniv I, Cohen IJ, Shuper A (2009). "Optic-pathway glioma: natural history demonstrated by a new empirical score". Pediatr Neurol. 40 (6): 432–6. doi:10.1016/j.pediatrneurol.2008.12.015. PMID 19433276.
  3. ↑ Parsa CF, Hoyt CS, Lesser RL, Weinstein JM, Strother CM, Muci-Mendoza R; et al. (2001). "Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging". Arch Ophthalmol. 119 (4): 516–29. PMID 11296017.
  4. ↑ Dalla Via P, Opocher E, Pinello ML, Calderone M, Viscardi E, Clementi M; et al. (2007). "Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program". Neuro Oncol. 9 (4): 430–7. doi:10.1215/15228517-2007-031. PMC 1994100. PMID 17704361.
  5. ↑ Moreno L, Bautista F, Ashley S, Duncan C, Zacharoulis S (2010). "Does chemotherapy affect the visual outcome in children with optic pathway glioma? A systematic review of the evidence". Eur J Cancer. 46 (12): 2253–9. doi:10.1016/j.ejca.2010.03.028. PMID 20400294.
  6. ↑ Optic nerve glioma. Radiopedia(2015) http://radiopaedia.org/articles/optic-nerve-glioma Accessed on October 5 2015
  7. ↑ Spoor TC, Kennerdell JS, Zorub D, Martinez AJ (1981). "Progressive visual loss due to glioblastoma: normal neuroroentgenorgraphic studies". Arch Neurol. 38 (3): 196–7. PMID 7469853.
  8. ↑ Spoor TC, Kennerdell JS, Martinez AJ, Zorub D (1980). "Malignant gliomas of the optic nerve pathways". Am J Ophthalmol. 89 (2): 284–92. PMID 6243868.
  9. ↑ Dario A, Iadini A, Cerati M, Marra A (1999). "Malignant optic glioma of adulthood. Case report and review of the literature". Acta Neurol Scand. 100 (5): 350–3. PMID 10536925.
  10. ↑ Taphoorn MJ, de Vries-Knoppert WA, Ponssen H, Wolbers JG (1989). "Malignant optic glioma in adults. Case report". J Neurosurg. 70 (2): 277–9. doi:10.3171/jns.1989.70.2.0277. PMID 2643688.
  11. ↑ Janss AJ, Grundy R, Cnaan A, Savino PJ, Packer RJ, Zackai EH; et al. (1995). "Optic pathway and hypothalamic/chiasmatic gliomas in children younger than age 5 years with a 6-year follow-up". Cancer. 75 (4): 1051–9. PMID 7842408.

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