Uveal melanoma natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
If left untreated, 50% of patients with uveal melanoma may progress to develop [[metastasis]]. Common [[complications]] of uveal melanoma include [[glaucoma]], [[vision loss]], and [[metastasis]]. [[Prognosis]] is generally good for [[Patient|patients]] with [[iris]] [[melanoma]], and the 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal [[tumors]] is 77 percent respectively. | |||
==Natural History, Complications, and Prognosis== | |||
==Natural | === Natural history === | ||
== | * The clinical course is unpredictable and [[metastatic]] [[disease]] can develop very late after a long [[disease]]-free interval. <ref name="pmid10661768">{{cite journal| author=Wöll E, Bedikian A, Legha SS| title=Uveal melanoma: natural history and treatment options for metastatic disease. | journal=Melanoma Res | year= 1999 | volume= 9 | issue= 6 | pages= 575-81 | pmid=10661768 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10661768 }} </ref><ref name="pmid23826405">{{cite journal| author=Jovanovic P, Mihajlovic M, Djordjevic-Jocic J, Vlajkovic S, Cekic S, Stefanovic V| title=Ocular melanoma: an overview of the current status. | journal=Int J Clin Exp Pathol | year= 2013 | volume= 6 | issue= 7 | pages= 1230-44 | pmid=23826405 | doi= | pmc=PMC3693189 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23826405 }} </ref> | ||
*[[Uvea (anatomy)|Uveal]] melanoma [[Metastasis|metastasizes]] haematogenously, predominantly to the [[liver]]. | |||
=== | * Less than 4% of [[Patient|patients]] with uveal melanoma have detectable [[metastatic]] [[disease]], at the time of [[diagnosis]]. | ||
* However in further course about half of [[Patient|patients]] will develop [[Metastasis|metastases]]. | |||
=== | * When [[metastatic]] disease appears it unavoidably leads to death because of lack of effective systemic treatment. | ||
===Complications=== | |||
[[Complications]] of eye [[melanoma]] include the following:<ref>Singh, Arun D., and Bertil Damato. Clinical ophthalmic oncology : uveal tumors. Heidelberg: Springer, 2014. Print.</ref> | |||
=== | |||
*[[Glaucoma]] | |||
*[[Cataract]] | |||
*[[Anterior chamber]] [[hemorrhage]] | |||
*[[Corneal]] decompensation with [[edema]] and band [[keratopathy]] | |||
*[[Metastasis]] | |||
**Eye melanoma can spread outside of the [[Eye-eating amoebas|eye]] and to distant areas of the [[body]], including the [[liver]], [[lungs]] and [[bones]]. | |||
*[[Vision loss]] | |||
:* Eye melanomas that are large often cause [[vision loss]] in the affected [[Acanthamoeba|eye]] and may cause [[complications]], such as [[retinal detachment]], that also cause [[vision loss]]. | |||
:* If small [[eye]] melanomas occur in critical parts of the eye they may cause some [[vision loss]]. | |||
:* There may be difficulty seeing in the center of the [[vision]] or on the side. | |||
:* Advanced eye melanomas can cause complete [[vision loss]]. | |||
===Complications of therapy of uveal melanoma=== | |||
*[[Complications]] of [[radiotherapy]] include the following:<ref name="SummanenImmonen1996">{{cite journal|last1=Summanen|first1=P|last2=Immonen|first2=I|last3=Kivela|first3=T|last4=Tommila|first4=P|last5=Heikkonen|first5=J|last6=Tarkkanen|first6=A|title=Radiation related complications after ruthenium plaque radiotherapy of uveal melanoma.|journal=British Journal of Ophthalmology|volume=80|issue=8|year=1996|pages=732–739|issn=0007-1161|doi=10.1136/bjo.80.8.732}}</ref> | |||
**[[Radiation]] [[retinopathy]] | |||
**[[Radiation]] [[maculopathy]] | |||
**[[Radiation]] opticopathy | |||
**[[Maculopathy]] | |||
**[[Cataract]] | |||
**[[Neovascular glaucoma]] | |||
**[[Vitreous]] [[haemorrhage]] | |||
**[[Retinal detachment]] and dryness | |||
**[[Radiation therapy|Radiation]] [[retinopathy]], opticopathy and neovascular [[glaucoma]] are responsible for the majority of secondary [[Vision loss|visual loss]] and secondary enucleations after therapy. | |||
===Prognosis=== | |||
*The 5-year [[mortality rate]] associated with [[metastasis]] from [[ciliary body]] or choroidal melanoma is approximately 30%, compared with a rate of 2% to 3% for [[iris]] [[Melanoma|melanomas]].<ref name="ShieldsMellen2013">{{cite journal|last1=Shields|first1=CarolL|last2=Mellen|first2=PhoebeL|last3=Morton|first3=SpenserJ|title=American joint committee on cancer staging of uveal melanoma|journal=Oman Journal of Ophthalmology|volume=6|issue=2|year=2013|pages=116|issn=0974-620X|doi=10.4103/0974-620X.116652}}</ref><ref name="pmid23664467">{{cite journal| author=Shields CL, Kaliki S, Furuta M, Fulco E, Alarcon C, Shields JA| title=American Joint Committee on Cancer classification of posterior uveal melanoma (tumor size category) predicts prognosis in 7731 patients. | journal=Ophthalmology | year= 2013 | volume= 120 | issue= 10 | pages= 2066-71 | pmid=23664467 | doi=10.1016/j.ophtha.2013.03.012 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23664467 }} </ref> | |||
*Overall [[Survival rate|survival]] depends on [[tumor]] size, extraocular spread, and [[metastases]]. Even small (<10 mm diameter, <3 mm thickness) [[tumors]] still carry a 10-15% 5-year [[Mortality rate|mortality]]. | |||
*The 10 year-[[Survival rate|survival]] for [[iris]] [[melanoma]] is approximately 95 percent and for ciliochoroidal tumors is 77 percent respectively. | |||
*The [[tumor]] node [[metastasis]] (TNM) staging system of the American Joint Committee on Cancer takes into account the key factors involved which are known to be of [[Prognosis|prognostic]] significance. | |||
*The [[prognosis]] is usually poor when local control is not achieved with the initial treatment.<ref name="pmid12605964">{{cite journal| author=Egger E, Zografos L, Schalenbourg A, Beati D, Böhringer T, Chamot L et al.| title=Eye retention after proton beam radiotherapy for uveal melanoma. | journal=Int J Radiat Oncol Biol Phys | year= 2003 | volume= 55 | issue= 4 | pages= 867-80 | pmid=12605964 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12605964 }} </ref><ref name="pmid9514491">{{cite journal| author=Egan KM, Ryan LM, Gragoudas ES| title=Survival implications of enucleation after definitive radiotherapy for choroidal melanoma: an example of regression on time-dependent covariates. | journal=Arch Ophthalmol | year= 1998 | volume= 116 | issue= 3 | pages= 366-70 | pmid=9514491 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9514491 }} </ref> | |||
*Detection of circulating [[tumor]] cells at the time of [[diagnosis]] is an independent [[risk factor]] for relapse and shortened survival in patients at high-risk based upon clinical parameters.<ref name="pmid17317826">{{cite journal| author=Schuster R, Bechrakis NE, Stroux A, Busse A, Schmittel A, Scheibenbogen C et al.| title=Circulating tumor cells as prognostic factor for distant metastases and survival in patients with primary uveal melanoma. | journal=Clin Cancer Res | year= 2007 | volume= 13 | issue= 4 | pages= 1171-8 | pmid=17317826 | doi=10.1158/1078-0432.CCR-06-2329 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17317826 }} </ref><ref name="pmid16314735">{{cite journal| author=Boldin I, Langmann G, Richtig E, Schwantzer G, Ardjomand N, Wegscheider B et al.| title=Five-year results of prognostic value of tyrosinase in peripheral blood of uveal melanoma patients. | journal=Melanoma Res | year= 2005 | volume= 15 | issue= 6 | pages= 503-7 | pmid=16314735 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16314735 }} </ref> | |||
*The [[prognosis]] for any patient with recurring or relapsing disease is poor, regardless of [[cell type]] or stage. | |||
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== References == | == References == | ||
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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, 50% of patients with uveal melanoma may progress to develop metastasis. Common complications of uveal melanoma include glaucoma, vision loss, and metastasis. Prognosis is generally good for patients with iris melanoma, and the 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal tumors is 77 percent respectively.
Natural History, Complications, and Prognosis
Natural history
- The clinical course is unpredictable and metastatic disease can develop very late after a long disease-free interval. [1][2]
- Uveal melanoma metastasizes haematogenously, predominantly to the liver.
- Less than 4% of patients with uveal melanoma have detectable metastatic disease, at the time of diagnosis.
- However in further course about half of patients will develop metastases.
- When metastatic disease appears it unavoidably leads to death because of lack of effective systemic treatment.
Complications
Complications of eye melanoma include the following:[3]
- Glaucoma
- Cataract
- Anterior chamber hemorrhage
- Corneal decompensation with edema and band keratopathy
- Metastasis
- Vision loss
- Eye melanomas that are large often cause vision loss in the affected eye and may cause complications, such as retinal detachment, that also cause vision loss.
- If small eye melanomas occur in critical parts of the eye they may cause some vision loss.
- There may be difficulty seeing in the center of the vision or on the side.
- Advanced eye melanomas can cause complete vision loss.
Complications of therapy of uveal melanoma
- Complications of radiotherapy include the following:[4]
- Radiation retinopathy
- Radiation maculopathy
- Radiation opticopathy
- Maculopathy
- Cataract
- Neovascular glaucoma
- Vitreous haemorrhage
- Retinal detachment and dryness
- Radiation retinopathy, opticopathy and neovascular glaucoma are responsible for the majority of secondary visual loss and secondary enucleations after therapy.
Prognosis
- The 5-year mortality rate associated with metastasis from ciliary body or choroidal melanoma is approximately 30%, compared with a rate of 2% to 3% for iris melanomas.[5][6]
- Overall survival depends on tumor size, extraocular spread, and metastases. Even small (<10 mm diameter, <3 mm thickness) tumors still carry a 10-15% 5-year mortality.
- The 10 year-survival for iris melanoma is approximately 95 percent and for ciliochoroidal tumors is 77 percent respectively.
- The tumor node metastasis (TNM) staging system of the American Joint Committee on Cancer takes into account the key factors involved which are known to be of prognostic significance.
- The prognosis is usually poor when local control is not achieved with the initial treatment.[7][8]
- Detection of circulating tumor cells at the time of diagnosis is an independent risk factor for relapse and shortened survival in patients at high-risk based upon clinical parameters.[9][10]
- The prognosis for any patient with recurring or relapsing disease is poor, regardless of cell type or stage.