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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 History==
=== 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>


==Complications==
*[[Glaucoma]]
Complications of eye melanoma may include:
*[[Cataract]]
===Glaucoma===
*[[Anterior chamber]] [[hemorrhage]]
Eye melanoma that is growing may cause glaucoma. Signs and symptoms of glaucoma may include the following:
*[[Corneal]] decompensation with [[edema]] and band [[keratopathy]]
*Eye pain
*[[Metastasis]]
*Redness
**Eye melanoma can spread outside of the [[Eye-eating amoebas|eye]] and to distant areas of the [[body]], including the [[liver]], [[lungs]] and [[bones]].
*Blurry vision
*[[Vision loss]]
===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.


===Metastasis===
:* 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]].
Eye melanoma can spread outside of the eye and to distant areas of the body, including the liver, lungs and bones.
:* 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==
===Prognosis===
Poor prognostic factors for systemic disease include:<ref name="radio">  Uveal melanoma. Radiopedia(2015) http://radiopaedia.org/articles/primary-uveal-malignant-melanoma Accessed on October 21, 2015</ref>
*Older age: >60 years of age
*Larger tumors
*Anterior location within the globe
*Epithelioid cells (i.e. mixed or epithelioid histology)
*Extraocular extension
*Systemic metastases may be wide spread, with the liver > lung > bone > kidney > brain being a common sites for tumor deposits.
*Cell type is the most commonly used predictor of outcome following enucleation, with spindle-A cell melanomas carrying the best prognosis and epithelioid cell melanomas carrying the least favorable prognosis. Nevertheless, most tumors have an admixture of cell types, and there is no clear consensus regarding the proportion of epithelioid cells that constitutes designation of a tumor as mixed or epithelioid.
*Overall survival depends on tumour 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.
*Several additional microscopic features can affect the prognosis of intraocular melanoma, including:<ref name="NIH">  Uveal melanoma. National Cancer Institute(2015) http://www.cancer.gov/types/eye/hp/intraocular-melanoma-treatment-pdq#link/_304_toc Accessed on October 23, 2015</ref>
**Mitotic activity.
**Lymphocytic infiltration.
**Fibrovascular loops
**Extraocular extension, recurrence, and metastasis are associated with an extremely poor prognosis, and long-term survival cannot be expected.
**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.
Uveal melanomas arise from melanocytes in the uvea. These melanomas have significant differences from cutaneous melanomas that guide their management [1].
 
Uveal melanomas can develop in any part of the uveal tract, including the iris, ciliary body, and choroid. Iris melanomas are less common than choroidal melanomas (approximately 5 versus more than 80 percent of cases). Iris melanomas are also associated with a better prognosis than ciliochoroidal tumors (10 year-survival approximately 95 versus 77 percent). Because of the less aggressive nature of iris melanomas, these tumors are usually managed conservatively (ie, close monitoring of the tumor). Fast-growing tumors may be excised. This topic will emphasize the management of uveal melanoma as it pertains to choroidal and ciliary body melanoma, the more common and life-threatening forms of the disease.


*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 ==  
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Latest revision as of 03:25, 15 August 2019

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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

Complications

Complications of eye melanoma include the following:[3]

Complications of therapy of uveal melanoma

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.

References

  1. ↑ Wöll E, Bedikian A, Legha SS (1999). "Uveal melanoma: natural history and treatment options for metastatic disease". Melanoma Res. 9 (6): 575–81. PMID 10661768.
  2. ↑ Jovanovic P, Mihajlovic M, Djordjevic-Jocic J, Vlajkovic S, Cekic S, Stefanovic V (2013). "Ocular melanoma: an overview of the current status". Int J Clin Exp Pathol. 6 (7): 1230–44. PMC 3693189. PMID 23826405.
  3. ↑ Singh, Arun D., and Bertil Damato. Clinical ophthalmic oncology : uveal tumors. Heidelberg: Springer, 2014. Print.
  4. ↑ Summanen, P; Immonen, I; Kivela, T; Tommila, P; Heikkonen, J; Tarkkanen, A (1996). "Radiation related complications after ruthenium plaque radiotherapy of uveal melanoma". British Journal of Ophthalmology. 80 (8): 732–739. doi:10.1136/bjo.80.8.732. ISSN 0007-1161.
  5. ↑ Shields, CarolL; Mellen, PhoebeL; Morton, SpenserJ (2013). "American joint committee on cancer staging of uveal melanoma". Oman Journal of Ophthalmology. 6 (2): 116. doi:10.4103/0974-620X.116652. ISSN 0974-620X.
  6. ↑ Shields CL, Kaliki S, Furuta M, Fulco E, Alarcon C, Shields JA (2013). "American Joint Committee on Cancer classification of posterior uveal melanoma (tumor size category) predicts prognosis in 7731 patients". Ophthalmology. 120 (10): 2066–71. doi:10.1016/j.ophtha.2013.03.012. PMID 23664467.
  7. ↑ Egger E, Zografos L, Schalenbourg A, Beati D, Böhringer T, Chamot L; et al. (2003). "Eye retention after proton beam radiotherapy for uveal melanoma". Int J Radiat Oncol Biol Phys. 55 (4): 867–80. PMID 12605964.
  8. ↑ Egan KM, Ryan LM, Gragoudas ES (1998). "Survival implications of enucleation after definitive radiotherapy for choroidal melanoma: an example of regression on time-dependent covariates". Arch Ophthalmol. 116 (3): 366–70. PMID 9514491.
  9. ↑ Schuster R, Bechrakis NE, Stroux A, Busse A, Schmittel A, Scheibenbogen C; et al. (2007). "Circulating tumor cells as prognostic factor for distant metastases and survival in patients with primary uveal melanoma". Clin Cancer Res. 13 (4): 1171–8. doi:10.1158/1078-0432.CCR-06-2329. PMID 17317826.
  10. ↑ Boldin I, Langmann G, Richtig E, Schwantzer G, Ardjomand N, Wegscheider B; et al. (2005). "Five-year results of prognostic value of tyrosinase in peripheral blood of uveal melanoma patients". Melanoma Res. 15 (6): 503–7. PMID 16314735.

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