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When you see
uveal melanoma,
think
fast

Uveal melanoma (UM) is a rare tumor of the eye^1,2

UM is the most common primary intraocular malignancy³

While UM can arise from melanocytes anywhere in the uveal tract, it most commonly occurs in the choroid.²

Occurrence by site²

Other, less common primary intraocular malignancies include vitreoretinal lymphoma and retinoblastoma.⁴

UM accounts for only

≈4%

of all melanomas.^1,3

≈8,000

people per year diagnosed

with UM globally⁵

1,600-2,000

diagnosed in the US^6,7

≈2,500

diagnosed in the EU27⁸

EU27, the 27 countries constituting the European Union.

Think uveal melanoma brochure

Please download the brochure for more information on uveal melanoma.

Download

Would you recognize a patient at risk?

Risk factors that predispose patients to developing UM include:

Caucasian²
light-colored irides⁹
median age: 62 years, with peak incidence at 55 years^2,10,11,a
BAP1 mutation¹²

^a Patients carrying the BAP1 mutation typically present at a younger age, between 30 and 59 years of age.¹⁰ BAP1, BRCA1-associated protein 1; BRCA1, breast cancer 1.

Additional risk factors may include:

Preexisting medical conditions such as congenital ocular melanocytosis, melanocytoma, neurofibromatosis, and ocular nevi (benign melanocytic lesions that can rarely develop into melanomas).^2,9,13

Symptoms of UM

UM can be asymptomatic or present with a variety of symptoms, making annual dilated eye exams ideal.³

Click below to see how patients may experience symptoms of UM. If your patients are experiencing these symptoms, a closer investigation may be warranted.

Blurred vision

Visual field defects

Photopsia

Metamorphopsia

Other symptoms

Approximately 30% of patients with UM present without symptoms. Patients can also present with headache, experience ocular pain, or report seeing flashes of light. Care must be taken to consider UM as a source of these symptoms, since attributing these symptoms to migraine, sinusitis, dental infection, or emotional distress may result in delayed diagnosis.^3,14

The vast majority of cases, more than 90%, show no signs of systemic disease at diagnosis.¹⁵

Given that patient survival correlates with tumor size and staging, early and accurate diagnosis is particularly important for patient outcomes.^13,16-18

Detecting UM

Watch Bertil Damato, MD, PhD, discuss the importance of systematic examinations.

Detection and diagnosis of UM can be challenging depending on the size, location, and appearance of the lesion²

Location of UM by type

Different tumors

Watch Bertil Damato, MD, PhD, discuss diagnosis and timely referral.

In addition to growth of lesions, ophthalmologists pay especially close attention to other attributes when distinguishing small melanomas from benign lesions, including¹⁹:

thickness
subretinal fluid
orange pigment
tumor margin
ultrasound hollowness
absence of a halo of lighter pigmentation around the growth

Patient treatment and prognosis

Surgery and radiation are the primary treatment options for UM^10,20

Enucleation (removal of the eye) does not seem to improve survival compared with brachytherapy (focal delivery of radiation to the tumor). Therefore, treatment for primary disease has focused on vision- and eye-preserving techniques.¹⁰

Most primary UM tumors are treated with plaque brachytherapy.¹⁰
Surgical approaches include transretinal endoresection and transscleral exoresection (which can still lead to partial sight loss or blindness).^10,21
Other therapies include proton beam radiotherapy, stereotactic radiotherapy, transpupillary thermotherapy, and photodynamic therapy.^10,21
Enucleation is performed in approximately 20% of cases.²²

Although local control of tumors is associated with impressive response rates,

up to 50%

of patients will go on to develop metastatic UM (mUM).^1,10

Sites of metastasis

UM cells spread hematogenously, most commonly metastasizing to the liver and becoming a systemic disease that can also involve the lungs, skin, and bones.^5,23

Studies have indicated that the risk for metastasis varies and is influenced by clinical characteristics of both the patient and the tumor, including^24,25:

somatic mutations in the tumor
histopathological features
tumor diameter and thickness
ciliary body involvement
advanced age
male gender

Need more information?

We’ve gathered some resources to help you assist patients and caregivers.

Resources

References:

Carvajal RD, Schwartz GK, Tezel T, Marr B, Francis JH, Nathan PD. Metastatic disease from uveal melanoma: treatment options and future prospects. Br J Ophthalmol. 2017;101(1):38-44. doi:10.1136/bjophthalmol-201‌6-30‌9034
Krantz BA, Dave N, Komatsubara KM, Marr BP, Carvajal RD. Uveal melanoma: epidemiology, etiology, and treatment of primary disease. Clin Ophthalmol. 2017;11:279-289. doi:10.2147/OPTH.S89591
Mahendraraj K, Lau CSM, Lee I, Chamberlain RS. Trends in incidence, survival, and management of uveal melanoma: a population-based study of 7,516 patients from the Surveillance, Epidemiology, and End Results database (1973–2012). Clin Ophthalmol. Published online October 25, 2016. 2016;10:2‌113-21‌19. doi:10.2147/OPTH.S1‌13623
Nagarkatti-Gude N, Wang Y, Ali MJ, Honavar SG, Jager MJ, Chan CC. Genetics of primary intraocular tumors. Ocul Immunol Inflamm. Published online September 7, 2012. 2012;201(4):244-254. doi:10.3109/09273948.2012.702843
Pandiani C, Béranger GE, Leclerc J, Ballotti R, Bertolotto C. Focus on cutaneous and uveal melanoma specificities. Genes Dev. 2017;31(8):724-743. doi:10.1101/gad.296962.117
Jovanovic P, Mihajlovic M, Djordjevic-Jocic J, Vlajkovic S, Cekic S, Stefanovic V. Ocular melanoma: an overview of the current status. Int J Clin Exp Pathol. 2013;6(7):123‌0-124‌4. Accessed April 14, 2020. http://www.ijcep.com/files/ijcep1304033.pdf
About ocular melanoma. Ocular Melanoma Foundation. Accessed April 14, 2020. http://ww‌w.ocularmelanoma.o‌rg/about-om.h‌tm
Non skin melanoma—malignant melanoma of uvea—tabular results. Search results. RARECARE: Surveillance of Rare Cancers in Europe. Accessed April 14, 2020. http://dcnapp4.dcn.ed.ac.uk/rcnet/searchpage.aspx
Nathan P, Cohen V, Coupland S, et al. Uveal melanoma UK national guidelines. Eur J Cancer. 2015;51(16):2‌404-241‌2. doi:10.1016/j.ejca.2015.07.013
Yang J, Manson DK, Marr BP, Carvajal RD. Treatment of uveal melanoma: where are we now? Ther Adv Med Oncol. 2018;10:175883‌4018757‌175. doi:10.1177/1758‌8340187‌57175
Virgili G, Gatta G, Ciccolallo L, et al; EUROCARE Working Group. Incidence of uveal melanoma in Europe. Ophthalmology. 2007;114(12):23‌09-23‌15, 2315.e1-23‌15.e2. doi:10.1016/j.ophtha.2007.01.032
Barker CA, Salama AK. New NCCN Guidelines for uveal melanoma and treatment of recurrent or progressive distant metastatic melanoma. J Natl Compr Canc Netw. 2018;16(5.5):646-650. doi:10.6004/jnccn.2018.0042
Weis E, Salopek TG, McKinnon JG, et al. Management of uveal melanoma: a consensus-based provincial clinical practice guideline. Curr Oncol. 2016;23(1):e57-e64. doi:10.374‌7/co‌.23.2859
Rishi PR, Shields CL, Khan MA, Patrick K, Shields JA. Headache or eye pain as the presenting feature of uveal melanoma. Ophthalmology. 2013;120(9):1946-1947, 1946.e1-1947.e2. doi:10.10‌16/‌j.ophtha.2013.06.015
Francis JH, Shoushtari AN, Barker CA, Abramson DH. Uveal melanoma: diagnosis, prognosis and current treatments for primary and metastatic disease. Melanoma Lett. 2016;34(3). Accessed December 13, 2018. ht‌tps://ww‌w.skincancer.or‌g/publications/the-mela‌noma-letter/fall-201‌6-vol-34-no-3/uveal-melanoma
Kivelä T, Simpson ER, Grossniklaus HE, et al. Uveal melanoma. In: Amin MB, Edge SB, Greene FL, et al, eds. AJCC Cancer Staging Manual. 8th ed. Springer; 2017:805-817.
Damato B, Eleuteri A, Taktak AFG, Coupland SE. Estimating prognosis for survival after treatment of choroidal melanoma. Prog Retin Eye Res. 2011;30(5):285-295. doi:10.1016/j.preteyeres.2011.05.003
DeParis SW, Taktak A, Eleuteri A, et al. External validation of the Liverpool Uveal Melanoma Prognosticator Online. Invest Ophthalmol Vis Sci. 2016;57(14):6‌116-6‌122. doi:10.1167/iovs.16-19654
Kivelä T. Diagnosis of uveal melanoma. In: Jager MJ, Desjardins L, Kivelä T, Damato BE, eds. Current Concepts in Uveal Melanoma. Karger; 2012. Bandello F, ed. Developments in Ophthalmology; vol 49.
Collaborative Ocular Melanoma Study (COMS) Group. The COMS randomized trial of iodine 125 brachytherapy for choroidal melanoma, V: twelve-year mortality rates and prognostic factors: COMS report no. 28. Arch Ophthalmol. 2006;124:1684-1693, E1-E3. doi:10.1001‌/arc‌hopht.12‌4.12.1684
Singh M, Durairaj P, Yeung J. Uveal melanoma: a review of the literature. Oncol Ther. 2018;6(1):87-104. doi:10.1007‌/s404‌87-0‌18-00‌56-8
Bensoussan E, Thariat J, Maschi C, et al. Outcomes after proton beam therapy for large choroidal melanomas in 492 patients. Am J Ophthalmol. 2016;165:78-87. doi:10.1016/j.‌ajo.201‌6.02.027
Carvajal RD. Management of metastatic uveal melanoma. UpToDate. Updated September 30, 2019. Accessed April 14, 2020. htt‌ps://w‌ww.uptodate.co‌m/contents/management-of-metas‌tatic-uveal-melanoma
Vaquero-Garcia J, Lalonde E, Ewens KG, et al. PRiMeUM: a model for predicting risk of metastasis in uveal melanoma. Invest Ophthalmol Vis Sci. 2017;58(10):40‌96-41‌05. doi:10.‌1167/iovs.1‌7-2‌2255
Corrêa ZM. Assessing prognosis in uveal melanoma. Cancer Control. 2016;23(2):93-98. doi:10.1177/10‌7327‌48‌16‌0230‌0202
Francis JH, Patel SP, Gombos DS, Carvajal RD. Surveillance options for patients with uveal melanoma following definitive management. In: Dizon DS, ed. American Society of Clinical Oncology Educational Book. Vol 33. American Society of Clinical Oncology; 2013:382-387. doi:10.14694/EdBook_AM.2013.33.382

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When you see
uveal melanoma,
think
fast

Uveal melanoma (UM) is a rare tumor of the eye^1,2

UM is the most common primary intraocular malignancy³

Occurrence by site²

Think uveal melanoma brochure

Would you recognize a patient at risk?

Additional risk factors may include:

Symptoms of UM

UM can be asymptomatic or present with a variety of symptoms, making annual dilated eye exams ideal.³

Blurred vision

Visual field defects

Photopsia

Metamorphopsia

Other symptoms

The vast majority of cases, more than 90%, show no signs of systemic disease at diagnosis.¹⁵

Given that patient survival correlates with tumor size and staging, early and accurate diagnosis is particularly important for patient outcomes.^13,16-18

Detecting UM

Detection and diagnosis of UM can be challenging depending on the size, location, and appearance of the lesion²

Location of UM by type

Different tumors

Patient treatment and prognosis

Surgery and radiation are the primary treatment options for UM^10,20

Sites of metastasis

UM cells spread hematogenously, most commonly metastasizing to the liver and becoming a systemic disease that can also involve the lungs, skin, and bones.^5,23

Need more information?

Get the latest updates in your inbox

Unsubscribe

When you seeuveal melanoma,thinkfast

Uveal melanoma (UM) is a rare tumor of the eye1,2

UM is the most common primary intraocular malignancy3

Occurrence by site2

Think uveal melanoma brochure

Would you recognize a patient at risk?

Additional risk factors may include:

Symptoms of UM

UM can be asymptomatic or present with a variety of symptoms, making annual dilated eye exams ideal.3

Blurred vision

Visual field defects

Photopsia

Metamorphopsia

Other symptoms

The vast majority of cases, more than 90%, show no signs of systemic disease at diagnosis.15

Given that patient survival correlates with tumor size and staging, early and accurate diagnosis is particularly important for patient outcomes.13,16-18

Detecting UM

Detection and diagnosis of UM can be challenging depending on the size, location, and appearance of the lesion2

Location of UM by type

Different tumors

Patient treatment and prognosis

Surgery and radiation are the primary treatment options for UM10,20

Sites of metastasis

UM cells spread hematogenously, most commonly metastasizing to the liver and becoming a systemic disease that can also involve the lungs, skin, and bones.5,23

Need more information?

Get the latest updates in your inbox

Unsubscribe

You are now leaving this website.

Thank you for signing up.

Thank you.

Please tell us what you think

Thank you

Get the latest updates in your inbox

Unsubscribe

When you see
uveal melanoma,
think
fast

Uveal melanoma (UM) is a rare tumor of the eye^1,2

UM is the most common primary intraocular malignancy³

Occurrence by site²

UM can be asymptomatic or present with a variety of symptoms, making annual dilated eye exams ideal.³

The vast majority of cases, more than 90%, show no signs of systemic disease at diagnosis.¹⁵

Given that patient survival correlates with tumor size and staging, early and accurate diagnosis is particularly important for patient outcomes.^13,16-18

Detection and diagnosis of UM can be challenging depending on the size, location, and appearance of the lesion²

Surgery and radiation are the primary treatment options for UM^10,20

UM cells spread hematogenously, most commonly metastasizing to the liver and becoming a systemic disease that can also involve the lungs, skin, and bones.^5,23