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When you see uveal melanoma, think
fast

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

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

Eye illustration Eye illustration

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

ink drop sequence
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 US6,7
≈2,500
diagnosed in the EU278

EU27, the 27 countries constituting the European Union.

Would you recognize a patient at risk?

patient image
Risk factors that predispose patients to developing UM include:
  • Caucasian2
  • light-colored irides9
  • median age: 62 years, with peak incidence at 55 years2,10,11,a
  • BAP1 mutation12
a Patients carrying the BAP1 mutation typically present at a younger age, between 30 and 59 years of age.10 BAP1BRCA1-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

“I was fitted for the contacts, and afterward, during the eye exam, the ophthalmologist said, ‘There’s a shadow in the back of your left eye. May I dilate your eye?’ He came back and said, ‘I’ve scheduled an appointment for you to see a specialist . . . I was hoping I wouldn’t need to tell you this, but this may be a matter of life and death. I’m very serious—you need to keep this eye appointment.’”—Patient with UM

Symptoms of UM

illustration of eye

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.

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

Location of UM by type

Eye depicting tumors Eye depicting tumors

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 UM10,20

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
ink drop sequence
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

Lungs Liver Skin Bones

Studies have indicated that the risk for metastasis varies and is influenced by clinical characteristics of both the patient and the tumor, including24,25:

  • 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

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Specialty
  • Ocular oncologist
  • Ophthalmologist
  • Optometrist
  • Retina specialist
  • Dermatologic oncologist
  • Medical oncologist
  • Other healthcare professional

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    References:
  1. 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.
  2. 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.
  3. 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) [published online for public access]. Clin Ophthalmol. 2016;10:2113-2119.
  4. Nagarkatti-Gude N, Wang Y, Ali MJ, Honavar SG, Jager MJ, Chan C-C. Genetics of primary intraocular tumors [published online for public access]. Ocul Immunol Inflamm. 2012;201(4):244-254.
  5. Pandiani C, Béranger GE, Leclerc J, Ballotti R, Bertolotto C. Focus on cutaneous and uveal melanoma specificities. Genes Dev. 2017;31(8):724-743.
  6. 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.
  7. About ocular melanoma. Ocular Melanoma Foundation website. www.ocularmelanoma.org​​/about​-om.htm. Accessed December 13, 2018.
  8. Search of malignant melanoma of uvea—Tabular results. RARECARE: Surveillance of rare cancers in Europe website. http://dcnapp4.dcn.ed.ac.uk​/rcnet​/searchpage.aspx. Accessed August 1, 2019.
  9. Nathan P, Cohen V, Coupland S, et al. Uveal melanoma UK national guidelines. Eur J Cancer. 2015;51(16):2404-2412.
  10. Yang J, Manson DK, Marr BP, Carvajal RD. Treatment of uveal melanoma: where are we now? Ther Adv Med Oncol. 2018;10:1758834018757175.
  11. 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.
  12. 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.
  13. 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.
  14. 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.e2.
  15. 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). Skin Cancer Foundation website. https://www.skincancer.org​/publications​/the-melanoma-letter​/fall-2016-vol-34-no-3​/uveal-melanoma. Accessed December 13, 2018.
  16. 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.
  17. 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.
  18. 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.
  19. Kivelä T. Diagnosis of uveal melanoma. In: Jager MJ, Desjardins L, Kivelä T, eds. Current Concepts in Uveal Melanoma. Basel, Switzerland: Karger; 2012. Bandello F, ed. Developments in Ophthalmology; vol. 49.
  20. 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.
  21. Singh M, Durairaj P, Yeung J. Uveal melanoma: a review of the literature. Oncol Ther. 2018;6(1):87-104.
  22. 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.
  23. Carvajal RD. Management of metastatic uveal melanoma. Up To Date website. https://www.uptodate.com​​/contents​​/management​-of​-metastatic​-uveal​-melanoma​. Updated October 31, 2018. Accessed December 13, 2018.
  24. 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.
  25. Corrêa ZM. Assessing prognosis in uveal melanoma. Cancer Control. 2016;23(2):93-98.
  26. Francis JH, Patel SP, Gombos DS, Carvajal RD. Surveillance options for patients with uveal melanoma following definitive management. In: ASCO Educational Book. Alexandria, VA: American Society of Clinical Oncology; 2013:382-387.
The information and other content contained on this website are not intended to be, nor should they be, used as medical advice, diagnosis, or treatment. Each patient must be evaluated, diagnosed, and treated based upon that individual patient’s specific circumstances.