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When you see uveal melanoma, you can't afford to wait

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Though it may start as an isolated tumor of the eye, uveal melanoma (UM) metastasizes in up to 50% of patients,
becoming a systemic disease.1

As metastatic UM runs its course, treatment options are limited and patients face a median survival of up to 12 months.2 So, when you see mUM, think fast.

Immunocore is acting too—we're dedicated to finding new approaches that could transform the lives of patients with rare diseases.

UM is a rare tumor of the eye1,3

UM is the most common primary intraocular malignancy4

While UM can arise from melanocytes anywhere in the uveal tract, it most commonly occurs in the choroid.3

Occurrence by site3

Eye illustration Eye illustration
ink drop sequence
UM accounts for only
≈4%
of all melanoma cases.1,4
≈8,000
people per year diagnosed with UM globally5
1,600-2,000
diagnosed in the US6,7

Would you recognize a patient at risk?

patient iamge patient silhouette
Risk factors that can predispose patients to developing UM include:
  • Caucasian3
  • Male (30% more likely than female)3
  • Light-colored irides2
  • Age (median age at diagnosis: 62 years)3,8,a
a Patients carrying the BAP1 mutation typically present at a younger age, between 30 and 59 years of age.8 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 sometimes develop into melanomas) and genetic mutations including germ line BAP12,3,9

“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 overdilate 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

Annual routine, dilated eye exams would be ideal, as UM can be asymptomatic or present with a variety of symptoms4

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%, present with localized disease at diagnosis.11

Diagnosing UM can be challenging depending on the size, location, and appearance of the lesion3

Location of UM by type

Eye depicting tumors Eye depicting tumors

In addition to observing growth patterns of lesions, ophthalmologists pay especially close attention to other attributes when distinguishing small melanomas from benign lesions, including12:

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

Patient treatment and prognosis

Surgery and radiation are the primary treatment options for UM8,13

For many years, treatment for primary disease has focused on vision- and eye-preserving techniques because enucleation (removal of the eye) has not seemed to demonstrate a survival benefit compared with brachytherapy (focal delivery of radiation to the tumor).8

  • Plaque BrachytherapyMost primary UM tumors are treated with plaque brachytherapy.8
  • SurgicalPreferred surgical approaches include transretinal endoresection and transscleral resection (which can still lead to partial sight loss or blindness).8
  • Other non-specificOther therapies include charged-particle radiotherapy, stereotactic proton beam therapy, and photocoagulation or transpupillary thermotherapy.8
  • EnucleationEnucleation is performed in approximately 20% of cases.14
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).1,8

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 bones5,15

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, including16,17:

  • cytogenetic abnormalities
  • type of 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
  • Dermatologic oncologist
  • Medical oncologist
  • Ocular oncologist
  • Ophthalmologist
  • Optometrist
  • Retina specialist
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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 [published online for public access]. Br J Ophthalmol. 2017;101(1):38-44. 2. Nathan P, Cohen V, Coupland S, et al. Uveal melanoma UK National Guidelines. Eur J Cancer. 2015;51(16):2404-2412. 3. Krantz BA, Dave N, Komatsubara KM, Marr BP, Carvajal RD. Uveal melanoma: epidemiology, etiology, and treatment of primary disease [published online for public access]. Clin Ophthalmol. 2017;11:279-289. 4. 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. 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. Yang J, Manson, DK, Marr BP, Carvajal RD. Treatment of uveal melanoma: where are we now? Ther Adv Med Oncol. 2018;10:1758834018757175. 9. Weis E, Salopek T, McKinnon JG, et al. Management of uveal melanoma: a consensus-based provincial clinical practice guideline. Curr Oncol. 2016;23(1):e57-e64. 10. 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. 11. 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. 12. Kivelä T. Diagnosis of uveal melanoma. In: Jager MJ, Desjardins L, Kivelä T, Damato BE, eds. Current Concepts in Uveal Melanoma. Basel, Switzerland: Karger; 2012. Bandello F, ed. Developments in Ophthalmology; vol. 49. 13. 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. 14. 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. 15. Carvajal RD. Management of metastatic uveal melanoma. Up To Date website. https://www.uptodate.com​/contents​/management-of-metastatic-uveal-melanoma​?search=management%20of%20metastatic%20uveal%20melanoma​&source=search_result​&selectedTitle=1~4​&usage_type=default​&display_rank=1. Updated October 31, 2018. Accessed December 13, 2018. 16. 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. 17. Corrêa ZM. Assessing prognosis in uveal melanoma. Cancer Control. 2016;23(2):93-98. 18. 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.