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When you see uveal melanoma progress, think
collaboratively

While guidelines for the management of uveal melanoma (UM) are emerging, evidence-based treatments for progressive disease are still needed.1,2

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Uveal Melanoma were first published in 2018 and were most recently updated in May 2020.3,4

Other national guidelines include5-9:

  • Guidance from the National Cancer Institute (NCI)

  • Uveal Melanoma UK National Guidelines (NICE-accredited)

  • The French Cancer Society

  • The Netherlands’ Recommendations for Uveal Melanomas Treatment Guidelines

  • A consensus-based guideline from Canada

A recent analysis of UM guidelines published between 2004 and 2019 appraised the methodological quality of assessed guidelines using AGREE II and AGREE-REX tools.1

Click here to access the full publication.

Poor prognosis for advanced disease

While effective therapies targeting the primary tumor in the eye have been developed, up to half of patients develop metastatic disease, most commonly spreading to the liver.1,10 The prognosis for metastatic uveal melanoma (mUM) remains poor, with a median survival of up to 12 months.2,11-13

ink drop sequence
50% of patients with mUM
survive for one year after diagnosis.13
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Surveillance defined by metastatic risk

Doctor with patient

Studies have indicated that the risk for metastasis varies and is influenced by clinical and histological characteristics of the patient and the tumor, including14-16:

  • tumor diameter

  • degree of pigmentation

  • high mitotic rate

  • somatic mutations in the tumor

  • tumor thickness

  • ciliary body involvement

  • advanced age

  • male gender

Guidelines recommend surveillance by a multidisciplinary team to identify mUM, but acknowledge that patients should understand both the benefits and the risks of surveillance.4,6,8,17 Unfortunately, surveillance and early detection have generally not been linked to improved outcomes,18 likely due to the lack of universally accepted effective therapies for mUM.10,12,13 Several factors do support surveillance, and its use is likely to become more important as more effective treatments for metastatic disease become available.19

ink drop sequence
Therapeutic options
for mUM offer
only modest efficacy,
and clinical trials
are currently
the recommended
course of treatment,
when clinically
appropriate.4,10,12,13
Overall survival for mUM treated with conventional chemotherapy or immunotherapy

From Melanoma Res (2019) 29(6), 561-568. Used with permission.

CHT, conventional chemotherapy (fotemustine, treosulfan + gemcitabine, cisplatin + treosulfan + gemcitabine, dacarbazine, DHA-paclitaxel, or temozolomide + bevacizumab); CPI, checkpoint inhibitor (anti-CTLA4 antibody, anti–PD-1/anti–PD-L1 antibody).

NCCN Guidelines® for Uveal Melanoma recommend stratification by risk for distant metastasis to determine frequency of systemic imaging during follow-up.4

Risk stratification to determine the frequency of follow-up systemic imaging should be based on the highest risk factor present4

AJCC, American Joint Committee on Cancer.

a The most frequent sites of metastasis are liver, lungs, skin/soft tissue, and bones. For patients who elect to have surveillance imaging, options include contrast-enhanced magnetic resonance or ultrasound of the liver, with modality preference determined by expertise at the treating institution. Additional imaging modalities may include chest/abdominal/pelvic computed tomography with contrast. However, screening should limit radiation exposure whenever possible. Scans should be performed with IV contrast unless contraindicated. Recognizing that there are limited options for systemic recurrence and that regular imaging may cause patient anxiety, patients should discuss with their treating physician the benefits of surveillance imaging, and some patients may elect to forgo surveillance imaging. Participation in a clinical trial is strongly encouraged.4

ink drop sequence
Liver-specific imaging* is common
due to the high rate of metastasis to the liver. Liver function tests are likely inadequate based on current evidence.3,6,8,18
*Eg, ultrasound, magnetic resonance imaging, and computed tomography.

Taking a multidisciplinary approach

Care team collaborating

While no approved therapy has demonstrated a substantial benefit specifically for patients with mUM,2,12 guidelines recommend a multidisciplinary approach to the management of metastatic disease.4,6,8,17

Multidisciplinary teams may include expertise from the following areas6,8,17:

  • Medical oncology

  • Ophthalmology

  • Radiology and radiotherapy

  • Pathology

  • Surgical oncology

  • Nursing specialist

  • Hepatology

A collaborative approach may help optimize outcomes for patients with mUM.10

Watch Bertil Damato, MD, PhD, discuss standards of care

Watch more videos that offer professional guidance and clinical perspectives on UM Learn more about presentation, symptoms, 
and management of UM
Understand the intrinsic differences 
between UM and cutaneous melanoma (CM)
Explore UM resources and 
clinical perspectives

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Specialty
  • Ocular oncologist
  • Ophthalmologist
  • Optometrist
  • Retina specialist
  • Dermatologic oncologist
  • Medical oncologist
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    References:
  1. Steeb T, Hayani KM, Fӧrster P, et al. Guidelines for uveal melanoma: a critical appraisal of systematically identified guidelines using the AGREE II and AGREE-REX instrument. J Cancer Res Clin Oncol. 2020;146(4):1‌0‌79-10‌88. doi:10.10‌07/s00‌432‌-02‌0-03‌14‌1-w
  2. 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
  3. 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
  4. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Uveal Melanoma V.1.2020. © National Comprehensive Cancer Network, Inc. 2020. All rights reserved. Accessed August 6, 2020. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.
  5. Mathis T, Cassoux N, Tardy M, et al. Management of uveal melanomas, guidelines for oncologists. Article in French. Bull Cancer. Published online September 11, 2018. 2018;10‌5(10):967-980. doi:10.10‌16/‌j.bulca‌n.20‌18.07‌.011
  6. The Netherlands’ recommendations for uveal melanoma (eye melanoma) treatment guidelines. Published 2017.
  7. 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
  8. 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
  9. Intraocular (uveal) melanoma treatment (PDQ®)–health professional version. National Cancer Institute. Updated December 17, 2019. Accessed April 15, 2020. https://ww‌w.cancer.go‌v/types/ey‌e/hp/intraocular-melanoma-tre‌atment-pdq
  10. 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-20‌16-30‌034
  11. 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
  12. Khoja L, Atenafu EG, Suciu S, et al. Meta-analysis in metastatic uveal melanoma to determine progression free and overall survival benchmarks: an International Rare Cancers Initiative (IRCI) ocular melanoma study. Ann Oncol. 2019;30(8):13‌70-1‌380. doi:10.1093/annonc/mdz176
  13. Rantala ES, Hernberg M, Kivelä TT. Overall survival after treatment for metastatic uveal melanoma: a systematic review and meta-analysis. Melanoma Res. Published online. 2019;29(6):561-568. doi:10.1097/C‌MR.00‌0000‌00000‌00575
  14. 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
  15. Corrêa ZM. Assessing prognosis in uveal melanoma. Cancer Control. 2016;23(2):93-98. doi:10.11‌77/10‌3‌2748‌160‌2300‌202
  16. Gill HS, Char DH. Uveal melanoma prognostication: from lesion size and cell type to molecular class. Can J Ophthalmol. 2012;47(3):246-253. doi:10.1016/j.jcjo.2012.03.038
  17. Nathan P, Cohen V, Coupland S, et al. Uveal melanoma national guidelines. Melanoma Focus. Published January 2015. Accessed April 15, 2020. http://melanomafocus.c‌om/wp-content/uploads/20‌15/01/Uveal-Melanoma-Nationa‌l-Guidelines-Full-v5.3.pdf
  18. 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.
  19. 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
  20. Onken MD, Worley LA, Char DH, et al. Collaborative Ocular Oncology Group report no. 1: prospective validation of a multi-gene prognostic assay in uveal melanoma. Ophthalmology. Published online August 1, 2013. 201‌2;119‌(8):159‌6-16‌03. doi:10.10‌16/j.ophtha.20‌12.0‌2.017
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.