2. Academic Validation
  3. Uveal melanoma

Uveal melanoma

  • Nat Rev Dis Primers. 2020 Apr 9;6(1):24. doi: 10.1038/s41572-020-0158-0.
Martine J Jager 1,  Carol L Shields 2,  Colleen M Cebulla 3,  Mohamed H Abdel-Rahman 3 4,  Hans E Grossniklaus 5 6,  Marc-Henri Stern 7,  Richard D Carvajal 8,  Rubens N Belfort 9,  Renbing Jia 10,  Jerry A Shields 2,  Bertil E Damato 11
Affiliations

Affiliations

  • 1 Department of Ophthalmology, Leiden University Medical Center, Leiden, The Netherlands. m.j.jager@lumc.nl.
  • 2 Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, USA.
  • 3 Havener Eye Institute, Department of Ophthalmology and Visual Science, The Ohio State University Wexner Medical Center, Columbus, OH, USA.
  • 4 Division of Human Genetics, Department of Internal Medicine, The Ohio State University Columbus, Columbus, OH, USA.
  • 5 Department of Ophthalmology, Emory University, Atlanta, GA, USA.
  • 6 Department of Pathology and Laboratory Medicine, Emory University Atlanta, GA, USA.
  • 7 Inserm U830, DNA Repair and Uveal Melanoma (D.R.U.M.), Equipe labellisée par la Ligue Nationale Contre le Cancer, Institut Curie, PSL Research University, Paris, France.
  • 8 Department of Medicine, Columbia University Medical Center, New York, NY, USA.
  • 9 Department of Ophthalmology and Visual Sciences, Federal University of São Paulo, São Paulo, Brazil.
  • 10 Department of Ophthalmology, Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
  • 11 Nuffield Department of Clinical Neurosciences, University of Oxford, West Wing, John Radcliffe Hospital, Oxford, UK.
PMID: 32273508 DOI: 10.1038/s41572-020-0158-0
Abstract

Uveal melanoma (UM) is the most common primary intraocular malignancy in adults. UMs are usually initiated by a mutation in GNAQ or GNA11, unlike cutaneous melanomas, which usually harbour a BRaf or NRAS mutation. The annual incidence in Europe and the USA is ~6 per million population per year. Risk factors include fair skin, light-coloured eyes, congenital ocular melanocytosis, ocular melanocytoma and the BAP1-tumour predisposition syndrome. Ocular treatment aims at preserving the eye and useful vision and, if possible, preventing metastases. Enucleation has largely been superseded by various forms of radiotherapy, phototherapy and local tumour resection, often administered in combination. Ocular outcomes are best with small tumours not extending close to the optic disc and/or fovea. Almost 50% of patients develop metastatic disease, which usually involves the liver, and is usually fatal within 1 year. Although UM metastases are less responsive than cutaneous melanoma to chemotherapy or immune checkpoint inhibitors, encouraging results have been reported with partial hepatectomy for solitary metastases, with percutaneous hepatic perfusion with melphalan or with tebentafusp. Better insight into tumour immunology and metabolism may lead to new treatments.

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