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Malignant melanoma of uveal tract

Last edited: 4/14/2026

Overview

Malignant melanoma of the uveal tract is a rare but aggressive intraocular malignancy arising from melanocytes of the uveal layer, often leading to metastasis with poor prognosis. Recent advancements include the approval of tebentafusp for HLA-A*02:01-positive metastatic cases 12.

Diagnosis

  • Clinical Presentation: Yellowish-gray lesions in the choroid, ciliary body, or iris 33.
  • Diagnostic Imaging: Ultrasound biomicroscopy (UBM), optical coherence tomography (OCT), and high-field MRI for tumor dimensions 20.
  • Histopathology: Essential for definitive diagnosis, including cytogenetic analysis for prognostic markers 18.
  • Genomic Testing: Gene expression profiling (GEP) to predict metastatic risk 13.
  • Immunohistochemistry: Expression of HLA antigens assessed to evaluate immune response potential 31.

    • Management

  • Primary Treatment:
    • - Surgical Resection: Localized tumors may be treated with surgical resection or local therapies 7. - Radiation Therapy: Charged-particle therapy (CPT) and brachytherapy are common, with CPT showing comparable efficacy 5.
  • Metastatic Disease:
    • - Tebentafusp: First systemic therapy improving survival in HLA-A*02:01-positive metastatic uveal melanoma 112. - Immune Checkpoint Inhibitors (ICIs): Variable efficacy reported; further research needed 38.
  • Biological Adjuncts: Limited evidence; experimental approaches like MEK inhibitors show promise 21.

    • Special Populations

  • Pregnancy: Limited data; management typically deferred until postpartum 7.
  • Elderly: Multidisciplinary care crucial due to comorbidities and treatment tolerance 15.
  • Comorbidities: Integrated care between ophthalmology, radiation oncology, and oncology essential 15.

    • Key Recommendations

  • Use Tebentafusp for HLA-A*02:01-positive metastatic uveal melanoma patients to improve survival outcomes (Evidence: Strong 112).
  • Implement gene expression profiling (GEP) for prognostic stratification in newly diagnosed uveal melanoma patients (Evidence: Moderate 13).
  • Consider charged-particle therapy (CPT) as a standard option for primary tumor treatment, balancing efficacy and preservation of vision (Evidence: Moderate 5).
  • Monitor for second primary malignancies in uveal melanoma survivors due to increased risk 4.
  • Utilize advanced imaging techniques such as high-field MRI for precise tumor assessment in treatment planning (Evidence: Moderate 20).

    • References

    1 Saldanha EF, Noronha MM, Reis PCA, Passos PRC, Filho VOC, Cappellaro AP et al.. Tebentafusp in Metastatic Uveal Melanoma: A Meta-analysis. Targeted oncology 2026. link 2 Mies G, Tsao NL, Houy A, Coupland SE, Kalirai H, Försti A et al.. Meta-analysis of uveal melanoma genome-wide association studies identifies novel risk loci and population effect size heterogeneity. HGG advances 2025. link 3 Yamada K, Takeuchi M, Fukumoto T, Suzuki M, Kato A, Mizuki Y et al.. Immune checkpoint inhibitors for metastatic uveal melanoma: a meta-analysis. Scientific reports 2024. link 4 de Esteban Maciñeira E, Fernández Fernández P, Conde González I, Bande Rodríguez MF, Blanco Teijeiro MJ. Multiple neoplasms in patients with uveal melanoma: a systematic review. International ophthalmology 2024. link 5 Tseng YH, Hsu CA, Chou YB. Comparing efficacy of charged-particle therapy with brachytherapy in treatment of uveal melanoma. Eye (London, England) 2024. link 6 Liu B, Yao X, Shang Y, Dai J. The multiple roles of autophagy in uveal melanoma and the microenvironment. Journal of cancer research and clinical oncology 2024. link 7 Weis E, Surgeoner B, Salopek TG, Cheng T, Hyrcza M, Kostaras X et al.. Management of Uveal Melanoma: Updated Cancer Care Alberta Clinical Practice Guideline. Current oncology (Toronto, Ont.) 2023. link 8 Pham JP, On L, Ardolino L, Hurwitz J, Salaun H, Sim HW et al.. Efficacy of immune checkpoint inhibition in metastatic uveal melanoma: a systematic review and meta-analysis. Melanoma research 2023. link 9 Davies C, Brown SL, Fisher P, Hope-Stone L, Fisher D, Morgan A et al.. Predictors of emotional distress in uveal melanoma survivors: a systematic review. Eye (London, England) 2023. link 10 Weis E, Aghazadeh H, Roelofs K, Agi J. Sunlamp use is a risk factor for uveal melanoma: a meta-analysis. Canadian journal of ophthalmology. Journal canadien d'ophtalmologie 2022. link 11 Naseripoor M, Azimi F, Mirshahi R, Khakpoor G, Poorhosseingholi A, Chaibakhsh S. Global Incidence and Trend of Uveal Melanoma from 1943-2015: A Meta-Analysis. Asian Pacific journal of cancer prevention : APJCP 2022. link 12 . First Drug Approved for Rare Eye Cancer. Cancer discovery 2022. link 13 Berry DE, Schefler AC, Seider MI, Materin M, Stinnett S, Mruthyunjaya P. CORRELATION OF GENE EXPRESSION PROFILE STATUS AND AMERICAN JOINT COMMISSION ON CANCER STAGE IN UVEAL MELANOMA. Retina (Philadelphia, Pa.) 2020. link 14 Wong W, Sundar G, Chee C, Zhao PS, Rajagopalan R, Gopal L. Clinical spectrum, treatment and outcomes of uveal melanoma in a tertiary centre. Singapore medical journal 2019. link 15 Tsai KK, Bollin KB, Patel SP. Obstacles to improving outcomes in the treatment of uveal melanoma. Cancer 2018. link 16 Kivelä TT. The first description of the complete natural history of uveal melanoma by two Scottish surgeons, Allan Burns and James Wardrop. Acta ophthalmologica 2018. link 17 Zozolou M, Tsoucalas G, Karamanou M, Laios K, Georgalas I, Douzenis A et al.. The distinguished Austrian ophthalmologist Ernst Fuchs (1851-1930) and the "sarcom des uvealtractus". Journal of B.U.ON. : official journal of the Balkan Union of Oncology 2018. link 18 Berus T, Halon A, Markiewicz A, Orlowska-Heitzman J, Romanowska-Dixon B, Donizy P. Clinical, Histopathological and Cytogenetic Prognosticators in Uveal Melanoma - A Comprehensive Review. Anticancer research 2017. link 19 Jager MJ, Magner JA, Ksander BR, Dubovy SR. Uveal Melanoma Cell Lines: Where do they come from? (An American Ophthalmological Society Thesis). Transactions of the American Ophthalmological Society 2016. link 20 Beenakker JW, Ferreira TA, Soemarwoto KP, Genders SW, Teeuwisse WM, Webb AG et al.. Clinical evaluation of ultra-high-field MRI for three-dimensional visualisation of tumour size in uveal melanoma patients, with direct relevance to treatment planning. Magma (New York, N.Y.) 2016. link 21 . Uveal melanoma therapy on the horizon?. Cancer discovery 2014. link 22 Classic KL, Furutani KM, Stafford SL, Pulido JS. Radiation dose to the surgeon during plaque brachytherapy. Retina (Philadelphia, Pa.) 2012. link 23 Weber A, Cordini D, Stark R, Heufelder J. The influence of silicone oil used in ophthalmology on the proton therapy of uveal melanomas. Physics in medicine and biology 2012. link 24 Folberg R. The molecular classification of uveal melanocytic lesions: an important discovery in context. The Journal of molecular diagnostics : JMD 2010. link 25 Bove R, Char DH. Nondiagnosed uveal melanomas. Ophthalmology 2004. link 26 Gombos DS, Van Quill KR, Uusitalo M, O'Brien JM. Geographic disparities in diagnostic screening for metastatic uveal melanoma. Ophthalmology 2004. link 27 Eskelin S, Kivelä T. Mode of presentation and time to treatment of uveal melanoma in Finland. The British journal of ophthalmology 2002. link 28 Tallberg T, Uusitalo R, Sarna S, Seregard S, Werschnik C. Improvement of the recurrence-free interval using biological adjuvant therapy in uveal melanoma. Anticancer research 2000. link 29 Nauraye C, Mazal A, Delacroix S, Bridier A, Chavaudra J, Rosenwald JC. An experimental approach to the design of a scattering system for a proton therapy beam line dedicated to ophthalmological applications. International journal of radiation oncology, biology, physics 1995. link00006-k) 30 Donoso LA, Shields JA, Augsburger JJ, Whitman J, Arbizo V. Antigenic and cellular heterogeneity of primary uveal malignant melanomas. Archives of ophthalmology (Chicago, Ill. : 1960) 1986. link 31 Jager MJ, de Wolff-Rouendaal D, Breebaart AC, Ruiter DJ. Expression of HLA antigens in paraffin sections of uveal melanomas. Documenta ophthalmologica. Advances in ophthalmology 1986. link 32 Vrabec F, Kuchynka P. Anterior chamber angle recession in uveal melanomas. Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie. Albrecht von Graefe's archive for clinical and experimental ophthalmology 1978. link 33 Shields JA. Modern methods in the diagnosis of uveal melanomas. Transactions of the ophthalmological societies of the United Kingdom 1977. link 34 Bucek J, Vrba M. Ultrastructure of human uveal melanomas cultivated in vitro. Neoplasma 1976. link

    Original source

    1. [1]
      Tebentafusp in Metastatic Uveal Melanoma: A Meta-analysis. — Saldanha EF, Noronha MM, Reis PCA, Passos PRC, Filho VOC, Cappellaro AP et al. Targeted oncology (2026)
    2. [2]
      Meta-analysis of uveal melanoma genome-wide association studies identifies novel risk loci and population effect size heterogeneity. — Mies G, Tsao NL, Houy A, Coupland SE, Kalirai H, Försti A et al. HGG advances (2025)
    3. [3]
      Immune checkpoint inhibitors for metastatic uveal melanoma: a meta-analysis. — Yamada K, Takeuchi M, Fukumoto T, Suzuki M, Kato A, Mizuki Y et al. Scientific reports (2024)
    4. [4]
      Multiple neoplasms in patients with uveal melanoma: a systematic review. — de Esteban Maciñeira E, Fernández Fernández P, Conde González I, Bande Rodríguez MF, Blanco Teijeiro MJ International ophthalmology (2024)
    5. [5]
      Comparing efficacy of charged-particle therapy with brachytherapy in treatment of uveal melanoma. — Tseng YH, Hsu CA, Chou YB Eye (London, England) (2024)
    6. [6]
      The multiple roles of autophagy in uveal melanoma and the microenvironment. — Liu B, Yao X, Shang Y, Dai J Journal of cancer research and clinical oncology (2024)
    7. [7]
      Management of Uveal Melanoma: Updated Cancer Care Alberta Clinical Practice Guideline. — Weis E, Surgeoner B, Salopek TG, Cheng T, Hyrcza M, Kostaras X et al. Current oncology (Toronto, Ont.) (2023)
    8. [8]
      Efficacy of immune checkpoint inhibition in metastatic uveal melanoma: a systematic review and meta-analysis. — Pham JP, On L, Ardolino L, Hurwitz J, Salaun H, Sim HW et al. Melanoma research (2023)
    9. [9]
      Predictors of emotional distress in uveal melanoma survivors: a systematic review. — Davies C, Brown SL, Fisher P, Hope-Stone L, Fisher D, Morgan A et al. Eye (London, England) (2023)
    10. [10]
      Sunlamp use is a risk factor for uveal melanoma: a meta-analysis. — Weis E, Aghazadeh H, Roelofs K, Agi J Canadian journal of ophthalmology. Journal canadien d'ophtalmologie (2022)
    11. [11]
      Global Incidence and Trend of Uveal Melanoma from 1943-2015: A Meta-Analysis. — Naseripoor M, Azimi F, Mirshahi R, Khakpoor G, Poorhosseingholi A, Chaibakhsh S Asian Pacific journal of cancer prevention : APJCP (2022)
    12. [12]
      First Drug Approved for Rare Eye Cancer. Cancer discovery (2022)
    13. [13]
      CORRELATION OF GENE EXPRESSION PROFILE STATUS AND AMERICAN JOINT COMMISSION ON CANCER STAGE IN UVEAL MELANOMA. — Berry DE, Schefler AC, Seider MI, Materin M, Stinnett S, Mruthyunjaya P Retina (Philadelphia, Pa.) (2020)
    14. [14]
      Clinical spectrum, treatment and outcomes of uveal melanoma in a tertiary centre. — Wong W, Sundar G, Chee C, Zhao PS, Rajagopalan R, Gopal L Singapore medical journal (2019)
    15. [15]
      Obstacles to improving outcomes in the treatment of uveal melanoma. — Tsai KK, Bollin KB, Patel SP Cancer (2018)
    16. [16]
      The first description of the complete natural history of uveal melanoma by two Scottish surgeons, Allan Burns and James Wardrop. — Kivelä TT Acta ophthalmologica (2018)
    17. [17]
      The distinguished Austrian ophthalmologist Ernst Fuchs (1851-1930) and the "sarcom des uvealtractus". — Zozolou M, Tsoucalas G, Karamanou M, Laios K, Georgalas I, Douzenis A et al. Journal of B.U.ON. : official journal of the Balkan Union of Oncology (2018)
    18. [18]
      Clinical, Histopathological and Cytogenetic Prognosticators in Uveal Melanoma - A Comprehensive Review. — Berus T, Halon A, Markiewicz A, Orlowska-Heitzman J, Romanowska-Dixon B, Donizy P Anticancer research (2017)
    19. [19]
      Uveal Melanoma Cell Lines: Where do they come from? (An American Ophthalmological Society Thesis). — Jager MJ, Magner JA, Ksander BR, Dubovy SR Transactions of the American Ophthalmological Society (2016)
    20. [20]
      Clinical evaluation of ultra-high-field MRI for three-dimensional visualisation of tumour size in uveal melanoma patients, with direct relevance to treatment planning. — Beenakker JW, Ferreira TA, Soemarwoto KP, Genders SW, Teeuwisse WM, Webb AG et al. Magma (New York, N.Y.) (2016)
    21. [21]
      Uveal melanoma therapy on the horizon? Cancer discovery (2014)
    22. [22]
      Radiation dose to the surgeon during plaque brachytherapy. — Classic KL, Furutani KM, Stafford SL, Pulido JS Retina (Philadelphia, Pa.) (2012)
    23. [23]
      The influence of silicone oil used in ophthalmology on the proton therapy of uveal melanomas. — Weber A, Cordini D, Stark R, Heufelder J Physics in medicine and biology (2012)
    24. [24]
      The molecular classification of uveal melanocytic lesions: an important discovery in context. — Folberg R The Journal of molecular diagnostics : JMD (2010)
    25. [25]
      Nondiagnosed uveal melanomas. — Bove R, Char DH Ophthalmology (2004)
    26. [26]
      Geographic disparities in diagnostic screening for metastatic uveal melanoma. — Gombos DS, Van Quill KR, Uusitalo M, O'Brien JM Ophthalmology (2004)
    27. [27]
      Mode of presentation and time to treatment of uveal melanoma in Finland. — Eskelin S, Kivelä T The British journal of ophthalmology (2002)
    28. [28]
      Improvement of the recurrence-free interval using biological adjuvant therapy in uveal melanoma. — Tallberg T, Uusitalo R, Sarna S, Seregard S, Werschnik C Anticancer research (2000)
    29. [29]
      An experimental approach to the design of a scattering system for a proton therapy beam line dedicated to ophthalmological applications. — Nauraye C, Mazal A, Delacroix S, Bridier A, Chavaudra J, Rosenwald JC International journal of radiation oncology, biology, physics (1995)
    30. [30]
      Antigenic and cellular heterogeneity of primary uveal malignant melanomas. — Donoso LA, Shields JA, Augsburger JJ, Whitman J, Arbizo V Archives of ophthalmology (Chicago, Ill. : 1960) (1986)
    31. [31]
      Expression of HLA antigens in paraffin sections of uveal melanomas. — Jager MJ, de Wolff-Rouendaal D, Breebaart AC, Ruiter DJ Documenta ophthalmologica. Advances in ophthalmology (1986)
    32. [32]
      Anterior chamber angle recession in uveal melanomas. — Vrabec F, Kuchynka P Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie. Albrecht von Graefe's archive for clinical and experimental ophthalmology (1978)
    33. [33]
      Modern methods in the diagnosis of uveal melanomas. — Shields JA Transactions of the ophthalmological societies of the United Kingdom (1977)
    34. [34]
      Ultrastructure of human uveal melanomas cultivated in vitro. — Bucek J, Vrba M Neoplasma (1976)
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