Skip to main content
SpringerLink
Log in

Uveal Melanoma: Epidemiologic Aspects

  • Chapter
  • First Online:
Clinical Ophthalmic Oncology

Abstract

Uveal and conjunctival melanomas comprise approximately 5% of all melanomas, of which the majority (95%) are uveal in origin. The incidence of uveal melanoma in the United States is similar to that in European countries. The overall mean incidence of uveal melanoma in the United States is 5.2 per million per year with a higher rate in males (6.0 per million per year) as compared to females (4.5 per million per year). Uveal melanoma is more common in the older age group, with a progressively rising age-specific incidence that peaks at the age of 70 years. The age-adjusted annual incidence of uveal melanoma in the United States has remained stable for the last 50 years. Known causes of uveal melanoma include congenital ocular melanocytosis, melanocytoma, and the BAP1 cancer predisposition syndrome, which is associated with cutaneous atypical nevi and melanoma and specific forms of SMN such as malignant mesothelioma, meningioma, and lung adenocarcinoma. In the large majority of cases, the etiology is unknown, although ocular melanomas are most common in fair individuals with light-colored eyes.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 99.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 139.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Chang AE, Karnell LH, Menck HR. The National Cancer Data Base report on cutaneous and noncutaneous melanoma: a summary of 84,836 cases from the past decade. The American College of Surgeons Commission on Cancer and the American Cancer Society. Cancer. 1998;83(8):1664–78.

    Article  CAS  PubMed  Google Scholar 

  2. Singh AD, Topham A. Incidence of uveal melanoma in the United States: 1973-1997. Ophthalmology. 2003;110(5):956–61.

    Article  PubMed  Google Scholar 

  3. Singh AD, Turell ME, Topham AK. Uveal melanoma: trends in incidence, treatment, and survival. Ophthalmology. 2011;118(9):1881–5.

    Article  PubMed  Google Scholar 

  4. Strickland D, Lee JA. Melanomas of eye: stability of rates. Am J Epidemiol. 1981;113(6):700–2.

    Article  CAS  PubMed  Google Scholar 

  5. Kivela T. The epidemiological challenge of the most frequent eye cancer: retinoblastoma, an issue of birth and death. Br J Ophthalmol. 2009;93(9):1129–31.

    Article  PubMed  Google Scholar 

  6. Scotto J, Fraumeni JF Jr, Lee JA. Melanomas of the eye and other noncutaneous sites: epidemiologic aspects. J Natl Cancer Inst. 1976;56(3):489–91.

    Article  CAS  PubMed  Google Scholar 

  7. Aronow ME, Topham AK, Singh AD. Uveal melanoma: 5-year update on incidence, treatment, and survival (SEER 1973–2013). Ocul Oncol Pathol. 2018;4(3):145–51.

    Article  PubMed  Google Scholar 

  8. Reis L, Eisner MP, Kosary CL, et al. SEER cancer statistics review, 1973–2000. Bethesda: National Cancer Institute; 2003.

    Google Scholar 

  9. Barzilai DA, Koroukian SM, Neuhauser D, et al. The sensitivity of Medicare data for identifying incident cases of invasive melanoma (United States). Cancer Causes Control. 2004;15(2):179–84.

    Article  PubMed  Google Scholar 

  10. McLaughlin CC, Wu XC, Jemal A, et al. Incidence of noncutaneous melanomas in the U.S. Cancer. 2005;103(5):1000–7.

    Article  PubMed  Google Scholar 

  11. Stang A, Parkin DM, Ferlay J, et al. International uveal melanoma incidence trends in view of a decreasing proportion of morphological verification. Int J Cancer. 2005;114(1):114–23.

    Article  CAS  PubMed  Google Scholar 

  12. Singh AD, Bergman L, Seregard S. Uveal melanoma: epidemiologic aspects. Ophthalmol Clin N Am. 2005;18(1):75–84, viii

    Article  Google Scholar 

  13. Keenan TD, Yeates D, Goldacre MJ. Uveal melanoma in England: trends over time and geographical variation. Br J Ophthalmol. 2012;96(11):1415–9.

    Article  PubMed  Google Scholar 

  14. Vajdic CM, Kricker A, Giblin M, et al. Sun exposure predicts risk of ocular melanoma in Australia. Int J Cancer. 2002;101(2):175–82.

    Article  CAS  PubMed  Google Scholar 

  15. Michalova K, Clemett R, Dempster A, et al. Iris melanomas: are they more frequent in New Zealand? Br J Ophthalmol. 2001;85(1):4–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Bergman L, Seregard S, Nilsson B, et al. Incidence of uveal melanoma in Sweden from 1960 to 1998. Invest Ophthalmol Vis Sci. 2002;43(8):2579–83.

    PubMed  Google Scholar 

  17. Seddon JM, MacLaughlin DT, Albert DM, et al. Uveal melanomas presenting during pregnancy and the investigation of oestrogen receptors in melanomas. Br J Ophthalmol. 1982;66(11):695–704.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Hartge P, Tucker MA, Shields JA, et al. Case-control study of female hormones and eye melanoma. Cancer Res. 1989;49(16):4622–5.

    CAS  PubMed  Google Scholar 

  19. Gallagher RP, Elwood JM, Rootman J, et al. Risk factors for ocular melanoma: Western Canada Melanoma Study. J Natl Cancer Inst. 1985;74(4):775–8.

    CAS  PubMed  Google Scholar 

  20. Holly EA, Aston DA, Ahn DK, et al. Uveal melanoma, hormonal and reproductive factors in women. Cancer Res. 1991;51(5):1370–2.

    CAS  PubMed  Google Scholar 

  21. Behrens T, Kaerlev L, Cree I, et al. Hormonal exposures and the risk of uveal melanoma. Cancer Causes Control. 2010;21(10):1625–34.

    Article  PubMed  Google Scholar 

  22. Lea CS, Holly EA, Hartge P, et al. Reproductive risk factors for cutaneous melanoma in women: a case-control study. Am J Epidemiol. 2007;165(5):505–13.

    Article  PubMed  Google Scholar 

  23. Egan KM, Quinn JL, Gragoudas ES. Childbearing history associated with improved survival in choroidal melanoma. Arch Ophthalmol. 1999;117(7):939–42.

    Article  CAS  PubMed  Google Scholar 

  24. Foss AJ, Alexander RA, Guille MJ, et al. Estrogen and progesterone receptor analysis in ocular melanomas. Ophthalmology. 1995;102(3):431–5.

    Article  CAS  PubMed  Google Scholar 

  25. Lee CS, Yang WI, Shin KJ, et al. Rapid growth of choroidal melanoma during pregnancy. Acta Ophthalmol. 2011;89(3):e290–1.

    Article  PubMed  Google Scholar 

  26. Shields CL, Shields JA, Eagle RC Jr, et al. Uveal melanoma and pregnancy. A report of 16 cases. Ophthalmology. 1991;98(11):1667–73.

    Article  CAS  PubMed  Google Scholar 

  27. Aziz K. Ocular melanomas in pregnancy. BMJ. 1993;307(6901):448.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Baergen RN, Johnson D, Moore T, et al. Maternal melanoma metastatic to the placenta: a case report and review of the literature. Arch Pathol Lab Med. 1997;121(5):508–11.

    CAS  PubMed  Google Scholar 

  29. Driscoll MS, Grant-Kels JM. Hormones, nevi, and melanoma: an approach to the patient. J Am Acad Dermatol. 2007;57(6):919–31. quiz 32-6.

    Article  PubMed  Google Scholar 

  30. Marsh RD, Chu NM. Placental metastasis from primary ocular melanoma: a case report. Am J Obstet Gynecol. 1996;174(5):1654–5.

    Article  CAS  PubMed  Google Scholar 

  31. Egan KM, Seddon JM, Glynn RJ, et al. Epidemiologic aspects of uveal melanoma. Surv Ophthalmol. 1988;32(4):239–51.

    Article  CAS  PubMed  Google Scholar 

  32. Nayman T, Bostan C, Logan P, et al. Uveal melanoma risk factors: a systematic review of meta-analyses. Curr Eye Res. 2017;42(8):1085–93.

    Article  PubMed  Google Scholar 

  33. Hu DN, Yu GP, McCormick SA, et al. Population-based incidence of uveal melanoma in various races and ethnic groups. Am J Ophthalmol. 2005;140(4):612–7.

    Article  PubMed  Google Scholar 

  34. Kaneko A. Malignant ophthalmic tumors. Nippon Rinsho. 1993;51 Suppl:1013–20.

    CAS  PubMed  Google Scholar 

  35. Tucker MA, Shields JA, Hartge P, et al. Sunlight exposure as risk factor for intraocular malignant melanoma. N Engl J Med. 1985;313(13):789–92.

    Article  CAS  PubMed  Google Scholar 

  36. Holly EA, Aston DA, Ahn DK, et al. Intraocular melanoma linked to occupations and chemical exposures. Epidemiology. 1996;7(1):55–61.

    Article  CAS  PubMed  Google Scholar 

  37. Saornil MA. Iris colour and uveal melanoma. Can J Ophthalmol. 2004;39(4):448–52.

    Article  PubMed  Google Scholar 

  38. Ferguson R, Vogelsang M, Ucisik-Akkaya E, et al. Genetic markers of pigmentation are novel risk loci for uveal melanoma. Sci Rep. 2016;6:31191.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Salopek TG. The dilemma of the dysplastic nevus. Dermatol Clin. 2002;20(4):617–28.

    Article  PubMed  Google Scholar 

  40. Clark WH Jr, Reimer RR, Greene M, et al. Origin of familial malignant melanomas from heritable melanocytic lesions: ‘The B-K mole syndrome’. Arch Dermatol. 1978;114(5):732–8.

    Article  PubMed  Google Scholar 

  41. NIH. NIH Consensus conference. Diagnosis and treatment of early melanoma. JAMA. 1992;268(10):1314–9.

    Article  Google Scholar 

  42. Singh AD, Damato B, Howard P, et al. Uveal melanoma: genetic aspects. Ophthalmol Clin N Am. 2005;18(1):85–97. viii

    Article  Google Scholar 

  43. Singh AD, Shields CL, Shields JA, et al. Uveal melanoma and familial atypical mole and melanoma (FAM-M) syndrome. Ophthalmic Genet. 1995;16(2):53–61.

    Article  CAS  PubMed  Google Scholar 

  44. de Snoo FA, Bergman W, Gruis NA. Familial melanoma: a complex disorder leading to controversy on DNA testing. Familial Cancer. 2003;2(2):109–16.

    Article  PubMed  Google Scholar 

  45. Ohta M, Nagai H, Shimizu M, et al. Rarity of somatic and germline mutations of the cyclin-dependent kinase 4 inhibitor gene, CDK4I, in melanoma. Cancer Res. 1994;54(20):5269–72.

    CAS  PubMed  Google Scholar 

  46. Njauw CN, Kim I, Piris A, et al. Germline BAP1 inactivation is preferentially associated with metastatic ocular melanoma and cutaneous-ocular melanoma families. PLoS One. 2012;7(4):e35295.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  47. Wiesner T, Obenauf AC, Murali R, et al. Germline mutations in BAP1 predispose to melanocytic tumors. Nat Genet. 2011;43(10):1018–21.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Wiesner T, Murali R, Fried I, et al. A distinct subset of atypical Spitz tumors is characterized by BRAF mutation and loss of BAP1 expression. Am J Surg Pathol. 2012;36(6):818–30.

    Article  PubMed  PubMed Central  Google Scholar 

  49. Bataille V, Sasieni P, Cuzick J, et al. Risk of ocular melanoma in relation to cutaneous and iris naevi. Int J Cancer. 1995;60(5):622–6.

    Article  CAS  PubMed  Google Scholar 

  50. Horn EP, Hartge P, Shields JA, et al. J Natl Cancer Inst. 1994;86(19):1476.

    Article  CAS  PubMed  Google Scholar 

  51. Harbour JW, Brantley MA Jr, Hollingsworth H, et al. Association between posterior uveal melanoma and iris freckles, iris naevi, and choroidal naevi. Br J Ophthalmol. 2004;88(1):36–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  52. Naumann G, Yanoff M, Zimmerman LE. Histogenesis of malignant melanomas of the uvea. I. Histopathologic characteristics of nevi of the choroid and ciliary body. Arch Ophthalmol. 1966;76(6):784–96.

    Article  CAS  PubMed  Google Scholar 

  53. Yanoff M, Zimmerman LE. Histogenesis of malignant melanomas of the uvea. II. Relationship of uveal nevi to malignant melanomas. Cancer. 1967;20(4):493–507.

    Article  CAS  PubMed  Google Scholar 

  54. Sahel JA, Pesavento R, Frederick AR Jr, et al. Melanoma arising de novo over a 16-month period. Arch Ophthalmol. 1988;106(3):381–5.

    Article  CAS  PubMed  Google Scholar 

  55. Albert DM, Robinson NL, Fulton AB, et al. Epidemiological investigation of increased incidence of choroidal melanoma in a single population of chemical workers. Int Ophthalmol Clin. 1980;20(2):71–92.

    CAS  PubMed  Google Scholar 

  56. Gordon-Shaag A, Barnard S, Millodot M, et al. Prevalence of choroidal naevi using scanning laser ophthalmoscope. Ophthalmic Physiol Opt. 2014;34(1):94–101.

    Article  PubMed  Google Scholar 

  57. Qiu M, Shields CL. Choroidal nevus in the United States adult population: racial disparities and associated factors in the National Health and Nutrition Examination Survey. Ophthalmology. 2015;122(10):2071–83.

    Article  PubMed  Google Scholar 

  58. Csoma RZ, Toth-Molnar E, Varga A, et al. Risk factors and relationship of cutaneous and uveal melanocytic lesions in monozygotic and dizygotic twin pairs. PLoS One. 2016;11(8):e0160146.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  59. Sumich P, Mitchell P, Wang JJ. Choroidal nevi in a white population: the Blue Mountains eye study. Arch Ophthalmol. 1998;116(5):645–50.

    Article  CAS  PubMed  Google Scholar 

  60. Singh AD, Kalyani P, Topham B. Estimating the risk of malignant transformation of a choroidal nevus. Ophthalmology. 2005;112(10):1784–9.

    Article  PubMed  Google Scholar 

  61. Ponder BAJ. Inherited cancer syndromes. In: Carney D, Sikora K, editors. Genes and cancer. New York: Wiley; 1990.

    Google Scholar 

  62. Jay M, McCartney AC. Familial malignant melanoma of the uvea and p53: a Victorian detective story. Surv Ophthalmol. 1993;37(6):457–62.

    Article  CAS  PubMed  Google Scholar 

  63. Singh AD, Donoso LA, Jackson L, et al. Familial uveal melanoma: absence of constitutional cytogenic abnormalities in 14 cases. [comment]. Arch Ophthalmol. 1996;114(4):502–3.

    Article  CAS  PubMed  Google Scholar 

  64. Singh AD, Demirci H, Shields CL, et al. Concurrent choroidal melanoma in son and father. Am J Ophthalmol. 2000;130(5):679–80.

    Article  CAS  PubMed  Google Scholar 

  65. Singh AD, Shields CL, Shields JA, et al. Uveal melanoma in young patients. Arch Ophthalmol. 2000;118(7):918–23.

    CAS  PubMed  Google Scholar 

  66. Rai K, Pilarski R, Boru G, et al. Germline BAP1 alterations in familial uveal melanoma. Genes Chromosomes Cancer. 2017;56(2):168–74.

    Article  CAS  PubMed  Google Scholar 

  67. Barr CC, McLean IW, Zimmerman LE. Uveal melanoma in children and adolescents. Arch Ophthalmol. 1981;99(12):2133–6.

    Article  CAS  PubMed  Google Scholar 

  68. Greer CH. Congenital melanoma of the anterior uvea. Arch Ophthalmol. 1966;76:77–8.

    Article  CAS  PubMed  Google Scholar 

  69. Singh AD, Schoenfield LA, Bastian BC, et al. Congenital uveal melanoma? Surv Ophthalmol. 2016;61(1):59–64.

    Article  PubMed  Google Scholar 

  70. Al-Jamal RT, Cassoux N, Desjardins L, et al. The pediatric choroidal and ciliary body melanoma study. Ophthalmology. 2016;123(4):898–907.

    Article  PubMed  Google Scholar 

  71. Verdaguer J Jr. Prepuberal and puberal melanomas in ophthalmology. Am J Ophthalmol. 1965;60:1002–11.

    Article  PubMed  Google Scholar 

  72. Singh AD, Shields JA, Eagle RC, et al. Iris melanoma in a ten-year-old boy with familial atypical mole-melanoma (FAM-M) syndrome. Ophthalmic Genet. 1994;15(3–4):145–9.

    Article  CAS  PubMed  Google Scholar 

  73. Cebulla CM, Binkley EM, Pilarski R, et al. Analysis of BAP1 germline gene mutation in young uveal melanoma patients. Ophthalmic Genet. 2015;36(2):126–31.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  74. Broadway D, Lang S, Harper J, et al. Congenital malignant melanoma of the eye. Cancer. 1991;67(10):2642–52.

    Article  CAS  PubMed  Google Scholar 

  75. Posnick JC, Chen P, Zuker R, et al. Extensive malignant melanoma of the uvea in childhood: resection and immediate reconstruction with microsurgical and craniofacial techniques. Ann Plast Surg. 1993;31(3):265–70.

    Article  CAS  PubMed  Google Scholar 

  76. Palazzi MA, Ober MD, Abreu HF, et al. Congenital uveal malignant melanoma: a case report. Can J Ophthalmol. 2005;40(5):611–5.

    Article  PubMed  Google Scholar 

  77. Rai A, Al-Jaradi M, Burnier M, et al. Congenital choroidal melanoma in an infant. Can J Ophthalmol. 2011;46(2):203–4.

    Article  PubMed  Google Scholar 

  78. Pukrushpan P, Tulvatana W, Pittayapongpat R. Congenital uveal malignant melanoma. J AAPOS. 2014;18(2):199–201.

    Article  PubMed  Google Scholar 

  79. Shammas HF, Watzke RC. Bilateral choroidal melanomas. Case report and incidence. Arch Ophthalmol. 1977;95:617–23.

    Article  CAS  PubMed  Google Scholar 

  80. Singh AD, Shields CL, Shields JA, et al. Bilateral primary uveal melanoma. Bad luck or bad genes? Ophthalmology. 1996;103(2):256–62.

    Article  CAS  PubMed  Google Scholar 

  81. Seregard S, Daunius C, Kock E, et al. Two cases of primary bilateral malignant melanoma of the choroid. Br J Ophthalmol. 1988;72(4):244–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  82. Bergman L, Nilsson B, Ragnarsson-Olding B, et al. Uveal melanoma: a study on incidence of additional cancers and familial cases in the Swedish population. Invest Ophthalmol Vis Sci. 2006;47(1):72–7.

    Article  PubMed  Google Scholar 

  83. Scott JF, Vyas R, Galvin J, et al. Primary bilateral uveal melanoma: a population-based study and systematic review. Clin Exp Ophthalmol. 2018;46(5):502–10.

    Article  PubMed  Google Scholar 

  84. Singh AD, Rundle PA, Slater DN, et al. Paraneoplastic melanocytic proliferation: uveal and cutaneous involvement. Arch Ophthalmol. 2003;121:1637–40.

    Article  PubMed  Google Scholar 

  85. Bell RW, Ironside JW, Fleck BW, et al. Cutaneous malignant melanoma metastatic to the choroid: a clinicopathological case report. Eye (Lond). 1995;9. (Pt 5:650–3.

    Article  Google Scholar 

  86. Singh AD, Shields JA, Shields CL, et al. Choroidal melanoma metastatic to the contralateral choroid. Am J Ophthalmol. 2001;132(6):941–3.

    Article  CAS  PubMed  Google Scholar 

  87. Filloy A, Caminal JM, Bruzual MC, et al. Cytogenetical study of a multifocal uveal melanoma. Semin Ophthalmol. 2014;29(3):163–5.

    Article  PubMed  Google Scholar 

  88. Dithmar S, Volcker HE, Grossniklaus HE. Multifocal intraocular malignant melanoma: report of two cases and review of the literature. Ophthalmology. 1999;106(7):1345–8.

    Article  CAS  PubMed  Google Scholar 

  89. Rao R, Pointdujour-Lim R, Ganguly A, et al. Multifocal choroidal melanoma in a patient with germ line Brca-associated protein 1 mutation. Retin Cases Brief Rep. 2018;12(1):1–4.

    Article  PubMed  Google Scholar 

  90. Honavar SG, Shields CL, Singh AD, et al. Two discrete choroidal melanomas in an eye with ocular melanocytosis. Surv Ophthalmol. 2002;47(1):36–41.

    Article  PubMed  Google Scholar 

  91. Echegaray JJ, Medina CA, Biscotti CV, et al. Multifocal primary uveal melanoma: clinical and molecular characteristics. Ocul Oncol Pathol. 2018; https://doi.org/10.1159/000487891.

    Article  PubMed  PubMed Central  Google Scholar 

  92. Gonder JR, Shields JA, Albert DM, et al. Uveal malignant melanoma associated with ocular and oculodermal melanocytosis. Ophthalmology. 1982;89(8):953–60.

    Article  CAS  PubMed  Google Scholar 

  93. Singh AD, De Potter P, Fijal BA, et al. Lifetime prevalence of uveal melanoma in white patients with oculo(dermal) melanocytosis. Ophthalmology. 1998;105(1):195–8.

    Article  CAS  PubMed  Google Scholar 

  94. Van Raamsdonk CD, Bezrookove V, Green G, et al. Frequent somatic mutations of GNAQ in uveal melanoma and blue naevi. Nature. 2009;457(7229):599–602.

    Article  PubMed  CAS  Google Scholar 

  95. Gerami P, Pouryazdanparast P, Vemula S, et al. Molecular analysis of a case of nevus of ota showing progressive evolution to melanoma with intermediate stages resembling cellular blue nevus. Am J Dermatopathol. 2010;32(3):301–5.

    PubMed  Google Scholar 

  96. Shields CL, Kaliki S, Livesey M, et al. Association of ocular and oculodermal melanocytosis with the rate of uveal melanoma metastasis: analysis of 7872 consecutive eyes. JAMA Ophthalmol. 2013;131(8):993–1003.

    Article  PubMed  Google Scholar 

  97. Ota M, Kawamura T, Ito N. Phakomatosis pigmentovascularis Ota. Jpn J Dermatol. 1947;52:1–3.

    Google Scholar 

  98. Happle R. Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol. 2005;141(3):385–8.

    Article  PubMed  Google Scholar 

  99. Tran HV, Zografos L. Primary choroidal melanoma in phakomatosis pigmentovascularis IIa. Ophthalmology. 2005;112(7):1232–5.

    Article  PubMed  Google Scholar 

  100. Honavar SG, Singh AD, Shields CL, et al. Iris melanoma in a patient with neurofibromatosis. Surv Ophthalmol. 2000;45(3):231–6.

    Article  CAS  PubMed  Google Scholar 

  101. Foster WJ, Fuller CE, Perry A, et al. Status of the NF1 tumor suppressor locus in uveal melanoma. Arch Ophthalmol. 2003;121(9):1311–5.

    Article  CAS  PubMed  Google Scholar 

  102. Li FP, Fraumeni JF, Mulvihill JJ, et al. A cancer family syndrome in twenty-four kindreds. Cancer Res. 1988;48:5358–62.

    CAS  PubMed  Google Scholar 

  103. Turner BJ, Siatkowski RM, Augsburger JJ, et al. Other cancers in uveal melanoma patients and their families. Am J Ophthalmol. 1989;107(6):601–8.

    Article  CAS  PubMed  Google Scholar 

  104. Lischko AM, Seddon JM, Gragoudas ES, et al. Evaluation of prior primary malignancy as a determinant of uveal melanoma. A case-control study. Ophthalmology. 1989;96(12):1716–21.

    Article  CAS  PubMed  Google Scholar 

  105. Holly EA, Aston DA, Ahn DK, et al. No excess prior cancer in patients with uveal melanoma. Ophthalmology. 1991;98(5):608–11.

    Article  CAS  PubMed  Google Scholar 

  106. Diener-West M, Reynolds SM, Agugliaro DJ, et al. Second primary cancers after enrollment in the COMS trials for treatment of choroidal melanoma: COMS report no. 25. Arch Ophthalmol. 2005;123(5):601–4.

    Article  PubMed  Google Scholar 

  107. Harbour JW, Onken MD, Roberson ED, et al. Frequent mutation of BAP1 in metastasizing uveal melanomas. Science. 2010;330(6009):1410–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  108. Yoshikawa Y, Sato A, Tsujimura T, et al. Frequent inactivation of the BAP1 gene in epithelioid-type malignant mesothelioma. Cancer Sci. 2012;103(5):868–74.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  109. Abdel-Rahman MH, Cebulla CM, Verma V, et al. Monosomy 3 status of uveal melanoma metastases is associated with rapidly progressive tumors and short survival. Exp Eye Res. 2012;100:26–31.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  110. Buecher B, Gauthier-Villars M, Desjardins L, et al. Contribution of CDKN2A/P16 (INK4A), P14 (ARF), CDK4 and BRCA1/2 germline mutations in individuals with suspected genetic predisposition to uveal melanoma. Familial Cancer. 2010;9(4):663–7.

    Article  CAS  PubMed  Google Scholar 

  111. Easton DF, Steele L, Fields P, et al. Cancer risks in two large breast cancer families linked to BRCA2 on chromosome 13q12-13. Am J Hum Genet. 1997;61(1):120–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  112. Sinilnikova OM, Egan KM, Quinn JL, et al. Germline brca2 sequence variants in patients with ocular melanoma. Int J Cancer. 1999;82(3):325–8.

    Article  CAS  PubMed  Google Scholar 

  113. Iscovich J, Abdulrazik M, Cour C, et al. Prevalence of the BRCA2 6174 del T mutation in Israeli uveal melanoma patients. Int J Cancer. 2002;98(1):42–4.

    Article  CAS  PubMed  Google Scholar 

  114. Cruz C, Teule A, Caminal JM, et al. Uveal melanoma and BRCA1/BRCA2 genes: a relationship that needs further investigation. J Clin Oncol. 2011;29(34):e827–9.

    Article  PubMed  Google Scholar 

  115. Singh AD, Rennie IG, Seregard S, et al. Sunlight exposure and pathogenesis of uveal melanoma. Surv Ophthalmol. 2004;49(4):419–28.

    Article  PubMed  Google Scholar 

  116. Seddon JM, Gragoudas ES, Glynn RJ, et al. Host factors, UV radiation, and risk of uveal melanoma. A case-control study. Arch Ophthalmol. 1990;108(9):1274–80.

    Article  CAS  PubMed  Google Scholar 

  117. Ikehata H, Ono T. The mechanisms of UV mutagenesis. J Radiat Res. 2011;52(2):115–25.

    Article  CAS  PubMed  Google Scholar 

  118. Rivolta C, Royer-Bertrand B, Rimoldi D, et al. UV light signature in conjunctival melanoma; not only skin should be protected from solar radiation. J Hum Genet. 2016;61(4):361–2.

    Article  CAS  PubMed  Google Scholar 

  119. Royer-Bertrand B, Torsello M, Rimoldi D, et al. Comprehensive genetic landscape of uveal melanoma by whole-genome sequencing. Am J Hum Genet. 2016;99(5):1190–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  120. Ajani UA, Seddon JM, Hsieh CC, et al. Occupation and risk of uveal melanoma. An exploratory study. Cancer. 1992;70(12):2891–900.

    Article  CAS  PubMed  Google Scholar 

  121. Behrens T, Lynge E, Cree I, et al. Occupational exposure to electromagnetic fields and sex-differential risk of uveal melanoma. Occup Environ Med. 2010;67(11):751–9.

    Article  PubMed  Google Scholar 

  122. Behrens T, Lynge E, Cree I, et al. Occupational exposure to endocrine-disrupting chemicals and the risk of uveal melanoma. Scand J Work Environ Health. 2012;38(5):476–83.

    Article  PubMed  Google Scholar 

  123. Behrens T, Lynge E, Cree I, et al. Pesticide exposure in farming and forestry and the risk of uveal melanoma. Cancer causes & control : CCC. 2012;23(1):141–51.

    Article  PubMed  Google Scholar 

  124. Stang A, Schmidt-Pokrzywniak A, Lash TL, et al. Mobile phone use and risk of uveal melanoma: results of the risk factors for uveal melanoma case-control study. J Natl Cancer Inst. 2009;101(2):120–3.

    Article  PubMed  PubMed Central  Google Scholar 

  125. Mulla ZD, Margo CE. Season of birth and the risk of uveal melanoma. Ophthalmic Epidemiol. 2009;16(2):74–7.

    Article  PubMed  Google Scholar 

  126. Guenel P, Laforest L, Cyr D, et al. Occupational risk factors, ultraviolet radiation, and ocular melanoma: a case-control study in France. Cancer Causes Control. 2001;12(5):451–9.

    Article  CAS  PubMed  Google Scholar 

  127. Vidal JL, Bacin F, Albuisson E, et al. “Melanoma 92”. Epidemiological study of uveal melanoma in France. J Fr Ophtalmol. 1995;18(8–9):520–8.

    CAS  PubMed  Google Scholar 

  128. Ge YR, Tian N, Lu Y, et al. Occupational cooking and risk of uveal melanoma: a meta- analysis. Asian Pac J Cancer Prev. 2012;13(10):4927–30.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. School of Medicine, Case Western University, Cleveland, OH, USA

    Nakul Singh

  2. Ophthalmic Pathology and Oncology Service and Department of Clinical Neuroscience, St. Erik Eye Hospital and Karolinska Institutet, Stockholm, Sweden

    Stefan Seregard

  3. Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA

    Arun D. Singh

Authors
  1. Nakul Singh
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Stefan Seregard
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Arun D. Singh
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Arun D. Singh .

Editor information

Editors and Affiliations

  1. University of Oxford, Oxford, UK

    Bertil E. Damato

  2. Cole Eye Institute Cleveland Clinic, Cleveland, OH, USA

    Arun D. Singh

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Singh, N., Seregard, S., Singh, A.D. (2019). Uveal Melanoma: Epidemiologic Aspects. In: Damato, B., Singh, A. (eds) Clinical Ophthalmic Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-17879-6_4

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-17879-6_4

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-17878-9

  • Online ISBN: 978-3-030-17879-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation