Original Article

Uveal Melanoma in the First 4 Decades of Life

Authors: Merve Sagus, Agop Y. Bedikian, MD


Objectives: According to reports in the clinical literature, metastatic uveal melanoma in young adults has not been well studied. This article describes the clinical characteristics and natural history of patients who were diagnosed as having uveal melanoma in the first 4 decades of life.

Methods: This was a chart review of patients aged 40 years or younger who were treated for metastatic uveal melanoma. The eligibility criteria included an established diagnosis of primary uveal melanoma that was treated with localized plaque radiation therapy or enucleation, the presence of radiologically confirmed systemic tumor metastasis, and available therapy and follow-up information. Sixty-five patients met the eligibility criteria and were included in our study.

Results: The interval between the time of diagnosis of the primary tumor to the diagnosis of systemic metastasis and the median survival duration from diagnosis of the primary and metastatic disease were significantly longer than were those of patients with uveal melanoma patients overall.

Conclusions: Patients with uveal melanoma who are diagnosed within the first 4 decades of life have a better prognosis than do patients with uveal melanoma overall; however, the prognosis of patients with metastatic uveal melanoma remains poor.

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1. Seregard S. Posterior uveal melanoma: the Swedish perspective. Acta Ophthalmol Scand 1996;74:315-329.
2. Wilkes S, Kurland L, Campbell RJ, et al. Incidence of uveal malignant melanoma. Am J Ophthalmol 1979;88(3 Pt 2):629-630.
3. The Collaborative Ocular Melanoma Study (COMS) randomized trial of pre-enucleation radiation of large choroidal melanoma II: initial mortality findings. COMS report no. 10.Am J Ophthalmol 1998;125:779-796.
4. Mooy CM, De Jong PT. Prognostic parameters in uveal melanoma: a review. Surv Ophthalmol 1996;41:215-228.
5. McLean IW, Foster WD, Zimmerman LE, et al. Inferred natural history of uveal melanoma. Invest Ophthalmol Vis Sci 1980;19:760-770.
6. Kolandjian NA, Wei C, Patel SP, et al. Delayed systemic recurrence of uveal melanoma. Am J Clin Oncol 2013;36:443-449.
7. Kujala E, Makitie T, Kivela¨ T. Very long-term prognosis of patients with malignant uveal melanoma. Invest Ophthalmol Vis Sci 2003;44:4651-4659.
8. Bedikian AY, Legha SS, Mavligit G, et al. Treatment of uveal melanoma metastatic to the liver: a review of the M. D. Anderson Cancer Center experience and prognostic factors. Cancer 1995;76:1665-1670.
9. Gragoudas ES, Egan KM, Seddon JM, et al. Survival of patients with metastases from uveal melanoma. Ophthalmology 1991;98:383-389.
10. Hsueh EC, Essner R, Foshag LJ, et al. Prolonged survival after complete resection of metastases from intraocular melanoma. Cancer 2004;100:122-129.
11. Kath R, Hayungs J, Bornfeld N, et al. Prognosis and treatment of disseminated uveal melanoma. Cancer 1993;72:2219-2223.
12. Bedikian AY. Metastatic uveal melanoma therapy: current options. Int Ophthalmol Clin 2006;46:151-166.
13. Varela M, Real MI, Burrel M, et al. Chemoembolization of hepatocellular carcinoma with drug eluting beads: efficacy and doxorubicin pharmacokinetics. J Hepatol 2007;46:474-481.
14. Nathan FE, Berd D, Sato T. et al. BOLD+interferon in the treatment of metastatic uveal melanoma: first report of active systemic therapy. J Exp Clin Cancer Res 1997;16:201-208.
15. Handbook for Reporting Results of Cancer Treatment. Geneva: World Health Organization; 1979.
16. Eisenhauer EA, Therasse P, Bogaerts J, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer 2009;45:228-247.
17. Singh AD, Topham A. Incidence of uveal melanoma in the United States:1973-1997. Ophthalmology 2003;110:956-61.
18. Weis E, Shah CP, Lajous M, et al. The association between host susceptibility factors and uveal melanoma: a meta-analysis. Arch Ophthalmol 2006;124:54-60.
19. Zimmerman LE, McLean IW. An evaluation of enucleation in the management of uveal melanomas. Am J Ophthalmol 1979;87:741-760.
20. Robertson DM, Earle JD, Kline RW. Brachytherapy for choroidal melanoma. In: Ryan SJ, ed. The Retina. 4th ed. New York: Elsevier-Mosby, 2010:747-759.
21. Virgili G, Gatta G, Ciccolallo L, et al. Incidence of uveal melanoma in Europe. Ophthalmology 2007;114:2309-2315.
22. Kolandijan NA, Wei C, Burke A, et al. Malignant melanoma in teenagers and young adults. J Pediatr Hematol Oncol 2014;36:552-558.
23. Strouse JJ, Fears TR, Tucker MA, et al. Pediatric melanoma: risk factor and survival analysis of the surveillance, epidemiology and end results database. J Clin Oncol 2005;23:4735-4741.
24. Wehner MR, Chren MM, Nameth D, et al. International prevalence of indoor tanning: a systematic review and meta-analysis. JAMA Dermatol 2014;150:390-400.
25. Crowley NJ, Seigler HF. Late recurrence of malignant melanoma. Analysis of 168 patients. Ann Surg 1990;212:173-177.
26. Callaway MP, Briggs JC. The incidence of late recurrence (greater than 10 years); an analysis of 536 consecutive cases of cutaneous melanoma. Br J Plast Surg 1989;42:46-49.
27. Diener-West M, Reynolds SM, Agugliaro DJ, et al. Development of metastatic disease after enrollment in the COMS trials for treatment of choroidal melanoma: Collaborative Ocular Melanoma study group report no. 26. Arch Ophthalmol 2005;123:1639-1643.
28. Kim IK, Lane AM, Gragoudas ES. Survival in patients with presymptomatic diagnosis of metastatic uveal melanoma. Arch Ophthalmol 2010;128:871-875.
29. Hansel G, Schonlebe J, Haroske G, et al. Late recurrence (10 years or more) of malignant melanoma in south-east Germany (Saxony). A single-centre analysis of 1881 patients with a follow-up of 10 years or more. J Eur Acad Dermatol Venereol 2010;24:833-836.
30. Aguirre-Ghiso JA. Models, mechanisms and clinical evidence for cancer dormancy. Nat Rev Cancer 2007;7:834-846.
31. Raderman D, Giler S, Rothem A, et al. Late metastases (beyond ten years) of cutaneous malignant melanoma. Literature review and case report. J Am Acad Dermatol 1986;15(2 Pt 2):374-378.
32. Rietschel P, Panageas KS, Hanlon C, et al. Variates of survival in metastatic uveal melanoma. J Clin Oncol 2005;23:8076-8080.
33. Onken MD, Worley LA, Long MD, et al. Oncogenic mutations in GNAQ occur early in uveal melanoma. Invest Ophthalmol Vis Sci 2008;49:5230-5234.
34. Van Raamsdonk CD, Bezrookove V, Green G, et al. Frequent somatic mutations of GNAQ in uveal melanoma and blue naevi. Nature 2009;457:599-602.
35. Abdel-Rahman MH, Boru G, Massengill J, et al. MET oncogene inhibition as a potential target of therapy for uveal melanomas. Invest Ophthalmol Vis Sci 2010;51:3333-3339.
36. All-Ericsson C, Girnita L, Seregard S, et al. Insulin-like growth factor-1 receptor in uveal melanoma: a predictor for metastatic disease and a potential therapeutic target. Invest Ophthalmol Vis Sci 2002;43:1-8.