Current Management of Uveal Melanoma

Kamalesh J Ramaiya; J William Harbour

Disclosures

Expert Rev Ophthalmol. 2007;2(6):939-946. 

In This Article

Abstract and Introduction

Uveal melanoma is the most common primary malignancy of the eye and frequently leads to metastatic death. Until the mid-20th Century, the only treatment for uveal melanoma was enucleation. Since then, many new globe-sparing options have been developed, including plaque brachytherapy, charged particle radiotherapy, transpupillary thermotherapy, stereotactic radiotherapy and local resection. Despite these advances, it remains unclear what effect they are having on patient survival. Recent research has identified highly accurate molecular markers of metastasis and has provided new insights into the molecular pathogenesis of uveal melanoma. Based on these discoveries, a new treatment paradigm is emerging that combines prompt treatment of the primary tumor with molecular predictive testing and, eventually, prophylactic systemic treatment in patients at high risk of metastasis. Here, we present an update of the outcomes, indications and complications of the current treatment options for uveal melanoma and speculate on likely improvements in management over the next 5 years.

Uveal melanomas can arise anywhere in the uveal tract, including the iris, ciliary body and choroid (Figure 1). Melanomas of the ciliary body and choroid are often considered together as ‘posterior uveal melanoma,' since their clinical features overlap and are somewhat distinct from iris melanoma. Uveal melanoma is the most common primary intraocular malignancy in humans, with an incidence of approximately 1200–1500 new cases per year in the USA.[1] Uveal melanoma comprises approximately 5–12% of all melanomas, over 90% of which are posterior uveal melanomas. Risk factors include light skin color, blonde hair and blue eyes, and there is a slight male preponderance.[2] Rare familial cases have been reported,[3,4] but no single disease-causing gene appears to be responsible for all cases. UV light exposure as a risk factor remains controversial.[2] Median survival after clinical detection of metastases is approximately 9 months[5] and 15-year mortality is approximately 50%.[6] Here, we review current options for the management of uveal melanoma and discuss likely future directions in management of both the primary tumor and metastatic disease.

Clinical appearance of uveal melanoma. (A) Fundus photograph of choroidal melanoma (arrows). (B) Ultrasonography (B-scan mode) of the same choroidal melanoma showing dome-shaped choroidal tumor (arrow). (C) Clinical photograph of an iris melanoma (arrow). (D) Goniophotograph of the same iris melanoma, showing the location of the tumor in the iris periphery and extending into the anterior chamber angle.

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