The Case of the Affluent Artist With Rapidly Deteriorating Vision

Albert Lowenfels, MD; Patrick Maisonneuve, Eng

Disclosures

August 10, 2010

How Would the Disease Be Considered and Treated Today?

What Is Known About the Causes of Ocular Melanoma?

Although most melanomas arise from melanocytes located in the skin, these cells are also found within the eye, particularly in the pigmented cells that line the eyeball. Melanomas arising in the eye are rare: The ratio of ocular to cutaneous melanoma is about 1 to 50. Despite its rarity, melanoma is the most common primary ocular tumor of adults.

Although ocular and cutaneous melanomas arise from similar progenitor cells, their causes are somewhat different. Skin melanomas are strongly related to sun exposure; ocular melanomas are not. As with cutaneous melanomas, ocular melanomas are more frequent in persons with light-colored irises (blue or gray) and fair skin color and in individuals who easily tan. However, neither red nor blond hair color nor latitude -- risk factors for cutaneous melanoma -- seem to be risk factors for ocular melanoma.[4]

Several studies have shown that cooks and welders have an increased risk for ocular melanoma.[5,6,7] The possible explanation may be that the eyes are often exposed to strong light or heat in both occupations.

One strange characteristic of this tumor is its propensity to metastasize to the liver -- often many years after treatment of the original tumor. The factors responsible for keeping the tumor suppressed for so long are unknown.

One should check for ocular melanoma in a patient with an enlarged liver and a prior eye problem, which helps explain the probable diagnosis in Sir Joshua Reynolds' case. Very rarely a clinician will encounter a patient with unilocular jaundice that is best explained by an unusual sequence of events: enucleation of the eye, replacement with a prosthetic device, and subsequent development of jaundice related to replacement of the liver with metastatic melanoma. Of course, the prosthetic eye will remain clear.

How Is Ocular Melanoma Treated Today?

Fundoscopic examination of the eye is now a routine part of an eye examination, and ophthalmoscopy can detect most ocular melanomas. The presence of a suspected ocular melanoma can be confirmed by ultrasonography. Other tests include fluorescein angiography, computed tomography, and magnetic resonance imaging. With these tests, the accuracy of diagnosing ocular melanoma is nearly 100%; confirmatory biopsy is rarely necessary.

After diagnosis of an ocular melanoma, several treatment options are available.[8] In 1985, many centers joined together to form the Collaborative Ocular Melanoma Study (COMS), a joint project of the National Eye Institute and the National Cancer Institute.[9] The study aim was to determine the most effective way to manage these rare tumors. The initial project was a randomized trial comparing radiotherapy with enuculation of the eye.

The study found that most of these tumors can be managed with local radiotherapy, termed "plaque therapy." Small radioactive pellets inside a container that is sutured to the sclera deliver radiotherapy to the tumor. The COMS trial, based on more than 1300 patients with medium-sized ocular melanomas, showed that radiotherapy with iodine-125 yielded 5-year survival rates of about 80% -- equal to that obtained by enucleation -- the traditional form of treatment for this tumor.[10]

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