An expert on uveal melanoma discusses the presentation, diagnosis, and prognosis of the disease.
How does uveal melanoma typically present and how is it diagnosed?
Marlana M. Orloff, M.D.: Patients with uveal melanoma can present in a couple of different ways. Some patients have a freckle in the eye that’s followed over time. Other patients just show up for a routine eye exam and it’s found. But most patients do have some sort of visual disturbance that brings them first to the eye doctor. Oftentimes, it’s retinal detachment symptoms like flashes and floaters. Patients will first see an eye doctor, are often then referred to a retina specialist, and then ultimately an ocular oncologist, who uses ultrasound and direct visualization to diagnose the eye melanoma.
What is the typical prognosis for patients diagnosed with uveal melanoma? How is the primary tumor treated?
Marlana M. Orloff, M.D.: Once the diagnosis of uveal melanoma is made, the ocular oncologist often discusses with the patient options for treatment of the primary tumor. This could [be] plaque radiation, nucleation, removal of the eye, or external radiation. Primary treatment of the eye tumor, regardless of the method, is very effective. The risk of it coming back in the eye is actually quite low. Patients are then often referred to a medical oncologist for surveillance to make sure that the cancer doesn’t come back in other parts of the body.
Despite the very effective treatment of the primary tumor, about half of the patients—and this is all comers—can recur with the cancer most commonly in the liver at some point after the primary eye diagnosis. We think that before the eye tumor is even treated, it’s possible that some cells could have gotten out and could lead to metastasis in the future. Because we don’t have the ability to really search for those single cells floating around, we rely on information about the primary tumor like the size and genetics to try to estimate what a patient’s risk is. For patients with very large tumors, we think their risk may be a little bit higher. Patients with things like monosomy 3, 8q amplification, or something called a class 2 genetic signature may have a greater than 50% risk of the cancer returning. Depending on a patient’s risk assessed by tumor size and genetics, we then monitor patients. The higher the risk, the closer we follow them. And often following these patients involves a series of things like MRIs, CT scans, and meeting with a medical oncologist.
Transcript edited for clarity.