I once read an article that stated that many rare diseases are only rare because they are often misdiagnosed because doctors are not familiar with them and misinterpret the symptoms for something else.

Case in point is the condition plaguing my oldest daughter. She has always had a host peculiar conditions her entire life but it wasn’t until she was in her later 30s that a doctor finally diagnosed her as having McCune Albright Syndrome (MAS). Although MAS was first diagnosed in 1937 by Donovan James McCune and Fuller Albright, the pediatrician who treated our daughter back in the 1970s and 1980s was totally unfamiliar with the non-hereditary genetic mutational disease, so he had no clue as to the cause of many of the conditions she displayed.

At one time, McCune Albright Syndrome was thought to occur in about 1 of every 1 million people, but that figure has been revised to closer to 1 in ever 500,000 and as more doctors learn about MAS that probability could continue to be greater.

One has to wonder if something similar is the case with a rare form of eye cancer known as ocular melanoma. Currently, it is believed that ocular melanoma only occurs in 6 of every 1 million people. This is why doctors and researchers are puzzled and concerned about the recent reports of a couple of clusters of this rare eye cancer that have suddenly appeared.

At the time of this writing, at least 18 people in the Huntersville, North Carolina area and another 30+ people in the Auburn, Alabama area that have been recently diagnosed with ocular melanoma. The incidents made national news when three women who have been diagnosed with ocular melanoma are friends and attended Auburn University together. That may not be just a coincidence and has doctors scratching their heads trying to find out.

Dr. Marlana Orloff, an oncologist who is treating some of these patients commented:

“Most people don’t know anyone with this disease. We said, ‘OK, these girls were in this location, they were all definitively diagnosed with this very rare cancer — what’s going on’?”

So, what is ocular melanoma?

According to the Ocular Melanoma Foundation:

“OM is an aggressive form of cancer that can involve any of three areas of the eye: the iris (the pigmented area surrounding your pupil), the ciliary body (a thin tissue layer in your eye responsible for aqueous humor production), and/or the choroid or posterior uvea (the vascular layer of the eye between the retina and the white outer layer known as the sclera; this pigmented tissue full of blood vessels nourishes the retina). These three areas are collectively known as the uvea or uveal tract, and OM can occur in any combination of the three. Iris melanomas have the best prognosis, whereas melanomas of the ciliary body have the worst. Most uveal tract melanomas originate in the choroid; the ciliary body is less commonly a site of origin, and the iris is the least common. Melanoma can also occur in the thin lining over the white part of the eye (the conjunctiva) or on the eyelid, but this is very rare.”

Is ocular melanoma curable?

Sorry but the answer is no, it is not. No one knows what causes OM so they consequently have no way of knowing how to cure it.

Is ocular melanoma fatal?

Sorry, again. According to the Ocular Melanoma Foundation:

”Approximately 50% of patients with OM will develop metastases by 10 to 15 years after diagnosis (a small percentage of people will develop metastases even later i.e. 20-25 years after their initial diagnosis). Metastatic disease is universally fatal. This 50% mortality rate is unchanged despite treatment advances in treating the primary eye tumor.”

They do know that OM tends to occur more often in people with light colored skin and lighter eye color, especially blue eyes. The frequency of developing OM also tends to increase with age but can occur in anyone at any age and with any eye or skin color.

Chances are with these two clusters hitting the news and becoming more well known, the rare eye form of eye cancer will probably not be as rare as once thought.