A Cure In Sight™
Melanomas are cancerous tumors arising from melanocytes, which are cells that produce melanin, a brown pigment that absorbs light. Ocular Melanomas comprise uveal and conjunctival melanomas. Uveal Melanoma, or UM, develops within the uveal tract, which comprises the choroid, the ciliary body and the iris. The choroid is a cup-shaped tissue under the retina. The ciliary body is a muscular ring that adjusts the focus of the lens, to maintain clear vision. The inner lining of the ciliary body pumps fluid into the eye to maintain the shape of the eyeball. The iris adjusts the size of the pupil, regulating the amount of light reaching the retina. Ocular melanomas are rare, developing in about six people per million per year, or about 2500 persons in the United States each year. These tumors represent approximately one twentieth (i.e., 5%) of all melanomas in the body. About nine-out-of-ten uveal melanomas are choroidal, the rest originating in ciliary body or iris. Intraocular melanomas are the most common form of cancer arising in adult eyes.
Ocular melanomas are most common in individuals with white skin and blue eyes. Some uveal melanomas arise from moles or, rarely, in individuals inheriting the ‘BAP1 cancer predisposition syndrome’. Similarly, conjunctival melanomas may develop ‘out from nowhere’ or from moles or from brown areas in the conjunctiva called ‘primary acquired melanosis’. Ocular melanomas almost always present in adulthood, especially around the age of 60 years. Men and women are affected in equal numbers. Whereas conjunctival and iris melanomas are visible to the naked eye, detection of choroidal and ciliary body melanomas requires ophthalmoscopic examination, usually performed by an optometrist or ophthalmologist. The most common symptoms of uveal melanomas at the back of the eye are blurred vision, blind-spots, and seeing flashing lights.
A Cure In Sight™ – For the Patients, by the Patients.
A Cure in Sight (ACIS) supports families who have ocular melanoma and focuses on research for a cure. Through public and private partnerships we provide clear and reliable education information providing a support community to patients and their caregivers.
ACIS educates the public about ocular melanoma, treatment options, clinical trials that may improve their prognosis, making it less overwhelming.
ACIS is dedicated to ongoing research. We know we can accelerate viable research projects that otherwise would stall and not be completed, missing opportunities to advance research for effective treatments and possibly a cure.
Overall, about seven-in-ten patients can expect to live more than ten years after diagnosis and treatment of the ocular melanoma. The remainder will develop metastatic disease, which occurs when the ocular melanoma circulates through the body to form new tumors (‘metastases’) in other organs. Metastases usually develop many months or years after apparent good health and are only rarely detectable when the ocular tumor is diagnosed and treated.
Uveal melanomas metastasize through the blood circulation, almost always to the liver. Such ‘metastatic disease’ develops almost exclusively in patients whose tumor shows particular genetic abnormalities, such as loss of chromosome 3 (‘monosomy 3’), BAP1 deletion, and a class 2 gene expression profile. These abnormalities are detected by laboratory analysis of a tumor sample, which is obtained by biopsy If the eye is retained by means of radiotherapy or laser treatment. Life expectancy also depends on the tumor size, location and extent. Keeping the eye does not increase the risk of metastatic disease, as long as the tumor is successfully eradicated. Regular liver scans are usually performed to detect metastases before symptoms develop so as to maximize any chances of prolonging life. There are several kinds of treatment, which include systemic therapies as well as liver-directed therapies, such as tumor resection, chemo-embolization and hepatic perfusion. Unfortunately, once metastases develop, the prognosis is poor, although clinical trials are showing some promise.
Conjunctival melanomas are quite distinct from uveal melanomas, both genetically and in their metastatic behavior, resembling skin melanomas in these respects. Conjunctival melanomas can spread through lymph channels to glands in the neck or directly to other parts of the body. Metastatic disease is more likely to develop if the tumor is large or if it involves the eyelid conjunctiva or the caruncle. Metastases from conjunctival melanoma are more responsive to systemic therapy than uveal melanomas, especially if they show mutations of the BRAF gene. Impressive advances are occurring in this field.