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Rare Eye Cancer Responds to New Drug, But What is Uveal Melanoma

Uveal melanoma

Early indications are that some individuals with a rare eye cancer called uveal melanoma may respond to a new drug, selumetinib. What is this novel medication and what is uveal melanoma?

Uveal melanoma has been a challenge

If you have not heard of uveal melanoma, it’s not surprising since this rare eye cancer affects about 5.3 to 10.9 individuals per million. It is the most common primary intraocular (inside the eyeball) disease, and it can be deadly.

According to a recent study published in Cancer Treatment Reviews, up to 50 percent of patients with primary uveal melanoma eventually develop metastasis, and 90 percent of the time the liver is affected. Average survival is then 4 to 5 months. However, those with metastasis to the lung or bone have an average survival of 19 to 28 months.

Despite advances in other cancer treatments, the same cannot be said about uveal melanoma, as survival rates have not changed in 30 years. Therefore, the new phase II trial results concerning selumetinib, a novel kinase inhibitor, provide some hope for patients who develop the disease, which is diagnosed in about 2,000 people per year in the United States.

Here’s a look at the findings from the trial, which involved 98 patients. Participants were administered either selumetinib or temozolomide (Temodar), a standard cancer drug given to patients with skin melanoma.

  • Half of the patients taking selumetinib experienced at least some reduction in the size of their tumor compared with 11 percent who took temozolomide
  • 15 percent of patients taking selumetinib had at least a 30 percent reduction in their tumor compared with no patients who took temozolomide
  • Progression-free survival was 54 percent better among selumetinib patients than among those taking temozolomide (16 weeks vs 7 weeks)
  • Overall survival was somewhat better for selumetinib (75 mg taken twice daily) compared with temozolomide (150 mg/m2 taken four times daily): 11 months vs 9 months

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Research into the potential use of selumetinib for uveal melanoma will continue. For now, as noted by melanoma specialist Michael Atkins, MD, deputy director of Georgetown Lombardi Comprehensive Cancer Center, “This is the first time any systemic therapy has been shown to work in patients with ocular melanoma,” a disease that “has been viewed as practically untreatable until now.”

Other treatments for uveal melanoma
Thus far, uveal melanoma has been resistant to conventional systemic chemotherapy, and so researchers have explored other options. In addition to selumetinib, other target therapies under investigation include bevacizumab (Avastin), imatinib, and ipilimumab.

In the earlier stages of uveal melanoma, treatment options such as proton beam radiotherapy and plaque brachytherapy (a type of radiation) can successfully preserve the eye and vision in some cases. Enucleation (removal of the eye) is usually recommended for advanced melanomas that take up most of the intraocular space or that have affected the optic nerve.

Unfortunately, once the cancer has spread (metastasis), prognosis is poor. Therefore, it is hoped that new drug treatments and other therapies will be found to successfully manage this rare eye disease.

American Society of Clinical Oncology
Carvajal RD et al. Phase II study of selumetinib (sel) versus temozolomide (TMZ) in gnag/Gna11 (Gg/11) mutant (mut) uveal melanoma. ASCO 2013
Papastefanou VP, Cohen VML. Uveal melanoma. Journal of Skin Cancer 2011; 2011:573974. Published online June 30, 2011
Spagnolo F et al. Uveal melanoma. Cancer Treatment Reviews 2012 Aug; 38(5): 549-53

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