Routine Surveillance Necessary for Young Patients with Posterior Uveal Melanoma

Young patients diagnosed with posterior uveal melanoma may have similar, if not worse, prognoses compared with the overall patient population, according to a retrospective, descriptive case series published in JAMA Ophthalmology.

Posterior uveal melanoma, or cancer of the eye, is the most common intraocular tumor in adults and represents approximately 5 percent of all melanoma cases. It also comes with a relatively high mortality rate due to metastasis. But because of its rarity in patients younger than 21 years old, there is a lack of clinical data in this patient population.

That’s why researchers from the department of ophthalmology in the College of Medicine at the University of Cincinnati used a statistical analysis of data to describe the baseline clinical features, treatment and clinical course of this patient population.

Of the 2,265 patients diagnosed with posterior uveal melanoma between July 1980 and December 2013, 18 patients (0.8 percent) were younger than 21 years.

Patients were an average age of 16.6 years — including one patient who was 5 years old and another who was younger than 10 – of which the majority (89 percent) were teenagers and experienced puberty at the time of diagnosis. Overall, nine patients were affected in the right eye and nine in the left. Additionally, none of these patients had a family history of uveal melanoma.

Enucleation (removal of the eye) was performed in 50 percent of patients, with tumor resection being done in only 17 percent of patients. These percentages are higher than what is found in older patient populations, the researchers wrote, adding that this could be a result of parents’ concerns of the long-term effects of radiation therapy in this subgroup.

Eight patients developed metastatic disease (44 percent), and all eight died as a result. Where the median overall survival was 11.9 years after treatment of the primary intraocular tumor, the median survival time after detection of metastasis was 2.3 months.

The study noted that three of the eight patients who developed metastatic disease did not show evidence of metastasis until more than five years post-treatment. Previous studies have indicated that metastasis may sometimes not emerge until ten or even 20 years after enucleation, leading the researchers to note that some surviving patients in their initial group of 18 may ultimately develop metastasis.

“What this tells us is that young persons who appear to be survivors of posterior uveal melanoma for five years or longer posttreatment should not be assured of an ultimate cure,” the researchers wrote. “Clinicians should advise such patients and their families about the potential for late emergence of uveal melanoma metastasis after an extended latent interval posttreatment.”

The researchers acknowledged they had limited ability to fully understand why this patient group had a rate of survival comparable with that of adult patients. “To better determine metastatic risk and patient survival probabilities, future studies should include gene expression profile testing and next-generation sequencing for mutation analysis of these tumors,” they added.