A recent study published in the Journal of AAPOS investigated clinical manifestations of ocular melanoma (OM) in pediatric patients via a meta-analysis.
Give me some background first.
Prior research into pediatric OM has largely focused on patients up to age 21 diagnosed with uveal melanoma (UM)—the most common primary intraocular malignancy in adults.
Researchers set out to assess the clinical manifestations and prognoses within OM patients under the age of 12 in order to gain insight into the different elements of the disease.
Now, talk about the study.
In this meta-analysis, investigators pulled from both published and unpublished cases, from databases, and international oncology centers.
In all, they included 133 eyes from 133 pediatric patients with the following types of melanoma:
- Choroidal or ciliary body (50%)
- Iris (25%)
- Conjunctival (19%)
- Eyelid (6%)
Findings?
The mean presentation age of the disease was 7 years old and 49% of the patients were male. There was no variation of family history relating to OM by the different tumor sites investigated.
Of the choroidal or ciliary body, 39% was found in boys.
The authors suggested that this was due to girls reaching puberty earlier, as puberty is associated with an increased presence in uveal melanoma.
Tell me more.
Conjunctival melanoma had the highest death rate (8%), followed by choroidal or ciliary body (5%)..
Additionally, an association with ocular melanocytosis (a congenital eye disease that is a risk factor for UM) was found at the following percentages per site:
- Choroidal or ciliary body (15%)
- Iris (11%)
- Conjunctival (4%)
- Eyelid (0%)
Go on…
Ocular melanocytosis was associated with 5.6 times higher mortality.
According to the study authors, “Melanocytosis was reported in 15% of children in the present study, compared to 0.04% in the general population.”
Anything else?
Mean follow-up was conducted and metastasis was seen in each site at the respective levels:
- Choroidal or ciliary body: 12% at 74 months
- Iris: 9% at 85 months
- Conjunctival: 19% at 50 months
- Eyelid: 13% and 105 months
Expert opinion?
The authors concluded that ocular melanoma in the pediatric population is rare, with unique clinical features and outcomes.
Per the study authors, “Although we report the largest cohort of pediatric OM to date, small sample size in younger patients is an inherent challenge.”
They continued: “The age cutoff for inclusion means that actual pre- or post-pubertal status of patients was un-known.”
Take home.
Ultimately the researchers concluded that the features and consequences emerging from OM have a number of variations.
Due to this variation, and also to the rareness of the disease in children, further research is necessary to establish causal relationships and discover additional factors.