Posterior uveal melanoma in adolescents and children: current perspectives

Uveal melanoma (UM) is a global disease which especially occurs in elderly people. Its incidence varies widely between populations, with the highest incidence among Caucasians, and a South-to-North increase in Europe. As northern Europeans often have blond hair and light eyes, we wondered whether iris colour may be a predisposing factor for UM and if so, why. We compared the distribution of iris colour between Dutch UM patients and healthy Dutch controls, using data from the Rotterdam Study (RS), and reviewed the literature regarding iris colour. We describe molecular mechanisms that might explain the observed associations. When comparing a group of Dutch UM patients with controls, we observed that individuals from Caucasian ancestry with a green/hazel iris colour (Odds Ratio (OR) = 3.64, 95% Confidence Interval (CI) 2.57–5.14) and individuals with a blue/grey iris colour (OR = 1.38, 95% CI 1.04–1.82) had a significantly higher crude risk of UM than those with brown eyes. According to the literature, this may be due to a difference in the function of pheomelanin (associated with a light iris colour) and eumelanin (associated with a brown iris colour). The combination of light-induced stress and aging may affect pheomelanin-carrying melanocytes in a different way than eumelanin-carrying melanocytes, increasing the risk of developing a malignancy.

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“…Males have a slightly higher incidence of UM than females [ 23 ]. The reported 5-year survival rate ranges from 66% to 82% [ 24 , 25 , 26 , 27 ].…”

Section: Incidencementioning

confidence: 99%

Iris Colour and the Risk of Developing Uveal Melanoma

Houtzagers

Wierenga

Ruys

et al. 2020

IJMS

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“…Some nevi can enlarge in adulthood; however, such growth is rare and tends to be subtle (i.e., <0.5 mm/year) [13, 14]. Growth before adulthood is not usually a sign of malignancy but nevertheless requires monitoring as choroidal melanoma can develop at a young age [15]. Some patients present with what appears to be a common nevus that was not noted previously.…”

Section: Discussionmentioning

confidence: 99%

Distinguishing Choroidal Nevi from Melanomas Using the MOLES Algorithm: Evaluation in an Ocular Nevus Clinic

et al. 2021

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Objective: The aim of this study was to determine the sensitivity and specificity of the MOLES scoring system in differentiating choroidal melanomas from nevi according to Mushroom shape, Orange pigment, Large tumor size, Enlarging tumor, and Subretinal fluid (SRF). Methods: Color photographs, fundus-autofluorescence images, and optical coherence tomography of 222 melanocytic choroidal tumors were reviewed. Each MOLES feature was retrospectively scored between 0 and 2 and tumors categorized as “common nevus,” “low-risk nevus,” “high-risk nevus,” and “probable melanoma” according to the total score. MOLES scores were compared with the experts’ diagnosis of melanoma. Results: The MOLES scoring system indicated melanoma in all 81 tumors diagnosed as such by ocular oncologists (100% sensitivity) and nevus in 135 of 141 tumors given this diagnosis by these experts (95.7% specificity). Of the 6 tumors with discordant diagnoses, 4 had basal diameters exceeding 6 mm, all with SRF and/or orange pigment, and 2 small tumors showed either significant SRF with traces of orange pigment, or vice versa. Conclusions: The MOLES system for diagnosing melanocytic choroidal tumors compares well with expert diagnosis but needs to be evaluated when deployed by ophthalmologists and community optometrists in a wide variety of working environments.

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“…Choroidální melanom u dětí je velmi vzácný [4]. V Čechách a na Slovensku se problematikou uveálního melanomu, zejména dospělých pacientů, zabývá profesorka Furdová a spol.…”

Section: Diskuzeunclassified

“…Většina praktických oftalmologů se za celou svoji praxi nikdy s případem dětského uveálního melanomu nesetká. Incidence uveálního melanomu u dětí a dospělých vykazuje obrovské rozdíly [1,4]. Incidence uveálního melanomu na milion obyvatel je u dětí 0-4 roky 0, 10-14 let 0,2 % a 15-20 let 0,4 %.…”

Section: Diskuzeunclassified

Uveal Melanoma In A 15-Year-Old Girl. Case Report

Žajdlíková¹,

Autrata²,

Krejčířová³

et al. 2021

CSO

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Uveal melanoma is the most common intraocular tumour in adults, it is a form of cancer that affects mostly older adults, as the average age at detection of this tumour is 60 years, but it can occur in any age group with no significant gender difference. However, uveal melanoma is very rare in children compared to the adult population, accounting for 1 % of all cases. In pediatric patients, malignant uveal melanoma is more frequently manifested during puberty, leading to speculation of an association between uveal melanoma and growth hormone levels. Prognostic factors for uveal melanoma include tumour histology, chromosomal abnormalities, tumour size, extrascleral spread and tumour location. Risk factors for uveal melanoma include melanocytosis, neurofibromatosis type 1 and dysplastic naevus syndrome. Some studies point to a significantly lower risk of developing metastases in younger patients, but the prognosis of uveal melanoma in children is not yet fully known. Clinical signs and treatment options for malignant uveal melanoma in children are still under discussion. Differential diagnosis of uveal lesions in children can sometimes be very difficult, as evidenced by following case report in which authors describe a case of choroidal melanoma in a 15-year-old girl.

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Posterior uveal melanoma in adolescents and children: current perspectives

Cited by 8 publications

References 32 publications

Iris Colour and the Risk of Developing Uveal Melanoma

Iris Colour and the Risk of Developing Uveal Melanoma

Distinguishing Choroidal Nevi from Melanomas Using the MOLES Algorithm: Evaluation in an Ocular Nevus Clinic

Uveal Melanoma In A 15-Year-Old Girl. Case Report

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