Uveal melanoma in children and adolescents: clinical features and treatment approaches according to 30-years’ experience

Relevance. The number of uveal melanomas (UM) in the structure of melanomas of the visual organ and its adnexa is approximately 85 %. This malignant intraocular neoplasm affects mainly the adult population. UM is a rather rare phenomenon among children and, according to various authors, accounts for only 0.5–1.3 % of all UM. The relevance of studying the topic of UM in children and adolescents lies in studying the issue of treatment with an organ-preserving purpose, since this aspect has medical and social significance.

Purpose of the study – to analyze clinical and histological features, course of disease and treatment approaches in the children and adolescents under 20 years.

Materials and methods. The study includes 29 patients with UM, treated and observed from 1992 to 2023. Median patients’ age at the time of treatment was 17 years (from 10 to 20 years old). 45 % patients were male, 55 % – female. The analyzed data included age at the time of treatment, gender, affected eye, tumor’s localization, size and other characteristics (optic nerve and ciliary body (CB) involvement, extraocular spread) and the treatment method.

Results. Mostly, UM had choroidal localization (n = 17, 59 %), fewer UM had panuveal localization (n = 5, 17 %); iris and CB melanoma took place in 14 % (n = 4), CB and choroidal melanoma – in 7 % (n = 2) and in only 1 (3 %) case we observed iris melanoma. TNM (AJCC) classification of our patients with UM was the following: T1 – 5 (17 %) eyes; T2 – 8 (28 %); T3 – 13 (45 %); T4 – 3 (10 %).

Organ-preserving methods of treatment were preferable (n = 20, 69 %). Enucleation was performed in 9 (31 %) cases. Organ-preserving treatment included surgical treatment (n = 9), brachytherapy (BT) (n = 6), BT with transpupillary thermotherapy (TTT) (n = 1), TTT (n = 3) and Gamma Knife stereotactic radiosurgery (n = 1). After organ-preserving treatment we confronted complications in 10 (34 %) cases: macular edema (n = 4), posterior subcapsular cataract (n = 3), neuropathy (n = 2) and phtisis bulbi (n = 1).

According to histopathological examination 50 % of eyes (after surgical treatment and enucleation) had spindle cell type of UM and only 7 % had epithelioid cell type. 6 patients (43 %) had mixed cell type of UM. The mean follow-up was 68 months (from 6 mo. to 21 year). No patients died during follow-up. One (1 %) patient developed liver metastasis. Disease-free survival at 3 and 5 years of follow-up was 94 %.

Conclusion. UM in children and adolescents possesses differences compared to UM in adults. Survival prognosis of UM in children and adolescents is better than in adults. However, true reasons of this feature require further research.

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