Characterization, treatment, and outcome of uveal melanoma in the first two years of life.

BACKGROUND AND OBJECTIVES Features and characteristics of uveal melanoma are well described in adults, but little is known about the presentation of uveal melanoma in infancy. DESIGN Systematic literature review. METHODS A review of published, peer-reviewed literature reporting on uveal melanoma presenting during the first two years of life. Outcome measures included demographics, clinical features, histopathological findings, extent of the disease, therapeutic interventions, management outcomes, association with skin lesions or systemic diseases, and survival data. RESULTS This review revealed 13 reported cases (seven boys and six girls) of uveal melanoma diagnosed within the first two years of life. The median age at diagnosis was seven months. Orbital mass and proptosis were the most common presentations (38%); only one tumor (8%) was melanotic, and pathologically 10 tumors (77%) had epithelioid component. Associated pigmented skin lesions (cutaneous disease) were seen in six cases (46%). All affected eyes were surgically removed; three patients received chemotherapy, and one received radiotherapy. At a median follow-up of 25months, two patients (15%) had metastasis, and one of them (8%) was dead at six months' follow-up with liver and multi-organ metastasis. CONCLUSIONS Uveal melanoma can present within the first two years of life. In very rare cases, it can present as an intraocular tumor that simulates retinoblastoma, but it can also present as an orbital tumor. It has a tendency to affect patients with cutaneous diseases like familial atypical mole, melanoma syndrome, and dysplastic nevus syndrome. Despite this, uveal melanoma in this group has a more favorable prognosis than adult melanoma.