Primary uveal melanoma is extremely rare in children and also among blacks. Uveal melanomas may be associated with various preexisting conditions, including congenital ocular melanocytosis and dysplastic nevus syndrome. We report the case of a 4-year-old black girl who presented with pronounced proptosis and no light perception secondary to a massive intraocular tumor with extrascleral extension. Biopsy of the tumor confirmed the diagnosis of primary uveal melanoma, and the patient was treated by enucleation and external beam radiation therapy. Although metastatic disease was ruled out at the time, the patient died approximately 3 months later.
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health