Pigmented Peril: A fatal case of Primary intracranial melanoma in a paediatric patient

Abstract

Primary CNS melanoma is an extremely rare and aggressive malignancy, especially in pediatric patients. We present the case of a 12-year-old girl who initially presented with sudden-onset severe headache and neurological deficits, and imaging revealed a left frontotemporal mass, suspected to be an arteriovenous malformation. After developing spontaneous intracerebral hemorrhage, she underwent emergency craniectomy and partial resection, with pathology confirming primary CNS melanoma. Despite treatment with immunotherapy, her condition deteriorated, leading to progressive hydrocephalus and leptomeningeal metastasis, ultimately resulting in her death. This case highlights the diagnostic and therapeutic challenges of primary CNS melanoma in children, emphasizing its rapid progression, limited treatment efficacy, and the need for early recognition, multidisciplinary management, and further research for definitive treatment.