Introduction Melanocytes, responsible for melanin production, originate from the neural crest and can give rise to malignant tumors known as melanoma. Primary Central Nervous System Melanoma (pCNSM) is a rare occurrence, with an incidence of 0.005 cases per 100,000 individuals annually. Considering the variations in tumor biology, a comprehensive treatment strategy has not been introduced yet. Immunotherapy is an emerging treatment avenue warranting further exploration.

Aim of Study In this paper, we conducted a literature review on patients with pCNSM including brain and spine to figure out the most successful treatment. We shared our case of a girl diagnosed initially as an arteriovenous malformation; however, she was found to have a pCNSM Melanoma.

Methods A systematic review of literature of all published articles from 1980 till 2023 was conducted. The data were collected from PubMed and Google Scholar, and electronic medical records.

Results The systematic review found 138 articles with 148 pCNSMs (77 cases of brain and 71 cases of spinal primay melanoma). The vast majority of patients (95%) underwent therapeutic gross total or partial resection of the tumors followed by radiotherapy. Only two cases with brain melanomas underwent immunotherapy including our case. The treatment strategies of primary brain melanomas were similar to those of Primary Spinal Melanoma (PSM) but exhibited better outcomes in PSM cases.

Conclusion Our findings suggest that surgical intervention, followed by adjuvant radiotherapy ± immuntherapy, yields favourable outcomes in PSM cases, but not in primary brain melanomas. Further multicentric studies are needed.

Disclosure of Interest no.