Meningeal melanocytoma of the central nervous system in children.

Purpose: This study aimed to summarize the existing English-language literature on central nervous system (CNS) meningeal melanocytomas in children, and additionally describe our institutional case report.

Methods: PubMed database was screened on September 2, 2024, for English-language papers reporting on pediatric patients with CNS meningeal melanocytoma.

Results: A total of 17 papers reporting on 18 patients with 19 CNS meningeal melanocytomas were found in the literature. Additionally, we reported on a 15-year-old male patient with C2-C6 meningeal melanocytoma. Pediatric cohort analysis showed nearly equal sex distribution and a mean age at diagnosis of 11.9 years. There were fifteen intracranial (75%) and five spinal tumors (25%). Four lesions (20%) were diagnosed as intermediate-grade melanocytomas, while the remaining sixteen (80%) were benign meningeal melanocytomas. Most tumors were hyperintense on T1-weighted imaging (85%) and hypointense on T2-weighted imaging (73%). All tumors showed positivity for S100 and Melan-A. Most tumors were characterized by a lack of CNS invasion (91%). Gross-total resection (GTR) was performed in 61% of tumors. Adjuvant radiotherapy (RT) was applied in 50% of patients with incomplete tumor resection. Postoperatively, 62% of patients achieved a favorable outcome. We found 1, 2, 3, and 4-year overall survival of 80%, 71%, 71%, and 50%, respectively. The recurrence rate was 15% after a mean time of 10 months.

Conclusions: Meningeal melanocytomas constitute a rare subgroup of CNS tumors. Surgical tumor removal aiming at maximally safe GTR remains a standard approach, resulting in favorable postoperative outcomes. Considering high recurrence rate, long-term follow-up is needed.