Journal of Neurological Surgery Reports, Table of Contents CC BY 4.0 · J Neurol Surg Rep 2025; 86(04): e249-e252DOI: 10.1055/a-2753-9601 Case Report Aggressive Clinical Course and Malignant Transformation of a Meningeal Melanocytoma of the Pontomedullary Region: Diagnostic and Therapeutic Implications Authors Author Affiliations Oyku Ozturk 1 Department of Neurosurgery, Gazi University Faculty of Medicine, Ankara, Türkiye Mehmet A. Inan 2 Department of Pathology, Gazi University Faculty of Medicine, Ankara, Türkiye Muammer M. Sahin 3 Department of Otorhinolaryngology/Head and Neck Surgery, Gazi University Faculty of Medicine, Ankara, Türkiye Emrah Celtikci 1 Department of Neurosurgery, Gazi University Faculty of Medicine, Ankara, Türkiye Recommend Article Abstract Abstract Background Primary melanocytic tumors of the central nervous system (CNS) are rare neoplasms that range from benign melanocytomas to aggressive malignant melanomas. Although meningeal melanocytomas are generally considered indolent lesions, malignant transformation and distant metastasis can occur. Case Description We report the case of a 33-year-old male with a bulbopontine meningeal melanocytoma who developed systemic metastases, culminating in a fatal outcome. Despite initial histopathologic features of benignity and absence of BRAF mutation, the lesion showed aggressive behavior. Conclusion This case underscores the diagnostic pitfalls associated with primary CNS melanocytic tumors and highlights the importance of long-term vigilance, even for histologically benign lesions. Keywords Keywordsbrainstem tumor - CNS melanoma - leptomeningeal tumors - malignant transformation - meningeal melanocytoma Full Text References References 1 Louis DN, Perry A, Reifenberger G. et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 2016; 131 (06) 803-820 2 Küsters-Vandevelde HVN, Küsters B, van Engen-van Grunsven ACH. et al. Primary melanocytic tumors of the CNS: a review with focus on molecular aspects. Brain Pathol 2015; 25 (02) 209-226 3 Wen L, Cai L. Primary diffuse meningeal melanomatosis. N Engl J Med 2023; 388 (20) 1892 4 Hannan EJ, O'Leary DP, MacNally SP. et al. The significance of BRAF V600E mutation status discordance between primary cutaneous melanoma and brain metastases: the implications for BRAF inhibitor therapy. Medicine (Baltimore) 2017; 96 (48) e8404 5 Heinzerling L, Baiter M, Kühnapfel S. et al. Mutation landscape in melanoma patients clinical implications of heterogeneity of BRAF mutations. Br J Cancer 2013; 109 (11) 2833-2841 6 Nicolaides P, Newton RW, Kelsey A. Primary malignant melanoma of meninges: atypical presentation of subacute meningitis. Pediatr Neurol 1995; 12 (02) 172-174 7 Bang OY, Kim DI, Yoon SR, Choi IS. Idiopathic hypertrophic pachymeningeal lesions: correlation between clinical patterns and neuroimaging characteristics. Eur Neurol 1998; 39 (01) 49-56 8 Mohapatra A, Choudhury P. An uncommon case of primary leptomeningeal melanoma in a 66-year-old white caucasian male. Cureus 2020; 12 (10) e10793
