Abstract
ABSTRACT
Primary Meningeal Melanoma: A Case Report and Literature Review
Background/Objectives: Primary meningeal melanoma is an extremely rare malignant tumor with aggressive biological behavior and poor prognosis. We report a case of rapidly progressive primary meningeal melanoma with liver metastases after gross total resection to contribute additional real-world data on this rare disease.
Case report: A 41-year-old male patient was diagnosed with primary meningeal melanoma in the left occipital region and underwent gross total microsurgical resection. Three months after surgery, the disease progressed with liver metastases. The patient received three cycles of pembrolizumab monotherapy; however, follow-up evaluation demonstrated continued progression of hepatic lesions according to RECIST 1.1 criteria.
Conclusions: This case highlights the aggressive clinical course, early distant metastasis, and limited response to immunotherapy in primary meningeal melanoma. Close postoperative surveillance, multidisciplinary management, and molecular profiling may play important roles in optimizing treatment strategies.
Keywords: primary meningeal melanoma; pembrolizumab; immunotherapy;
References
Pellerino A, Verdijk RM, Nichelli L, Andratschke N, Idbaih A, Goldbrunner R. Diagnosis and treatment of primary CNS melanoma: A EURACAN consensus recommendation. Cancers (Basel). 2024;16(14):2508.
Brat DJ, Giannini C, Scheithauer BW, Burger PC. Primary melanocytic neoplasms of the central nervous system. Am J Surg Pathol. 1999;23(7):745–54.
Agarwalla PK, Koch MJ, Mordes DA, Codd PJ, Coumans JV. Pigmented lesions of the nervous system and the neural crest: Lessons from embryology. Neurosurgery. 2016;78(1):142–55.
Naik H, Kharosekar H, Velho V. Black brain caused by primary intracranial malignant melanoma. Neurol India. 2016;64(1):193–4.
Burgos R, Cardona AF, Santoyo N, et al. Case report: Differential genomics and evolution of a meningeal melanoma treated with ipilimumab and nivolumab. Front Oncol. 2021;11:691017.
Küsters-Vandevelde HV, Kruse V, Van Maerken T, et al. Copy number variation analysis and methylome profiling of a GNAQ-mutant primary meningeal melanocytic tumor and its liver metastasis. Exp Mol Pathol. 2017;102(1):25–31.
Misir Krpan A, Rakusic Z, Herceg D. Primary leptomeningeal melanomatosis successfully treated with PD-1 inhibitor pembrolizumab: A case report. Medicine (Baltimore). 2020;99(50):e22928.
Ricchizzi A, et al. How should we treat meningeal melanocytoma? A retrospective analysis of potential treatment strategies. Cancers (Basel). 2022;14(23):5851.
Berzero G, Diamanti L, Di Stefano AL, et al. Meningeal melanomatosis: A challenge for timely diagnosis. BioMed Research International. 2015;2015:948497.
Wang J, Guo ZZ, Wang YJ, Zhang SG, Xing DG. Microsurgery for the treatment of primary malignant intracranial melanoma: A surgical series and literature review. Eur J Surg Oncol. 2014;40(9):1062–71.
Rades D, Heidenreich F, Tatagiba M, Brandis A, Karstens JH. Therapeutic options for meningeal melanocytoma. Case report. J Neurosurg. 2001;95(2 Suppl):225–31.
El Habnouni C, Bléchet C, Bens G. Pembrolizumab for primary malignant melanoma of the central nervous system. J Neurooncol. 2018;139(1):225–7.
Smith AB, Rushing EJ, Smirniotopoulos JG. Pigmented lesions of the central nervous system: Radiologic-pathologic correlation. Radiographics. 2009;29(5):1503–24.
Ginat DT, Meyers SP. Intracranial lesions with high signal intensity on T1-weighted MR images: Differential diagnosis. Radiographics. 2012;32(2):499–516.
Hayward RD. Malignant melanoma and the central nervous system. A guide for classification based on the clinical findings. J Neurol Neurosurg Psychiatry. 1976;39(6):526–30.
Mayer S, Mauer UM, Mathieu R, Hackenbroch C, Knupfer J, Schulz C. Primary amelanotic CNS melanoma: Case report and literature review. J Neurol Surg A Cent Eur Neurosurg. 2018;79(1):96–100.
Buckland A, Green C, Kho LK, Prentice D. Primary leptomeningeal melanoma: The prognostic significance of its genetic signature and embryological origin. BMJ Case Rep. 2021;14(3):e239496.
Steininger J, Pfohler C, Eigentler TK, et al. Leptomeningeal metastases in melanoma patients. Cancers. 2023;15(15):3897.
| Published | 18-08-2026 | |
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| Issue | Vol. 18 No. 5 (2026) | |
| Section | Case report | |
| DOI | 10.38103/jcmhch.18.5.16 | |
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