Circumscribed Meningeal Melanocytoma of the Craniovertebral Junction: A Case Report and Focused Literature Review

Background and Clinical Significance: Meningeal melanocytoma is an exceptionally rare, typically benign melanocytic tumor of the central nervous system. Its clinical management becomes particularly challenging when located at the craniovertebral junction, where proximity to critical neurovascular structures limits the extent of safe surgical resection. Due to its rarity and complex anatomical location, each case contributes valuable insight into optimal diagnostic and therapeutic strategies. We report a case of circumscribed meningeal melanocytoma arising at the craniovertebral junction and provide a focused review of previously reported cases, with particular emphasis on clinical presentation, radiological characteristics, surgical challenges, treatment strategies, and outcomes; Case Presentation: A 65-year-old Caucasian woman presented with progressive binocular diplopia. Magnetic resonance imaging revealed two contrast-enhancing extra-axial lesions at the level of the foramen magnum, with one dominant mass. The patient underwent near-total microsurgical resection of the dominant lesion under intraoperative neurophysiological monitoring. Histopathological and immunohistochemical evaluation confirmed the diagnosis of meningeal melanocytoma. In the postoperative period, the patient developed transient lower cranial nerve dysfunction, which improved with conservative treatment. Follow-up imaging demonstrated a stable residual tumor. Given the subtotal nature of resection and the anatomically complex location, adjuvant proton beam radiotherapy was recommended; Conclusions: Meningeal melanocytoma of the craniovertebral junction poses distinctive diagnostic and surgical challenges because of its proximity to critical neurovascular structures. The available literature supports maximal safe resection as the primary treatment strategy, with adjuvant radiotherapy considered in selected patients with residual disease. Long-term radiological surveillance is warranted because late recurrence and malignant transformation have been reported.