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Primary melanocytic tumors of the central nervous system (CNS) are extremely rare and account for only 1% of all melanomas and 0.05% of primary brain tumors. In case of diffusely invasive lesions of the pia mater with a tumor of melanocytic origin (without signs of extracranial metastases), the tumors are classified as primary diffuse meningeal melanomatosis (PDMM). The latter is an extremely rare subtype of CNS malignant tumor with an incidence rate of 1 in 20 million people. Despite the development of neuroimaging techniques, today a morphological examination remains the main and most accurate method for verifying CNS melanocytic tumors. The paper describes a fatal case of PMMD manifested as epileptic syndrome, with a rapidly progressive course.

The paper presents the experience of using DNA methylation status in patients with meningiomas of the craniovertebral junction area in a neurosurgical clinic. A clinical case of combined treatment of a patient with meningioma of the craniovertebral junction and the choice of tactics based on the result of DNA methylation analysis of meningioma are described.