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[Primary diffuse meningeal melanomatosis: a literature review and a case report]
D A Sitovskaya1,2, O P Verbitsky2, Yu A Petrova2
1Prof. A.L. Polenov Russian Research Institute of Neurosurgery - Branch V.A. Almazov National Medical Research Center of the Ministry of Health of Russia, St. Petersburg, Russia.
Abstract:
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.
Insights
Primary diffuse meningeal melanomatosis (PDMM) is an extremely rare brain tumor. This case highlights its fatal presentation as epilepsy, emphasizing morphological examination for diagnosis.
Area of Science:
- Neuro-oncology
- Pathology
Background:
- Primary melanocytic tumors of the central nervous system (CNS) are rare, comprising 1% of melanomas and 0.05% of brain tumors.
- Primary diffuse meningeal melanomatosis (PDMM) is an exceptionally rare CNS malignant tumor, affecting 1 in 20 million people.
- PDMM presents as diffusely invasive lesions of the pia mater without extracranial metastases.
Observation:
- This paper details a fatal case of PDMM.
- The patient presented with an epileptic syndrome.
- The disease course was rapidly progressive.
Findings:
- Morphological examination remains the gold standard for diagnosing CNS melanocytic tumors, despite advancements in neuroimaging.
- PDMM can manifest with neurological symptoms such as epilepsy.
- The rapid progression of the disease contributed to the fatal outcome.
Implications:
- Accurate diagnosis of rare CNS tumors like PDMM relies on histopathological analysis.
- Understanding the clinical presentation of PDMM is crucial for timely intervention.
- This case underscores the aggressive nature and poor prognosis associated with PDMM.
