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[Primary diffuse meningeal melanomatosis. Case report]
Anna Bajer-Czajkowska1, Przemysław Nowacki
1Katedra i Klinika Neurologii, Pomorska Akademia Medyczna w Szczecinie, Szczecin. bajeranka@wp.pl
Abstract:
Melanoma malignum constitutes only 0.1% of central nervous system neoplasms. It can occur either as a solid tumour or as a diffuse meningeal melanomatosis. A case of the latter form of central nervous system melanoma is presented in a 44-year-old man, suffering from headaches, cerebrospinal fluid protein elevation, optic disc oedema, hydrocephalus, seizures, cranial nerves and multilevel spinal root damage. Above mentioned neurological manifestations gradually increased within 18 months after onset of first symptoms of the disease (headache). The clinical course in our patient suggested diffuse leptomeningeal involvement. Despite the use of detailed diagnostic procedures, the correct diagnosis of primary diffuse meningeal melanomatosis was established at postmortem examination. We present our case because of the casuistic rarity of primary diffuse meningeal melanomatosis coexisting with obstructive hydrocephalus resulting from, among other things, extensive neoplastic infiltration of the vertebral channel, especially the cauda equina.
Insights
Meningeal melanomatosis, a rare form of central nervous system melanoma, can present with diverse neurological symptoms. This case highlights the diagnostic challenges and postmortem confirmation of this rare condition.
Area of Science:
- Neuro-oncology
- Neuropathology
Background:
- Melanoma malignum is a rare central nervous system neoplasm, accounting for only 0.1% of cases.
- It can manifest as a solid tumor or diffuse meningeal melanomatosis.
Observation:
- A 44-year-old male presented with progressive neurological deficits including headaches, elevated cerebrospinal fluid protein, optic disc edema, hydrocephalus, seizures, cranial nerve palsies, and spinal root damage over 18 months.
- Clinical presentation suggested diffuse leptomeningeal involvement.
Findings:
- Despite extensive diagnostic efforts, primary diffuse meningeal melanomatosis was definitively diagnosed postmortem.
- The case exhibited obstructive hydrocephalus secondary to extensive neoplastic infiltration, particularly affecting the cauda equina.
Implications:
- This case underscores the diagnostic difficulties in identifying primary diffuse meningeal melanomatosis, often leading to delayed or missed diagnosis.
- The rarity of this condition, especially with obstructive hydrocephalus and cauda equina involvement, warrants increased awareness among clinicians.
- Postmortem examination remains crucial for confirming rare neuropathological diagnoses like diffuse meningeal melanomatosis.
