Central Nervous System Involvement and Neuroradiological Imaging Insights of Neurocutaneous Melanocytosis in Congenital Melanocytic Nevi

Congenital melanocytic nevi (CMN) are pigmented lesions present at birth, varying widely in size and clinical impact. In rare instances, these nevi become visible during the first months of life, a phenomenon known as tardive melanocytic nevi (tardive CMN). Giant congenital melanocytic nevi (GCMN) a...

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Bibliographic Details
Published in:Diagnostics (Basel) 2024-10, Vol.14 (21), p.2345
Main Authors: Ruff, Christer, Gohla, Georg, Nägele, Thomas, Batra, Marion
Format: Article
Language:English
Subjects:
Central nervous system
central nervous system (CNS)
congenital melanocytic nevi (CMN)
Diagnostic imaging
Females
Genetic disorders
Genotype & phenotype
giant congenital melanocytic nevi (GCMN)
Interesting Images
Localization
magnetic resonance imaging (MRI)
Melanoma
Nervous system
neurocutaneous melanocytosis (NCM)
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Summary:Congenital melanocytic nevi (CMN) are pigmented lesions present at birth, varying widely in size and clinical impact. In rare instances, these nevi become visible during the first months of life, a phenomenon known as tardive melanocytic nevi (tardive CMN). Giant congenital melanocytic nevi (GCMN) are defined as nevi larger than 40 cm in projected adult size (PAS). Their association with the central nervous system (CNS) poses significant risks, including melanoma and neurocutaneous melanocytosis (NCM), where melanocytes infiltrate the CNS, potentially causing seizures, hydrocephalus, and, rarely, CNS melanoma. MRI is recommended for GCMN patients, particularly those with numerous satellite nevi or neurological symptoms, to detect CNS involvement. The Nevosurgery Network recommends MRI examinations in cases of GCMN (>40 cm PAS), the presence of over 20 concomitant nevi, and neurological symptoms requiring clarification. CMN can be associated with melanocyte accumulations and melanin deposits in the brain, spinal cord, and leptomeninges.
ISSN:2075-4418
2075-4418
DOI:10.3390/diagnostics14212345