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KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Neurocutaneous melanosis (NCM)
Definitions
- Congenital phakomatosis characterized by multiple congenital melanocytic nevi (CMN) + benign or malignant melanotic lesions of CNS
- Parenchymal CNS disease
- Melanosis: Focal collection of benign melanotic cells
- Malignant melanoma (MM)
- Leptomeningeal (LM) CNS disease
- Leptomeningeal melanosis (LMs): Excess of benign melanotic cells in leptomeninges
- Leptomeningeal melanoma (LMm): Malignant melanoma of leptomeninges
- Parenchymal CNS disease
- Congenital phakomatosis characterized by multiple congenital melanocytic nevi (CMN) + benign or malignant melanotic lesions of CNS
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Barbarot S: Revisiting neurocutaneous melanosis spectrum: do we have to undertake systematic magnetic resonance imaging in children with congenital melanocytic naevi? Br J Dermatol. 173(3):639-40, 2015
- Sibbald C et al: Neurocutaneous melanosis and congenital melanocytic naevi: a retrospective review of clinical and radiological characteristics. Br J Dermatol. 173(6):1522-4, 2015
- Siddiqui MA et al: Neurocutaneous melanosis: Review of a rare non-familial neuroectodermal dysplasia with newer association of cerebellopontine angle cistern lipoma. Neuroradiol J. 28(2):222-6, 2015
- Waelchli R et al: Classification of neurological abnormalities in children with congenital melanocytic naevus syndrome identifies magnetic resonance imaging as the best predictor of clinical outcome. Br J Dermatol. 173(3):739-50, 2015
- Jain P et al: Encephalocraniocutaneous lipomatosis with neurocutaneous melanosis. J Child Neurol. 29(6):846-9, 2013
- Kinsler VA et al: Multiple congenital melanocytic nevi and neurocutaneous melanosis are caused by postzygotic mutations in codon 61 of NRAS. J Invest Dermatol. 133(9):2229-36, 2013
- Alikhan A et al: Congenital melanocytic nevi: where are we now? Part I. Clinical presentation, epidemiology, pathogenesis, histology, malignant transformation, and neurocutaneous melanosis. J Am Acad Dermatol. 67(4):495, 2012
- Ramaswamy V et al: Spectrum of central nervous system abnormalities in neurocutaneous melanocytosis. Dev Med Child Neurol. 54(6):563-8, 2012
- Johnson JM et al: Ultrasonographic detection of intracranial melanocytosis in an infant. Pediatr Radiol. 40(2):210-4, 2010
- Acosta FL Jr et al: Neurocutaneous melanosis presenting with hydrocephalus. Case report and review of the literature. J Neurosurg. 102(1 Suppl):96-100, 2005
- Hale EK et al: Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi--results from the NYU-LCMN registry. Br J Dermatol. 152(3):512-7, 2005
- Di Rocco F et al: Neurocutaneous melanosis. Childs Nerv Syst. 20(1):23-8, 2004
- Hayashi M et al: Diffuse leptomeningeal hyperintensity on fluid-attenuated inversion recovery MR images in neurocutaneous melanosis. AJNR Am J Neuroradiol. 25(1):138-41, 2004
- Byrd SE et al: MR imaging of symptomatic neurocutaneous melanosis in children. Pediatr Radiol. 27(1):39-44, 1997
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Neurocutaneous melanosis (NCM)
Definitions
- Congenital phakomatosis characterized by multiple congenital melanocytic nevi (CMN) + benign or malignant melanotic lesions of CNS
- Parenchymal CNS disease
- Melanosis: Focal collection of benign melanotic cells
- Malignant melanoma (MM)
- Leptomeningeal (LM) CNS disease
- Leptomeningeal melanosis (LMs): Excess of benign melanotic cells in leptomeninges
- Leptomeningeal melanoma (LMm): Malignant melanoma of leptomeninges
- Parenchymal CNS disease
- Congenital phakomatosis characterized by multiple congenital melanocytic nevi (CMN) + benign or malignant melanotic lesions of CNS
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Barbarot S: Revisiting neurocutaneous melanosis spectrum: do we have to undertake systematic magnetic resonance imaging in children with congenital melanocytic naevi? Br J Dermatol. 173(3):639-40, 2015
- Sibbald C et al: Neurocutaneous melanosis and congenital melanocytic naevi: a retrospective review of clinical and radiological characteristics. Br J Dermatol. 173(6):1522-4, 2015
- Siddiqui MA et al: Neurocutaneous melanosis: Review of a rare non-familial neuroectodermal dysplasia with newer association of cerebellopontine angle cistern lipoma. Neuroradiol J. 28(2):222-6, 2015
- Waelchli R et al: Classification of neurological abnormalities in children with congenital melanocytic naevus syndrome identifies magnetic resonance imaging as the best predictor of clinical outcome. Br J Dermatol. 173(3):739-50, 2015
- Jain P et al: Encephalocraniocutaneous lipomatosis with neurocutaneous melanosis. J Child Neurol. 29(6):846-9, 2013
- Kinsler VA et al: Multiple congenital melanocytic nevi and neurocutaneous melanosis are caused by postzygotic mutations in codon 61 of NRAS. J Invest Dermatol. 133(9):2229-36, 2013
- Alikhan A et al: Congenital melanocytic nevi: where are we now? Part I. Clinical presentation, epidemiology, pathogenesis, histology, malignant transformation, and neurocutaneous melanosis. J Am Acad Dermatol. 67(4):495, 2012
- Ramaswamy V et al: Spectrum of central nervous system abnormalities in neurocutaneous melanocytosis. Dev Med Child Neurol. 54(6):563-8, 2012
- Johnson JM et al: Ultrasonographic detection of intracranial melanocytosis in an infant. Pediatr Radiol. 40(2):210-4, 2010
- Acosta FL Jr et al: Neurocutaneous melanosis presenting with hydrocephalus. Case report and review of the literature. J Neurosurg. 102(1 Suppl):96-100, 2005
- Hale EK et al: Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi--results from the NYU-LCMN registry. Br J Dermatol. 152(3):512-7, 2005
- Di Rocco F et al: Neurocutaneous melanosis. Childs Nerv Syst. 20(1):23-8, 2004
- Hayashi M et al: Diffuse leptomeningeal hyperintensity on fluid-attenuated inversion recovery MR images in neurocutaneous melanosis. AJNR Am J Neuroradiol. 25(1):138-41, 2004
- Byrd SE et al: MR imaging of symptomatic neurocutaneous melanosis in children. Pediatr Radiol. 27(1):39-44, 1997
