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Neurocutaneous Melanosis
Gilbert Vézina, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Neurocutaneous melanosis (NCM)
            • Definitions

              • Congenital phakomatosis characterized by giant &/or multiple melanocytic nevi (GCMN) of the skin in association with melanotic lesions of CNS
                • CNS disease: Parenchymal
                  • Melanosis or melanocytosis: Focal collection of benign melanotic cells
                  • Malignant melanoma or melanomatosis (MM)
                • CNS disease: Leptomeningeal (LM)
                  • Primary leptomeningeal melanocytic neoplasms (LMNs)
                    • Can be circumscribed (usually adults) or diffuse (more often children)
                    • Benign or malignant
                    • Leptomeningeal melanocytosis (LMs): Excess of benign melanotic cells in leptomeninges (most frequently in children with NCM)
                    • Leptomeningeal melanoma (LMm) or melanomatosis: Malignant melanoma of leptomeninges
                • Genetic studies show NCM is a RASopathy

            IMAGING

            • General Features

              • CT Findings

                • MR 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

                                        1. Araújo C et al: Giant congenital melanocytic nevi and neurocutaneous melanosis. Case Rep Med. 2015:545603, 2015
                                        2. Küsters-Vandevelde HV et al: Primary melanocytic tumors of the central nervous system: a review with focus on molecular aspects. Brain Pathol. 25(2):209-26, 2015
                                        3. Bekiesinska-Figatowska M et al: Neurocutaneous melanosis in children with giant congenital melanocytic nevi. Clin Imaging. 38(2):79-84, 2014
                                        4. van Engen-van Grunsven AC et al: Update on Molecular Pathology of Cutaneous Melanocytic Lesions: What is New in Diagnosis and Molecular Testing for Treatment? Front Med (Lausanne). 1:39, 2014
                                        5. Jain P et al: Encephalocraniocutaneous lipomatosis with neurocutaneous melanosis. J Child Neurol. 29(6):846-9, 2013
                                        6. 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
                                        7. 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
                                        8. Ramaswamy V et al: Spectrum of central nervous system abnormalities in neurocutaneous melanocytosis. Dev Med Child Neurol. 54(6):563-8, 2012
                                        9. Schreml S et al: Neurocutaneous melanosis in association with Dandy-Walker malformation: case report and literature review. Clin Exp Dermatol. 33(5):611-4, 2008
                                        10. 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
                                        11. Di Rocco F et al: Neurocutaneous melanosis. Childs Nerv Syst. 20(1):23-8, 2004
                                        12. 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
                                        13. Byrd SE et al: MR imaging of symptomatic neurocutaneous melanosis in children. Pediatr Radiol. 27(1):39-44, 1997
                                        Related Anatomy
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                                        Related Differential Diagnoses
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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 giant &/or multiple melanocytic nevi (GCMN) of the skin in association with melanotic lesions of CNS
                                                      • CNS disease: Parenchymal
                                                        • Melanosis or melanocytosis: Focal collection of benign melanotic cells
                                                        • Malignant melanoma or melanomatosis (MM)
                                                      • CNS disease: Leptomeningeal (LM)
                                                        • Primary leptomeningeal melanocytic neoplasms (LMNs)
                                                          • Can be circumscribed (usually adults) or diffuse (more often children)
                                                          • Benign or malignant
                                                          • Leptomeningeal melanocytosis (LMs): Excess of benign melanotic cells in leptomeninges (most frequently in children with NCM)
                                                          • Leptomeningeal melanoma (LMm) or melanomatosis: Malignant melanoma of leptomeninges
                                                      • Genetic studies show NCM is a RASopathy

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR 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

                                                                              1. Araújo C et al: Giant congenital melanocytic nevi and neurocutaneous melanosis. Case Rep Med. 2015:545603, 2015
                                                                              2. Küsters-Vandevelde HV et al: Primary melanocytic tumors of the central nervous system: a review with focus on molecular aspects. Brain Pathol. 25(2):209-26, 2015
                                                                              3. Bekiesinska-Figatowska M et al: Neurocutaneous melanosis in children with giant congenital melanocytic nevi. Clin Imaging. 38(2):79-84, 2014
                                                                              4. van Engen-van Grunsven AC et al: Update on Molecular Pathology of Cutaneous Melanocytic Lesions: What is New in Diagnosis and Molecular Testing for Treatment? Front Med (Lausanne). 1:39, 2014
                                                                              5. Jain P et al: Encephalocraniocutaneous lipomatosis with neurocutaneous melanosis. J Child Neurol. 29(6):846-9, 2013
                                                                              6. 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
                                                                              7. 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
                                                                              8. Ramaswamy V et al: Spectrum of central nervous system abnormalities in neurocutaneous melanocytosis. Dev Med Child Neurol. 54(6):563-8, 2012
                                                                              9. Schreml S et al: Neurocutaneous melanosis in association with Dandy-Walker malformation: case report and literature review. Clin Exp Dermatol. 33(5):611-4, 2008
                                                                              10. 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
                                                                              11. Di Rocco F et al: Neurocutaneous melanosis. Childs Nerv Syst. 20(1):23-8, 2004
                                                                              12. 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
                                                                              13. Byrd SE et al: MR imaging of symptomatic neurocutaneous melanosis in children. Pediatr Radiol. 27(1):39-44, 1997