Congenital melanocytic nevi: update in genetics and management.
The article intends to review recent updates in the management of congenital melanocytic nevi (CMN) and the evolving genomic landscape that has begun to shed light on the nature of nevogenesis, malignant potential, and possible therapeutic targets for those with melanoma and neurologic involvement.



CMN are the result of postzygotic somatic mutations involving key proteins in the mitogen-activated protein kinase pathway, primarily NRAS and BRAF. Complications include a spectrum of neurologic findings and development of melanoma. Patients with extracutaneous involvement may be better termed as having 'CMN syndrome'. MRI findings in high risk patients with multiple CMN may best predict clinical outcome.



The management of patients with CMN is complex. Neurologic involvement and melanoma remain the two most devastating complications. New, genetically targeted therapies for patients with complications may be of value, and research for potential therapies is ongoing.
aDivision of Dermatology, Phoenix Children's Hospital bUniversity of Arizona College of Medicine, Department of Child Health, Phoenix, AZ, USA.
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D049109:Cell Proliferation; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000473:pathology; D004252:DNA Mutational Analysis; D020558:GTP Phosphohydrolases / Q000235:genetics; D006801:Humans; D020935:MAP Kinase Signaling System / Q000235:genetics; D008545:Melanoma / Q000235:genetics / Q000473:pathology*; D008565:Membrane Proteins / Q000235:genetics; D058990:Molecular Targeted Therapy / Q000639:trends*; D009154:Mutation / Q000235:genetics; D009508:Nevus, Pigmented / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology / Q000628:therapy*; D010641:Phenotype; D017410:Practice Guidelines as Topic; D011230:Precancerous Conditions / Q000235:genetics / Q000473:pathology*; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D012307:Risk Factors; D012878:Skin Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000473:pathology* / Q000628:therapy
D008565:Membrane Proteins; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf; D020558:GTP Phosphohydrolases; C579846:NRAS protein, human