Congenital melanocytic nevi: where are we now? Part I. Clinical presentation, epidemiology, pathogenesis, histology, malignant transformation, and neurocutaneous melanosis

J Am Acad Dermatol. 2012 Oct;67(4):495.e1-17; quiz 512-4. doi: 10.1016/j.jaad.2012.06.023.


Congenital melanocytic nevi (CMN) are present at birth or arise during the first few weeks of life. They are quite common, may have a heritable component, and can present with marked differences in size, shape, color, and location. Histologic and dermatoscopic findings may help suggest the diagnosis, but they are not entirely specific. CMN are categorized based on size, and larger lesions can have a significant psychosocial impact and other complications. They are associated with a variety of dermatologic lesions, ranging from benign to malignant. The risk of malignant transformation varies, with larger CMN carrying a significantly higher risk of malignant melanoma (MM), although with an absolute risk that is lower than is commonly believed. They may also be associated with neuromelanosis, which may be of greater concern than cutaneous MM. The information presented herein aims to help dermatologists determine when it is prudent to obtain a biopsy specimen or excise these lesions, to obtain radiographic imaging, and to involve other specialists (eg, psychiatrists and neurologists) in the patient's care.

Publication types

  • Review

MeSH terms

  • Cell Transformation, Neoplastic / pathology
  • Education, Medical, Continuing
  • Humans
  • Melanoma / epidemiology
  • Melanoma / pathology
  • Melanosis / epidemiology*
  • Melanosis / pathology*
  • Neural Tube Defects / epidemiology
  • Neural Tube Defects / pathology
  • Neurocutaneous Syndromes / epidemiology*
  • Neurocutaneous Syndromes / pathology*
  • Nevus, Pigmented / congenital
  • Nevus, Pigmented / epidemiology*
  • Nevus, Pigmented / pathology*
  • Skin Neoplasms / congenital
  • Skin Neoplasms / epidemiology*
  • Skin Neoplasms / pathology*

Supplementary concepts

  • Neurocutaneous melanosis