Congenital melanocytic nevi: an update for the pediatrician.

While melanoma is uncommon in childhood, recent evidence suggests that its incidence is increasing in both adolescence and adulthood. Risk factors may be identifiable during childhood and include congenital nevi. Large congenital nevi carry a significantly increased risk for the development of melanoma, both cutaneous and extracutaneous. This risk appears to be greatest during early childhood. Large congenital nevi, particularly those overlying the head and neck, may also be associated with neurocutaneous melanosis. Symptomatic neurocutaneous melanosis, although rare, carries a poor prognosis. Conversely, asymptomatic neurocutaneous melanosis may be more common than previously recognized. For the most part, large congenital nevi are treated with primary excisions and closures, assisted by tissue-expansion techniques and skin grafting. Until the associated risks are better defined, therapy of small congenital nevi should be individualized, with consideration given to additional melanoma risk factors.