Congenital melanocytic nevi-when to worry and how to treat: Facts and controversies.

Congenital melanocytic nevi (CMN) are evident in 1% to 6% of neonates. In some studies, nevi with clinical, dermatoscopic, and histologic features identical to CMN have had a prevalence of more than 15% in older children and adults, possibly reflecting the "tardive" appearance of nevi programmed from birth. There is ongoing debate about the magnitude of the risk of melanoma and other complications associated with CMN of various sizes and the best approach to management of these lesions. We review the natural history of CMN, including proliferative nodules and erosions during infancy, neurotization, and spontaneous regression, and features of variants such as speckled lentiginous and congenital blue nevi. The risk of melanoma arising within small-sized (<1.5 cm) and medium-sized CMN is low (likely <1% over a lifetime) and virtually nonexistent before puberty. Recent data suggest that melanoma (cutaneous or extracutaneous) develops in approximately 5% of patients with a large (>20 cm) CMN, with about half of this risk in the first few years of life. Melanoma and neurocutaneous melanocytosis (NCM) are most likely in patients with CMN that have a final size of >40 cm in diameter, numerous satellite nevi, and a truncal location. One-third of individuals with NCM have multiple medium-sized (but no large) CMN. In patients at risk for NCM, a screening gadolinium-enhanced magnetic resonance imaging, preferably before age 6 months, and longitudinal neurologic assessment are recommended. Management of CMN depends on such factors as the ease of monitoring (more difficult for large, dark, thick nevi) and cosmetic and psychologic benefits of excision or other procedures. CMN require lifelong follow-up. Periodic total body skin examinations are necessary for all patients with large CMN, even when complete resection (often impossible) has been attempted. Copyright 2010. Published by Elsevier Inc.