Patient Story
A small congenital nevus (Figure 163-1) was noted on this 6-month-old child by his new family physician during a routine examination. The parents acknowledged that it was present from birth and asked if it needed to be cut out. They were reassured that nothing needs to be done about it at this time.
Introduction
Synonyms
- Garment nevus, bathing trunk nevus, giant hairy nevus, giant pigmented nevus, pigmented hairy nevus, nevus pigmentosus, nevus pigmentosus et pilosus, and Tierfell nevus.1
- Tardive congenital nevus refers to a nevus with similar features to congenital nevi, but appears at age 1 to 2 years.
Epidemiology
- Congenital melanocytic nevi develop in 1% to 6% of newborns and are present at birth or develop during the first year of life.1
- In an Italian prevalence study of more than 3000 children ages 12 to 17 years, congenital melanocytic nevi or congenital nevus-like nevi were found in 17.5%; most (92%) were small (<1.5 cm).2
- Congenital nevi are also seen in neurocutaneous melanosis, a rare syndrome characterized by the presence of congenital melanocytic nevi and melanotic neoplasms of the central nervous system.
- The development of melanoma within congenital nevi (Figure 163-8) is believed to occur at a higher rate than in normal skin. Estimates range from 4% to 10%, with smaller lesions having lowest risk.1
- In a systematic review, 46 of 651 patients with congenital melanocytic nevi (0.7%), who were followed for 3.4 to 23.7 years, developed melanomas, representing a 465-fold increased relative risk of developing melanoma during childhood and adolescence.3 The mean age at diagnosis of melanoma was 15.5 years (median: 7 years).
- Patients with giant congenital melanocytic nevi appear to be at highest risk where subsequent melanoma has been reported in 5% to 7% by age 60 years.4 In one study, 70% of patients with a large congenital melanocytic nevi diagnosed with melanoma were diagnosed within the first 10 years of life.5
- In a prospective study of 230 medium-size congenital nevi (1.5 to 19.9 cm) in 227 patients from 1955 to 1996, no melanomas occurred. The average follow-up period being 6.7 years to an average age of 25.5 years.6
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- In a systematic review, 46 of 651 patients with congenital melanocytic nevi (0.7%), who were followed for 3.4 to 23.7 years, developed melanomas, representing a 465-fold increased relative risk of developing melanoma during childhood and adolescence.3 The mean age at diagnosis of melanoma was 15.5 years (median: 7 years).