Bulky perineal naevocytoma with a rare variant of giant congenital melanocytic nevus in a female neonate

Abstract

Background: Congenital melanocytic nevi are common and vary in size. Some can be quite extensive but are usually benign. However, a giant congenital nevus is rare and has the potential for malignant transformation. Giant nevus could occur in any part of the body; though it is most common on the trunk, it rarely covers over three-quarters of the skin. It is known to be associated with other congenital malformations such as spina bifida and foot deformity. Very few cases of bulky perineal naevocytoma associated with giant congenital melanocytic naevi have been reported in the literature. Case summary: We report a case of a 2 day old female neonate with a giant melanocytic nevus that covers about 85% of the whole body associated with a bulky perineal naevocytoma arising within the lesion. Conclusion: Such extensive lesion is at high risk of malignant transformation and has a major psychosocial impact on the caregivers.