Large congenital melanocytic nevus

What is Large congenital melanocytic nevus?

A rare skin hamartoma characterized by at least one pigmented skin lesion present at birth of more than 20 cm (large congenital melanocytic nevus; LCMN) or 40 cm (giant; GCMN) projected adult diameter. The primary lesion is composed of mutated melanocytes and often locally disorganized epidermal annexes or dermis, and presents with an elevated risk of malignant transformation to melanoma or, more rarely, other neoplasms in skin or central nervous system.

Other condition names

  • Congenital pigmented nevus
  • GMN
  • Giant congenital melanocytic nevus
  • Giant pigmented hairy nevus
  • LCMN

Inheritance type

Not applicable

Prevalence

  • Europe: 1-9 in 100,000

Age of Onset

  • Infancy
  • Neonatal
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.