Aggressive melanoma in an infant with congenital melanocytic nevus syndrome and multiple, NRAS and BRAF mutation-negative nodules

Pediatr Dermatol. 2018 Sep;35(5):e281-e285. doi: 10.1111/pde.13595. Epub 2018 Jul 12.

Abstract

We report the case of a newborn boy with multinodular NRAS and BRAF mutation-negative congenital melanocytic nevi and cerebral lesions compatible with congenital intraparenchymal melanosis. Histopathology from skin lesions showed atypical nodular melanocytic proliferation with marked melanocytic atypia and a large number of mitoses and apoptosis, indicating aggressive proliferation. The child developed several new subcutaneous tumors and multiple internal lesions, which were confirmed to be metastases, and died at 5 months of age. This case may represent an infantile melanoma developing from a giant congenital melanocytic nevus or a congenital melanoma.

Keywords: neoplasms-malignant; nevi-melanocytic.

Publication types

  • Case Reports

MeSH terms

  • Fatal Outcome
  • GTP Phosphohydrolases / genetics*
  • Humans
  • Infant
  • Infant, Newborn
  • Magnetic Resonance Imaging
  • Male
  • Melanoma / pathology*
  • Melanosis / pathology
  • Membrane Proteins / genetics*
  • Mutation
  • Nevus, Pigmented / genetics
  • Nevus, Pigmented / pathology*
  • Proto-Oncogene Proteins B-raf / genetics*
  • Skin / pathology
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology*
  • Ultrasonography

Substances

  • Membrane Proteins
  • BRAF protein, human
  • Proto-Oncogene Proteins B-raf
  • GTP Phosphohydrolases
  • NRAS protein, human

Supplementary concepts

  • Melanocytic nevus syndrome, congenital