[Epidermal nevus associated with a type I neurofibromatosis and a nephroblastoma: a new epidermal nevus syndrome?]

Ann Pathol. 2000 Dec;20(6):616-9.
[Article in French]


We report the case of a 6-year-old boy who showed a large epidermal nevus mixed with a plexiform neurofibroma, which was associated with "café au lait" macules and a nephroblastoma. This association could not be classified in one of the five well defined epidermal nevus syndrome. To our knowledge this is the first time that this type of epidermal nevus syndrome has been described, which raises the question of the relationship between neurofibromatosis 1, nephroblastoma and epidermal nevus.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Child
  • Humans
  • Kidney Neoplasms / complications*
  • Kidney Neoplasms / pathology
  • Male
  • Neurofibromatosis 1 / complications*
  • Neurofibromatosis 1 / pathology
  • Nevus / complications*
  • Nevus / pathology
  • Skin Neoplasms / complications*
  • Skin Neoplasms / pathology
  • Wilms Tumor / complications*
  • Wilms Tumor / pathology