Child's Nervous System

, Volume 20, Issue 1, pp 23–28

Neurocutaneous melanosis

  • F. Di Rocco
  • G. Sabatino
  • M. Koutzoglou
  • D. Battaglia
  • M. Caldarelli
  • G. Tamburrini
Case-based Update

DOI: 10.1007/s00381-003-0835-9

Cite this article as:
Di Rocco, F., Sabatino, G., Koutzoglou, M. et al. Childs Nerv Syst (2004) 20: 23. doi:10.1007/s00381-003-0835-9

Abstract

Background

Neurocutaneous melanosis (NCM) is a rare phakomatosis characterized by a focal or diffuse proliferation of melanin-producing cells in both the skin and the leptomeninges. This syndrome is believed to result from an error in the morphogenesis of embryonal neuroectoderm.

Features

Two-thirds of patients with NCM have giant congenital melanocytic nevi, and the remaining third have numerous lesions but no giant lesions. Most patients present neurological manifestations early in life, which can be secondary to intracranial hemorrhages, to impairment of cerebrospinal fluid circulation, and to malignant transformation of the melanocytes.

Prognosis

The prognosis of patients with symptomatic neurocutaneous melanosis is extremely poor, even in the absence of malignancy. Chemotherapy has been ineffective in the few patients in whom it has been tried.

Keywords

PhakomatosisCongenital neviLeptomeningeal melanosisNeurocutaneous syndrome

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • F. Di Rocco
    • 1
  • G. Sabatino
    • 1
  • M. Koutzoglou
    • 2
  • D. Battaglia
    • 3
  • M. Caldarelli
    • 1
  • G. Tamburrini
    • 1
  1. 1.Section of Pediatric Neurosurgery, Institute of NeurosurgeryCatholic University Medical SchoolRomeItaly
  2. 2.Division of Neurosurgery“Agia Sophia” Children’s HospitalAthens (Goudi)Greece
  3. 3.Child Neurology and Psychiatry UnitCatholic University Medical SchoolRomeItaly