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June 16, 2015; 84 (24) Resident and Fellow Section

Teaching NeuroImages: Neurocutaneous melanosis

Manish Kumar, Radhika Dhamija
First published June 15, 2015, DOI: https://doi.org/10.1212/WNL.0000000000001689
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A 3-month-old boy presented with a giant congenital melanocytic nevus involving his back. He was developing normally with no neurologic symptoms. Brain MRI demonstrated a T1-bright lesion in right amygdala without necrosis, hemorrhage, edema, or enhancement (figure). MRI spine was normal. The imaging findings in the clinical setting are consistent with a diagnosis of neurocutaneous melanosis.

Figure
Figure Axial and coronal views of multiplanar reconstructed images of the brain MRI and photograph of the melanocytic nevus

(A) Axial and (B) coronal views of multiplanar reconstructed images of the brain MRI demonstrate a single 8 mm × 7 mm × 6 mm lesion within the right amygdala with T1 shortening with no evidence of necrosis, hemorrhage, or edema. (C) The giant congenital melanocytic nevus.

First described by Von Rokitanski in 1861, neurocutaneous melanosis is a rare sporadic embryonic neuroectodermal dysplasia characterized by congenital melanocytic nevi and melanotic tumors of the leptomeninges or brain parenchyma.1 It most commonly results from an embryonic postzygotic somatic mutation in NRAS.2

AUTHOR CONTRIBUTIONS

Dr. Kumar: drafting the manuscript, chart review, analysis and interpretation of data. Dr. Dhamija: acquisition of data, study supervision, critical revision of the manuscript.

STUDY FUNDING

No targeted funding reported.

DISCLOSURE

The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

Footnotes

REFERENCES

  1. 1.↵
    1. Kadonaga JN,
    2. Frieden IJ
    . Neurocutaneous melanosis: definition and review of the literature. J Am Acad Dermatol 1991;24:747–755.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Kusters-Vandevelde HV,
    2. Willemsen AE,
    3. Groenen PJ,
    4. et al
    . Experimental treatment of NRAS-mutated neurocutaneous melanocytosis with MEK162, a MEK-inhibitor. Acta Neuropathol Commun 2014;2:41.
    OpenUrlCrossRefPubMed
View Abstract

Letters: Rapid online correspondence

  • Neurocutaneous melanosis
    • Manish Kumar, Resident, University of Virginia
    • Radhika Dhamija, Charlottesville, VA, USA
    Submitted July 08, 2015
  • Neurocutaneous melanocytosis
    • Yasmin Khakoo, Child Neurology Director, Memorial Sloan Kettering Cancer Center
    • New York
    Submitted June 30, 2015
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