• Pankaj K Agarwalla, Matthew J Koch, Daniel A Mordes, Patrick J Codd, and Jean-Valery Coumans.
• Departments of *Neurosurgery and‡Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
• Neurosurgery. 2016 Jan 1;78(1):142-55.

Unlabelled: Neurosurgeons encounter a number of pigmented tumors of the central nervous system in a variety of locations, including primary central nervous system melanoma, blue nevus of the spinal cord, and melanotic schwannoma. When examined through the lens of embryology, pigmented lesions share a unifying connection: They occur in structures that are neural crest cell derivatives. Here, we review the important progress made in the embryology of neural crest cells, present 3 cases of pigmented tumors of the nervous system, and discuss these clinical entities in the context of the development of melanoblasts. Pigmented lesions of the nervous system arise along neural crest cell migration routes and from neural crest-derived precursors. Awareness of the evolutionary clues of vertebrate pigmentation by the neurosurgical and neuro-oncological community at large is valuable for identifying pathogenic or therapeutic targets and for designing future research on nervous system pigmented lesions. When encountering such a lesion, clinicians should be aware of the embryological basis to direct additional evaluation, including genetic testing, and to work with the scientific community in better understanding these lesions and their relationship to neural crest developmental biology.AbbreviationsDRG, dorsal root gangliaEMT, epithelium-to-mesenchyme transitionNC, neural crestNCM, neurocutaneous melanosisSCP, Schwann cell precursor.

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