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Neurocristic hamartoma in Adult
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Neurocristic hamartoma in Adult

Contributors: Lindsey Mohney BA, Jeffrey Globerson DO, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Neurocristic hamartoma (NCH) is an extremely uncommon, benign hamartoma of the skin and soft tissue that arises from abnormal migration and differentiation of pluripotential cells from the neural crest that show differentiation toward Schwann cells, pigmented dendritic spindle cells, and melanocytes.

NCH presents as a blue-to-black pigmented plaque most commonly located on the scalp that may be found on other areas of the head, face, neck, and back. When on the scalp, there is often accompanying alopecia; however, lesions may rarely show local hypertrichosis. Most cases are congenital, but NCH may be acquired.

Recognition of NCH is critical as malignant transformation to melanoma and subsequent metastatic disease can occur. Thirty-eight percent of reported cases to date have shown the development of melanoma, and 19% of patients displayed metastatic disease. Patients with congenital NCH tend to develop malignant degeneration with melanoma occurring within 15-67 years, whereas acquired lesions show more rapid progression to melanoma within 1-6 years.

Codes

ICD10CM:
D23.9 – Other benign neoplasm of skin, unspecified

SNOMEDCT:
399921006 – Hamartoma of skin

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Last Reviewed:11/01/2021
Last Updated:11/23/2021
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Neurocristic hamartoma in Adult
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A medical illustration showing key findings of Neurocristic hamartoma
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