Neurocristic hamartoma in Adult
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Synopsis
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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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Aplasia cutis
- Common blue nevus
- Cellular blue nevus
- Pigmented epithelioid melanocytoma
- Spitz nevus
- Pigmented nerve sheath tumor
- Dermatofibrosarcoma protuberans
- Melanoma
- Nevus of Ota
- Nevus of Ito
- Becker nevus
- Smooth muscle hamartoma
- Plexiform neurofibroma
- Neurofibromatosis type 1
- Neurocutaneous melanosis
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Therapy
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References
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Last Reviewed:11/01/2021
Last Updated:11/23/2021
Last Updated:11/23/2021