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J Cutan Pathol. 2014 May;41(5):457-61. doi: 10.1111/cup.12297. Epub 2014 Feb 26.

Cutaneous epithelioid melanocytic neurofibroma arising in a patient with neurofibromatosis-1.

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University of Pennsylvania, Department of Dermatology, Philadelphia, PA, USA.


Tumors expressing both melanocytic and neural features can pose a diagnostic challenge to the dermatopathologist and provoke questions regarding their lineage. We report a case of a tumor arising on the right cheek of a 9-year-old boy with neurofibromatosis type 1 (NF-1). This neoplasm featured nests of non-pigmented epithelioid cells arising within a neurofibroma. The entire tumor stained strongly with S100, whereas the epithelioid population stained with MART-1, HMB-45 and MiTF. The neoplasm shows only scattered Ki-67 positivity. This tumor represents a neurofibroma with portions that have undergone melanocytic differentiation (melanocytic neurofibroma). This exceedingly rare tumor represents a distinct entity from neurotized melanocytic nevi, combined melanocytic nevi or pigmented neurofibromas and provides further evidence that melanocytes arise indirectly from ventromedial neural crest-derived Schwann cell precursors.


immunohistochemistry; melanocytic lesions; neural crest

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