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A 12-year-old female presented with a 1-year history of a slow-growing lesion on the frontal scalp. The patient reported intermittent headaches increasing in frequency localized under the lesion. Physical exam revealed a firm, fixed, 5-mm orange-pink papule on the right frontal scalp. Excisional biopsy was performed using a 6-mm punch biopsy, after which the patient reported a cessation of headaches. Histopathology showed a diffuse proliferation of spindled and epithelioid cells within the superficial and deep reticular dermis demonstrating a nested as well as fascicular growth pattern. There was also focal myxoid dermal change. Immunohistochemical staining showed the tumor to be strongly positive for NK1C3 and negative for S-100. A diagnosis of cellular neurothekeoma was made. Cellular neurothekeomas are slow-growing, asymptomatic nodules that most frequently appear on the head and neck of young adults. Cellular neurothekeomas rarely present with symptoms beyond mild tenderness upon palpation. Our case represents a unique presentation of a neurothekeoma of the scalp with complaint of headaches, previously unpublished in the literature.

A Case of a Cellular Neurothekeoma Presenting with Headaches and Review of the Literature