A 52-year-old man requests removal of an asymptomatic growth on his nose that has been gradually increasing in size over the past year. He denies history of skin cancer or warts. Examination reveals a 2-mm flesh-colored papule. Scattered skin tags are noted on his face, and seborrheic keratoses are found on his back.
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Neurothekeoma is an uncommon benign tumor that is believed to originate from the nerve sheath. The condition was coined neurothekeoma by Gallager and Helwing in 1980, from the Greek Oeke meaning “sheath.”1 Histology reveals a myxoid, cellular, or combination pattern. Lesions lack a capsule and are composed of cellular nodules separated by bands of collagen. Differentiation from related tumors such as neurofibroma and schwannoma is facilitated by immunohistochemical analysis.2
Neurothekeoma usually occurs on the face and neck, and the majority of cases occur in 20- to 30-year-old women.1 Extremely rare locations include the oral mucosa and conjunctiva.3,4 The lesion presents as a solitary, slow-growing, asymptomatic, dome-shaped pink to flesh-colored papule.5 Consistency may be either soft or firm. Full excision is curative.6 Malignant transformation has not been reported.
Stephen Schleicher, MD, is director of the DermDox Center for Dermatology, associate professor of medicine at Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.
1. Gallager RL, Helwig EB. Neurothekeoma–a benign cutaneous tumor of neural origin. Am J Clin Pathol. 1980;74(6):759-764.
2. Laskin WB, Fetsch JF, Miettinen M. The “neurothekeoma”: immunohistochemical analysis distinguishes the true nerve sheath myxoma from its mimics. Hum Pathol. 2000;31(10):1230-1241.
3. Tham AC, Chilagondanahalli NL, Bundele MM, Kanagalingam J. Oral neurothekeoma of the right buccal mucosa. Case Rep Otolaryngol. 2016;2016:4709753.
4. See TRO, Stålhammar G, Grossniklaus HE. Neurothekeoma of the eye, conjunctiva, and periorbital adnexa: a report of two cases and brief review. Surv Ophthalmol. 2019;64(6):852-857.
5. Maktabi AMY, Al-Hussain H, Khoja H, Alkatan HM. Unusual recurrent lateral canthus mass in a 16-year-old male patient: neurothekeoma. Case Rep Oncol. 2019;12(3):693-697.
6. Boukovalas S, Rogers H, Boroumand N, Cole EL. Cellular neurothekeoma: A7676767676 rare tumor with a common clinical presentation. Plast Reconstr Surg Glob Open. 2016;4(8):e1006.