Cellular neurothekeoma: case report and its (un) relation with nerve sheath myxoma

Abstract

Neurothekeoma is an uncommon, benign neoplasm presenting in young adults, primarily on the head and neck. It was initially related to nerve sheath myxoma but with the advent of immunohistochemistry, new insights into its cellular differentiation and origin have emerged, unlinking Neurothekeoma and nerve sheath myxoma. Herein we describe a 19-year-old male who had had a frontal, fl esh-colored, asymptomatic papule for 2 years. Histology showed a dermal fusocellular-spindle cell tumor, including an eosinophilic cytoplasm with mild cellular pleomorphism and moderately dense fi brous stroma. IHQ was positive for CD10 and negative for S100 and Claudin-1. These fi ndings were compatible with cellular Neurothekeoma. The lesion was completely extirpated and at the 6-month follow-up, the patient was asymptomatic and had experienced no recurrences.

Pediatric cellular neurothekeoma: Seven cases and systematic review of the literature

“…They also highlight the utility of NK1/C3 positivity and S‐100 protein negativity in differentiating CNT from conventional neurothekeoma (nerve sheath myxoma). Our findings also validate the previously reported high incidence of CD10 positivity in CNTs . Some of the less well‐described markers with high rates of positivity in this population included vimentin, KBA.62 and CD56.…”

“…Historically, neurothekeomas and nerve sheath myxomas were synonymous and neurothekeomas were postulated to be of nerve sheath origin . However, recent studies indicate that neurothekeomas may be of fibrohystiocytic differentiation and suggest that nerve sheath myxomas and neurothekeomas are morphologically and histopathologically distinct …”

Neurothekoma of the scalp

“…Neurothekoma (NT) is an uncommon and benign dermal tumor, originating from the sheath of peripheral nerves. It is mainly located on the face, neck, arms and shoulders of young adults, and is more frequent in females [1,2].…”

“…Clinically, it manifests as an asymptomatic, pinkerythematous, dome-shaped, solitary papule or nodule, with a slow growth rate [2]. The clinical differential diagnosis of NT includes epidermal cyst, dermatofibroma, adnexal tumor (e.g.…”

“…The clinical differential diagnosis of NT includes epidermal cyst, dermatofibroma, adnexal tumor (e.g. pilomatricoma), Fibrohistiocytic tumor, Basal cell carcinoma, hemangioma, amelanotic melanoma and cutaneous metastasis [2].…”