Pathological Features of Atypical Cellular Neurothekeoma of the Nasal Ala

DOI: https://doi.org/10.62517/jmhs.202505226

Author(s)

Qinxue Pu1,2, Zhongping Huan1, Juan Xiang1,2, Feiyu Liu1,2,*

Affiliation(s)

1The First College of Clinical Medical Science, China Three Gorges University, Yichang, Hubei, China 2Department of pathology, Yichang Central People's Hospital, Yichang, Hubei, China

Abstract

Atypical cellular neurothekeoma (NTK) is a rare soft tissue tumor primarily arising in the skin. This study aimed to investigate its clinicopathological features, immunophenotype, differential diagnosis, and prognosis. We analyzed the clinical and pathological data of a 6-year-old boy who presented with a painless, red, round lesion measuring approximately 0.5 cm on the right nasal ala for two years. The lesion was moderately soft with rich surface vascularization and no ulceration. Histologically, the tumor was located in the dermis without connection to the epidermis and composed of epithelioid, mononuclear, and short spindle-shaped cells arranged in cord-like or small nodular structures separated by extensively hyalinized collagen fibers. Tumor cells exhibited mild atypia, eosinophilic cytoplasm, round and vacuolated nuclei with prominent nucleoli, and visible mitotic figures, with occasional eccentric nuclei and scattered multinucleated giant cells. Focal infiltration into deep striated muscle and perineural invasion was observed. Immunohistochemistry revealed strong positivity for PGP9.5 and CD10, focal positivity for CD99, scattered positivity for CD68, and negativity for S-100, SMA, Desmin, MyoD1, MelanA, ALK (1A4), and PCK. The Ki-67 proliferation index was 3%. Following complete surgical excision, the patient remained free of recurrence or metastasis over a 7-year follow-up period. The diagnosis of atypical cellular NTK relies on a combination of characteristic histological morphology and immunophenotypic profile.

Keywords

Neurothekeoma; Fibrohistiocytic Tumor; CD10

References

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