Cellular Neurothekeoma with Atypical Histologic Features in the Thigh: A Case Report
PDF

Keywords

Cellular neurothekeoma
Immunohistochemistry
Neurothekeoma
Soft tissue tumor
Case report

Categories

Data Availability Statement

The data supporting the findings of this case report are not publicly available in order to protect patient privacy and confidentiality. All relevant clinical, histopathological, and immunohistochemical information supporting the conclusions of this report is included within the article. Additional de-identified details may be made available from the corresponding author upon reasonable request and subject to institutional and ethical considerations.

How to Cite

1.
Hasan‬‏ ‪Abdulkarim, Nafie K, Merabet O, et al. Cellular Neurothekeoma with Atypical Histologic Features in the Thigh: A Case Report. ASIDE Case Reports. 2026;3(1):31-35. doi:10.71079/ASIDE.CR.022826385

Abstract

Neurothekeoma is a rare, benign neoplastic process that most commonly presents on the head and neck of young adults. We report a case of cellular neurothekeoma in a middle-aged woman with a thigh lesion showing histologic features that closely mimicked malignant tumors, particularly undifferentiated pleomorphic sarcoma, sarcomatoid carcinoma, and amelanotic melanoma.

A dermal/subcutaneous thigh lesion demonstrated marked cytologic atypia and an infiltrative growth pattern on histologic evaluation. Immunohistochemistry supported the diagnosis of cellular neurothekeoma: tumor cells were positive for CD10 and showed very focal MITF staining, and negative for S100, SOX10, desmin, EMA, AE1/AE3, SMA, NSE, and factor XIIIa. The lesion was completely excised with clear margins. At 12 months of clinical follow-up, no recurrence was observed.

Cellular neurothekeoma should be considered in the differential diagnosis of atypical dermal soft-tissue masses with worrisome histologic features. Ancillary studies, particularly immunohistochemistry, are essential to exclude aggressive mimics and avoid overtreatment, although conclusions remain limited by the single-case nature of this report and the available literature.

PDF

References

1. Harkin JC, Reed RJ. Tumors of the peripheral nervous system: Armed Forces Institute of Pathology; 1969.

2. Vered M, Fridman E, Carpenter WM, Buchner A. Classic neurothekeoma (nerve sheath myxoma) and cellular neurothekeoma of the oral mucosa: immunohistochemical profiles. J Oral Pathol Med. 2011: 174 [PMID: 20923450, https://doi.org/10.1111/j.1600-0714.2010.00952.x]

3. Gallager RL, Helwig EB. Neurothekeoma--a benign cutaneous tumor of neural origin. Am J Clin Pathol. 1980: 759 [PMID: 7446487, https://doi.org/10.1093/ajcp/74.6.759]

4. Murphrey M, Huy Nguyen A, White KP, Krol A, Bernert R, Yarbrough K. Pediatric cellular neurothekeoma: Seven cases and systematic review of the literature. Pediatr Dermatol. 2020: 320 [PMID: 31930561, https://doi.org/10.1111/pde.14043]

5. Wilson AD, Rigby H, Orlando A. Atypical cellular neurothekeoma--a diagnosis to be aware of. J Plast Reconstr Aesthet Surg. 2008: 186 [PMID: 17709307, https://doi.org/10.1016/j.bjps.2007.01.078]

6. Chen KH, Fang CL, Chen CC. Cellular-type neurothekeoma over the chest wall of a woman. Tzu Chi Med J. 2016: 33 [PMID: 28757716, https://doi.org/10.1016/j.tcmj.2015.11.002]

7. Stratton J, Billings SD. Cellular neurothekeoma: analysis of 37 cases emphasizing atypical histologic features. Mod Pathol. 2014: 701 [PMID: 24186141, https://doi.org/10.1038/modpathol.2013.190]

8. Ojha HR, Verma N, Srivastava H, Raguram S. Cellular neurothekeoma of the eyelid: A rare tumor with a common clinical presentation. Indian Journal of Ophthalmology - Case Reports. 2024: 395 https://doi.org/10.4103/ijo.Ijo_1075_23]

9. Fetsch JF, Laskin WB, Hallman JR, Lupton GP, Miettinen M. Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information. Am J Surg Pathol. 2007: 1103 [PMID: 17592278, https://doi.org/10.1097/PAS.0b013e31802d96af]

10. Navarrete-Dechent C, Curi-Tuma M, Marin C, Gonzalez S, Sandoval-Osses M. Cellular neurothekeoma: case report and its (un) relation with nerve sheath myxoma. An Bras Dermatol. 2015: 156 [PMID: 26312702, https://doi.org/10.1590/abd1806-4841.20153402]

11. de Giorgi V, Alfaioli B, Franchi A, Gori A, Sestini S, Papi F, Lotti T. Cellular neurothekeoma in a girl: could oestrogens favour the development and growth of this rare tumour? J Eur Acad Dermatol Venereol. 2008: 1149 [PMID: 18462302, https://doi.org/10.1111/j.1468-3083.2008.02595.x]

12. D'Alessandris N, Picchetto A, Pignataro MG, Cerbelli B, Manzo D, Rocca CD, d'Amati G, Pernazza A. Atypical cellular neurothekeoma (ACN) of the elderly: case report and brief review of the literature. Pathologica. 2020: 210 [PMID: 33393524, https://doi.org/10.32074/1591-951X-104]

13. Wick MR. Cutaneous Tumors and Pseudotumors of the Head and Neck. Gnepp's Diagnostic Surgical Pathology of the Head and Neck. 2021: 1012 https://doi.org/10.1016/b978-0-323-53114-6.00014-6]

14. Hübner-Garretón ML, Ñanco Meléndez C, Muñoz Montes C, Riveros Marivil F. Diagnóstico diferencial de un tumor de partes blandas en el brazo. Piel. 2025: 26 https://doi.org/10.1016/j.piel.2024.04.004]

15. Bhatia S, Chu P, Weinberg JM. Atypical cellular neurothekeoma. Dermatol Surg. 2003: 1154 [PMID: 14641347, https://doi.org/10.1046/j.1524-4725.2003.29356.x]

16. de la Guardia V, Castro-Perez E, Porcell AI, de Tena-Davila SG, Pacheco M. Atypical cellular neurothekeoma: a case report with a novel NF1 mutation. Diagn Pathol. 2024: 151 [PMID: 39578817, https://doi.org/10.1186/s13000-024-01578-y]

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Copyright (c) 2026 ‪Abdulkarim Hasan‬‏, Khalid Nafie, Okba Merabet, Stephen Osasan, Samira Attar, Mohammed S. Abdelwahed, Ali Nagi