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Atypical cellular neurothekeoma.

Sonal Bhatia1, Paul Chu, Jeffrey M Weinberg

  • 1Department of Dermatology, St. Luke's-Roosevelt Hospital Center and Beth Israel Medical Center, 1090 Amsterdam Avenue, Suite 11D, New York, NY 10025, USA.

Dermatologic Surgery : Official Publication for American Society for Dermatologic Surgery [Et Al.]
|December 4, 2003
PubMed
Summary

A rare variant, atypical cellular neurothekeoma, can mimic malignancy but is curable with complete surgical excision. Dermatologists should recognize its distinctive features to ensure proper diagnosis and treatment.

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Area of Science:

  • Dermatopathology
  • Oncology
  • Surgical Pathology

Background:

  • Neurothekeoma, previously classified with benign peripheral nerve sheath tumors, encompasses rare variants like atypical cellular neurothekeoma.
  • This variant presents with concerning features such as large size, deep infiltration, infiltrative borders, vascular invasion, high mitotic rate, and significant cytologic pleomorphism.

Observation:

  • A case study details a 34-year-old woman with atypical cellular neurothekeoma, exhibiting both cellular and intermediate subtypes.
  • The cellular subtype showed cells with rounded/ovoid nuclei and abundant cytoplasm, high mitotic activity (8-12/HPF), and individual cell necrosis.
  • The intermediate subtype featured smaller nests with mucin, lower mitotic activity (1-2/HPF), and negative staining for S100, EMA, and SMA.

Findings:

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  • Histopathology revealed distinct cellular and intermediate subtypes within the atypical cellular neurothekeoma.
  • Immunohistochemical analysis showed negativity for S100, epithelial membrane antigen, and smooth muscle actin in the intermediate areas.
  • The neoplasm demonstrated features that could raise concern for malignancy, including high mitotic activity and pleomorphism.

Implications:

  • Dermatologists must be aware of atypical cellular neurothekeoma's characteristics to differentiate it from malignant lesions.
  • Despite concerning features, complete surgical excision is curative for atypical cellular neurothekeoma.
  • Early recognition and appropriate surgical management are crucial for favorable patient outcomes.