Epithelioid Sarcoma of the Peripheral Nerve: Clinicopathologic Series of Three Cases and Literature Review

Am J Clin Pathol. 2021 Apr 26;155(5):729-737. doi: 10.1093/ajcp/aqaa180.


Objectives: Epithelioid sarcoma (ES) rarely arises in the nerve. To increase our understanding of this unusual tumor originating in the nerve, we describe the features of three cases and review the literature.

Methods: Clinical data, imaging, pathology, treatment, and follow-up are detailed. A systematic literature review was conducted.

Results: Two patients were male and one female; the median age was 24 years. The patients had neurologic symptoms, and the tumors arose in large nerves and ranged from 2.4 to 5.8 cm. The tumors were avid on positron emission tomography-computed tomography and showed increased signal intensity on T2-weighted magnetic resonance imaging. Centered in the nerve, the tumors grew with an infiltrative pattern and encased the nerve fascicles. All were treated with wide resection, and adjuvant treatment included combinations of chemotherapy and radiation. One recurred, and the limb was amputated. Metastases were documented to lymph nodes, lung, pleura, and skin. One patient died of disease after 54 months. Literature review including our cases showed that tumors stained with pancytokeratin (9/9), EMA (4/4), and CD34 (7/7); there was loss of INI1 in all six cases tested.

Conclusions: ES rarely arises in the peripheral nerve, and its infiltrative nature often requires morbid surgery. The differential includes a variety of benign and malignant epithelioid neoplasms.

Keywords: Connective and soft tissue neoplasms; Epithelioid sarcoma; Nerve sheath neoplasms; Pathology; Peripheral nerves.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Biomarkers, Tumor / analysis
  • Female
  • Humans
  • Immunohistochemistry / methods
  • Male
  • Neoplasm Recurrence, Local / diagnosis
  • Neoplasm Recurrence, Local / pathology*
  • Neurilemmoma / metabolism*
  • Neurilemmoma / pathology
  • Sarcoma / diagnosis
  • Sarcoma / pathology*
  • Soft Tissue Neoplasms / diagnosis
  • Soft Tissue Neoplasms / pathology*


  • Biomarkers, Tumor