Case Report: Primary epithelioid sarcoma of orbit: first case in Asia and literature review - Takeaways - MDSpire

Case Report: Primary epithelioid sarcoma of orbit: first case in Asia and literature review

  • By

  • Guang Yang

  • Jingping Yuan

  • Fangfang Chen

  • Honglin Yan

  • Wen Liu

  • June 26, 2026

  • 0 min

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  • 1

    Primary epithelioid sarcoma is a rare malignant soft tissue neoplasm with distinct epithelioid morphology and unclear histogenesis.

  • 2

    This report presents the first documented case of primary epithelioid sarcoma in the orbit in Asia, involving a 25-year-old male.

  • 3

    Histological examination revealed multinodular growth with epithelioid and spindle cells, alongside necrotizing granuloma-like changes.

  • 4

    Immunohistochemical analysis showed loss of nuclear INI1 expression, critical for the diagnosis of primary epithelioid sarcoma.

  • 5

    The patient underwent tumor resection and local radiotherapy, with no local recurrence or distant metastasis observed at 7 months postoperatively.

Original Source(s)

Case Report: Primary epithelioid sarcoma of orbit: first case in Asia and literature review

  • by Guang Yang, Jingping Yuan, Fangfang Chen, Honglin Yan, Wen Liu

  • June 26, 2026

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