Case Report: Primary epithelioid sarcoma of orbit: first case in Asia and literature review - Scorecard - 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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Clinical Scorecard: Report on a Unique Case of Primary Epithelioid Sarcoma in the Orbit: The First Documented Instance in Asia with a Review of Existing Literature

At a Glance

CategoryDetail
ConditionPrimary Epithelioid Sarcoma of Orbit
Key MechanismsCharacterized by epithelioid morphology, multinodular growth, and loss of nuclear INI1 expression.
Target PopulationAsian population, specifically a 25-year-old male patient.
Care SettingClinical case report and literature review.

Key Highlights

  • First documented case of primary epithelioid sarcoma of the orbit in Asia.
  • Histological features include multinodular architecture with epithelioid and spindle cells.
  • Immunohistochemical profile shows loss of nuclear INI1 expression.
  • Postoperative management included extended tumor resection and local intensity-modulated radiotherapy.
  • No local recurrence or distant metastasis detected at 7 months postoperatively.

Guideline-Based Recommendations

Diagnosis

  • Pathological examination is the gold standard for diagnosis.

Management

  • Extended tumor resection combined with local intensity-modulated radiotherapy.

Monitoring & Follow-up

  • Regular follow-up for local recurrence and distant metastasis.

Risks

  • Potential for local recurrence and metastasis if not adequately treated.

Patient & Prescribing Data

25-year-old male with primary epithelioid sarcoma of the left orbit.

Combination of surgical resection and radiotherapy is crucial for management.

Clinical Best Practices

  • Consider differential diagnosis for orbital masses including vascular tumors and inflammatory lesions.
  • Utilize immunohistochemical markers for accurate diagnosis.

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