To report the first documented case of primary epithelioid sarcoma of the orbit in Asia and review existing literature on this rare tumor.
Approach:
Case Presentation: A 25-year-old male presented with painless proptosis of the left eye, diagnosed via MRI and histopathological examination.
Histopathological Analysis: The tumor exhibited multinodular growth with epithelioid and spindle cells, necrotizing changes, and invasion of skeletal muscle.
Immunohistochemical Findings: Tumor cells co-expressed epithelial and mesenchymal markers, with loss of nuclear INI1 expression, confirming the diagnosis.
Postoperative Management: The patient underwent tumor resection and local radiotherapy, with no recurrence or metastasis at 7 months follow-up.
Key Findings:
Primary epithelioid sarcoma of the orbit is extremely rare, with no prior documented cases in Asia.
Histological examination is crucial for diagnosis, highlighting the importance of characteristic features and immunophenotype.
The case enriches the clinicopathological data for this tumor in the Asian population.
Interpretation:
Pathological examination remains the gold standard for diagnosing primary epithelioid sarcoma.
Limitations:
As a single-case study, the findings may not be generalizable.
Further research with larger series is needed to confirm the results.
Conclusion:
This case provides insights into the clinical management and pathological diagnosis of primary epithelioid sarcoma of the orbit.
