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Updated: May 15, 2026
06:15
Anterior High-Resolution Optical Coherence Tomography in the Diagnosis and Therapeutic Monitoring of Ocular Surface Squamous Neoplasia
Published on: August 9, 2024
Primary extraosseous Ewing sarcoma of the orbit
Jorge L Alio1, Marco Sales-Sanz, Maria A Vaz
1Oculoplastic and Orbital Surgery Unit, Ophthalmology Department, Ramón y Cajal University Hospital, Madrid, Spain.
Ophthalmic Plastic and Reconstructive Surgery
|December 19, 2012
Summary
A rare primary orbital Ewing sarcoma in an adult male caused vision loss. Multimodal treatment including chemotherapy, surgery, and radiotherapy led to a disease-free outcome.
Area of Science:
- Oncology
- Ophthalmology
- Pathology
Background:
- Orbital tumors are rare, and primary Ewing sarcoma of the orbit is exceptionally uncommon in adults.
- Adult-onset orbital Ewing sarcoma presents diagnostic challenges due to its rarity and potential mimicry of other orbital pathologies.
Observation:
- A 40-year-old male presented with painless, progressive vision loss and mild proptosis of the right eye.
- CT imaging revealed an intraconal orbital mass with optic canal involvement.
- Histopathological examination showed a diffuse proliferation of small, round, blue cells.
Findings:
- Immunohistochemical analysis was positive for vimentin and MIC2 (CD99), consistent with Ewing sarcoma.
- Genetic testing detected the characteristic EWS gene translocation (22q12).
- Metastatic workup, including a bone scan, was negative, confirming a primary orbital origin.
Implications:
- This case highlights the importance of considering rare diagnoses like primary orbital Ewing sarcoma in adult patients with orbital masses.
- A multidisciplinary approach involving ophthalmology, oncology, and pathology is crucial for accurate diagnosis and effective management.
- The successful treatment with neoadjuvant chemotherapy, orbital exenteration, and adjuvant radiotherapy offers a potential therapeutic strategy for this rare malignancy.
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