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Orbital Ewing's sarcoma of the orbit
J J Dutton1, J G Rose, C M DeBacker
1The Department of Ophthalmology, Duke University Eye Center, Durham, North Carolina, USA.
Ophthalmic Plastic and Reconstructive Surgery
|August 3, 2000
Summary
Ewing's sarcoma is a rare childhood orbital bone tumor. Chemotherapy significantly improves survival rates for this rare malignancy, from less than 10% to approximately 50%.
Area of Science:
- Pediatric Oncology
- Ophthalmology
- Skeletal Tumors
Background:
- Ewing's sarcoma is a rare primary bone tumor in children, infrequently involving the orbit.
- Orbital involvement is often metastatic and can be misdiagnosed as other small round cell malignancies.
Observation:
- Two pediatric cases of orbital Ewing's sarcoma are presented, one with an occult frontal bone primary and another with a known clavicular primary.
- Clinical manifestations include proptosis, pain, visual loss, and motility restriction, often unsuspected before histologic evaluation.
Findings:
- Immunohistochemical and electron microscopic studies are crucial for accurate diagnosis of small round cell tumors.
- Histologic evaluation is essential, as the diagnosis is typically unexpected.
Implications:
- Ewing's sarcoma of the orbit requires multimodal treatment, including chemotherapy, to improve survival.
- While surgery and radiotherapy alone yield poor outcomes (<10% 5-year survival), chemotherapy increases survival to approximately 50%.