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Rib-Origin Ewing Sarcoma With Cranial Nerve Involvement From Orbital Metastasis: A Rare Case Report
Muhammad Noraiz Abbas Khan1, Khubaib Ghufran2, Muhammad Tausif1
1King Edward Medical University Lahore Pakistan.
This case study details a rare instance of Ewing sarcoma (ES) presenting with orbital and cranial involvement. Early detection and aggressive treatment are crucial for improving survival rates in this aggressive bone tumor.
Area of Science:
- Oncology
- Pathology
- Medical Imaging
Background:
- Ewing sarcoma (ES) is an aggressive bone tumor in young adults, often originating in the pelvis or long bones.
- It is characterized by the EWSR1-FLI1 fusion gene due to a specific chromosomal translocation.
- While lung metastasis is common, orbital and cranial involvement is exceptionally rare.
Purpose of the Study:
- To report a rare case of Ewing sarcoma with unusual orbital and cranial involvement.
- To emphasize the importance of early detection and aggressive treatment for improving patient outcomes in ES.
- To highlight the diagnostic utility of immunohistochemistry in resource-limited settings.
Main Methods:
- Case report of a 23-year-old male with progressive shortness of breath, chest swelling, and proptosis.
- Diagnostic imaging included Computed Tomography (CT) and Magnetic Resonance Imaging (MRI).
- Histopathological analysis involved CT-guided biopsy, immunohistochemistry (CD99, NKX2.2), and Ki-67 index assessment. Pleural fluid analysis was also performed.
Main Results:
- The patient presented with a large chest mass, pleural effusion, and orbital mass causing proptosis and diplopia.
- Initial pleural fluid analysis mimicked tuberculosis but biopsy confirmed small round blue cell tumor.
- Immunohistochemistry confirmed Ewing sarcoma with a high Ki-67 proliferation index.
Conclusions:
- Ewing sarcoma with orbital and cranial involvement is a rare but critical presentation.
- Prompt diagnosis and multimodal therapy (chemotherapy, radiation, surgery) are vital for survival.
- Immunohistochemistry plays a key role in diagnosing ES, particularly in resource-limited environments.
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