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Rare case of Infiltrative multifocal Ewing sarcoma presenting as neck mass
Shubhlaxmi Srivastava1, Gaurav Raj1, Kaustubh Gupta1
1Department of Radio-Diagnosis, Dr Ram Manohar Lohia Institute Of Medical Sciences, Lucknow, Uttar Pradesh, India.
Radiology Case Reports
|August 22, 2025
Summary
Extra skeletal Ewing sarcoma, a rare aggressive tumor, can present atypically in the neck. Early diagnosis and chemotherapy are crucial for managing this challenging cancer in adolescents and young adults.
Area of Science:
- Oncology
- Pathology
- Radiology
Background:
- Ewing sarcoma is a rare, aggressive small round cell tumor predominantly affecting adolescent and young adult bone. Extraskeletal involvement is uncommon.
- Typical presentations involve long bones or pelvis, with head and neck involvement being exceedingly rare.
Observation:
- An 18-year-old male presented with a painful neck mass, diagnosed as extraskeletal Ewing sarcoma involving the occipital bone and C1 vertebra.
- Imaging demonstrated extensive intracranial, intraspinal, and paravertebral extension with widespread skeletal metastases.
- Histopathology and immunohistochemistry confirmed the diagnosis, with positive NKX 2.2 expression.
Findings:
- The patient received systemic chemotherapy with the VDC/IE regimen.
- Significant clinical and radiological improvement was observed following treatment.
Implications:
- This case highlights the importance of considering extraskeletal Ewing sarcoma in atypical cranial and paraspinal presentations.
- Early multimodal imaging and prompt chemotherapy are vital for managing aggressive extraskeletal Ewing sarcoma.
- The findings emphasize the need for a broad differential diagnosis in young patients with unexplained neck masses.
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