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Extraskeletal Ewing sarcoma of the buccal space
Sarah L Spaulding1, Monica H Xing1, Gabriella T Seo1
1Thyroid, Head, and Neck Cancer (THANC) Foundation, 10 Union Square East, Suite 5B, New York, NY 10003, USA.
Clinical Imaging
|December 28, 2020
Summary
Extraskeletal Ewing sarcoma (EES) of the head and neck is rare. This case highlights an unusual EES presentation in the buccal space, aiding future diagnosis.
Area of Science:
- Oncology
- Radiology
- Pathology
Background:
- Extraskeletal Ewing sarcoma (EES) is a rare soft tissue malignancy.
- EES in the head and neck region presents unique diagnostic challenges due to anatomical complexity and infrequent occurrence.
Observation:
- A 19-year-old male presented with facial swelling initially presumed to be a lymphatic malformation.
- Initial imaging revealed a rim-enhancing lesion in the left buccal space without bone or muscle involvement.
- Follow-up imaging demonstrated significant growth with destruction of the zygomatic arch and orbital wall.
Findings:
- Pathology confirmed a small round blue cell tumor, with FISH testing revealing the EWSR1 gene rearrangement characteristic of EES.
- The case presented an atypical EES imaging phenotype: a rim-enhancing, centrally hypointense mass without initial bony erosion or muscular invasion.
Implications:
- This case underscores the importance of considering EES in the differential diagnosis of head and neck masses, even with atypical imaging features.
- The unusual presentation offers valuable insights for radiologists and oncologists in the early detection and management of buccal space EES.
- Successful treatment with surgical resection followed by chemoradiation resulted in a disease-free outcome at three years postoperatively.
