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Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Ewing's sarcoma/primitive neuroectodermal tumour occurring in the maxillary sinus.
Masaki Kawabata1, Kosuke Yoshifuku, Yukari Sagara
1Department of Otolaryngology Head and Neck Surgery, Kagoshima University Graduate School of Medical and Dental Sciences, Japan. supercar@almond.ocn.ne.jp
Rhinology
|May 1, 2008
Summary
A rare Ewing's sarcoma/primitive neuroectodermal tumor in a child presented as cheek swelling. Successful treatment with chemotherapy and radiotherapy led to significant tumor reduction and no recurrence after 20 months.
Area of Science:
- Oncology
- Pediatric Oncology
- Radiology
Background:
- Ewing's sarcoma/primitive neuroectodermal tumor (PNET) is a rare malignant bone and soft tissue tumor.
- Maxillary sinus tumors are uncommon, especially in pediatric patients.
Observation:
- A 12-year-old boy presented with left cheek swelling.
- Fiberscopy revealed a nasal mass; CT and MRI showed a large maxillary sinus tumor with bone destruction and soft tissue invasion.
Findings:
- Histopathology revealed atypical cells positive for CD99 and weakly for NSE, consistent with Ewing's sarcoma/PNET.
- The tumor originated in the maxillary sinus and extended into the nasal cavity and cheek.
Implications:
- This case highlights the importance of considering rare tumors in the differential diagnosis of pediatric facial swelling.
- Multimodal treatment including chemotherapy and radiotherapy can be effective in managing sinonasal Ewing's sarcoma/PNET.
- Early diagnosis and prompt treatment are crucial for favorable outcomes in pediatric oncology.
