Key result
Rare ethmoidal sinus synovial sarcoma demonstrates an aggressive clinical course in two reported cases.
Why the study?
Synovial sarcoma is rare in the head and neck and rarest in the ethmoidal sinus, with only three cases reported till date.
Case Report (n=2)
Synovial sarcoma of the ethmoidal sinus is a rare and aggressive cancer with poor prognosis requiring aggressive management.
Two additional ethmoidal synovial sarcoma cases support inclusion in sinonasal differentials; leaves open optimal multimodal management strategies.
Background Synovial sarcoma is an aggressive soft tissue cancer of extremities mainly and rare in head and neck region, whereas rarest in ethmoidal sinus as only three cases have been reported till date. Case Reports We managed two cases of synovial sarcoma who presented with nasal obstruction, epistaxis, and swelling around the nasofacial region. Endoscopic nasal biopsy and immunohistochemistry markers confirmed synovial sarcoma in both the cases. While one case was managed by surgery and chemoradiation, the second patient received two cycles of ifosfamide-based chemotherapy and succumbed after 6 weeks of diagnosis. Conclusion Head and neck sarcomas are aggressive and carry a poor prognosis. Surgical resection with postoperative radiotherapy is the standard treatment. However, they have a high risk of recurrence and hence aggressive management and close follow-up is warranted for the optimal outcome.
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Dhiman et al. (2021) conducted a case report in Synovial sarcoma of ethmoidal sinus (n=2). Surgery, chemoradiation, and ifosfamide-based chemotherapy was evaluated on Clinical outcome. Two cases of rare ethmoidal sinus synovial sarcoma demonstrated aggressive disease, with one patient managed by surgery and chemoradiation and the other succumbing 6 weeks after diagnosis.
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