【摘 要】
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Background: Synovial cell sarcomas represent a rare group of cancers, particularly in the head and neck region.Furthermore, only a few cases of retropharyngeal and laryngeal synovial cell sarcomas hav
【机 构】
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Nepean Hospital, The University of Sydney Australia
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Background: Synovial cell sarcomas represent a rare group of cancers, particularly in the head and neck region.Furthermore, only a few cases of retropharyngeal and laryngeal synovial cell sarcomas have been documented to date.The primary translocation identified in these cancers is t(X;18)(p 11 ;q 11).Histologically they may be divided into monophasic, biphasic or poorly differentiated subtypes.Methods: We presented a case report with operative and specimen photographs of a patient with biphasic synovial cell sarcoma in the larynx and retropharynx and conducted a systematic review on the epidemiology, diagnosis and management of synovial sarcomas using multiple databases including Pubmed, Current Contents, Medline.Results: Twelve cases of laryngeal and retropharyngeal synovial sarcomas were identified via our literature search.It affects people in the third to fifth decade of life and affects males/females in the ratio of 2∶1.Diagnosis is based on fine needle aspiration biopsy, immunohistochemistry and molecular typing.Prognosis varies with patient age, tumour site and size, degree of necrosis, level of mitotic activity and neurovascular invasion.Complete tumour resection is first line treatment in locally invasive disease.CO2 lasers useful in controlling localised disease.There is also a role for adjuvant radiotherapy.Ifosfamide based chemotherapy is most useful for metastatic disease.Conclusion: Head and neck synovial sarcomas are rare, posing challenging diagnostic and management issues.Multidisciplinary approach to management and long term follow up is essential to monitor for recurrence and improve disease free survival.
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