头颈部滑膜肉瘤临床诊治分析

Diagnosis and treatment of synovial sarcoma in head and neck

目的 探讨头颈部滑膜肉瘤的临床表现、病理特点及诊治情况。方法 分析我院2009年12月~2011年11月诊治的4例滑膜肉瘤患者的临床、病理资料及预后情况。结果 4例滑膜肉瘤患者均行手术治疗,术后2例行化疗,1例行放化疗;术后3例患者局部复发,其中2例发生远处转移（肺及肋骨）。免疫组织化学结果示细胞角蛋白和波形蛋白多为阳性,SYT/SSX融合基因可用来确诊该病。随访至今,2例患者死亡。结论 头颈部的滑膜肉瘤多发于青中年,极易误诊,需病理形态学、免疫组织化学及分子遗传学确诊。由于该病的高侵袭性,应早期诊断和综合治疗。

Objective To analyze the clinical presentations, pathological features, diagnosis and treatment of synovial sarcoma in head and neck. Methods Data including clinical findings, pathologic changes and prognosis of four patients suffering from synovial sarcoma and hospitalized in our department during Dec 2009 to Nov 2011 were analyzed retrospectively. Results Immunohistoehemical analysis showed that most tumor cells were positive for cytokeratin and vimentin. Detection of SYT/SSX fusion transcript was used for confirming the diagnosis of synovial sarcoma. All 4 patients underwent primary surgical excision. After surgery, two patients received chemotherapy and one received combined chemotherapy and radiotherapy. Three of them had local recurrence, and distant metastases （lung and bone） occurred in two. Two of the patients died, and two were alive. Conclusions Synovial sarcoma of head and neck is a disease of young people and carries a poor prognosis. Correct pre-operative diagnosis is difficult. Accurate diagnosis depends on morphologic, immunohistochemical examinations and proper molecular analysis. Due to the aggressive nature of primary synovial sarcoma, early diagnosis and comprehensive treatment including extensive resection and postoperative chemoradiation are required.