摘要目的 总结头颈部原始神经外胚层肿瘤的临床特征、诊断及治疗方法.方法 回顾性分析北京协和医院耳鼻咽喉科2004年7月至2014年6月收治的5例原发于头颈部的外周原始神经外胚层肿瘤患者的临床资料.结果 5例患者中1例病变局限于颞部,采取肿瘤根治性切除手术,术后给予放射治疗,随访24个月无复发.3例病变范围广泛,分别侵及上颌骨、颞下窝、面颊部等结构,3例患者均经活检明确诊断,其中1例采取术前放疗+肿瘤根治性切除+术后化疗方案,至今已无瘤生存106个月;1例采取术前化疗+肿瘤根治性切除+术后放疗方案,术后15个月肿瘤复发,后因回当地治疗失访;另l例患者采取术前化疗+放疗+肿瘤扩大切除手术,术后5个月出现肺转移,并于发病25个月后死于脑转移.l例外籍患者病变累及颌下、口底及舌体,经活检明确诊断后给予化疗,肿物明显缩小,后因患者回国失访.结论 原发于头颈部的原始神经外胚层肿瘤非常少见,临床主要表现为局部占位性病变及压迫症状,确诊有赖于病理检查,治疗上以综合治疗为宜,包括手术切除、局部放疗和全身化疗.

abstractsObjective To review the clinical characteristics,diagnosis and treatments of primitive neuroectodermal tumor (PNET) in head and neck.Methods A retrospective review of the medical records for the cases of PNET in head and neck from 2004 to 2014.General clinical information including diagnosis and treatments was obtained and analyzed.A literature review was also conducted.Results A total of 5 cases diagnosed with PNET were included.Of 5 patients,one patient with lesion in the temporal field was treated with radical resection,followed by radiotherapy,and a 24-month follow-up showed no recurrence.Three patients presented with maxillary,infratemporal fossa or cheek invasion respectively,after diagnosed with pathological examination,one patient received preoperation radiotherapy plus radical resection and postoperative chemotherapy,and the lesions had no obvious progress with follow-up of 106 months;one patient was applied with preoperative chemotherapy plus extensive resection and postoperation radiotherapy,showed recurrence 15 months later,and was lost to follow-up;and another patient underwent chemotherapy plus radiotherapy and extensive resection,presented with pulmonary metastasis 5 months later,and died of brain metastasis within 25 months.One foreign patient presented with tumor involved submaxillary,mouth and tongue,the tumor was reduced obviously after chemotherapy,but he was lost to follow-up after getting home.Conclusions PNET in head and neck is rare and the clinical profile of PNET is the presence of occupying and compression.The pathology examination is an only way to confirm the diagnosis of PNET.The combination of excision,radiotherapy and chemotherapy is the treatment choice.