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目的:探讨蝶窦原发肿瘤的临床特点、诊断及治疗，提高临床医师对其认识水平。方法:收集并回顾2014年8月至2017年6月中国医科大学附属盛京医院以病理确诊的5例蝶窦原发肿瘤患者的临床病例资料，分析其临床特点、诊断及治疗方式。结果:5例患者中，1例以头痛首诊于神经内科，2例以眶周胀痛首诊于眼科，2例以嗅觉下降、鼻塞、涕中带血、咽异物感等鼻部症状首诊于耳鼻喉科。所有患者术前均无头颈部手术外伤史，均行鼻窦CT和增强MRI检查，怀疑恶性者行ECT检查，明确无远处转移病灶和手术禁忌，全身麻醉下鼻内窥镜下行蝶窦开放术，其中3例辅助影像导航系统。术后病理回报1例脊索瘤，1例腺样囊性癌，1例浆细胞瘤，1例低分化癌，1例内翻性乳头状瘤局灶恶变。脊索瘤、腺样囊性癌、低分化癌及内翻性乳头状瘤局灶恶变术后转入肿瘤科行放化疗，浆细胞瘤单纯手术，因个人原因未进一步治疗，术后随访16~50个月，随访截止于2018年10月1日，低分化癌患者随访12个月出现肝转移死亡，其余4例均生活良好，继续随访中。结论:蝶窦原发肿瘤临床表现无特异性，就诊时很多已属晚期。影像学检查对疾病的诊断及手术方法的制定有重要作用，手术是治疗本病的主要方法，术后辅助放化疗对提高患者生存率有重要意义。“,”Objective:To study the clinical features, diagnosis and treatment of primary sphenoid sinus tumors and to improve the understanding of clinicians.Methods:The clinical data of 5 cases of primary sphenoid sinus tumors diagnosed by pathology from August 2014 to June 2017 in Affiliated Shengjing Hospital of China Medical University were collected and reviewed. The clinical characteristics, diagnosis and treatment were analyzed.Results:Of the 5 patients, 1 patient was first diagnosed in neurology with headache, 2 patients were first diagnosed in ophthalmology with periorbital pain, and 2 patients were diagnosed with nasal symptoms such as decreased olfactory, nasal congestion, blood stasis, and pharyngeal foreign body sensation. In the otolaryngology department, all patients had no history of head and neck surgery before surgery. All patients underwent sinus CT and MRI. They were suspected of malignant ECT examination. There were no distant metastatic lesions and surgical contraindications. General anesthesia and endoscopic sphenoid sinus surgery was used. And assisted image navigation systems were used in 3 patients. Postoperative pathological returns showed chordoma in 1 case, adenoid cystic carcinoma in 1 case, plasmacytoma in 1 case, poorly differentiated carcinoma in 1 case, and focal malignant transformation of inverted papilloma in 1 case. Patients with chordoma, adenoid cystic carcinoma, poorly differentiated carcinoma and inverted papilloma received foci surgery and were transferred to our hospital for oncology and chemotherapy. Patients with plasmacytoma only had surgery, and received no further treatment for personal reasons. Follow-up lasted 16-50 months and ended on October 1, 2018. Patients with poorly differentiated cancer were followed up for 12 months and died after liver metastases, and the other 4 patients lived well. Our department continued to follow up.Conclusions:The clinical manifestations of primary sphenoid sinus tumors are non-specific. Many of them are in advanced stage. Imaging examination plays an important role in the diagnosis of diseases and the development of surgical methods. Surgery is the main method for the treatment of this disease. Postoperative adjuvant chemotherapy and radiotherapy is of great significance to improve the survival rate of patients.