鼻腔鼻窦间叶组织恶性肿瘤

The sinonasal mesenchymal malignant tumors

目的 分析鼻腔鼻窦间叶组织恶性肿瘤的临床特征，总结诊疗经验。方法 回顾分析我院1990年1月～2003年12月收治的16例鼻腔鼻窦间叶组织恶性肿瘤，15例行手术广泛切除，1例在鼻内镜下切除上颌窦肿瘤和部分鼻腔外侧壁；14例在术后接受放疗和化疗。结果 骨肉瘤2例，软骨肉瘤3例，横纹肌肉瘤和血管肉瘤各4例，恶性血管外皮瘤、恶性纤维组织细胞瘤和纤维肉瘤各1例。12例随访完整，生存期最短2个月，最长8年。4例在治疗后2年内、5例在治疗后第2～5年期间因肿瘤复发死亡，3例超过5年存活期。结论 鼻腔鼻窦间叶组织恶性肿瘤临床表现无特异性，病理切片结合免疫组化可确诊，手术加放疗和化疗为基本的治疗模式，预后差。

Objective To explore the clinical features of sinonasal mesenchymal malignant tumors and summarize the diagnosis and management experiences. Methods Clinical data of 16 patients with sinonasal mesenchymal tumors who were treated in our hospital from Jan 1, 1990 to Dec 30, 2003 were analyzed retrospectively. 15 cases were treated with radical excision and the whole tumor in the maxillary sinus and partial lateral wall of the ipsilateral nasal cavity was excised in 1 case. Postoperative radiotherapy and chemotherapy were applied to 14 cases. Results All the 16 cases, the case numbers of osteosarcoma, chondrosarcoma, rhabdomyosarcoma, hemangiosarcoma, malignant hemangiopericytomas, malignant fibrous histiocytoma and fibrosarcoma were 2,3, 4, 4, 1, 1 and 1. 12 cases were completely followed up. The shortest survial time was 2 months and the longest has been 8 years, while 4 patients died of tumor relapse in 2 years, 5 patients died during 2 to 5 years after treatment and 3 patients survied longer than 5 years. Conclusion Sinonasal mesenchymal malignant tumors have no specific clinical situations. The diagnosis of the tumors depends on the pathological immunohistochemical examinations. The basic treatment model is operation combined with radiotherapy and chemotherapy, and the prognosis is gloomy.