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目的探讨原发性肺部少见肿瘤的临床特征及治疗。方法回顾性分析福建省立医院2008年1月—2012年1月收治的原发性肺部少见肿瘤患者30例,术前借助增强CT扫描检查明确肿瘤位置、大小、浸润程度及与纵隔淋巴结的关系,判断手术切除的可能性,根据个体情况进行开胸手术或胸腔镜手术治疗,部分恶性肿瘤患者术后实行化疗。分析患者临床预后,统计其1、3、5年生存率。结果患者开胸手术19例,胸腔镜手术11例;全肺切除术3例,肺叶切除术21例,肺楔形切除术6例,术中均无不良事件发生。患者术后进行常规病理诊断和免疫组织化学检测,明确肺部肿瘤的性质,其中良性肿瘤5例,包括肺血管瘤3例,肺成熟性畸胎瘤1例,肺透明细胞糖原瘤1例;恶性肿瘤25例,包括上皮来源的肺肉瘤样癌13例,间叶来源的肺肉瘤7例,淋巴系统来源3例,杂类肿瘤2例。TNM分期为Ⅰb期4例,Ⅱa期5例,Ⅱb期8例,Ⅲa期6例,Ⅳ期2例。25例恶性肿瘤中,18例接受术后化疗。5例肺部良性肿瘤患者术后获得了较好的生活质量及良好的预后。25例恶性肿瘤患者中,18例接受4~6个化疗周期,其中采用吉西他滨联合顺铂10例,紫杉醇联合顺铂5例,环磷酰胺联合多柔比星2例,顺铂联合恩度1例。随访5年,25例恶性肿瘤患者1年生存率为64%,3年生存率为36%,5年生存率为16%。结论原发性肺部少见肿瘤的临床表现缺乏特异性,易被误诊,外科综合治疗是其首选的治疗方式,而肿瘤恶性程度是影响术后预后的重要因素。
Objective To investigate the clinical features and treatment of rare primary lung cancer. Methods Thirty patients with primary rare lung cancer who were admitted to Fujian Provincial Hospital from January 2008 to January 2012 were retrospectively analyzed. The location, size, infiltration and location of the tumor with the mediastinal lymph nodes Relationship, to determine the possibility of surgical resection, according to individual circumstances for thoracic surgery or thoracoscopic surgery, some patients with malignant tumor after chemotherapy. The clinical prognosis of patients was analyzed and their 1, 3, 5 year survival rates were calculated. Results Thoracotomy in 19 cases, thoracoscopic surgery in 11 cases; pneumonectomy in 3 cases, lobectomy in 21 cases, pulmonary wedge resection in 6 cases, no adverse events during operation. The patients were routinely pathologically diagnosed and immunohistochemically detected to determine the nature of the lung tumor. Among them, 5 were benign tumors including 3 pulmonary hemangiomas, 1 lung mature teratoma, 1 lung clear cell glycoma ; 25 cases of malignant tumors, including epithelial-derived pulmonary sarcomatoid carcinoma in 13 cases, mesenchymal sarcoma in 7 cases, lymphatic system in 3 cases, 2 cases of miscellaneous tumors. TNM stage Ⅰb 4 cases, Ⅱ a 5 cases, Ⅱ b 8 cases, Ⅲ a 6 cases, Ⅳ 2 cases. Of 25 malignant tumors, 18 received postoperative chemotherapy. Five patients with benign lung tumors achieved better quality of life and good prognosis after operation. Of the 25 patients with malignant tumors, 18 received 4 to 6 cycles of chemotherapy, including 10 gemcitabine plus cisplatin, 5 paclitaxel plus cisplatin, 2 cyclophosphamide plus doxorubicin, example. The follow-up of 5 years, 25 cases of malignant tumor patients one-year survival rate was 64%, 3-year survival rate was 36%, 5-year survival rate was 16%. Conclusions The clinical manifestations of rare primary lung cancer are not specific and easily misdiagnosed. Comprehensive surgical treatment is the first choice of treatment and the malignant degree of the tumor is an important factor affecting the prognosis.