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目的:研究脊柱原发尤文家族肿瘤的临床特点、治疗方式及预后。方法:对1997年5月~2010年6月收治的28例脊柱尤文肉瘤与原始神经外胚瘤进行回顾性分析。男17例,女11例;年龄5~45岁,平均19岁。尤文肉瘤18例,原始神经外胚瘤10例。累及颈椎4例,胸椎5例,胸腰椎多发1例,腰椎6例,骶椎12例。23例患者入院时有神经症状及体征。所有患者均行化疗,27例接受手术治疗,22例于术后接受放疗。根据肿瘤位置行前路或后路肿瘤切除、内固定手术,其中7例行整块切除手术。结果:术后随访3~12年。平均6.7年。1例胸椎后路减压术后于化疗期间因肿瘤进展、多器官功能衰竭死亡。2例术前Frankel C级和1例Frankel A级术后无改变,其余20例(86.9%)术前有不同神经症状者术后Frankel分级均改善1~2个等级。局部复发及转移共13例,包括局部复发9例(9/27,33.3%),肺转移10例(10/27,37.1%),骨转移3例(3/27,11.1%)。整块切除7例中局部复发1例(14.3%)、肺转移2例(28.6%)。转移及局部复发时间5个月~6年,局部复发及转移者全部接受二线化疗,局部复发及骨转移者6例接受再次手术。至末次随访已死亡11例,2年生存率为74.3%,5年生存率为53.3%。结论:脊柱原发尤文家族肿瘤是一类高度恶性的脊柱肿瘤,应当采取手术、放疗和化疗相结合的综合治疗模式。整块切除对于降低复发率和提高生存率有重要意义。
Objective: To study the clinical features, treatment and prognosis of the spine primary Juvenile tumors. Methods: 28 cases of spine ewing sarcoma and primitive neuroectodermal tumors admitted to our hospital from May 1997 to June 2010 were retrospectively analyzed. 17 males and 11 females; aged 5 to 45 years, mean 19 years. Ewing sarcoma in 18 cases, 10 cases of primitive neuroectodermal. 4 cases involving the cervical spine, 5 cases of thoracic spine, 1 case of thoracolumbar multiple, 6 cases of lumbar, 12 cases of sacral vertebra. Twenty-three patients had neurological symptoms and signs at admission. All patients underwent chemotherapy, 27 patients underwent surgical treatment and 22 received radiotherapy after surgery. According to the tumor location of anterior or posterior tumor resection, internal fixation surgery, of which 7 cases underwent block operation. Results: The patients were followed up for 3 to 12 years. Average 6.7 years. One case of thoracic posterior decompression after chemotherapy due to tumor progression, multiple organ failure. There were no changes in 2 cases of Frankel C and 1 case of Frankel A, and the other 20 cases (86.9%) had different preoperative neurological symptoms. Frankel classification improved by 1 or 2 grades. There were 13 cases of local recurrence and metastasis, including 9 cases (9/27, 33.3%) of local recurrence, 10 cases (10/27, 37.1%) of lung metastasis and 3 cases (3 / 27,11.1%) of bone metastasis. One patient (14.3%) had partial tumor recurrence in 7 patients, and 2 patients (28.6%) had pulmonary metastasis. Metastasis and local recurrence time of 5 months to 6 years, local recurrence and metastasis were all accepted second-line chemotherapy, local recurrence and bone metastasis in 6 patients underwent reoperation. Eleven patients died at the last follow-up. The 2-year survival rate was 74.3% and the 5-year survival rate was 53.3%. Conclusion: The spine primary Juventus tumor is a kind of highly malignant tumor of the spine. It should be combined with surgery, radiotherapy and chemotherapy. Block resection for reducing the recurrence rate and improve the survival rate of great significance.