脊柱原发尤文家族肿瘤的治疗及预后

The treatment and prognosis of primary spinal Ewing′s sarcoma family tumor

中文关键词:  脊柱  尤文肉瘤  原始神经外胚瘤  治疗方式

英文关键词:Spine  Ewing′s sarcoma  PNET  Treatment strategy

中文摘要:

  【摘要】 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。

英文摘要:

  【Abstract】 Objectives: To investigate the clinical features, treatment and prognosis of patient with primary spinal Ewing′s sarcoma family tumor(ESFT). Methods: 28 patients were retrospectively investigated between May 1997 and June 2010, 18 patients(17 males and 11 females) with Ewing′s sarcoma and 10 patients with peripheral malignant neuroectodermal tumor(PNET). The age of patients ranged from 5 to 45 years with an average of 19 years. The tumor involved cervical vertebrae(4 cases), thoracic vertebrae(5 cases), lumbar vertebrae(6 cases)， thoracic-lumbar segments(1 case) and sacral vertebrae(12 cases). The treatment included surgery, chemotherapy and/or radiotherapy. A five-drug regimen of chemotherapy(vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide) was administered to all patients. Surgery was performed on 27 patients, surgery followed by radiotherapy on 22. 7 cases underwent en bloc surgery.　　Results: The follow-up period was from 3 to 12 years with an average of 6.7 years. One patient died of tumor progression during chemotherapy. Twenty patients(20/23, 86.9%) obtained satisfied outcomes with nerurofunction improved for 1-2 Frankel grade. Nine(33.3%) patients developed isolated recurrence and 10(37.1%) patients developed lung metastasis. Only 3 patients developed multiple bone metastasis. The treatment after relapse included administration of second line chemotherapy(13 cases) and combined surgery(6 cases). 11 patients died at final follow-up. The 2-year and 5-year survival rate was 74.3% and 53.3%, respectively. Conclusions: The spinal Ewing′s sarcoma family tumor is a highly malignant tumor with poor prognosis. The local control and chemotherapy is necessary for improving the prognosis of the ESFT. Surgery plays an important role especially for the cases in which the tumor can be resected. En bloc resection is useful to decrease the recurrence. Radiotherapy is applicable for the patient whose tumor can not be resected.