CAJ | 학술논문

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。
목적：연구척주원발우문가족종류적림상특점、치료방식급예후。방법：대1997년5월～2010년6월수치적28례척주우문육류여원시신경외배류진행회고성분석。남17례，녀11례；년령5～45세，평균19세。우문육류18례，원시신경외배류10례。루급경추4례，흉추5례，흉요추다발1례，요추6례，저추12례。23례환자입원시유신경증상급체정。소유환자균행화료，27례접수수술치료，22례우술후접수방료。근거종류위치행전로혹후로종류절제、내고정수술，기중7례행정괴절제수술。결과：술후수방3～12년。평균6.7년。1례흉추후로감압술후우화료기간인종류진전、다기관공능쇠갈사망。2례술전Frankel C급화1례Frankel A급술후무개변，기여20례（86.9%）술전유불동신경증상자술후Frankel분급균개선1～2개등급。국부복발급전이공13례，포괄국부복발9례（9/27，33.3%），폐전이10례（10/27，37.1%），골전이3례（3/27，11.1%）。정괴절제7례중국부복발1례（14.3％）、폐전이2례（28.6％）。전이급국부복발시간5개월～6년，국부복발급전이자전부접수이선화료，국부복발급골전이자6례접수재차수술。지말차수방이사망11례，2년생존솔위74.3%，5년생존솔위53.3%。결론：척주원발우문가족종류시일류고도악성적척주종류，응당채취수술、방료화화료상결합적종합치료모식。정괴절제대우강저복발솔화제고생존솔유중요의의。
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 verte-brae (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, cy-clophosphamide, ifosfamide and etoposide) was administered to all patients. Surgery was performed on 27 pa-tients, 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 adminis-tration of second line chemotherapy(13 cases) and combined surgery(6 cases). 11 patients died at final fol-low-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. Ra-diotherapy is applicable for the patient whose tumor can not be resected.