Fu Jun, Guo Zheng, Wang Zhen, Li Xiang-dong, Li Jing, Chen Guo-jing, Wu Zhi-gang
Department of Oncologic Orthopaedics, Xijing Hospital, Xi'an 710032, China.
Zhonghua Zhong Liu Za Zhi. 2012 Dec;34(12):927-31. doi: 10.3760/cma.j.issn.0253-3766.2012.12.010.
To explore the effect of neo-adjuvant chemotherapy and computer-assisted surgery on children and adolescents with primary pelvic Ewing's sarcoma, and assess the therapeutic effect on the pelvic skeletal growth and development.
This is a retrospective analysis of 10 children with primary pelvic Ewing's sarcoma treated between Jan 2001 and Oct 2010 at the Department of Oncologic Orthopaedics at Xijing Hospital. There were 3 girls and 7 boys in the age of 7 to 16 years (average 12.7 years). All patients were pathologically diagnosed as Ewing's sarcoma. There were two cases in the sacroiliac joint, one in the ilium, one in the pubic bone, and 6 cases in peri-acetabular area including 5 below the triradiate cartilage and one above the triradiate cartilage, without cartilage invasion. All patients underwent neo-adjuvant chemotherapy, resection and reconstruction surgery and postoperative chemotherapy. CDP, ADM and IFO regimen chemotherapy were given as the main treatment. Five cases were treated by traditional resection and reconstruction, and after 2008, five cases were treated by computer-assisted surgery. During the reconstruction, the hip rotation center was put at a depressed location. All of the 10 cases underwent postoperative radiotherapy in a dose of 45-55 Gy.
All patients were followed-up for 12-72 months (mean: 37.8 months). One child had tumor recurrence and lung metastasis and 9 patients had no evidence of disease (NED). After neo-adjuvant chemotherapy, the oncologic statuses (RECIST) were: 1 CR, 8 PR and 1 SD. The functional recoveries after surgery (Enneking's) were: 4 cases excellent, 4 good, 1 fair and 1 poor. Five cases who underwent computer-assisted surgery achieved a good reconstruction without local recurrence. There were no effects on skeletal growth in 8 cases. An unbalanced hip rotational center occurred in one case, and a compemsatory scoliosis was found in another case. There were no serious complications in all patients.
The comprehensive treatment including neo-adjuvant chemotherapy, resection-reconstruction surgery and postoperative chemoradiotherapy may give a good control to primary pelvic Ewing's sarcomas in children and adolescents. The computer-assisted surgery used for accurate tumor resection and pelvic reconstruction is a good alternative when treating young patients with malignant pelvic tumors. The triradiate cartilage in children's acetabulum could be a natural barrier resistant to the invasion of Ewing's sarcomas.
探讨新辅助化疗及计算机辅助手术对儿童及青少年原发性骨盆尤文肉瘤的治疗效果，并评估其对骨盆骨骼生长发育的影响。
对2001年1月至2010年10月在西京医院骨肿瘤科接受治疗的10例原发性骨盆尤文肉瘤患儿进行回顾性分析。其中女孩3例，男孩7例，年龄7至16岁（平均12.7岁）。所有患者均经病理诊断为尤文肉瘤。骶髂关节2例，髂骨1例，耻骨1例，髋臼周围6例（其中5例在髋臼三联软骨下方，1例在髋臼三联软骨上方），均无软骨侵犯。所有患者均接受新辅助化疗、手术切除及重建手术和术后化疗。以环磷酰胺、多柔比星和异环磷酰胺方案化疗为主。5例采用传统切除重建手术，2008年后5例采用计算机辅助手术。重建时，将髋关节旋转中心置于较低位置。10例患者术后均接受45 - 55 Gy的放疗。
所有患者随访12至72个月（平均37.8个月）。1例患儿出现肿瘤复发及肺转移，9例无疾病证据（NED）。新辅助化疗后，肿瘤情况（RECIST）为：1例完全缓解（CR），8例部分缓解（PR），1例疾病稳定（SD）。术后功能恢复情况（Enneking标准）为：优4例，良4例，可1例，差1例。5例接受计算机辅助手术的患者重建效果良好，无局部复发。8例对骨骼生长无影响。1例出现髋关节旋转中心不平衡，另1例发现代偿性脊柱侧弯。所有患者均无严重并发症。
新辅助化疗、切除重建手术及术后放化疗的综合治疗可较好地控制儿童及青少年原发性骨盆尤文肉瘤。计算机辅助手术用于精确肿瘤切除及骨盆重建，是治疗年轻恶性骨盆肿瘤患者的良好选择。儿童髋臼的三联软骨可能是抵抗尤文肉瘤侵犯的天然屏障。
