【摘要】 目的研究骶骨原发尤文肉瘤的临床特点、治疗方式及预后。方法 1998年6月至2011年6月,对收治的15例骶骨原发尤文肉瘤进行回顾性分析。男9例,女6例,其年龄11~45岁,平均18岁。所有患者术前均有骶尾部疼痛症状,11例伴有会阴区麻木、下肢疼痛等神经症状,3例可触及局部肿块。侵及单个节段者2例,累及2个节段及以上者13例。术前x线片及CT提示骶椎多呈不规则溶骨性破坏,CT和MRI检查经常可见巨大的软组织肿块。所有患者均行系统化疗及手术治疗,10例于术后接受放疗。9例行骶骨肿瘤分块切除、重建术,6例行整块切除术,其中4例行一期前后路联合全骶骨切除重建术。结果术后随访2~10年。平均5.2年。所有患者局部疼痛均得到缓解。围手术期并发症5例,其中伤口并发症4例。11例均存在不同程度大小便功能障碍。在随访期内出现肿瘤局部复发及转移10例,局部复发9例(9/15,60%),肺转移5例(5/15,33.3%),其中包括局部复发伴肺转移4例。其中行肿瘤整块切除6例中骶骨局部复发1例,局部复发伴肺转移1例。转移及局部复发时间7个月至5年,局部复发及转移者中全部10例均接受二线治疗。至末次随访已死亡9例,2例带瘤生存。2年无瘤生存率60%,5年无瘤生存率33.3%,5年总生存率为47%。结论骶骨原发尤文肉瘤是一类高度恶性的肿瘤,必须采取手术、放疗和化疗相结合的综合治疗模式。骶骨肿瘤术后并发症较多。骶骨肿瘤整块切除术是对于骶骨原发高度恶性肿瘤的重要局部治疗方法。更多还原

【Abstract】 Objective To investigate the clinical features,treatment strategies and prognoses of primary Ewing’s sarcoma of the sacrum.Methods The data of 15 patients with primary Ewing’s sarcoma of the sacrum who were adopted from June 1998 to June 2011 were retrospectively analyzed.There were 9 males and 6 females,whose average age was 18 years old(range;11-45 years).All the patients had sacrococcygeal pain,and 11 patients had perineal numbness and pain in the lower extremities.Three patients had palpable masses.The single segment was invaded in 2 cases,and double or more segments were invaded in 13 cases.The preoperative X-ray films and CT scans indicated that there was much irregular osteolytic bone destruction in the sacral vertebra.The CT and MRI examination often showed huge soft tissue masses.The systemic chemotherapy and surgical treatment were performed on all the patients,and the radiotherapy on 10 patients postoperatively.Nine patients underwent block resection of sacral tumors and reconstruction,and 6 patients underwent en bloc resection,among whom 4 patients received one-stage total sacrectomy by a combined anterior-posterior approach and rod-screw reconstruction.Results All patients were followed up for an average period of 5.2 years(range,2-10 years).The local pain in all the patients got relieved.Five patients had perioperative complications and 4 of them had wound healing problems.Eleven patients had bladder and bowel dysfunction in varying degrees.During the follow-up,10 cases of local recurrence and metastasis were found.The local recurrence rate was 60%(9/15)and the lung metastasis rate was 33.3%(5/15),including 4 cases of both local recurrence and lung metastasis.Among the 6 patients treated by en bloc resection,1 patient had local recurrence in the sacrum and 1 patient had both local recurrence and lung metastasis.The duration of metastasis and local recurrence was 7-60 months.The 10 patients who had local recurrence and lung metastasis received the second-line chemotherapy.Nine patients died in the latest follow-up,and 2 patients survived with tumors.The 2-year and 5-year disease-free survival rate were 60% and 33.3%respectively.The 5-year overall survival rate was 47%.Coneluslons The primary Ewing’s sarcoma of the sacrum is highly malignant,for which the comprehensive therapy including the surgery,radiotherapy and chemotherapy is necessary.The en bloc resection of sacral tumors is an important local treatment method for primary malignant tumors in spite of many complications.更多还原