Chordoma: Natural history and results in 28 patients treated at a single institution
Chordoma: Natural history and results in 28 patients treated at a single institution
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DOI：
10.1245/aso.2003.06.002
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发表时间：
2003-04-01
影响因子：
3.7
通讯作者：
Santinami, M
中科院分区：
文献类型：
--
作者：
Baratti, D;Gronchi, A;Santinami, M
背景：脊索瘤的自然病史特点是失败率高，功能预后差。本研究的目的是回顾我们机构经验的长期结果。方法：回顾性分析56例脊索瘤患者的临床特点、治疗方式、病理评价及随访。结果：本中心于1933年1月至2000年12月间治疗了50例骶骨脊索瘤和6例脊索瘤（中位尺寸为13 cm，范围2-30 cm）。本研究以1977年以后手术的28例骶尾脊索瘤患者为基础。手术切缘宽11例，边缘13例，局部内4例。中位随访时间为71个月（15-200个月）。17例患者复发。10个病人死于疾病。9名患者持续无病。估计的5年和10年总生存率分别为87.8%和48.9%；无病生存率分别为60.6%和24.2%。考虑对边缘和局部切除进行放射治疗。结论：骶尾部脊索瘤的高位截肢术可获得较好的宽切缘切除率。辅助放疗可以抵消边缘切除对预后的负面影响。
Background: The natural history of chordoma is characterized by a high failure rate and a poor functional outcome. The purpose of this study was to review the long-term outcome of our institutional experience.Methods: The clinical features, type of treatment, pathologic assessment, and follow-up of 56 consecutive patients with chordoma were reviewed.Results: Fifty sacral and six mobile spine chordomas (median size, 13 cm; range, 2-30 cm) were treated at our center between January 1933 and December 2000. Twenty-eight patients affected by sacrococcygeal chordoma and operated on after 1977 form the basis of our study. Surgical margins were rated as wide in 11 cases, marginal in 13 cases, and intralesional in 4 cases. The median follow-up was 71 months (range, 15-200 months). Seventeen patients' disease recurred. Ten patients died as a result of disease. Nine patients remained continuously free of disease. The estimated 5- and 10-year overall survival was, respectively, 87.8% and 48.9%; disease-free survival was 60.6% and 24.2%. Radiotherapy was considered for marginal and intralesional resections.Conclusions: High sacral amputation can achieve a good rate of wide-margin resections for sacrococcygeal chordomas. Adjuvant radiotherapy may offset the negative effect in the prognosis of marginal resections.