Chordoma of the mobile spine: Fifty years of experience

Chordoma of the mobile spine: Fifty years of experience
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DOI:
10.1097/01.brs.0000200038.30869.27
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发表时间:
2006-02-15
期刊:
影响因子:
3
通讯作者:
Weinstein, JN
Weinstein, JN
中科院分区:
医学2区
文献类型:
--
作者:
Boriani, S;Bandiera, S;Weinstein, JN

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研究设计。在50年间连续观察52例活动脊柱脊索瘤,包括对1991年以前治疗的15例和1991年至2002年治疗的37例的前瞻性回顾。本系列回顾了脊索瘤的流行病学问题和临床模式。我们试图随着时间的推移将肿瘤的范围、治疗和结果联系起来。脊索瘤是活动脊柱最常见的原发肿瘤。由于生长缓慢,初发症状和治疗后复发均出现较晚,难以评估治疗方案的疗效。对37例患者进行前瞻性研究,并与1954年至1991年间观察到的15例患者进行回顾性比较。在前瞻性研究中,所有患者都进行了影像检查、肿瘤学和外科分期。当整块切除不可行时,肿瘤包膜内切除联合放射治疗。对预期的患者进行临床评估和成像。回顾组的患者通过图表和可获得的图像进行评估;在这些患者中,只进行了一次整体切除(病灶内切缘)。所有幸存者都接受了临床评估和放射学检查。48名患者可进行长期随访。4例死于术后并发症,6例死于治疗后不到2年的疾病。42例随访2年以上,26例随访5年以上。所有接受单纯放疗、病灶内切除或联合治疗的患者在不到2年的时间内复发,有些患者在长期存活后死亡。肿瘤囊外切除加放射治疗复发率高(12/16,平均30个月),但3例持续无病(CDF),平均52个月,5例有病存活,平均69个月(24~146个月)。18例全切除患者中12例CDF,平均8年(48~155个月)。其余6例复发,其中1例死亡。所有这些(6)都曾接受过治疗和/或有受污染的切缘整块切除。随访超过5年的CDF的唯一治疗方案是无边界整块切除。
Study Design. A consecutive series of 52 chordomas of the mobile spine observed over a 50-year period includes a retrospective review of 15 cases treated prior to 1991 and a prospective group of 37 cases treated from 1991 to 2002.Objectives. This series reviews epidemiologic issues as well as clinical patterns of spinal chordomas. We attempt to correlate tumor extent, treatment, and outcomes over time.Summary of Background Data. Chordoma is the most frequent primary tumor of the mobile spine. Due to slow growth, both initial symptoms and recurrences after treatment arise later, making it difficult to evaluate the effectiveness of treatment protocols.Methods. A prospective series of 37 cases is compared with a retrospective group of 15 patients observed between 1954 and 1991. In the prospective study, all patients had imaging studies, and oncologic and surgical staging. When en bloc resection was not feasible, intralesional extracapsular excision was combined with radiation therapy. The prospective patients were clinically evaluated and imaged. Patients in the retrospective group were evaluated by chart and available images; of these, only one en bloc resection ( intralesional margin) was performed. Survivors were all evaluated clinically and had radiographic studies.Results. Forty-eight patients were available for longterm follow-up. Four died due to post-operative complications, and six due to disease less than 2 years after treatment. Forty-two patients were followed over 2 years; 26 patients had over 5 years follow-up. All patients having radiation alone, intralesional excision, or a combination had recurrences in less than 2 years, and died in some cases after a long survival with symptomatic disease. Intralesional extracapsular excision with radiation had a high rate of recurrence ( 12 of 16 at average 30 months), but 3 patients are continuously disease-free (CDF) at mean 52 months and 5 are alive with disease at average 69 months ( ranging 24 to 146). Twelve of 18 patients having en bloc resection are CDF at average 8 years (48 to 155 months). The remaining 6 recurred and of these 1 died. All of these ( 6) had been previously treated and/or had en bloc resections with contaminated margins.Conclusions. The only treatment protocol associated with CDF at follow-up longer than 5 years is margin-free en bloc resection.