Sacral chordoma: 40-year experience at a major cancer center

Sacral chordoma: 40-year experience at a major cancer center
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DOI:
10.1097/00006123-199901000-00041
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发表时间:
1999-01-01
期刊:
影响因子:
4.8
通讯作者:
Gokaslan, ZL
Gokaslan, ZL
中科院分区:
医学1区
文献类型:
--
作者:
York, JE;Kaczaraj, A;Gokaslan, ZL

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目的:骶骨脊索瘤是相对罕见的局部侵袭性恶性肿瘤。尽管进行了手术切除、辅助放疗和化疗,复发仍很常见。本研究回顾了我们在德克萨斯大学 M.D. 安德森癌症中心过去 40 年的经验,以确定各种治疗方法对该疾病整个过程的影响。方法:进行了一项回顾性研究。自1954年至1994年,我院对27例骶骨脊索瘤患者进行了评估。结果:其中男19例,女8例,平均年龄56岁(范围27-80岁)。除一名患者外,所有患者均出现疼痛,27 名患者中有 17 名在初次就诊时表现出自主神经功能障碍的证据。根据手术标本边缘的显微镜检查,手术程序分为根治性切除或次全切除。所有患者均接受了至少 1 次外科手术,总共 67 次手术(28 次根治性切除和 39 次次全切除)。 12 名患者接受了 1 次手术,9 名患者接受了两次手术,6 名患者接受了两次以上手术(范围为 3-16 次手术)。 67 例外科手术中有 13 例结合放射治疗。 Kaplan-Meier 对整个组总生存时间的中位估计为 7.38 年(范围,4 个月至 34 年)。 67 例手术中有 47 例术后肿瘤复发。接受根治性切除术的患者每次手术的总无病间隔为 2.27 年,而次全切除患者每次手术的总无病间隔为 8 个月(两条曲线之间不等式的对数秩检验为 19.58;P < 0.0001)。添加放射治疗延长了接受次全切除患者的无病间隔(2.12 年与 8 个月;两条曲线之间不平等的对数秩检验为 5.82;P < 0.02)。结论:我们的结果表明大多数脊索瘤患者频繁复发。与肿瘤次全切除相比,根治性切除与显着更长的无病间隔相关。尽管放射治疗通常只能使用一次,但次全切除后添加放射治疗可改善无病间隔。基于这些发现,我们认为,只要有可能,根治性切除应该是骶骨脊索瘤的首选治疗方法。
OBJECTIVE: Sacral chordomas are relatively rare, locally invasive, malignant neoplasms. Despite surgical resection, adjuvant radiation therapy, and chemotherapy, recurrence is common. This study reviews our experience during the last 40 years at The University of Texas M.D. Anderson Cancer Center, to determine the effects of various treatment methods on the overall course of this disease process.METHODS: A retrospective study was performed. From 1954 to 1994, 27 patients with sacral chordomas were evaluated at our institution.RESULTS: There were 19 male and 8 female patients, with a mean age of 56 years (range, 27-80 yr). All except one of the patients presented with pain, and 17 of 27 showed evidence of autonomic dysfunction at initial presentation. Based on microscopic examination of surgical specimen margins, surgical procedures were categorized as either radical resection or subtotal excision. All patients underwent at least one surgical procedure, for a total of 67 procedures (28 radical resections and 39 subtotal excisions). Twelve patients underwent one operation, whereas nine underwent two procedures and six underwent more than two operations (range, 3-16 operations). Radiation therapy was used in conjunction with 13 of the 67 surgical procedures. The median Kaplan-Meier estimate of the overall survival time for the entire group was 7.38 years (range, 4 mo to 34 yr). Tumors recurred after 47 of the 67 procedures. The overall disease-free interval for patients undergoing radical resection was 2.27 years for each procedure, compared with 8 months for each procedure for patients treated with subtotal excision (log-rank test for the inequality between the two curves, 19.58; P < 0.0001). The addition of radiation therapy prolonged the disease-free interval for patients undergoing subtotal resection (2.12 yr versus 8 mo; log-rank test for the inequality between the two curves, 5.82; P < 0.02).CONCLUSION: Our results suggest frequent recurrences in the majority of patients with chordomas. Radical resection is associated with a significantly longer disease-free interval, compared with subtotal removal of the tumor. Addition of radiation after subtotal resection improves the disease-free interval, although radiation therapy can generally be used only once. Based on these findings, we think that, whenever possible, radical resection should be the treatment of choice for sacral chordomas.