York J E, Kaczaraj A, Abi-Said D, Fuller G N, Skibber J M, Janjan N A, Gokaslan Z L
Department of Neurosurgery, The University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
Neurosurgery. 1999 Jan;44(1):74-9; discussion 79-80. doi: 10.1097/00006123-199901000-00041.
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.
A retrospective study was performed. From 1954 to 1994, 27 patients with sacral chordomas were evaluated at our institution.
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).
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.
骶骨脊索瘤是相对罕见的、局部侵袭性恶性肿瘤。尽管采用了手术切除、辅助放疗和化疗，但复发仍很常见。本研究回顾了我们在德克萨斯大学MD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病程的影响。
进行了一项回顾性研究。1954年至1994年，我们机构对27例骶骨脊索瘤患者进行了评估。
男性19例，女性8例，平均年龄56岁（范围27 - 80岁）。除1例患者外，所有患者均有疼痛症状，27例中有17例在初次就诊时出现自主神经功能障碍迹象。根据手术标本切缘的显微镜检查，手术方式分为根治性切除或次全切除。所有患者至少接受了一次手术，共进行了67次手术（28例根治性切除和39例次全切除）。12例患者接受了一次手术，9例接受了两次手术，6例接受了两次以上手术（范围3 - 16次手术）。67次手术中有13次联合使用了放疗。整个组的中位Kaplan-Meier总生存时间估计为7.38年（范围4个月至34年）。67次手术中有47次肿瘤复发。接受根治性切除的患者每次手术的无病间期为2.27年，而接受次全切除治疗的患者每次手术的无病间期为8个月（两条曲线不等性的对数秩检验，19.58；P<0.0001）。放疗的加入延长了接受次全切除患者的无病间期（2.12年对8个月；两条曲线不等性的对数秩检验，5.82；P<0.02）。
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，根治性切除与显著更长的无病间期相关。次全切除后加用放疗可改善无病间期，尽管放疗通常只能使用一次。基于这些发现，我们认为，只要有可能，根治性切除应是骶骨脊索瘤的首选治疗方法。
