Hulen Christopher A, Temple H Thomas, Fox William P, Sama Andrew A, Green Barth A, Eismont Frank J
Department of Orthopaedic and Rehabilitation, University of Miami School of Medicine, Miami, FL 33101, USA.
J Bone Joint Surg Am. 2006 Jul;88(7):1532-9. doi: 10.2106/JBJS.D.02533.
Sacral chordoma is a rare, low to intermediate-grade tumor that poses substantial challenges in terms of timely diagnosis and adequate treatment. Few studies have examined the oncologic and functional outcomes of patients treated for sacral chordoma.
The clinical records of sixteen patients who had undergone sacrectomy for chordoma between 1985 and 2001 were evaluated retrospectively. All patients underwent resection by means of a sequential combined anterior and posterior approach. Patients were followed clinically at six-month intervals following recovery from the index surgical procedure. The disease onset, treatment, hospital stay, recurrence rates, survival, adjuvant therapy, functional outcome measures, and complications were evaluated.
The average age at the time of diagnosis was sixty-one years. The mean tumor size was 15.2 cm in diameter, and all patients had a resection involving S1 or S2. The mean duration of follow-up was sixty-six months, and the tumor recurred in twelve of the sixteen patients. The mean time to metastasis was fifty months. Four patients were clinically disease-free at a mean follow-up of 94.5 months, while five patients died as a result of progressive local or metastatic disease at a mean follow-up of 31.4 months. Only one patient had normal bowel and bladder control postoperatively, and only three were able to walk without assistive devices. Eight patients had wound complications, and one patient had a deep-vein thrombosis. With the numbers available, neither negative margins at the time of initial tumor resection nor adjuvant radiation therapy had a significant impact on survival or local recurrence. More cephalad levels of resection were associated with significantly worse bowel (p = 0.01) and bladder (p = 0.01) control. Complications were frequent and were more common with a larger tumor size at the time of presentation (p = 0.034).
The treatment of sacral chordoma is an arduous clinical undertaking that requires a multidisciplinary approach and attention to detail from the outset. Despite aggressive well-planned surgical management and adherence to strict surveillance protocols, frequent recurrence and the late onset of metastatic disease are to be expected in a substantial proportion of patients, especially those with a very large chordoma or one at a more cephalad level. Adequate surgical treatment results in substantial functional impairment and numerous complications; however, it does offer the possibility of long-term disease-free survival. We advocate an attempt at complete resection, when there is still a possibility of cure, and aggressive treatment of local recurrences.
Therapeutic Level IV. See Instructions to Authors for a complete description of levels of evidence.
骶骨脊索瘤是一种罕见的低至中级别的肿瘤,在及时诊断和充分治疗方面面临重大挑战。很少有研究探讨骶骨脊索瘤患者的肿瘤学和功能结局。
回顾性评估了1985年至2001年间因脊索瘤接受骶骨切除术的16例患者的临床记录。所有患者均采用前后联合序贯入路进行切除。患者在首次手术恢复后每隔6个月进行一次临床随访。评估疾病的发病情况、治疗、住院时间、复发率、生存率、辅助治疗、功能结局指标和并发症。
诊断时的平均年龄为61岁。肿瘤平均直径为15.2厘米,所有患者的切除均涉及S1或S2。平均随访时间为66个月,16例患者中有12例肿瘤复发。转移的平均时间为50个月。4例患者在平均94.5个月的随访中临床无病,而5例患者在平均31.4个月的随访中因局部或转移性疾病进展而死亡。只有1例患者术后肠道和膀胱控制正常,只有3例患者能够在无辅助设备的情况下行走。8例患者出现伤口并发症,1例患者发生深静脉血栓形成。就现有数据而言,初始肿瘤切除时的切缘阴性和辅助放疗对生存率或局部复发均无显著影响。切除平面越高,肠道(p = 0.01)和膀胱(p = 0.01)控制越差。并发症很常见,就诊时肿瘤越大越常见(p = 0.034)。
骶骨脊索瘤的治疗是一项艰巨的临床任务,从一开始就需要多学科方法并注重细节。尽管进行了积极且精心规划的手术管理并严格遵守监测方案,但预计相当一部分患者,尤其是那些脊索瘤非常大或位于较高平面的患者,会频繁复发且出现转移性疾病的晚期发作。充分的手术治疗会导致严重的功能损害和众多并发症;然而,它确实提供了长期无病生存的可能性。我们主张在仍有可能治愈时尝试进行完整切除,并积极治疗局部复发。
治疗性IV级。有关证据水平的完整描述,请参阅作者指南。