Surgical management of primary and metastatic sarcoma of the mobile spine

OBJECT: Sarcomas of the spine are a challenging problem due to their frequent and extensive involvement of multiple spinal segments and high recurrence rates. Gross-total resection to negative margins, with preservation of neurological function and palliation of pain, is the surgical goal and may be achieved using either intralesional resection or en bloc excision. The authors report outcomes of surgery for primary and metastatic sarcomas of the mobile spine in a large patient series. METHODS: A retrospective review of patients undergoing resection for sarcomas of the mobile spine between 1993 and 2005 was undertaken. Sarcomas were classified by histology study results and as either primary or metastatic. Details of the surgical approach, levels of involvement, and operative complications were recorded. Outcome measures included neurological function, palliation of pain, local recurrence, and overall survival. RESULTS: Eighty patients underwent 110 resections of either primary or metastatic sarcomas of the mobile spine. Twenty-nine lesions were primary sarcomas (36%) and 51 were metastatic sarcomas (64%). Intralesional resections were performed in 98 surgeries (89%) and en bloc resections were performed in 12 (11%). Median survival from surgery for all patients was 20.6 months. Median survival for patients with a primary sarcoma of the spine was 40.2 months and was 17.3 months for patients with a metastatic sarcoma. Predictors of improved survival included a chondrosarcoma histological type and a better preoperative functional status, whereas osteosarcoma and a high-grade tumor were negative influences on survival. Multivariate analysis showed that only a high-grade tumor was an independent predictor of shorter overall survival. American Spinal Injury Association scale grades were maintained or improved in 97% of patients postoperatively, and there was a significant decrease in pain scores postoperatively. No significant differences in survival or local recurrence rates between intralesional or en bloc resections for either primary or metastatic spine sarcomas were found. CONCLUSIONS: Surgery for primary or metastatic sarcoma of the spine is associated with an improvement in neurological function and palliation of pain. The results of this study show a significant difference in patient survival for primary versus metastatic spine sarcomas. The results do not show a statistically significant benefit in survival or local recurrence rates for en bloc versus intralesional resections for either metastatic or primary sarcomas of the spine, but this may be due to the small number of patients undergoing en bloc resections.     

“en-bloc” vertebrectomy in the mobile lumbar spine

BACKGROUND Primary tumors of the vertebral bodies have previously been treated with total or subtotal excision in a piecemeal fashion (intralesional excision). Radiation therapy has been used to help control tumor growth. Recurrence rates with an intralesional, piecemeal removal of vertebral tumors have been unacceptably high. This study describes a method to excise a lumbar vertebra “en-bloc,” and in the process, to perform a marginal (extralesional) resection of a primary tumor of the mobile lumbar spine that allows for a potential surgical cure.

METHODS A combined posterior-anterior procedure allows for an extralesional, marginal resection of the tumor and the involved vertebra. All posterior bony elements, including the pedicles and the adjacent intervertebral discs, are removed via a posterior approach. An anterior, retroperitoneal approach is then used to remove the vertebral body/tumor as a single specimen. The nerve roots at the involved levels are spared and the spine is instrumented and fused both posteriorly and anteriorly.

RESULTS Three patients successfully had combined posterior-anterior resections of lumbar vertebral chordomas. No permanent neurological complications occurred. Overall morbidity of the procedure was acceptable. At 31-month follow-up, no tumor recurrence has been detected.

CONCLUSIONS “En-bloc” resection of a primary vertebral tumor of the lumbar spine is technically demanding, but potentially curative. The alternative approaches—intralesional excision, radiation therapy, or a combination—are unable to cure these tumors. Long-term, 10-year follow-up will be necessary to confirm whether this en-bloc approach provides a surgical cure.     

Relationship between surgical margins and local recurrence in sarcomas of the spine

The combination of improved resection, stabilization, and fusion techniques allows for more aggressive removal of malignant spinal tumors with acceptable mortality and morbidity. Thirty consecutive patients with primary sarcomas of the mobile spine, who were operated on at the authors' institution from January 1970 to December 2000, were included in the current study. Demographic information, tumor location, type of resection, resection margins, local recurrence, and overall survival data were retrieved and analyzed. Treatment consisted of en bloc resection in 12 patients (40%) and piecemeal resections in 18 patients (60%). The resection was classified as wide in seven patients (23.3%), marginal in three patients (10%), and intralesional in 20 patients (66.7%). Pathology reports showed tumor-free resection margins in 12 patients (40%). In the remaining 18 patients (60%), resection margins were positive and resulted in a fivefold increase in the risk of a local recurrence. Ninety-two percent of the patients with local recurrence died of sequelae associated with the local recurrence. Primary sarcomas of the mobile spine in certain cases, can be removed completely with tumor-free resection margins. En bloc resection with tumor-free margins provides substantial improvement in overall survival.     

后路一期整块全脊椎切除治疗胸腰椎肿瘤

目的:总结后路一期整块全脊椎切除的手术经验、并发症和对神经功能的影响。方法:2006年8月至2007年9月对9例脊柱肿瘤患者进行后路一期整块全脊椎切除术,病理诊断包括4例骨巨细胞瘤,1例尤文肉瘤,1例孤立性浆细胞瘤,3例孤立性骨转移癌。8例患者肿瘤位于胸椎,1例位于腰椎。4例患者术前接受了病椎的选择性节段动脉栓塞。所有病例术前均根据Tomita脊柱肿瘤外科分期进行评估,2例患者为间室内,7例为间室外。对切除的肿瘤标本进行外科边界检查。采用Frankel分级对神经功能进行评价。结果:平均术中失血量5800ml,平均手术时间291min。2例患者获得广泛边界,其他为边缘性边界。1例术后血肿形成,1例脑脊液漏致胸腔积液,1例血肿形成合并脑脊液漏。随访14～36个月,平均25.8个月,在随访期内肿瘤无复发,2例转移癌及1例尤文肉瘤患者带瘤生存,1例转移癌死亡,4例无瘤生存。术后所有病例神经功能均获得改善或保持原有水平。结论:对具有适应证的胸腰椎肿瘤患者进行后路一期整块全脊椎切除手术可以获得满意的局部控制和功能恢复,但仍需要进一步随访并提高技术,减少合并症的发生。     

Sarcoma and the spinal column

Primary sarcomas are rare in the spine. Common primary sarcomas include osteosarcoma, Ewing's sarcoma, chondrosarcomas, and retroperitoneal soft-tissue sarcomas. These tumors tend to occur in adolescents and young adults. Surgery remains the mainstay of treatment, although the anatomy of spine and spinal cord often limits complete surgical resection with a wide margin. Chemotherapy and radiation therapy have variable effects on these tumors. With recent advances in surgical techniques and development of new chemotherapy protocols, survival and local control are both improving.     

Tumors of the spine

Spine tumors comprise a small percentage of reasons for back pain and other symptoms originating in the spine.The majority of the tumors involving the spinal column are metastases of visceral organ cancers which are mostly seen in older patients.Primary musculoskeletal system sarcomas involving the spinal column are rare.Benign tumors and tumor-like lesions of the musculoskeletal system are mostly seen in young patients and often cause instability and canal compromise.Optimal diagnosis and treatment of spine tumors require a multidisciplinary approach and thorough knowledge of both spine surgery and musculoskeletal tumor surgery.Either primary or metastatic tumors involving the spine are demanding problems in terms of diagnosis and treatment.Spinal instability and neurological compromise are the main and critical problems in patients with tumors of the spinal column.In the past,only a few treatment options aiming short-term control were available for treatment of primary and metastatic spine tumors.Spine surgeons adapted their approach for spine tumors according to orthopaedic oncologic principles in the last 20 years.Advances in imaging,surgical techniques and implant technology resulted in better diagnosis and surgical treatment options,especially for primary tumors.Also,modern chemotherapy drugs and regimens with new radiotherapy and radiosurgery options caused moderate to long-term local and systemic control for even primary sarcomas involving the spinal column.     

Treatment of an aneurysmal bone cyst of the lumbar spine in children and teenagers, about five cases

The aneurysmal bone cyst is a rare tumor. Its treatment is complex when localized to the lumbar spine, with neurological, mechanical, and tumoral complications. The aim of this study is to describe these tumors, their treatment, and their long-term evolution, as well as to define an appropriate therapeutic strategy. Four of the five cysts had anterior and posterior extension. Three patients had neurological symptoms at diagnosis and two of them presented with pathological fracture. Surgical treatment was performed by intralesional resection. Long-term progress was always favorable, without recurrence or functional limitation. Two patients had a stable, mild spine deformity.     

Tumoren und Metastasen an der Wirbelsäule

Metastases in the spine make up the largest proportion of the total number of spinal tumors. Primary bone tumors are very rare and represent only a marginal proportion of all primary malignant tumors. Tumors such as breast, renal cell, prostate and lung cancer are most frequently described, with the peak age for the occurrence of spinal metastases between the ages of 40–65 years. They are mostly found in the thoracic, followed by the lumbar spine and most rarely in the cervical spine. Pain is usually the first symptom described by tumor patients caused by instabilities with osteolysis, pathological fractures, and neurological function failures. Pain reduction, stability and avoiding progressive neurological deficits must be the aim of surgical therapy in patients with advanced tumor growth.     

Spinal tumors in coexisting degenerative spine disease—a differential diagnostic problem

The clinical presentation of spinal tumors is known to vary, in many instances causing a delay in diagnosis and treatment, especially with benign tumors. Neck or back pain and sciatica, with or without neurological deficits, are mostly caused by degenerative spine and disc disease. Spinal tumors are rare, and the possibility of concurrent signs of degenerative changes in the spine is high. We report a series of ten patients who were unsuccessfully treated for degenerative spine disease. They were subsequently referred for operative treatment to our department, where an initial diagnosis of a tumor was made. Two patients had already been operated on for disc herniations, but without long-lasting effects. In eight patients the diagnosis of a tumor was made preoperatively. In two cases the tumor was found intraoperatively. All patients showed radiological signs of coexisting degenerative spine disease, making diagnosis difficult. MRI was the most helpful tool for diagnosing the tumors. A frequent symptom was back pain in the recumbent position. Other typical settings that should raise suspicion are persistent pain after disc surgery and neurological signs inconsistent with the level of noted degenerative disease. Tumor extirpation was successful in treating the main complaints in all but one patient. There was an incidence of 0.5% of patients in which a spinal tumor was responsible for symptoms thought to be of degenerative origin. However, this corresponds to 28.6% of all spine-tumor patients in this series. MRI should be widely used to exclude a tumor above the level of degenerative pathology.     

En bloc spondylectomy in malignant tumors of the spine

En bloc spondylectomy is a technique that enables wide or marginal resection of malignant lesions of the spine. Both all posterior techniques as well as combined approaches are reported. Aim of the present study was to analyse the results of 21 patients with malignant lesions of the spine, all treated with en bloc excision in a combined posteroanterior (n = 19) or all posterior approach (n = 2). Twenty-one consecutive patients, operated between 1997 and 2005, were included into this retrospective study. Thirteen patients had primary malignant lesions, eight patients had solitary metastases, all located in the thoracolumbar spine. There were 16 single level, three two-level, one three-level and one four-level spondylectomy. The patients were followed clinically and radiographically (including CT studies) with an average follow-up of 4 years. Out of 11 patients with primary Ewing or osteosarcoma seven patients are alive without any evidence of disease. One patient died after 5 years from other causes and three are alive with evidence of disease. Latter had either a poor histologic response to the preoperative chemotherapy (n = 2) or an intralesional resection (n = 1). All three patients with solitary spinal metastases of Ewing or osteosarcoma died of the disease. Five patients with solitary metastases of mainly hypernephroma are alive. In total, six resections were intralesional, mainly due to large intraspinal tumor masses, with two patients having had previous surgery. In the remaining cases, wide (n = 10) or marginal (n = 5) resection was accomplished. There were one pseudarthrosis requiring extension of the fusion and two cases with local recurrences and repeated excisional surgery. At follow-up CT studies, all cages were fused. Health related quality of life analysis (SF-36) revealed only slightly decreased physical component and normal mental component scores compared to normals in those patients with no evidence of disease. En bloc spondylectomy enables wide or marginal resection of malignant lesions of the spine in most cases with acceptable morbidity. Intralesional resection, poor histologic response, and solitary spinal metastases of Ewing and osteosarcoma are associated with a poor prognosis.     

Is radiotherapy important for low-grade soft tissue sarcoma of the extremity?

Radiotherapy and limb-preserving surgery has replaced amputation and compartmental resection for treatment of soft tissue sarcomas. However, the role of radiotherapy in low-grade tumors remains unclear. This study reviews the outcomes of 132 patients who received multimodality treatment for low-grade soft tissue sarcoma. Large primary tumors (> 5 cm) and the absence of radiotherapy correlated with local recurrence. Radiotherapy was most effective in patients operated on with marginal margins. Patients who were treated with wide surgical margins or had small tumors (< or =5 cm) showed no benefit with adjuvant radiotherapy. Size greater than 5 cm and local recurrence correlated with metastasis. Radiotherapy appears to be important in the management of low-grade soft tissue sarcoma. The principles of local treatment for low-grade soft tissue sarcoma should be the same as for high-grade tumors with a combination of surgery and adjuvant radiotherapy. In a subset of patients with small and widely excised tumors, consideration may be given to withholding radiotherapy. Local recurrence and metastasis from low-grade soft tissue sarcoma may occur as long as 1 decade after primary tumor resection. Long-term review of patients with low-grade tumors may be indicated.     

En-bloc-Spondylektomie und Defektrekonstruktion bei Primärtumoren und Solitärmetastasen der Wirbelsäule

In primary tumors of the spine and, with limitations, solitary metastasis, the surgical approach should aim for curative treatment of the disease. Because the prognosis of malignant bone tumors is extremely limited, if an intralesional approach is performed, an extralesional en bloc resection is the treatment of choice. Therefore, it is mandatory to use an appropriate staging system. For the spine, the WBB staging system has been approved, which transfers the principles of the Enneking classification for treating primary malignant tumors of the limb to the spine. After en bloc spondylectomy, rigid and primary stable instrumented dorsoventral reconstruction must be performed - posteriorly with a dual-rod system using pedicle screws, and anteriorly in the ideal case by means of a vertebral body replacement cage. The possibility of extralesional (wide or marginal) resection of spinal tumors depends on tumor size and location. Extralesional resection and, if indicated, other neoadjuvant, adjuvant, or local therapeutic modalities have a strong positive influence on long-term survival rates. A good prognosis for primary tumors is associated with a good response to chemotherapy and extralesional resection. Solitary metastases have a much worse quod vitam prognosis. Therefore, local control of the disease in en bloc resections of solitary metastasis is a second relevant goal, although curative treatment is the primary aim.     

骨盆和骶骨骨巨细胞瘤的治疗策略

目的 探讨骨盆和骶骨骨巨细胞瘤(GCT)外科治疗的手术方法、局部复发率、并发症.方法 1997年12月至2005年12月我院共收治骶骨、骨盆GCT 46例,其中男性25例,女性21例.年龄17～64岁,平均32岁.骶骨GCT 24例、骨盆GCT 22例.骨盆Ⅰ区GCT(髂骨)8例,Ⅱ区(髋臼)10例,Ⅲ区(坐骨耻骨)4例.S1-5 GCT 2例,S1-4 4例,S1-3 12例,S1-2 5例,S3-5 1例.手术方法:行3次手术者2例,行2次手术者7例.骶骨GCT患者的治疗方式包括19例患者进行了病灶内边缘切除术,2例患者同时进行了病灶内边缘切除术和放疗,3例患者进行了广泛边缘切除术.骨盆22例GCT患者中,除2例坐骨及1例髂骨GCT患者采用刮除术外,其余19例均行大块切除术.结果 1例患者在外院手术后复发,肿瘤巨大、表面溃烂,再次手术后2周死于严重感染.余45例患者,随访时间12个月至8年,平均随访时间37个月.2例患者1年后死亡.1例骶骨GCT患者术后2年出现肺转移,化疗1周期,随访1年,肺部病灶无明显增大.1例髋臼部GCT患者刮除术后2年出现肺转移、局部复发,行肺部照射、髋臼部肿瘤广泛切除、人工半骨盆置换术,肺部病灶随访1年,控制良好.局部复发:骶骨:9/24(37.5%),其中复发2次的患者2例,复发1次的患者7例;骨盆:2/22(9.1%),2例坐骨GCT刮除后均局部复发;行大块切除的19例GCT均未复发.结论 对于骶骨GCT,由于刮除术后局部复发率高,治疗应该更具侵袭性.肿瘤广泛边缘切除术可能会引起骶神经损害,但由于局部复发率降低,所以仍为治疗的最佳选择.     

Combined posterior pelvis and lumbar spine resection for sarcoma

The oncologic outcome in the treatment of pelvic sarcomas is not comparable with that in the extremity. Particular problems arise when the tumor involves the posterior pelvis and the sacrum or even the lower lumbar spine. Because of the difficulty in achieving local control and the major functional deficits after extensive surgery, some authors suggest conservative, nonsurgical treatment for these patients. The purpose of the current study was to analyze the oncologic outcome of patients who were treated for a pelvic sarcoma necessitating resection of the ilium, part of the sacrum and part of the lower spine, which is defined as extended hemipelvectomy. Between 1979 and 1999, 11 males and seven females with a mean age of 34.5 years (range, 14-67 years) had an extended hemipelvectomy for a sarcoma of the posterior pelvis. The tumors included seven osteogenic sarcomas, six chondrosarcomas, and five fibrosarcomas: 13 were classified as high-grade lesions. The mean size of the tumor was 11 x 9 x 6 cm. In 11 patients, a hemivertebrectomy of L5 and in seven patients of L4 combined with L5 was done to achieve an adequate margin. In four patients, the resection was followed by reconstruction with a vascularized fibula or autograft. At a mean followup of 56 months (range, 3-220 months), 12 of 18 patients were alive and without disease. Six patients died; four of these patients had metastasis to the lung at presentation. The two remaining patients died of complications of adjuvant treatment. The authors conclude from this small series of patients that an aggressive surgical resection for localized, nonmetastatic, high-grade sarcomas of the posterior pelvis may be justified to provide local control and improve survival.     

Postradiation sarcoma involving the spine

Postradiation sarcomas arising many years after treatment of cancer are long term sequelae of therapy. We describe the clinical features, radiographic findings, and results of treatment in 13 patients with such sarcomas encountered over a 6-year period. Of these patients, 9 had bone sarcomas and the remaining 4 had paraspinal tumors arising from adjacent soft tissue and nerve. The primary cancer for which radiation was given included Hodgkin's disease (4 patients), breast cancer (2 patients), cervix cancer (2 patients), and a variety of others (5 patients). The latent interval to the occurrence of the second neoplasm varied from 6 to 30 years (median, 10 years) after treatment of the original tumor. Despite aggressive treatment, the overall prognosis was poor. The median survival was 8 months, with only 3 surviving more than 2 years. Although rare, postradiation sarcoma should be considered in the differential diagnosis of patients presenting with late onset of spinal pain or neurological symptoms after clinical remission of an original cancer.     

Giant cell tumor of the spine

Six patients with giant cell tumor of the spine had surgery between 1981 and 1995. Three lesions were located in the scrum, two lesions were in the thoracic spine, and one lesion was in the lumbar spine. Preoperatively, all patients had local pain and neurologic symptoms. Two patients had cement implanted after curettage or intralesional excision of the sacral tumor; one patient had a local relapse. After the second curettage and cement implantation, the tumor was controlled. One patient with a sacral lesion had marginal excision and spondylodesis; no relapse developed. Two patients with thoracic lesions had planned marginal excision and spondylodesis; the margins finally became intralesional, but no relapse developed. One patient with a lumbar lesion had incomplete removal of the tumor and received postoperative irradiation. At the final followup (median, 69 months), five of six patients were disease-free and one patient died of disease progression. Two of the five surviving patients had pain after standing or neurologic problems. Although some contamination occurred, planning a marginal excision of the lesion seems beneficial for vertebral lesions above the sacrum. Total sacrectomy of a sacral lesion seems to be too invasive when cement implantation can control the lesion.     

Pediatric spine tumors with spinal cord compromise.

Twenty-nine pediatric spine tumor patients with neurologic deficit were reviewed retrospectively to evaluate tumor control, neurologic course, spinal deformity, and response to treatment. Diagnoses included 10 neuroblastomas, nine sarcomas, eight astrocytomas, and two lymphomas. Mean duration of symptoms at presentation was 8 weeks. Mean clinical follow-up was 7 years 4 months, with patient survival of 66% and a local recurrence rate of 34%. Twenty-four percent (seven of 29) of patients recovered minimal neurologic function with treatment, 76% (22 of 29) showed significant recovery, and two thirds (20 of 29) developed spinal deformity. The initial clinical response to chemotherapy appeared to be most pronounced in sarcoma patients (66% positive response).     

Synovial sarcoma: the importance of size and location for survival

A retrospective analysis was done on 135 consecutive patients with extremity and truncal synovial sarcomas seen at our institutions between 1961 and 1996. Ninety-nine patients (73%) presented with a newly diagnosed localized primary sarcoma, nine patients (7%) presented with a locally recurrent sarcoma previously treated at another institution, and 27 patients (20%) presented with metastatic disease. The median followup for surviving patients was 78 months (range, 20-420 months). The overall survival for patients with localized primary and locally recurrent synovial sarcoma was 51% at 10 years. Patients with localized synovial sarcomas less than 5 cm in longest diameter had a survival at 10 years of 88% compared with a 10-year survival of 38% and 8% for patients with sarcomas 5 to 10 cm and greater than 10 cm in longest diameter, respectively. Distal location (hands and feet) and histologic grade also were statistically significant predictors of patient survival for localized synovial sarcoma on univariate analysis. On multivariate analysis size greater than 10 cm and size 5 to 10 cm were associated with an 18-fold and a 3.1-fold increased risk of death compared with tumors smaller than 5 cm. Grade and location were not found to be independent predictors of survival on multivariate analysis. Primary tumor size and initial status at presentation were prognostic for survival and patients with synovial sarcomas larger than 5 cm or locally recurrent at presentation should be considered for more aggressive surgical and adjunctive radiotherapy or chemotherapy.     

脊柱转移瘤的外科治疗效果

目的：探讨脊柱转移瘤患者手术治疗的适应证与疗效。方法：对1998年7月至2003年7月5年间收治的167例脊柱转移瘤患者的临床资料进行回顾性分析。胸椎转移99例，腰椎转移53例．颈椎转移15例。男性96例，女性71例。出现神经系统受损者112例，其中Frankel分级A级17例．B级11例．C级22例．D级62例。患者均有不同程度的疼痛，手术根据肿瘤破坏脊椎的部位行前路椎体切除、钢板内固定术或后路病变椎板切除、肿瘤切除、椎管减压、经椎弓根内固定术。结果：167例患者中，155例（92．8％）术后疼痛得到缓解。112例有神经功能损害的患中，67例（59．8％）术后Frankel分级有不同程度的改善。随访6～48个月．术后存活1年以上的患者117例，约占患者总数的70％。结论：由于转移瘤所致椎体塌陷或不稳定造成严重的神经损害或机械性不稳脊柱痛疼的患者，手术治疗可改善其神经功能，增加脊椎稳定性．提高生存质量。