The role of surgery and resection margins in the treatment of Ewing's sarcoma.

Because of the enormous progress in surgery in the treatment of patients with tumors, the current study analyzed the influence of wide surgical resection margins on the outcome of patients with Ewing's sarcoma. Between 1980 and 1994, 86 patients were treated with systemic therapy and surgery (biopsy in six patients, tumor resection in 80 patients). Forty-four patients also had radiation therapy. The 5-year overall survival was 56.8% (5-year disease-free survival, 59.4%). The 5-year overall survival after radical or wide resection was 60.2% (5-year disease-free survival, 58.2%), in comparison with 40.1% (46.7%) after marginal or intralesional resection. Two patients with inadequate resection margins had local recurrences. In addition to the influence of neoadjuvant chemotherapy for higher survival rates (5-year overall survival with a good response was 80.2% versus 41.7% with a poor response), adequate surgical margins significantly affect the outcome for patients with Ewing's sarcoma.     

The role of resection in the treatment of pulmonary metastases from Ewing's sarcoma

We have investigated the role of resection in the treatment of patients with isolated pulmonary metastases from Ewing's sarcoma. In a retrospective review, 19 patients with the diagnosis of Ewing's sarcoma treated at the National Cancer Institute from 1965 to 1985 who underwent chest exploration for resection of pulmonary nodules were evaluated. Ten patients (53%) were made disease free by resection of pulmonary metastases, six patients (32%) were found to have unresectable disease, and three patients (16%) were found to have benign pulmonary disease. The actuarial 5 year survival rate of the 10 patients successfully made disease free by resection was 15%, and the median survival of this group was 28 months. In contrast, the median survival of the six patients not made disease free was 12 months, and no patient with residual disease was alive 22 months after thoracotomy (p2 = 0.0047). There were no postoperative deaths and only three minor postoperative complications for 25 operative procedures. Various prognostic variables were analyzed to determine their influence on postmetastasectomy survival. There was no difference in postmetastasectomy survival between patients who underwent resection of synchronous versus metachronous pulmonary metastases (p2 = 0.90). Patients who underwent resection of fewer than four malignant nodules had a significantly longer postmetastasectomy disease-free survival (p2 = 0.0019) and overall survival (p2 = 0.06) than those undergoing resection of four or more nodules. Patients who underwent resection of metastases that developed during chemotherapy had a significantly shorter postmetastasectomy survival that those who underwent resection of metastases that developed after chemotherapy (p2 = 0.0295). Our data show that selected patients with Ewing's sarcoma metastatic to the lungs may benefit from an aggressive surgical approach. Also, a significant proportion of these patients will have benign pulmonary disease and can thus avoid additional intensive systemic therapy.     

Synovial sarcoma in children: surgical lessons from a single institution and review of the literature

BACKGROUND: Synovial sarcomas are malignant high-grade, soft-tissue neoplasms that account for 7% to 8% of all malignant soft-tissue tumors and are the most common nonrhabdomyosarcoma soft-tissue sarcomas in pediatric patients. STUDY DESIGN: A retrospective review of the records of children younger than 17 years with synovial sarcoma treated at the University of Texas MD Anderson Cancer Center from 1966 until 1999 was undertaken. Primary site, tumor size, tumor margins, surgical treatment, adjuvant therapy, local and distant recurrence, and survival were recorded for 42 patients. Overall survival (OS) and progression-free survival (PFS) rates were calculated by the Kaplan-Meier method. The PFS and OS comparisons were performed using the log-rank test. RESULTS: Forty-four patients were identified, but two patients were excluded because of incomplete records. The median followup duration for the 42 patients was 8.8 years (range 0.2 to 22.4 years). The 5-year progression-free survival and overall survival rates were 75.6% and 87.7%, respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. Intergroup Rhabdomyosarcoma Study (IRS) grouping and tumor invasiveness were found to be significant prognostic indicators (p < 0.01 and p = 0.02, respectively). Patients with initial gross total resection (IRS Groups I and II) and noninvasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (<5 cm) (p = 0.09) had better PFS and OS. Adjuvant radiation therapy appeared to be of benefit, and chemotherapy did not seem to impact PFS or OS. Tumors > or = 5 cm are associated with increased risk of local recurrence and distant metastases. CONCLUSIONS: Complete resection with clear, yet not necessarily large, margins remains the treatment of choice for synovial sarcoma in children. Adjuvant radiation therapy should possibly be considered in patients with clear margins (IRS Group I) and in patients with microscopic residual tumor (IRS Group II). Chemotherapy did not seem to impact PFS or OS. Lymph nodes should be evaluated for local regional disease.     

The treatment of soft-tissue sarcomas of the extremities: prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy.

Between May 1975 and April 1981, 43 adult patients with high-grade soft tissue sarcomas of the extremities were prospectively randomized to receive either amputation at or above the joint proximal to the tumor, including all involved muscle groups, or to receive a limb-sparing resection plus adjuvant radiation therapy. The limb-sparing resection group received wide local excision followed by 5000 rads to the entire anatomic area at risk for local spread and 6000 to 7000 rads to the tumor bed. Both randomization groups received postoperative chemotherapy with doxorubicin (maximum cumulative dose 550 mg/m2), cyclophosphamide, and high-dose methotrexate. Twenty-seven patients randomized to receive limb-sparing resection and radiotherapy, and 16 received amputation (randomization was 2:1). There were four local recurrences in the limb-sparing group and none in the amputation group (p1 = 0.06 generalized Wilcoxon test). However, there were no differences in disease-free survival rates (71% and 78% at five years; p2 = 0.75) or overall survival rates (83% and 88% at five years; p2 = 0.99) between the limb-sparing group and the amputation treatment groups. Multivariate analysis indicated that the only correlate of local recurrence was the final margin of resection. Patients with positive margins of resection had a higher likelihood of local recurrence compared with those with negative margins (p1 less than 0.0001) even when postoperative radiotherapy was used. A simultaneous prospective randomized study of postoperative chemotherapy in 65 patients with high-grade soft-tissue sarcomas of the extremities revealed a marked advantage in patients receiving chemotherapy compared with those without chemotherapy in three-year continuous disease-free (92% vs. 60%; p1 = 0.0008) and overall survival (95% vs. 74%; p1 = 0.04). Thus limb-sparing surgery, radiation therapy, and adjuvant chemotherapy appear capable of successfully treating the great majority of adult patients with soft tissue sarcomas of the extremity.     

Chemotherapy for malignant bone tumors

Chemotherapy has had a major impact in malignant bone tumors. In osteosarcoma, metastasis-free survival has been achieved in approximately 50 to 75 per cent of patients. Additional improvement based on the altered pattern of pulmonary metastases has also been reported. Preoperative chemotherapy has facilitated surgical resection of the primary tumor. The effects on the primary tumor may be utilized as a predictive factor and to design postoperative adjuvant therapy. Similar results have been achieved in Ewing's sarcoma with survival rates in the vicinity of 50 to 80 per cent. The interaction of chemotherapy with radiation has augmented the ability to achieve local control of the primary tumor. The tumoricidal properties of chemotherapy in destroying micrometastases may possibly also contribute to local control. Finally, initial treatment with chemotherapy may yield a complete response and facilitate definitive surgical treatment of the primary tumor. This may eliminate the need for radiation therapy and its delayed consequences.     

Adjuvant regional chemotherapy after hepatic resection for colorectal metastases.

BACKGROUND: This study explored the possibility of achieving a better survival rate and reduced recurrence in the remaining liver in patients with colorectal hepatic metastases undergoing hepatic resection. Adjuvant postoperative regional chemotherapy was administered via the hepatic artery or the portal vein. METHODS: A retrospective study was performed on 174 patients after hepatic resection for colorectal metastases. These comprised 78 patients who had hepatic artery infusion (HAI) chemotherapy (HAI group), 30 who had portal vein infusion (PVI) chemotherapy (PVI group) and 66 who had no regional chemotherapy (resection alone group). The three groups were compared with one another in terms of complications, survival rate and patterns of recurrence. RESULTS: Severe complications did not occur at any point during adjuvant HAI or PVI chemotherapy. The 5-year disease-free survival rate of patients in the HAI, PVI and resection alone groups were 35, 13 and 9 per cent respectively, including six hospital deaths. Patients in the HAI group showed significantly improved recurrence rates in the remaining liver compared with the resection alone group (P = 0.03), and more prolonged disease-free and overall survival than those in the PVI (P = 0.01 and P = 0.02 respectively) and resection alone (P = 0.0001 and P = 0.0006 respectively) groups. CONCLUSION: This study suggests that adjuvant HAI chemotherapy after hepatic resection may have therapeutic potential for improved management of patients with colorectal metastases.     

Chest wall resection and reconstruction for malignant conditions in childhood

This report evaluates the efficacy of extensive chest wall resection and prosthetic reconstruction in 15 children with chest wall malignancies. There were nine boys and six girls, with a mean age of 9.6 years. Eleven patients had primary chest wall tumors including Ewing's sarcoma (ES), six; rhabdomyosarcoma (RH), two; chondrosarcoma (CS), one; Askin's malignant neuroectodermal tumor, one; and mesenchymal sarcoma, one. Four children had metastases to chest wall and lung from Wilms' tumor (WT), two; osteogenic sarcoma (OS), one; and neuroblastoma (NB), one. Chest wall resection of two to six ribs and reconstruction with Marlex mesh (seven), lattisimus flap (two), prolene mesh (one), and more recently, a Gortex patch (five), was performed. Eight of the patients required concomitant en-bloc pulmonary resection (wedge, five; lobectomy, two; pneumonectomy, one) and two required resection of diaphragm. Fourteen received adjunctive therapy (chemotherapy, 14; irradiation, eight [preoperative, five; postoperative, three]. Six patients had second-look resections after chemotherapy. There was no operative mortality. Early pulmonary function was normal; however, pulmonary restrictive disease and scoliosis occurred with growth. One ES patient developed a radiation-induced second malignant tumor at age 10 and one ES child died at age 6 (no evidence of disease) of meningitis. Average survival length for ES patients was 77 months (range, 18 to 132 months.) Currently, eight patients are alive and five are free of disease. Extensive chest wall resection and reconstruction is useful in the treatment of primary chest wall tumors, but is palliative in metastatic cases. The Gortex patch is the current prosthetic of choice.     

Prognostic factors predictive of survival for truncal and retroperitoneal soft-tissue sarcoma.

OBJECTIVE: The authors identified prognostic factors relevant to clinical outcomes (especially survival) in truncal and retroperitoneal soft-tissue sarcoma. SUMMARY BACKGROUND DATA: These results can be used to optimize surgical management and select patients most likely to benefit from novel therapeutic strategies in future trials. METHODS: A retrospective analysis was performed of a prospectively compiled database of 183 consecutive patients with truncal and retroperitoneal sarcomas seen at the Brigham and Women's Hospital and the Dana Farber Cancer Institute between 1970 to 1994. RESULTS: For truncal sarcoma, multivariate analysis showed that high-grade histology was associated with an eightfold increased risk of death compared with low-grade histology (p = 0.001). In addition to grade, gross positive margin of resection (p = 0.001), microscopic positive margin (p = 0.023), and tumors greater than 5 cm in size (p = 0.018) were important independent prognostic factors for survival. In this series, postoperative radiation therapy for truncal sarcoma was associated with a 2.4-fold decreased risk of death compared with truncal sarcoma patients receiving no adjuvant radiation therapy, having adjusted for the other prognostic factors (p = 0.030). In contrast, for retroperitoneal sarcoma, multivariate analysis showed that high-grade and intermediate-grade histology were associated with a five- to sixfold increased risk of death compared with low-grade histology (p = 0.009). In addition to grade, gross positive margin of resection (p = 0.001) and microscopic positive margin (p = 0.004) were important independent prognostic factors for survival in retroperitoneal sarcoma. Patients who received either preoperative or postoperative chemotherapy for retroperitoneal sarcoma had a 4.6-fold (p = 0.002) and 3-fold (p = 0.010) increased risk of death, respectively, compared with patients receiving no adjuvant chemotherapy, having adjusted for the other prognostic factors. CONCLUSIONS: The histologic grade and the margin of resection are prognostic for survival in both truncal and retroperitoneal soft-tissue sarcoma. Tumor size was an independent prognostic factor for truncal sarcoma, but not for retroperitoneal sarcoma. Postoperative adjuvant radiation was beneficial to overall survival for truncal sarcoma. In this series of patients receiving a heterogeneous mixture of chemotherapeutic regimens-either as preoperative "neoadjuvant" therapy or as postoperative "adjuvant" therapy, there were no beneficial effects on survival compared with nonrandomized patients not receiving chemotherapy.     

Combined therapy for malignant tumors of the chest wall in children.

Of 1168 children with malignant solid tumors who were admitted to St. Jude Hospital between 1962 and 1976, 22 (1.8%) had primary malignancies of the chest wall, excluding the mediastinum. The treatment plan for these patients varied with the resectability of the tumor, but always included chemotherapy with or without irradiation. Six children had totally resectable tumors and another two had initially unresectable tumors converted to a resectable state with preoperative therapy. All 8 patients have been disease-free for 3–90 mo (median 18.5). Of 11 children with unresectable disease, 4 remain disease-free for 7–175 mo following chemotherapy and irradiation only (3 of 9) or both modalities plus partial resection (1 of 2). The 3 remaining children received en bloc resections for recurrent rhabdomyosarcoma following chemotherapy and irradiation: 2 died at 15 and 18 mo after diagnosis and 1 remains disease-free for 13 mo.The availability of effective combined therapy methods for local control of primary tumors and prevention of metastases made possible a more conservative surgical approach for 4 of 8 patients with resectable disease and permitted conversion of initially unresectable tumors to a resectable state in 2 patients. Moreover, these measures resulted in decreased morbidity without apparent compromise of tumor-free survival. Overall, 13 of 22 patients survive without evidence of disease: 3 of 3 patients with neuroblastoma, 4 of 6 with rhabdomyosarcoma, 4 of 10 with Ewing's sarcoma, 1 of 2 with osteosarcoma, and 1 patient with malignant histiocytoma.     

Preoperative chemotherapy and surgical resection in the management of posterior paraspinal tumors. Report of three cases

Three patients with posterior paravertebral tumors are presented to illustrate the value of preoperative chemotherapy prior to surgical resection. All three had massive tumors adjacent to the spine, extending over several vertebral segments, with myelographic evidence of epidural involvement in one patient. Tissue diagnosis by incisional biopsy confirmed the diagnosis of extraosseous osteogenic sarcoma in one, extraosseous Ewing's sarcoma in the second, and embryonal rhabdomyosarcoma in the third. All patients were treated with chemotherapy preoperatively. Marked regression in tumor size was noted, and surgical excision of the tumor along with involved segments of spine was easily accomplished. Histological examination following surgery revealed completely necrotic tumor in two patients. Preoperative chemotherapy for fully malignant tumors offers the following advantages: 1) extremely bulky and otherwise unresectable tumors can be reduced in size to enable complete surgical resection; 2) systemic chemotherapy is instituted earlier in the course of disease to prevent more effectively the appearance of metastases; and 3) the information gained regarding the sensitivity of the tumor to a particular chemotherapy regimen allows postoperative adjuvant chemotherapy to be used on a more rational basis.     

Chemotherapy of malignant bone tumors]

In several primary malignant tumors significant improvement of formely bad prognosis has been achieved by the introduction of new cytostatic compounds and the study of new cytostatic combination regimens. Adjuvant chemotherapy in osteosarcoma and Ewing's sarcoma led to remarkable increase in survival rates. Leaning on natural history and on remission rates reached by cytostatic treatment in metastasizing stages of disease, proposals for adjuvant chemotherapy are made and chemotherapy regimen appliable on out-patient basis is described.     

Prognostic factors predictive of survival and local recurrence for extremity soft tissue sarcoma.

OBJECTIVE: The authors sought to identify prognostic factors in the management of extremity soft tissue sarcoma. SUMMARY BACKGROUND DATA: The surgical management of soft tissue sarcoma has evolved because of advances in therapy, resulting in increased limb preservation and quality of life. However, identifying a subset of patients most likely to benefit from adjuvant chemotherapy has been difficult to achieve. METHODS: A retrospective analysis of a prospective data base of 182 patients with extremity sarcomas from 1970 to 1992 was performed. RESULTS: A histologic diagnosis of Ewing's sarcoma, synovial sarcoma, and angiosarcoma was associated with a 13-fold increased risk of death compared with liposarcoma, fibrosarcoma, and malignant peripheral nerve sheath histologic types after having adjusted for the other prognostic factors (p < 0.001). In addition to histologic type, high-grade sarcomas (p = 0.018), sarcomas greater than 10 cm in size (p = 0.006), and age at diagnosis (p = 0.016) were found to be important prognostic factors for survival but not for local recurrence. For the first time to their knowledge, the authors showed that mean mitotic activity has prognostic value after having adjusted for other prognostic factors, such as grade (p = 0.005). The only prognostic factors predictive for local recurrence were whether the patient presented with locally recurrent disease (p = 0.0001) or had microscopically positive margins (p = 0.052). CONCLUSIONS: The use of mitotic activity along with grade, size, histologic type, and age at diagnosis is prognostic for survival in extremity soft tissue sarcoma. The use of an objective pathologic feature, such as mean mitotic activity, is also useful in selecting patients for future systemic neoadjuvant or adjuvant trials and primary therapy.     

Development and treatment of pulmonary metastases in adult patients with extremity soft tissue sarcoma.

OBJECTIVE: The authors reviewed a series of adult patients with extremity soft tissue sarcoma to determine the incidence of pulmonary metastases and outcome after treatment. METHODS: Of 716 patients admitted between January 1983 and December 1990, 135 (19%) had isolated pulmonary metastases as the initial site of distant recurrence. Fifty-eight percent (78 of 135) of the patients were treated surgically, and 83% of them had their tumors completely resected. RESULTS: The median survival after complete resection was 19 months; incomplete resection, 10 months; and no operation, 8 months (p = 0.005). The 3-year survival rate after complete resection was 23%, compared with a 2% rate (1 of 57) in those treated nonsurgically (p < 0.001). Factors associated with an increased risk of pulmonary metastases included high tumor grade, tumor size greater than 5 cm, lower extremity site, and histologic type (spindle cell, tendosynovial, and extraskeletal osteosarcoma). Factors associated with complete resectability were the histologic types of spindle cell and extraskeletal osteosarcoma. CONCLUSIONS: Complete surgical resection remains the only possibility for cure from pulmonary metastases in soft tissue sarcoma; however, only 11% of the 19% of patients with an extremity sarcoma whose first distant recurrence is in the lung will be alive at 3 years, despite therapy. Complete resection and the development of more effective adjuvant treatments are imperative to improve outcome for this group of patients.     

ADULT PROSTATE SARCOMA: THE M. D. ANDERSON CANCER CENTER EXPERIENCE

PURPOSE: Sarcoma of prostate origin is rare. Historically, long-term survival rates for adult patients with prostate sarcoma are poor. We analyzed the experience of 1 institution with prostate sarcoma during the last 3 decades. MATERIALS AND METHODS: The records of 21 patients with prostate sarcoma were reviewed to identify symptoms at presentation, diagnostic procedures, presence and development of metastases, staging evaluation, histological subtype, grade and size of the primary tumor, and treatment sequence, including surgery, and preoperative and postoperative therapies. Several clinicopathological variables were assessed for prognostic importance. RESULTS: Most patients presented with urinary obstruction. The diagnosis of prostate sarcoma was usually established with ultrasound guided biopsy or transurethral resection. Histological subtypes were leiomyosarcoma in 12, rhabdomyosarcoma in 4, malignant fibrous histiocytoma in 1 and unclassified sarcoma in 4 patients. At last followup, 8 patients had no evidence of disease after a median of 81.5 months (range 10 to 197). The remaining 13 patients died of sarcoma (median survival 18 months, range 3 to 94). The 1, 3 and 5-year actuarial survival rates for all 21 patients were 81%, 43% and 38%, respectively. Factors predictive of long-term survival were negative surgical margins (p = 0.0005) and absence of metastatic disease at presentation (p = 0.0004). Tumor size and grade, and the histological subtype of prostate sarcoma had no significant influence on actuarial survival. CONCLUSIONS: The long-term disease specific survival rate for adults with prostate sarcoma is poor. Early diagnosis and complete surgical resection offer patients the best chance for cure.     

Ewing's sarcoma of the pelvis. Long-term survival and functional outcome

Fifty patients with Ewing's sarcoma of the pelvis were treated using a multidisciplinary approach; followup of surviving patients averaged 137 months (range, 40-276 months). The addition of surgical resection to the multidisciplinary treatment for all patients was associated with improved survival compared with survival of patients treated with chemotherapy and radiation therapy alone; the addition of surgery to the treatment regimen of 37 patients without metastases also was associated with improved survival. There were no significant differences between the surgical and nonsurgical groups in terms of tumor size, stage of disease, patient age, duration of symptoms before diagnosis, or anatomic site. Surgery was used more often in recently treated patients, but the year of diagnosis and treatment did not significantly affect overall survival, secondary to large confidence intervals. The Short Form-36 and the Musculoskeletal Tumor Society functional evaluation instruments showed a superior level of function in the nonsurgical group, but this difference was not statistically significant. There have been many advances in the treatment of patients with Ewing's sarcoma during the past 3 decades, resulting in improved survival for patients with Ewing's sarcoma of the pelvis. The addition of surgery significantly improved survival and did not show a significant difference in functional outcome.     

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.     

Knochen- und Weichteiltumoren des Fußes und Sprunggelenkes

BACKGROUND: This study reports the epidemiology of bone and soft-tissue tumors of the foot and ankle, presents therapy strategies, and evaluates mid-term clinicofunctional outcome after surgery for malignant tumors.METHODS: Two hundred four patients with tumors of the foot and ankle were analyzed (163 benign and 41 malignant). Epidemiology and surgical therapy are reported.RESULTS: The most frequent tumors were exostosis, bone cyst, and osteoid osteoma for benign tumors and metastases and chondrosarcoma and Ewing's sarcoma for malignant tumors. In more than 90% of the benign tumors, local resection could be carried out, whereas in malignant tumors, ablative procedures and arthrodeses were almost as common as limb- and joint-sparing techniques. However, follow-up revealed good functional results and a 5-year survival rate of 84% for patients with primary malignant tumors.CONCLUSION: Tumors of the foot and ankle require a thorough therapeutic strategy. Mid-term functional results and survival rate after surgical treatment are good, although a high percentage of ablative procedures or fusions could not be avoided in our patients.     

Surgical procedures for bone neoplasms in children]

The treatment of 40 patients with bone tumors have been presented. The primary tumors were located in the following sites: femur (14), tibia (8), fibula (4), humerus (4), scapula (1), clavicle (2), pelvis (5), hand (1). Investigated group were: osteosarcoma (18), Ewing's sarcoma (14), chondrosarcoma (2), fibrosarcoma (1), synovial sarcoma (1), chondroblastoma (4). In the most frequent malignant bone tumors, osteosarcoma and Ewing's sarcoma, unified management was adapted. The treatment was initiated with multidrug chemotherapy and followed by surgery or radiotherapy (Ewing's sarcoma) of the primary site. Surgery was performed in 30 cases: 19 mutilating operations because of the broad local invasion, 11 conservative surgical procedures (limb -- salvage operations). Satisfactory oncological and functional effect can be achieved after limb-salvage surgical procedures in the cases of localized, especially semimalignant bone tumors.     

Oncological outcomes of patients with Ewing's sarcoma: is there a difference between skeletal and extra-skeletal Ewing's sarcoma?

The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing's sarcoma. We identified 300 patients with new primary Ewing's sarcoma diagnosed between 1980 and 2005 from the centres' local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing's sarcomas. Although patients with skeletal Ewing's were younger (mean age 16.8 years) than those with extra-skeletal Ewing's sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing's sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not. This large series has shown that the oncological outcomes of Ewing's sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.     

Total vertebrectomy for primary malignant tumours of the spine

Primary malignant tumours should be resected with wide margins. This may be difficult to apply to lesions of the spine. We undertook total vertebrectomy on seven patients, four males and three females with a mean age at operation of 26.5 years (6.3 to 45.8). The mean follow-up was 52.3 months. Histological examination revealed a Ewing's sarcoma in two patients and osteosarcoma, leiomyosarcoma, spindle-cell sarcoma, chondrosarcoma and malignant schwannoma in one each. In five patients, histological examination showed that a wide resection had been achieved. At follow-up there was no infection and a permanent neurological deficit was only seen in those patients in whom the surgical procedure had required resection of nerve roots. Despite the high demands placed on the surgeon and anaesthetist and the length of postoperative care we consider total vertebrectomy to be an appropriate procedure for the operative treatment of primary malignant lesions of the spine.