Background: Sacral tumours are commonly diagnosed late and therefore are often large and at an advanced stage before treatment is instituted. The late presentation means that curative surgical excision is technically demanding. 1. Total en-bloc sacrectomy is fraught with potential complications: deep infection, substantial blood loss, large bone and soft tissue defects, bladder, bowel and sexual dysfunction, spinal-pelvic non-union, and gait disturbance. 2. The aim of the current study was two-fold: firstly to detail the technique used by the senior author and chronicle how this has evolved; and secondly to present the complications and outcome of nine total en bloc sacrectomies. Methods: We retrospectively analysed of total en-bloc sacrectomies between 1991 and 2004. Nine patients (2M, 7F, mean age at surgery 39 years, range 21 – 64yrs) with a diagnosis of primary sacral tumour underwent total en-bloc sacrectomy under the care of the senior author. The mean follow-up was 50.2 months (range: 3.5 – 161 mths). Patients’ functional outcome was evaluated using the Functional Independence Measure (FIM) instrument and the SF-36. Intra-operative and postoperative complications (including disease progression) were documented. Results: Surgical technique has evolved from single stage surgery without and with colostomy to two stage surgery with colostomy. Currently, the first stage includes an anterior lumbar interbody fusion at L4/L5 retaining the L5 nerve roots. In the second stage an L4 to pelvic fusion is performed posteriorally. The dura is tied and divided just below the L5 roots. The mean total operating time was 13.3 hrs (range: 8 – 20.1hrs); the mean total blood loss 14.1 ltrs (range: 4.2 – 33 ltrs). There were two revision L4 to pelvic fusions for pseudoarthroses. The mean length of hospital stay was 8.9mths (range: 2 – 36mths). One patient had a recurrence and died 2 years after her surgery. Of the surviving 8 patients the results from the functional outcome scores were variable. Three patients are able to walk independently; the remaining 5 are all mobile but require differing degrees of assistance to walk. Conclusion: Total en bloc sacrectomy is a major surgical undertaking but our series has shown that it is probably justified in view of the fact that 8 out of 9 patients have had no tumour recurrence and all are able to walk

Sacral tumours are rare and can present difficult diagnostic and therapeutic challenges even at an early diagnosis. Surgical resection margins have a reported prognostic role in local recurrence and improved survival. Successful management is achieved within a specialist multidisciplinary service and involves combination chemotherapy, radiotherapy and surgery. We present our experience of patients with sacral tumours referred to our unit, who underwent total and subtotal sacrectomy procedures. Materials and Methods. Between 1995 and 2010, we identified twenty-six patients who underwent a total or subtotal sacrectomy operation. Patients were referred from around the United Kingdom to our services. We reviewed all case notes, operative records, radiological investigations and histopathology, resection margins, post operative complications, functional outcomes and we recorded long-term survival outcomes. Patients who were discharged to local services for continued follow up or further oncological treatment were identified and information was obtained from their general practitioner or oncologist. We reviewed the literature available on total sacrectomy case series, functional outcomes and soft tissue reconstruction. Results. We reviewed 26 patients, 16 male and 10 female, with a mean age at presentation of 53.4 years (range 11–80 years). Duration of symptoms ranged from 2 weeks to 6 years; lower back pain and sciatica were amongst the most common presenting features. Histological diagnoses included chordoma, Ewing's, malignant peripheral nerve sheath tumour, chondromyxoid fibroma, spindle cell sarcoma, synovial sarcoma, chondrosarcoma. A combined approach was used in two-thirds of patients and most of these patients had a soft tissue reconstruction with pedicled vertical rectus myocutaneous flap. Complications were categorised into major and minor and subdivided into wound, bladder and bowel symptoms. Wound complications and need for further intervention were more common amongst the patient group who did not have simultaneous soft tissue reconstruction at operation. All patients had a degree of bladder dysfunction in the early postoperative period. We present survivorship curves including recurrence and development of metastases. Conclusion. Total sacrectomy procedures carry a high risk of associated morbidity but can improve survival amongst specific groups of patients. They present challenges in diagnosis and management, but must be referred to a specialist service, that will instigate appropriate investigations and treatment regimes within a multidisciplinary setting. The expansion of services from other specialties required for the postoperative and ongoing rehabilitation plays an important role in overall management and appropriate pathways to coordinate these services are necessary

46 Sacral chordoma patients treated between 1987 and 2004 are reviewed. The importance of early diagnosis, adequate surgical margin and post operative radiotherapy for optimum outcome and survival is stressed. There were 33 male and 13 female patients, with a mean age of 61 years (38–73 years). The surgical approach depended on the level and extent of the lesion, with an anteroposterior approach used in 23 and posterior approach in 17 patients. 20 had partial sacrectomy, 17 had subtotal sacrectomy and 3 underwent total sacrectomy. 6 patients were deemed inoperable and received palliative therapy. 14 patients received radiotherapy post-operatively. The length of average follow up was 4.27 years (range 2–15.7 years). Low back pain was the most common presenting symptom (80%), and 50% patients had a palpable mass. The mean duration of symptoms prior to diagnosis was 2 years (range 1 month–10 years). Examination revealed a palpable mass in 7 both externally and on rectal examination. 10 had a palpable mass on rectal examination but not externally. 2 patients presented with multiple metastases and another 2 with widespread local disease. Excision was complete in 23 patients and incomplete in 17. Histology revealed dedifferentiation in 4. Complete excision margin was achieved in 69.6% through combined approach and 52.9% through posterior approach only. 24 patients (52%) had local recurrence. Without adjuvant radiotherapy the mean disease free period following complete excision was 3.5 years, compared to 0.9 years following incomplete excision. Adjuvant radiotherapy extended the mean disease free period following incomplete excision to 1.8 years. The authors conclude that an early diagnosis and careful examination is important. Wide excision remains the mainstay of treatment. If excision is incomplete radiotherapy increases the disease free period although local recurrence is inevitable. The use of a combined approach increases the likelihood of complete excision

Purpose: To report the importance of early diagnosis, adequate surgical margin and postoperative radiotherapy for optimum outcome and survival. Study Design: A retrospective review of 46 sacral chordoma patients treated between 1987 and 2004. Methods: There were 33 male and 13 female patients, with mean age of 61 years (38 to 73 year). The surgical approach depended on the level and extent of the lesion, with an anteroposterior approach used in 23 and posterior approach in 17 patients. 20 had partial sacrectomy, 17 had subtotal sacrectomy and 3 underwent total sacrectomy. 6 patients were deemed inoperable and received palliative therapy. 14 patients received radiotherapy postoperatively. The length of average follow up was 4.27 years (range 2–15.7 years). Results: Low back pain was the most common presenting symptom (80%), and 50% patients had a palpable mass. The mean duration of symptoms prior to diagnosis was 2 years (range 1 month–10 years). Examination revealed a palpable mass in 7 both externally and on rectal examination. 10 had palpable mass on rectal examination but not externally. 2 patients presented with multiple metastases and another 2 with widespread local disease. Excision was complete in 23 patients and incomplete in 17. Histology revealed dedifferentiation in 4. Complete excision margin was achieved in 69.6% through combined approach and 52.9% through posterior approach only. 24 patients (52%) had local recurrence. Without adjuvant radiotherapy the mean disease-free period following complete excision was 3.5 years, compared to 0.9 years following incomplete excision. Adjuvant radiotherapy extended the mean disease-free period following incomplete excision to 1.8 years. Conclusion: An early diagnosis and careful examination is important. Wide excision remains the mainstay of treatment. If excision is incomplete radiotherapy increases the disease free period although local recurrence is inevitable. Use of combined approach increases the likelihood of complete excision

Purpose. Curative treatment of malignancies in the sacrum and lumbar spine frequently requires en-bloc spinopelvic resection. There is no standard classification of these procedures. We present a classification of these resections based on analysis of 45 consecutive cases of oncologic spinopelvic resections. This classification implies a surgical approach, staging algorithm, bony and soft tissue reconstruction, and functional outcomes following surgery. Method. We reviewed oncologic staging, surgical resections, and reconstructions of 45 consecutive patients undergoing spinopelvic resection with curative intent. Mean follow-up of surviving patients was 38 months. Common themes in these cases were identified to formulate the surgical classification. Results. Tumors included chondrosarcoma (n=11), other sarcomas (n=11), osteosarcoma (n=9), chordoma (n=6), locally invasive carcinoma (n=5), and others (n=3). Resections could be divided into 5 types based on the exent of the lumbosacral resection and the need for an associated external hemipelvectomy. Type 1 resections included a total sacrectomy +/− lumbar spine resection. Type 2 resections included hemisacrectomy +/− partial lumbar excision, and iliac wing resection. Type 3 resections encompassed external hemipelvectomy with hemisacrectomy +/− partial lumbar excision. Type 4 resections encompassed external hemipelvectomy with total sacrectomy +/− lumbar excision. Type 5 excisions involved hemicorporectomy type procedures. For each type of resection we have developed guidelines for trans- vs retroperitoneal surgical approaches, staging of the resections, bony and soft tissue reconstructive procedures to re-establish spinopelvic continuity, and predicted functional outcomes for patients. At mean 38 month follow-up on surviving patients, 28 are living and 17 are deceased. Twenty-two of 28 surviving patients are disease free. Nineteen of 26 surviving patients are independent in their activities of daily living. Conclusion. En bloc spinopelvic resections may be classified into five types based on the extent of lumbosacral excision and the need for concurrent hemipelvectomy. Using this classification system, we have formulated treatment strategies to guide surgical approach, procedural staging, bony and soft tissue reconstructive procedures, and expected functional outcomes. Long term survival and independent function can be achieved in this challenging patient population

Curative treatment of malignancies in the sacrum and lower lumbar spine frequently requires en bloc spinopelvic resection. There is no standard classification of these procedures. We present outcomes and a classification scheme with oncologic and reconstructive guidelines for spinopelvic tumors based on an analysis of 30 cases of en bloc resection and reconstruction performed with curative intent. Mean follow-up of surviving patients was 38 months. Tumors included osteosarcoma (n=9), chondrosarcoma (n=6), chordoma (n=5), other sarcomas (n=5), neurogenic tumors (n=4), and local extension of carcinoma (n=1). Resections could be divided into 4 types. Type 1 resections (n=12) included a total sacrectomy with lower lumbar spine and bilateral medial iliac resections. Type 2 resections (n=6) included hemisacrectomy, partial lumbar spine excision, and medial iliac resection. Type 3 resections (n=9) encompassed external hemipelvectomy with hemisacrectomy and partial lumbar spine excision. Type 4 resections (n=3) encompassed external hemipelvectomy, total sacrectomy, and lumbar spine excision. For each resection type, we have developed staged surgical approaches to allow resection with wide margins and reconstruction of spinopelvic continuity. Tumor free margins were achieved in all cases. Perioperative mortality was 3/30. Seven additional patients have died of disease, two died of other causes, two are alive with disease, and 16 have no evidence of disease. 13/18 surviving patients are independent in their activities of daily living. In our practice en bloc excision and reconstruction of spinopelvic neoplasms may be classified into four types. For each type, we have devised surgical treatment guidelines to allow for wide resection and reconstruction of spinopelvic continuity. Long term survival and independent function can be achieved in this challenging patient population. This represents the first standardised classification of oncologic spinopelvic resections and reconstructions

Purpose of the study: Diagnosis and treatment of primary malignant tumors of the pelvis raise difficult problems. The purpose of this retrospective study was to analyze the functional and cancerological results observed after surgical treatment in a single center. Material and methods: Between 1973 and 2002, 24 patients (16 men and 8 women) underwent surgery in our unit for histological proven malignant tumors. A posterior approach was used for curettages and sacrectomies of the apex. A combined anterior and posterior approach was used for total sacrectomy and hemisacrectomy. Oncological results were assessed in terms of local recurrence, presence of metastasis and patient status at last follow-up. Overall survival and disease-free survival were calculated with the Kaplan-Meier method. Results: Mean age was 53.38 years. Mean follow-up in our series was 54 months. Mean time to diagnosis was 16 months. Pain was the predominant symptom. Sixteen patients presented neurological manifestations and the digital rectal examination was positive in all. Chondroma was the most frequent histological type (18/24). None of the patients had metastatic disease at diagnosis. A posterior approach was used for 15 patients and a combined approach for the others. There was a clear correlation between type of resection and volume of blood loss (p=0.0002). Wide dissection was wide in five patients, marginal in five and oncologically insufficient in 14. Mean operative time was 1.34 hours for posterior approaches and 9 hours for combined approaches. The postoperative period was uneventful for ten patients. Infection was the most frequent complication. Adjuvant radiotherapy, delivered in 16 patients, effectively retarded the occurrence of local recurrence. Functional disorders were correlated with the level of the neurological sacrifice. At least one S3 root had to be preserved to limit the urological and digestive incapacity. At last follow-up, local recurrence was present in 12 patients. Mean time to first recurrence was 32 months. There was a strong correlation between quality of the resection and time to local recurrence. There was a significant difference between patients with a wide resection and those with an oncologically insufficient resection (p=0.0312). Five patients had metastases. Five-year actuarial survival was 73±12%. At ten years it was 32±14%. Local recurrence-free survival was 55±11% at five years and zero at 10 years. Discussion and conclusion: In light of these results, factors of poor prognosis were: late diagnosis, soft tissue invasion, proximal extension, marginal or insufficient resection

It is frequently difficult to diagnose and treat of malignant sacral bone tumors. This tumor is diagnosed with lumbar disc hernia, instability coccygitis, hemorrhoids. We reviewed the surgical treatment of primary malignant (14) and secondary (metastatic) sacral tumors (11) in 25 patients from 1983 to 2000. Primary tumors consisted of chordoma in 11 patients, chordoma with spindle cell sarcoma, malignant peripheral nerve sheath tumor (MPNST), giant cell tumor of bone in 1 patient each. The secondary tumors consisted of invading carcinoma in 7 patients, metastatic carcinoma in 4 patients. Location of the sacral tumor was showed total sacrum in 2 patients, below S2 in 18, S3 in 2 and S4 in 3. Preserving nerves were L5 in 1 patient, S1 in 17, S2 in 2, S3 in 3, and 2 performed curettage. Posterior approach was used in 8 patients, and an anterior and posterior combined approach in 17. Sacrectomy only in 7 patients, and sacrectomy and colostomy in 8, including with rectum was performed in 8, and 2 patients had extensive curettage and bone graft or hydroxyapatite (HA) transplantation. Six tumor excisions were used modified T-saw which pass through the sacral canal preserving nerve roots. Surgical margin of chordoma in primary sacral tumors had wide in 10, wide excision with partial contamination in 2, except curettage in 1. MPNST had curettage and giant cell tumor of bone had marginal in 1 each. Secondary sacral tumors had wide in 9, marginal in 2. Adjuvant therapy was used radiation therapy in 3 patients and chemotherapy in 2 and ethanol in 1. Musculocutaneous flap was reconstracted tensor fascia lata flap and gluteal muscle flap in 2 patients. Interval between initial chief complaints and diagnosis of chordoma detected from 6 months to 10 years, avarage 5 years 3 months by rectal examination, radiogram, genital ultra echo and MRI; invading carcinoma from 2 months to 3 years, avarage 8 months, and metastatic carcinoma from 2 months to 4 months, average 3 months. Six of 12 patients of chordoma in primary sacral tumors are alive from 6 months to 18 years, average 4 years 6 months; remaining patients were died 6 month to 8 years, average 3 years 2 months, except 2 patient died with infection. The patient with a MPNST died after 2 years 6 months, and a giant cell tumor of bone had no recurrence or lung metastases in 10 years. One of 11 patients of secondary sacral tumor (initial surgery) is alive in 14 years 6 months, remaining 10 patients died 3 months to 4 years 6 months, average 1 year 10 months, except 2 patients died with infection. Complications were much bleeding, infection, skin slough, nerve injury. We recommend better surgical method that anterior and posterir approach use above S3, and posterior approach blow S4, A modified T-saw performed an osteotomy of the pars lateral of the sacrum, proved to be easier and faster than osteotomies performed using the old method

Introduction/Aim. Numerous lumbo-pelvic reconstruction methods based on posterior construct and anterior cages have been proposed for cases involving total sacrectomy and lumbar vertebrectomy. These constructs create long lever arms and generate high cantilever forces across the lumbo-sacral junction resulting in implant failure or breakage. Biomechanical studies have shown that placing implants anterior to lumbo-sacral pivot point provide a more effective moment arm to resist flexion force and improve the ultimate strength of the construct. As a result more emphasis is placed on screws in the pelvis. We report a new and novel technique that allows for the placement of a pelvic ring construct to augment the posterior construct in a lumbo-pelvic reconstruction. Method. In the prone position, two contoured hard rods are passed along the inner table of the pelvis under the iliac muscle from a minor posterior approach. The rods are connected to the posterior lumbo-pelvic construct with T-junction clamps. The patient is turned supine and the anterior ends of the rods are connected to a sub-cutaneously placed hard rod along the anterior abdominal wall with T-junction clamps. This in turn is fixed to the AIIS (anterior inferior iliac spine) with two poly axial screws. The whole construct resembles an oblong ring. Results. At six months she is mobilising independently with a frame and X-rays show no failure of construct or implant. Conclusion. The construct is technically less morbid and bio-mechanically sound to effectively neutralise the flexion to a greater degree than previous constructs described in the literature. It shares and tolerates the flexion moments at the lumbo-pelvic junction by its anterior placement to the sacral pivot point

Aim: To investigate the outcome of our management of patients with giant cell tumour of the sacrum and draw lessons from this. Method: Retrospective review of medical records and scans for all patients treated at our unit over the past 20 years with a giant cell tumour (GCT) of the sacrum. Results: Of 517 patients treated at our unit for GCT over the past 20 years, only 9 (1.7%) had a GCT in the sacrum. 6 were female, 3 male with a mean age of 34 (range 15–52). All but two tumours involved the entire sacrum and there was only one purely distal to S3. The mean size was 10cm and the most common symptom was back or buttock pain. Five had abnormal neurology at diagnosis but only one presented with cauda equina syndrome. The first four patients were treated by curettage alone but two patients had intra-operative cardiac arrests and although both survived all subsequent curettages were preceeded by embolization of the feeding vessels. Of 7 patients who had curettage, 3 developed local recurrence but all were controlled with a combination of further embolisation, surgery or radiotherapy. One patient elected for treatment with radiotherapy and another had excision of the tumour distal to S3. All the patients are alive and only two patients have worse neurology than at presentation, one being impotent and one with stress incontinence. All are mobile and active at a follow up between 2 and 21 years. Conclusion: GCT of the sacrum can be controlled with conservative surgery rather than sacrectomy. Embolisation and curettage are the preferred first option with radiotherapy as a possible adjunct. Spino-pelvic fusion may be needed if the sacrum collapses

Primary solitary fibrous tumour (SFT) of bone is extremely rare with few cases reported in the literature. The incidence of the lesion is 0.08% of all primary bone tumours (0.1% of primary malignant bone neoplasms). Previously, such lesions may have been reported as haemangiopericytoma (HP). Despite being previously considered as separate entities, the two types of tumour (SFT and HP) are now generally accepted as related, sharing similar morphological and immunohistochemical features. Cytogenetic and molecular analysis has, so far, been unable to unite or divide the two. Although frequently having a histologically benign appearance or being labelled as intermediate grade, these tumours may exhibit an unpredictable clinical course and behave in an aggressive manner. We present two cases of osseous solitary fibrous tumour (cellular haemangiopericytoma). Using the histopathology and bone tumour databases at our institution, we identified two patients (one female aged 21 and one male aged 40) with a histopathological diagnosis of osseous SFT. The site of primary tumour in both patients was the sacrum. In the female patient, the lesion was confined to the sacrum and she underwent curettage. In the male patient, the tumour extended beyond the sacrum to the sacro-iliac joint, ilium and gluteal mass, therefore, total sacrectomy was performed. At presentation neither patient had evidence of metastatic spread. The female patient was disease free at four years with no evidence of recurrence of metastases. The male patient developed metastases in both lung fields and bone (ribs, vertebrae) three years post-operatively and died four years post-operatively. Orthopaedic surgeons and histopathologists should remain aware of SFT due to its erratic behaviour and the recent move towards unifying it with HP in a continuous spectrum. We recommend early staging and treatment of these tumours, even for histologically benign/low grade lesions, due to their potentially aggressive behaviour

Purpose: Dedifferentiated chordomas are a rare and aggressive variant of chordoma. They usually occur in recurrences or following radiotherapy. We describe the rare occurrence of three cases arising de novo within conventional chordoma. Methods and Results: 29 cases of histopathologically verified chordoma were identified from our unit database. From these, we identified three cases of primary dedifferentiated chordoma. The study group included three men, with an average age of 58.3 years (range 57–61). Presenting features were comparable and included lower back pain and rectal discomfort, with a mass palpable per rectum. A needle biopsy was undertaken in one patient, which demonstrated evidence of a pleomorphic spindle cell tumour without evidence of chordoma. Surgical management involved subtotal sacrectomy, with complete excision achieved in two cases. Histopathological examination confirmed dedifferentiated chordoma with varying amounts of sarcomatous elements in each case. Post-operative radiotherapy was administered in two patients and chemotherapy in one patient. Two patients died at 7 and 10 months following presentation with pulmonary metastases. The third patient remains well and alive at two years follow-up. This patient had a complete excision with post-operative radiotherapy, however histopathological examination revealed only a small focus of the dedifferentiated component. Conclusions: Dedifferentiated chordoma is a fatal tumour, with metastases and death the likely outcome. Most cases in the literature and the two cases reported in this series, uphold this ominous prognosis. However this may not always hold true. Small areas of dedifferentiation within the chordoma tumour may carry a more favourable prognosis

Purpose: Surgery is required for primary tumours invading the sacroiliac joint. The purpose of the present work was to analyse results obtained with surgical treatment in order to better ascertain indications. Material and methods: Forty patients (24 men and 16 women), mean age 24 years (range 12–56) underwent surgery for high-grade sarcoma (n=30, osteosarcoma 12, Ewing 13, chondrosarcoma five) or more differentiated tumours (n=10, low-grade S chondrosarcoma five, fibro-sarcoma two, others three). Resection was achieved in 37 cases via two approaches and via a lateral approach in three. Resection involved vertical sacrectomy either via the homolateral foramen (n=27) or via the midline (n=10). Reconstruction consisted in stabilising the iliosa-cral assembly generally associated with vertebral osteo-synthesis, an autologous graft in 36 cases, cement in one, and an allograft in three. Functional outcome was assessed with the MSTS (Enneking). Results: There were three infections (all three with extensive lateral approach) and five cases of postoperative lumbosacral trunk palsy. Late complications were three cases of spondylolisthesis and eight nonunions. Twenty patients died (eight local recurrences, ten metastases, one chemotherapy toxicity, one undetermined cause). Sixteen patients achieved complete remission at six years (follow-up 2–16 years) and four patients were lost to follow-up. Functional outcome was very good in eight, good in ten, fair in twelve, and poor in ten. Survival was 40% among patients with malignant tumours (38 patients) but only 20% for those with osteosarcomas. Discussion: Technical improvements (combined approach rather than wide lateral approach and omentum flap) have allowed a reduction of cutaneous and infectious complications. Mechanical complications can be prevented by systematic lumbosacral fusion on the side opposite the resection reconstruction. This provides good functional results despite sacrificing a hemi-sacral plexus if the lumbo-sacral trunk is preserved. Reconstruction after extension of the resection to the acetabulum raises an unresolved problem and yields mediocre results. The quality of the surgical resection is determinant since risk of local recurrence is 8/100 after a contaminated resection edge. Conclusion: Surgical resection of sacroiliac tumours is a source of numerous complications despite real technical improvements. This approach can be proposed if carcinological resection can be reasonably achieved. Local control is very poor in case of large osteo-osteogenic sarcomas

Purpose of the study: Chordoma is a malignant neoplasm believed to arise from notochord remnants. It accounts for approximately 3 to 4 p. 100 of primary bone tumors and is localized along the axial skeleton, 50 p. 100 being sacrococcygeal. Clinical, radiographical and histological findings have been well established since the first description by Ribbert in 1894. Sacral chordomas are however difficult to manage and remain a challenge for surgeons and radiotherapists alike. The purpose of this study was to evaluate the long-term results of surgical treatment and patterns of failure in patients treated for chordoma of the sacrum in our department. Materials and methods: This retrospective study included 11 cases of sacral chordomas treated from 1973 to 1998. Patient age ranged from 36 to 77 years (mean 59 years). Six patients were female and five male. The initial treatment was surgery in all cases including intralesional removal in two cases, marginal resection in seven and complete en bloc resection in two. Results: Median follow-up was 6 years (1 month to 14 years). Tumoral recurrences were observed in nine cases 5 months to 8 years after treatment. In two cases, recurrence was observed 8 years after radical sacrectomy. Treatment of recurrences was partial surgical removal with radiotherapy (40 to 70 Grays). Three patients developed metastases in lungs, liver and bone, respectively. Seven patients died, two from metastatic disease. The 5-year overall survival was 64 p. 100 but only 18 p. 100 of the patients survived 10 years. Average disease-free survival was 18 p. 100 at 5 years and 0 p. 100 at 10 years. Discussion: Chordoma is a slow-growing tumor allowing survival for several years despite recurrent disease. However, only 10 to 20 p. 100 of the patients survive free of disease at 5 years. Recurrences are frequent (45 to 80 p. 100) and often multiple. Chordoma inevitably recurs and eventually leads to death after intralesional removal or marginal resection. Radical surgery should be attempted whenever technically feasible. When performed early, particularly for smaller lesions, it offers the best chance for cure. However, tumoral recurrence can occur postoperatively despite a macroscopically complete resection. Because radiation therapy seems to be more successful in controlling microscopic disease, it should be considered as a pre- or postoperative adjuvant to a macroscopically complete resection