The purpose of our study was to examine the survival and functional outcome of endoprosthetic replacements for non-oncology limb salvage purposes. Although initially designed for bone tumours, such is the versatility of these implants they can be used to salvage failed joint replacements, peri-prosthetic fractures, failed internal fixation and non-union.

Thirty eight procedures were identified from September 1995 to June 2007 from a prospectively kept database, including 17 distal femoral replacements, 12 proximal femoral replacements, 4 proximal humeral replacements, 2 distal humeral replacements, 2 hemi-pelvic replacements and 1 total femoral replacement. The quality of patients’ mobility was used to assess functional outcome and the survival of the prosthesis was calculated using a Kaplan-Meier survival curve.

The Kaplan-Meier implant survival was 91.3% at 5 years, 68.5% at 10 years and 45.7% at 20 years. The limb salvage survival for all reconstructions was 75% at 10 years.

The best survival was as follows pelvic (n=0/2) and total femoral prostheses where there was no failure in either group (n=0/1). Distal femoral replacements survival was 91% at 5 years, a single humeral prosthesis failed at 11 years post surgery, and proximal femoral replacements had a survival at 87.5% at 5 years. Three implants failed, two as a result of infection and required staged revisions and 1 failed as a result of aseptic loosening. Two patients dislocated their proximal femoral replacements, both were treated successfully by closed reduction.

Endoprosthetic replacement appears to be effective and the medium term survival is encouraging. The aim of a pain free functional limb is achievable with this technique. The complication rates are acceptable considering the salvage nature of these patients. We recommend referral of complex cases to a tertiary centre with expertise in this type of surgery.

We aim to establish the likely functional scores following different types of surgery for bone tumours. All living patients who had undergone treatment for a bone tumour were contacted and invited to complete a TESS self-report questionnaire. The TESS contains 32 items of day-to-day activities which the patient scores themselves against. The final score is a percentage, with 100% being ‘normal’. The project was approved by the local ethical committee.

723 responses were obtained from just over 1000 letters that were sent. Thirty seven sets of data were incomplete leaving a total of 686. There were 369 males and 317 females, with an average age of 49 (range 15 to 93). The mean interval from their initial operation was 12 years (range 1 to 42). 590 of the procedures were in the lower limb and 96 in the upper limb. 126 patients had an amputation and 560 limb salvage (82%).

Mean scores obtained for each procedure were: EPR mid-femur 86%; EPR distal femur 77%; EPR proximal tibia 74%; EPR proximal humerus 71%; EPR proximal femur 71%; Below knee amputation 70%; EPR total femur 63%; Disarticulation of the hip 61%; Above knee amputation 60%; Hindquarter amputation 57%; EPR pelvis 54%.

Younger patients had much better scores (80% for those under 30 vs. 69% for those over 30, p< 0.0001) than those over 30. Additionally, the scores for those that had had an amputation following a EPR procedure were no worse than those who had had an amputation originally. Significantly, EPR proximal tibia was better than AKA (78% vs. 67% p = 0.0003), as was EPR distal femur (80% vs. 67% p < 0.0001).

This data from a large series is helpful in indicating likely outcomes following surgical treatment of sarcomas, both for patients and surgeons.

Aim: To identify factors leading to the success or failure of extracorporeal irradiation and reimplantation of bone (ECIR).

Method: Review of experience of this technique since 1996 documenting successes and failures.

Results: 35 patients with a variety of malignant bone tumours underwent ECIR from 1996 up to 2007. The most common diagnosis was Ewings’ sarcoma (15) followed by osteosarcoma (9) and chondrosarcoma (5) with the most common sites being the pelvis (15) followed by the tibia (10) and humerus (3). The age range was from 7 to 66 and 8 were skeletally immature at the time of surgery. The bones were sterilised with a dose of 90Gy before reimplantation.

The overall patient survival was 69% at 5 years and 62% at 10 years. Local recurrence arose in 4 cases, 3 of whom already had metastatic disease. None of the LR arose in the irradiated bone. The rate of non union was 9% at the 58 osteotomies, the greatest risk being in the tibia. There were four graft fractures of which 3 needed fixation and all united. There is one case of convincing graft resorbtion after 12 years. Two patients developed infections but there have been none since the done was routinely immersed in antibiotics whilst being irradiated.

Conclusion: ECIR is a useful technique with very limited indications. In the majority of cases it works well. It appears particularly useful in pelvic resections and diaphyseal resections. Distal tibial reconstructions do worst. The irradiated bone needs reinforcing with either cement or a bone graft for best results.

Patients who undergo soft tissue sarcoma excision accumulate serosanguinous fluid, potentially resulting in a seroma. This can lead to wound complications and delay post-op radiotherapy.

The purpose of this preliminary report is to assess the impact of routine application of Tisseel sealant prior to closure.

We investigated whether the sealant Tisseel is effective as a sealing agent to reduce the duration and volume of serosanguinous fluid drainage. Results were compared with individually matched controls.

Patients were split into 2 groups: those receiving 10mls sealant and those not receiving the sealant. Efficacy was evaluated by the number of days required for wound drainage, the volume of fluid drainage and the length of stay compared with matched controls.

The preliminary findings suggest that application of the sealant reduced the duration and quantity of fluid drainage after excision of the STS, allowing earlier discharge from hospital.

We present this work to suggest that the members of the British Orthopaedic Oncology Society should consider using this in a randomised controlled trial setting to evaluate its efficacy nationally.

Chondrosarcoma is treated with respect by oncology surgeons, given that it is relatively resistant to chemotherapy and radiotherapy. The aim was to study the outcomes of surgery for chondrosarcoma and determine the role of initial surgical margins and local recurrence on outcome.

Electronic patient records were retrieved on all patients seen with chondrosarcoma of bone with a minimum of two years follow up. A total of 532 patients were seen with Chondrosarcoma between 1970 and 2006. Patients were excluded if they had initial treatment in another unit (20 patients), a subdiagnosis of dedifferentiated chondrosarcoma (due to very poor prognosis, 43 patients), metastases at presentation (30 patients), if they presented with disseminated metastases prior to local recurrence (12 patients) or were not offered surgery, leaving 402 patients in the study group.

The mean age was 48 years old (range 6–89 years) with the most commonly sites of presentation being in the pelvis in 132 patients (29%), proximal femur in 81 patients (18%), distal femur in 40 patients (9%) and proximal humerus in 40 patients (9%). Grade at presentation was grade 1 in 44%, grade 2 in 44% and grade 3 in 12%. Surgical margins were radical in 3%, wide in 44%, marginal in 29%, planned incisional in 13% and unplanned incisional in 11%. Local recurrence occurred in 87 patients (22%). Local recurrence rates were significantly different for surgical margins on Fisher exact testing (p=0.003), which held true even when stratified by presenting grade of tumour. Surgery for local control was successful in 62% of cases.

Complex relationships exist between surgical margins, local recurrence and survival. Long term survival is possible in 1/3 patients who have local recurrence in intermediate and high grade chondrosarcomas and therefore ever effort should be made to regain local control following local recurrence.

We have compared the cost:benefit ratio of the new type of non invasive extendable prostheses with the old type which required lengthening under general anaesthetic with an invasive procedure.

Over the past four years we have inserted 27 non invasive endoprostheses (cost £14,000). Two have failed to lengthen due to problems with the inbuilt motor. So far there have been no infections, no loosenings and no patient has required revision. The lengthenings are painless and take half an hour. In the past 25 years we inserted 175 extendable endoprostheses (cost £7,000). All lengthenings were performed under a general anaesthetic. The risk of infection was initially 20% at ten years but had decreased to 8%. Pain and stiffness arose in about 10% requiring physiotherapy or occasionally manipulation under anaesthetic.

Assuming the following costs (current NHS cost) are accurate and appropriate, then the non invasive extendable prosthesis becomes cost effective when Cost EPR < Cost old EPR + (Additional risk physio(P) x cost) + (additional cost x number of lengthenings (L)) + (additional cost of revision for infection x risk of infection (R)). 14000 < 7000 + (300 x P) + (1500 x L) + (20000 x R). Assuming a 10% need for physiotherapy, four lengthenings and a 10% risk of infection gives: 14000 < 7000 + (30) +(6000) + (2000) = 15030.

Given the high complication rate of the old type of extendable procedure and assuming there are few if any with the non invasive type, then the non-invasive endoprostheses becomes cost effective if more than three lengthenings are required. They are certainly more popular with parents and children alike!

Pelvic reconstruction after tumour resection is challenging. Pelvic replacements are usually custom made at considerable expense and then need very careful positioning at the time of surgery. They have a very high rate of complications with up to 30% risk of infection and 10% dislocation. In 2003 we developed a new type of pelvic replacement which would be simple to make, simple to use and which would hopefully avoid the major complications of previous pelvic replacements whist being versatile to use even when there was very little pelvis remaining. The concept is based on the old design of Ring stemmed hip replacement and has become known as the ice-cream cone prosthesis. It is inserted into the remnant of pelvis or sacrum and is surrounded by bone cement containing antibiotics. One of the main advantages is it’s flexibility, allowing insertion after resection at a variety of levels. It is also suitable for patients with metastatic disease.

We have inserted 12 of these implants in the past 4 years, resolving very difficult reconstruction problems. There was one case that became infected but was cured with washout and antibiotics. In one patient there was excessive leg lengthening resulting in a sciatic and femoral nerve palsy and the prosthesis had to be revised. All patients can walk with one stick or less.

These results are encouraging and suggest that this versatile implant may be the way forward for pelvic reconstruction because of it’s flexibility of use and low complication rate.

Aim: To review our experience of managing patients with aneurysmal bone cysts (ABCs).

Method: We reviewed the medical records and radiographs of all patients with ABCs treated at our unit over a 25 year period. During that time the policy of the unit was to treat ABCs with biopsy/curettage without use of adjuvants or bone grafting. Patients were followed up with regular Xrays until healing had taken place. Local recurrences were again treated with curettage, occasionally supplemented with embolisation or bone grafting.

Results: 237 patients (128 female, 109 male), with a median age of 14 yrs (range 1 to 76), received treatment. The cyst size varied from 1 to 20 cm and the median duration of symptoms was 16 weeks (range 0 to 8 yrs). The most common sites were the tibia (55), followed by the femur (41), then the pelvis (29) and the humerus (27). Thirty-five (15%) of the patients presented with a pathological fracture.

Primary treatment was by curettage alone in 195, curettage and bone grafting in 7, aspiration and injection of steroids or bone marrow in 7, excision in 5 and observation alone in 17. The rate of local recurrence requiring further surgery was 12% with all local recurrences (but one) arising within 18 months. Local recurrence was not related to site, age, sex or whether the patient had previous treatment or not. Local recurrences were managed with curettage alone in 19 of the 23 cases, with one having embolisation, one excision and 2 curettage and bone grafting. This was successful in all but 3 cases who were controlled with a third procedure.

Conclusion: The local control rate of ABCs with simple curettage is 88%, which is as good as the results published for any other technique. We recommend biopsy in all cases with limited curettage at the same time, and many ABCs will heal with this simple procedure. Full curettage is needed for those showing no signs of healing within 4 weeks. Local recurrence is very unusual after 18 months.

Of 3000 patients diagnosed with primary malignant bone tumours and treated at our unit over the past 25 years, 234 (7.8%) were considered to be spindle cell sarcomas of bone (ie not osteosarcoma, chondrosarcoma, Ewing’s, chordoma or adamantinoma). We have analyzed their management and outcomes.

The diagnosis of these cases varied with fluctuations in the popularity of conditions such as MFH, fibrosarcoma and leiomyosarcoma with the passage of time. Treatment was with chemotherapy and surgery whenever possible. 36 patients had metastases at diagnosis and 17 had palliative treatment only because of age or infirmity. The most common site was the femur followed by the tibia, pelvis and humerus. The mean age was 45 and the mean tumour size 10.2cm at diagnosis. 25% of patients presented with a pathological fracture. Chemotherapy was used in 70% of patients the most common regime being cisplatin and doxorubicin. 35% of patients having neoadjuvant chemotherapy had a good (> 90% necrosis) response. The amputation rate was 22% and was higher in patients presenting with a fracture and in older patients not having chemotherapy.

With a mean follow up of 8 years the overall survival was 64% at 5 yrs and 58% at 10 yrs. Adverse prognostic factors included the need for amputation, older age and poor response to chemotherapy as well as a pathological fracture at presentation. The few patients with angiosarcoma fared badly but there was no difference in outcomes between patients with other diagnoses.

We conclude that patients with spindle cell sarcomas should be treated similarly to patients with osteosarcoma and can expect comparable outcomes. The histological diagnosis does not appear to predict behaviour.

Enchondromatosis is a non-hereditary disease, characterised by the presence of multiple enchondromas. While Ollier Disease is typified by multiple enchondromas, in Maffucci Syndrome they are combined with haemangioma.

Due to the rarity of these diseases, systematic studies on clinical behaviour providing information how to treat patients are lacking.

This study intends to answer the following questions: What are predictive factors for developing chondrosarcoma? When is extensive surgery necessary? How often patients die due to dedifferentiation or metastasis?

Twelve institutes in eight countries participated in this descriptive retrospective EMSOS-study. 118 Patients with Ollier Disease and 15 patients with Maffucci Syndrome were included. Unilateral localization of disease was found in 60% of Ollier patients and 40% of patients with Maffucci Syndrome.

One of the predictive factors for developing chondrosarcoma is the location of the enchondromas; the risk increases especially when enchondromas are located in the scapula (33%), humerus (18%), pelvis (26%) or femur (15%). For the phalanges, this risk is 14% in the hand and 16% in the feet. The decision whether or not to perform extensive surgery is difficult, especially in patients who suffer multiple chondrosarcomas.

Malignant transformation was found in fourty-four patients with Ollier Disease (37%) and eight patients with Maffucci Syndrome (53%). Multiple synchronous or metachronous chondrosarcomas were found in 15 patients.

Nine patients died (range 21–54 yrs). Seven of them died disease related due to pulmonary metastasis (2 humerus, 2 pelvis, 3 femur). Two patients died from glioma of the brain.

In conclusion, one important predictive factor for developing chondrosarcoma is the location of the enchondromas; interestingly, only patients with chondrosarcoma outside the small bones died of their disease. In this series, no dedifferentiation of chondrosarcoma was seen. A first design flow-chart how to approach chondrosarcoma in patients with Ollier Disease and Maffucci Syndrome is in preparation.

Synovial Sarcoma of the foot is the most common soft tissue sarcoma to present in the foot. Despite this, diagnosis is often delayed and treatment may be difficult. The aim of this paper is to review the presenting features, management and outcome of synovial sarcoma of the foot and to try and identify areas for improvement. 33 patients with synovial sarcoma were treated at out unit over a 25 year period. The average duration of symptoms was 125 weeks. The age range at presentation was 11 to 80 years (mean 44). The mean size of the tumour at diagnosis was 5cm in diameter (range 1–10cm). 75% of the tumours were deep at the time of diagnosis. Treatment was by amputation in 21 patients and limb salvage in 11 with 1 patient receiving palliative chemotherapy. Only one patient had local recurrence and presented with lung metastases. Four patients had metastases at diagnosis and nine developed them subsequently. Overall survival at 10 years was 53%. These results have shown that late diagnosis is common for synovial sarcoma of the foot and that by the time of diagnosis 75 % have invaded extra-compartmentally leading to a high risk for amputation. 20 patients had an inadvertent excision of the tumour before referral to our unit. Local control was best achieved with amputation. Overall survival was surprisingly poor despite successful local control. Earlier aggressive investigation of patients with foot pain or swelling but no other features may change their long term prognosis.

Low grade central osteosarcoma is a rare intramedullary bone producing tumour. It accounts for only 1–2% of all osteosarcomas. Due to the indolent nature of low grade central osteosarcoma, achieving a correct and prompt diagnosis is the real challenge both from imaging and histology, particularly as it may resemble a benign condition, i.e. Fibrous Dysplasia.

We have reviewed 15 cases of low grade central osteosarcoma with long term follow-up (2 to 22 years) to identify problems in diagnosis and treatment and to assess outcome.

There were 7 females and 8 males with a mean age of 37 yrs (range 11 to 72 years); 13 cases arose in the lower limb (8 femur, 4 tibia, 1 os calcis), 1 in the pelvis and 1 in the upper limb. The average duration of symptoms prior to presentation was over 2yrs. A primary diagnosis of low grade central osteosarcoma was achieved for only 6 cases (4 open and 2 needle biopsies), in the other 9 the primary diagnoses were GCT, cystic lesion or fibrous lesion (both benign and malignant) and all of them had undergone treatment (usually curettage with or without bone grafting for this). Definitive treatment was with surgery attempting to obtain wide margins. Marginal excision was associated with local recurrence in three cases but there were no local recurrences in patients who had a wide excision, even in those with prior treatment. Only one patient has died following the development of multiple metastases after 9 years. The survival rate is 90% at 15 years.

We present this study to show the difficulties in diagnosing this rare type of osteosarcoma and to highlight the importance of wide surgical margins to obtain local control.

Aim: To investigate the effectiveness of silver coated titanium prostheses in preventing periprosthetic infection in a group of very high risk patients.

Methods: Periprosthetic infection is one of the main problems in limb salvage surgery, especially for tibial and pelvic prostheses or following revision surgery, particularly if it has been done for a previous infection. We have used silver coated prostheses in 21 cases on a named patient basis and have now assessed the results.

Results: Between July 2006 and June 2008 21 patients had a silver coated prosthesis inserted. 11 patients were having a second stage revision after a previous infection, 6 were having a pelvic prosthesis inserted, 3 were having a primary tibial replacement and one a one stage proximal tibial revision. Three patients developed a postoperative infection, two of the pelvic replacements and one infected revision (a total femur replacement). Of these only one patient required removal of the prosthesis (for overwhelming coliform infection in a pelvic replacement) whilst the other two infections both settled with antibiotics and washout.

Discussion: The anticipated risk of infection in this high risk group would have been around 20%. The actual infection rate was 14% but two of the infections completely resolved with relatively modest treatment. This suggests that the silver coating may not only have a role in preventing infection but also enhancing control of infection should it arise. There were no other side effects and we believe that these preliminary results are encouraging and should lead to a further evaluation of silver for preventing infection around prostheses.

Malignant tumours of the radius compose only 3% of all upper limb tumours. Owing to their rarity they are often difficult to manage satisfactorily. Of the options for fixation available, endoprosthetic replacements have been scarcely utilized despite their success in limb preservation with malignant tumours in other parts of the body. At our centre we have used these when biological solutions (eg fibula graft) were not indicated due to extensive disease or the need for radiotherapy.

We performed four endoprosthetic replacements of the distal radius in three males and one female with ages ranging from 19–66 years (average= 42.25 years of age). Two were performed for varieties of osteosarcoma (parosteal and osteoblastic osteosarcomas), one for a large destructive giant cell tumour (GCT) and one for destructive renal metastases. Three were right sided (75%) and one left sided (25%).

Medical records were evaluated for information on local recurrence, metastases, complications and functional outcome using the Toronto Extremity Salvage Score (TESS). Follow up ranged from 22 to 205 months (average= 116.5 months). The average TESS score was 58.1% (range= 44.6–74.5%). Neither case of osteosarcoma recurred. The GCT recurred twice and the patient with renal metastases had nodules removed from his affected wrist on two further occasions. There were no cases of infection, but the two earlier cases had problems with metacarpal stems cutting out and jointsubluxatinos. The two earlier cases have since died at 205 (parosteal osteosarcoma) and 189 months (GCT) respectively of other disease.

We conclude that although this is a very small series of endoprosthetic replacement of the distal radius, the technique is a useful addition to the surgical options, with acceptable postoperative functional results and complication rates when a biological solution or preservation of the wrist joint is not indicated.

Background: ASPS is a rare, high grade sarcoma primarily affecting children and young adults. Its origin remains enigmatic and there has until recently been no diagnostic markers. Diagnostic problems particularly occur when presenting as metastasis before detection of the primary tumour and when there is morphologic overlap with other malignancies. Recently, identification ASPS/TFE3 fusion transcripts and immuno-detection of TFE3 have been reported as useful diagnostic tools.

Design: 17 ASPS were analysed in terms of clinicopatho-logic characteristics, treatment and follow up. Archival formalin-fixed and paraffin embedded tissues were used for TFE3 immuno-histochemistry and RNA extraction followed by RT-PCR analysis and sequencing. Novel primers to detect ASPS/TFE3 fusion transcripts, type 1 and 2, were designed.

Results: The patients, 9 females/8 males, ranged in age from 3 to 46 years (median 23 years); 16 involved the extremities (9 lower, 7 upper) and one the pelvis. All but one patient had primary, curative surgery; chemotherapy and radiotherapy was given for metastatic disease. Five had lung metastases at diagnosis and 3 developed lung and brain metastases later. Four patients died of disease (after 1–5 years), 4 are alive with metastases and 9 are alive and well (after 6 mos.-10 years). 15/15 ASPS showed ASPL/TFE3 fusion transcripts (8 type 1, 7 type 2) and TFE3 immuno-positivity. Of 26 control tumours, several of which with overlapping morphologic features, none had fusion transcripts, 4 showed immuno-positivity (all granular cell tumours).

Conclusions: Immuno-detection of TFE3 and RT-PCR based identification of ASPL/TFE3 fusion transcripts in formalin-fixed/paraffin embedded tissues are powerful tools in the diagnosis of ASPS.

Aim: To estimate the risk of bone malignancy arising in premalignant conditions.

Methods: There are quite a number of possible premalignant conditions with considerable uncertainty about the actual risk of a bone sarcoma developing. The incidence of these malignant conditions was identified from a prospective database containing 3000 primary bone sarcomas.

Results: 178 of the 3000 patients with newly diagnosed bone sarcomas had a pre-exiting condition which in all probability led to the sarcoma. These included 50 with previous radiotherapy treatment and 47 with Paget’s disease. 31 patients developed malignancy in HME, 8 with neurofibromatosis and 7 each with Ollier’s disease and retinoblastoma. There were 4 malignancies in patients with Mafucci’s syndrome, 3 in patients with fibrous dysplasia, 3 in patients with synovial chondromatosis and 2 in patients with Rothmund-Thomson syndrome.

Given that the incidence of bone sarcomas is 9/million population per year, our 3000 patients represent 333 million population years. When the incidence of a condition is known in the population this allows an estimation of the risk of malignancy compared with the normal population. Retinoblastoma for instance is known to arise in 1 in 16000 births. The 7 malignancies we saw thus represents a risk to individuals with retinoblastoma of 336/million/yr – a figure 37 times the risk of the normal population. Approximate figures of risk have been calculated for other entities.

Conclusion: Data from a supra-regional register allows an approximate estimate of the increased risk of bone tumours in premalignant conditions.

Disappearing bone disease is also known as vanishing bone disease, phantom bone disease, massive osteolysis, Gorham’s disease or Gorham-Stout disease. Basically, it is characterised by osteolysis in (contiguous) bone segments, due to localised proliferation of thin-walled vascular channels in the bone and surrounding soft tissues.

The etiology and pathophysiology of this condition remain poorly understood and largely unclear, but there is increasing evidence that disordered lymphangiogenesis plays a role. It is an extremely rare cause of osteolysis, so all other differential diagnoses should be considered and ruled out before retaining the diagnosis of disappearing bone disease.

Treatment is fairly disappointing and no single treatment modality has proven effective in actually arresting the disease. Conservative treatment includes ant-resorptive agents (bisphosphonates), immunomodulating substances and radiation therapy, whereas surgical treatment options include resection and reconstruction with bone grafts and/or prostheses versus amputation.

We report on the only two cases that were identified in our database between 1984 and 2008, both affecting the lower limb (one tibia, one femur). In an attempt to limb salvage, these patients initially underwent endoprosthetic replacement of the affected bone segment, but due to disease progression both eventually ended up with a hip disarticulation.

Conclusion: Although benign, this condition can be very aggressive, necessitating amputation to achieve local control.

Introduction: Limb salvage reconstruction evolved from the treatment of primary bone tumours. Endoprosthetic replacements (EPR) were originally designed for this purpose, but the versatility of these implants has resulted in an extension in the indications for their use. Severe bone loss, failed revision surgery and persistent deep infection present similar challenges and when a salvage procedure is required, EPR are occasionally used. The aim of our study was to assess the medium term survival and functional outcome of EPR.

Materials and Methods: 38 patients (23 females and 15 males), who underwent EPR for non-neoplastic conditions were identified from a prospectively kept database of all patient seen at the Royal Orthopaedic Hospital Oncology Service. The indications for replacement included failed joint replacement, fracture non-union, failed internal fixation and periprosthetic fractures.

The 38 procedures were identified from September 1995 to June 2007 and included 17 distal femoral replacements, 12 proximal femoral replacements, 4 proximal humeral replacements, 2 distal humeral replacements, 2 hemi-pelvic replacements and 1 total femoral replacement. EPR survivorship was calculated using a Kaplan-Meier survival curve. The quality of patients’ mobility and performance of activities of daily living was used to assess functional outcome.

Results: Patients had a mean age of 60 years (range 15–85 years) at surgery and had between 0 and 4 previous operations prior to EPR. Seven out of 38 patients had recorded deep infection prior to surgery (18%). The Kaplan-Meier implant survival was 91.3% at 5 years, 68.5% at 10 years and 45.7% at 20 years. The limb salvage survival for all reconstructions was 75% at 10 years.

87.4% of patients who underwent a lower limb EPR achieved a satisfactory or very satisfactory functional outcome. 100% of patients achieved a satisfactory or very satisfactory functional outcome in the upper limb EPR group.

3 implants failed, 2 as a result of infection and required staged revisions, 1 eventually requiring amputation, and 1 failed as a result of aseptic loosening. 2 patients dislocated their proximal femoral replacements, both were treated successfully by closed reduction. Despite the salvage surgery subsequent amputation was only required in one patient.

Conclusion: EPR appears to be effective and the medium term survival is encouraging. The aim of a pain free functional limb is achievable with this technique. The complication rates are acceptable considering the salvage nature of these patients. We recommend referral of complex cases to a tertiary centre with expertise in this type of surgery.

Aim: To review treatment outcomes in patients with sacral chordoma treated at our centre over the past 20 years.

Methods: Retrospective review of prospectively kept data. Previously treated patients were excluded. The surgical objective was to obtain clear margins. If sacrifice of S2,3,4 was necessary, this was usually combined with colostomy.

Results: 30 patients were treated (20 males, 10 females), median age 63.5 (28 to 94). Median duration of symptoms before presentation was 79 weeks (3–260), mean tumour size 11 cm. Most had neurological symptoms. Eight tumours involved the S2 roots, 1 the entire sacrum. Treatment was palliative in 7 patients, resection in 23. Operation time averaged 4.5 hours (1.5 to 8). Margins were wide in 7, marginal in 12, and intralesional in 4 patients. There was a high rate of postoperative complications, mostly wound problems (61% of patients). In 1 case this resulted in septicaemia and post-operative death. Average operative blood loss was 1600ml (0–3500). 65% of patients were incontinent of urine and/or faeces. Local recurrence (LR) occurred in 52% of operated patients at a median of 32 months (4–134). Incidence of LR was 60% after intralesional, 57% after marginal and 25% after wide surgery (p=0.49). LR was treated with re-excision, radiofrequency ablation, radio- and occasionally chemotherapy. Overall survival (Kaplan-Meier) of all patients was 57% at 5, and 40% at 10 years. Of operated patients this was 67% and 47%. There was a trend for better survival after wide resection margin. Metastatic disease only occurred in 3 patients.

Conclusion: Chordoma of the sacrum is frequently diagnosed late. Resection is associated with a high complication rate. Local recurrence is the most common cause of death. Early referral to a specialist centre is recommended to optimize treatment. The role of adjuvant therapy remains unclear.