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.

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.

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.

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.

Introduction: Renal cell carcinoma often metastasizes to the skeleton and less frequently soft tissues, leading to the increased risk of fracture. Previous studies have show that the survival of patients with bone metastases is frequently prolonged and that early failure of surgical implants designed for fracture fixation is high. The aim of the study was to investigate the outcomes from surgical procedures undertaken at a tertiary referral bone tumour unit.

Methods: A retrospective review of consecutive patients presenting with a musculoskeletal metastasis to our unit from October 1976 to June 2007 was undertaken. There were 179 patients seen over this period, 89 with solitary and 90 with multiple mets. The mean age at presentation was 60.0 yrs. The mean follow up was 16.9 months. Kaplan-Meyer implant and patient survival analysis was undertaken.

Results: The mean patient survival was 22 months (range 1 month to 16 years). The 1yr, 3yr and 10yr patient survival was 77 %, 44 % and 11 % respectively. The types of surgical treatment undertaken included EPR in 119 patients, conventional arthroplasty in 15 patients, fracture fixation in 14 patients, excision only in 12 patients, amputation in 9 patients, curettage and cementation in 9 patients, decompression stabilization of spine in 1 patient. Two femoral EPRs failed due to infection, requiring hindquarter amputations. Failure of fixation to the humerous lead to a forequarter amputation, two hip dislocations. Failure of curettage and cementation in the acetabulum and proximal tibia were treated with THR and EPR respectively.

Discussion: Patients with renal cell carcinoma metastases can survive for prolonged periods. Any surgical intervention for bone metastases has to outlive the patient and EPRs are reliable with a low rate of failure or complication. We would recommend resection and reconstruction with endoprostheses for solitary renal metastases in a tertiary referral unit.

Introduction: The use of extendible endoprosthetic implants in the skeletally immature has been used for just under 30 years. Limb salvage has become a realistic alternative to those children presenting with primary bone sarcomas. We aim to review the use of an implant which uses a non-invasive mechanism of adjusting the length of the prosthesis, during the growth phase.

Method: A retrospective review of consecutive patients undergoing primary or revision endoprosthetic replacement with non-invasive extensible implants, was undertaken. Between January 1993 and February 2008, 34 children were treated with non-invasive extensible endoprosthetic replacements, 26 distal femur, 5 total femurs, 3 proximal tibias and 1 proximal femur.

Results: The underlying pathology, requiring excision, was Ewings sarcoma in 4 patients and osteosarcoma in the remaining 30 patients. Most underwent pre-operative chemotherapy and 2 patients died of their disease.

Four operations were secondary procedures following previous non-grower implant failures (1 infection of previous EPR, 1 IM nail non-union, 1 failed allograft and a revision of a proximal femoral EPR to a total femoral prosthesis). Five patients required revision of the primary prosthesis (2 with motor failures, 3 due to prosthesis infections).

Mean time to start lengthening from surgery was 12.2 months. The mean number of lengthenings was 4 with an average total length of 30 mm achieved, mean leg length difference was 0.8 cm. All lengthenings were undertaken with the patient fully alert, no adverse incidents occurred at the time or after lengthening.

Discussion: The non-invasive prostheses show promise in handling the difficult problem of limb preservation in a growing child, with similar complication rates to that of an invasive type, but without the need for multiple anaesthetics for lengthening.

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.

Introduction: The aim of this study was to identify the pattern of symptoms in patients presenting with synovial sarcoma, examine how these corresponded to the symptoms outlined by the NICE guidelines on the rapid referral of patients with a suspected sarcoma and spot factors that led to long delays in diagnosis.

Methods: Early symptoms and the results of clinical and radiological investigation were reviewed along with the presumed diagnoses that had been made for 35 children. The total duration of symptoms was separated into patient delay and doctor delay.

Results: Using the four clinical findings suggestive of sarcoma according to the NICE guidance, only half of the patients had one or more of them at the time of initial symptoms. The most common presentation was a painless mass (n=16), and in 10 children there was no mass identified. Seven patients had an inexplicable joint contracture, many having been extensively investigated unsuccessfully. The mean duration of symptoms before the diagnosis was made was 98 weeks (range 2 to 364). The mean number of doctors seen prior to referral was 3 (range 1–6) and for 15 patients the diagnosis was obtained after inadvertent excision. The factors associated with long duration of symptoms before diagnosis were knee and elbow location (p=0.0047) or periarticular location (p=0.01), absence of lump (p=0.016) or painful mass as early symptom (p=0.04), the presence of calcifications on x-rays (p=0.01) and a fixed joint contracture (p=0.0003).

We could not show that delay in diagnosis led to a worse prognosis.

Discussion: This paper highlights the sometimes bizarre symptoms associated with synovial sarcoma and hopefully this will increase awareness of the condition among relevant sections of the medical profession and help to reduce the delay in diagnosing these cases.

Introduction: Modular tumour prosthetic replacement is especially useful in the region of proximal femur following pathological fractures and failed fixation. The aim of the study was to assess the clinical and functional outcomes following modular tumour prosthesis reconstruction of the proximal femur in 100 consecutive patients with metastatic tumours and to assess its cost effectiveness.

Methods: The study was a retrospective review of 100 consecutive patients who underwent modular tumour prosthetic reconstruction of the proximal femur using the METS prosthesis [Stanmore Implants Worldwide] for metastatic tumours from 2001 to 2008.

Results and conclusion: There were 45 male and 55 female patients. The mean age was 60.2 years. The indications were metastasis [23renal ca, 28 breast ca, 11 ca bronchus, 5 ca prostate and 31 others]. 75 patients presented with pathological fracture or with failed fixation and 25 patients were at a high risk of developing fracture. The mean follow up was 24.6 months [range0–74]. Three patients died within 2 weeks following surgery. Of the 60 patients who were dead 58 did not need revision surgery indicating that the implant provided single definitive treatment which outlived the patient. 1 patient had revision surgery. There were 2 dislocations. 6 patients had deep infections. The implant survival was 98% with revision or amputation as end point. The hospital cost of an endoprosthetic replacement is estimated to be £12,000. This procedure becomes cost effective when compared with no treatment if the patients’ life expectancy is more than 40 days and when compared with internal fixation if the patients’ life expectancy is more than 2 years.

We conclude that METS modular tumour prosthesis for proximal femur provides versatility; low implant related complications and acceptable function lasting the lifetime of the patients with metastatic tumours of the proximal femur providing a cost effective solution.

Improving outcomes is important for any patient with any disease. Defining the outcome measure will effect what steps need to be taken to improve it. In 2006 the National Institute of Clinical Excellence (NICE) published evidence based Guidance on measures to be taken in the UK to improve outcomes for patients with sarcomas. The Guidance established the following principles:

That all patients with sarcomas must be treated by a recognised and properly constituted multi disciplinary team

How this Guidance has been implemented and what implications this may have for other countries will be discussed.