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!

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.

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

Method: We reviewed the medical records and radiographs of all patienst with aneurismal bone cyst 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 humerus (27). 35 (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 (bar 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 those published for any other technique. We recommend biopsy in all cases and limited curettage at the same time, 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.

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.

Introduction: Parosteal osteosarcoma is a rare, mostly low grade tumor arising from the surface of the bone. The aim of the study was to establish risk factors for local recurrence (LR) and death.

Method: Retrospective review of a prospectively collected database with complete up-to-date follow up.

Results: 63 patients were diagnosed and treated at our centre from 1978 to 2007. The most common site was the femur (37) followed by the tibia (10) and humerus (9). The median size was 7cm (2 – 16). At the time of diagnosis 27 (43%) were low grade, 13 (21%) were low grade but had invaded the medullary canal and 8 (13%) had a high grade component; the data was missing in 15 patients (24%). All patients were operated.

LR arose in 11 patients (17.5%) at a mean of 38 months and was related to surgical margins. There were no LR in the 26 wide resections, but the risk of LR was 20% in those with a marginal and 46% in those with an intralesional excision. LR appeared as a higher grade than the primary tumor in 3 patients (27%).

9 (14.3%) patients developed metastases at a median of 22 months (6 – 123). Five patients developed both LR and metastases. Seven patients died from the tumor, 2 patients are alive with metastatic disease.

None of the patients with a low grade tumor and wide or marginal margins died of tumor, but 2 patients (18%) operated intralesional died of tumor.

Discussion: There was a statistically significant relationship between surgical margins and LR (p< 0.001). All patients with low grade parosteal osteosarcoma operated with wide margins have been cured. LR appeared often in higher grade than the primary tumor. Patients with low grade tumor and intralesional margins had 18% risk to tumor-ralated death.

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.

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.

Ewing’s sarcoma principally arises in bone but can also present as a soft tissue tumour. Very few studies have assessed the outcomes of extra-skeletal Ewing’s sarcomas. This study compares the oncological outcomes of the two forms of Ewing’s sarcomas to see if there is any difference in prognostic factors.

198 patients with primary, non metastatic Ewing’s sarcoma diagnosed between 1980 and 2005 were identified from our database. There were 118 males and 80 females with a median age of 15 years. The three most common sites of diagnosis were the femur (24%), pelvis (15%) and tibia (13%). There were 169(85%) bony Ewing’s and 29 (15%) extra-skeletal Ewing’s sarcomas. All patients received chemotherapy. 86% of the patients had surgery for local control but 28(14%) patients had radiotherapy.

The overall survival at five years was 89% and was related to the age of patient (92% < 16years p=0.005), size (p=0.03) and site of tumour (p=0.004) as well as the response to chemotherapy. There was no difference in the overall survival of patients with bony Ewing’s (90%) and extra-skeletal Ewing’s (85%) (p=0.85). There was a 10% risk of local recurrence at 5 years with site of tumour (p=0.01) and surgical excision (p=0.05) being significant prognostic factors. The risk of local recurrence was also not related to the type of Ewing’s sarcoma.

This large series has shown that the oncological outcomes of Ewing’s sarcoma is related to tumour characteristics, patient age and treatment factors and not determined by the tissue component.

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.

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.

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.

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.

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.

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: 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: Chondrosarcoma has always been treated with respect by oncology surgeons, given that it is relatively resistant to chemotherapy and radiotherapy. The importance of the adequacy of surgical resection margins has been previously reported, however, the aim of the study was to study the outcomes of surgery for Chondrosarcoma and determine the role of initial surgical margins and local recurrence on final outcome.

Methods: Electronic patient records were retrieved on all patients seen at a tertiary referral musculoskeletal oncology centre with Chondrosarcoma of bone with a minimum of 2 years follow up. A total of 532 patients were seen with Chondrosarcoma between 1970 and 1/1/2006. 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 (6 patients) and if they presented with disseminated metastases prior to local recurrence (12 patients). This left 451 patients in the study group and the clinicopathological records were reviewed on these patients.

Results: Of the 451 patients, the mean age was 48 years old at diagnosis (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 wide in 45%, marginal in 28% and incisional (including curettage procedures) in 27%. Local recurrence occurred in 88 patients (19.5%). 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. Local recurrence occurred at a mean of 2.8 years, however, 12.5% occurred more than 5 years from diagnosis. There was a significant difference in survival compared between those patients with local recurrent disease and those without on Kaplan Meier analysis; 10 year survival for those without recurrence was 73.1% compared to 41% for those with local recurrence(p< 0.0001, Logrank). On cox regression analysis significant factors affecting survival were pelvic location (p=0.004), local recurrence (p=0.007), age at presentation (p=0.01), marginal margins (p=0.04) and initial tumour grade (p=0.043). There was no significant difference between survival and initial surgical margin when stratified by grade of tumour, possibly as further surgery to improve adequacy of margins. Further sub-analysis is being performed.

Conclusions: There is a complex relationship between surgical margins, local recurrence and margins. It appears that long term survival is possible in approximately 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.

Introduction: The aim of this study was to investigate the results of a series of cases from a single institution with respect to local disease control and patient survival to determine prognostic factors.

Methods: Electronic patient records were reviewed on all patients with STS between February 1963 and January 2007. 2445 patients had over 30 types of STS. 1639 (67%) had not received any treatment prior to presentation, however, 770 patients (32%) had undergone a previous attempted excision. Survival analyses were done using Kaplan Meier and Cox regression analyses, however, for prognostic factor analysis, only patients presenting without prior treatment were included.

Results: Common diagnoses were liposarcoma (292 patients, 12%), synovial sarcoma (242 patients, 10%) and leiomyosarcoma (239 patients, 10%). Most presented in the thigh (950 patients, 39%), arm (325 patients, 13%) or lower leg (275 patients, 11%) and most were deep to fascia (1581 patients, 74%). The mean size was 10.2cm.

Overall cumulative patient survival was 58% at 5 years and 44% at 10 years. Locally recurrent disease occurred in 350 patients (14%), 204 patients (8%) presented with and 720 patients (30%) subsequently developed metastatic disease.

Prognostic factors for locally recurrent disease were arm tumours (p=0.003, HR=0.3), hip tumours (p=0.01, HR=0.31), thigh tumours (p=0.002, HR=0.52), intralesional margins (p< 0.0001, HR=3.7), high grade tumours (p=0.03, HR=1.8), tumour size 3–6cm (p=0.04, HR=0.54) and tumour size 6–10cm (p=0.03, HR=0.63).

Prognostic factors for patient survival were deep location (p=0.02, HR=1.6), high grade tumours (p< 0.0001, HR=4.7), intermediate grade tumours (p< 0.0001, HR=3.4), surgical margins (p=0.04), age at diagnosis (p< 0.0001, HR=1.02), size of tumour < 3cms (p=0.04, HR=0.29), 3–6cms (p< 0.0001, HR=0.41), 6–10cms (p=0.007, HR=0.63), no locally recurrent disease (p=0.0001, HR=0.59).

Conclusions: Significant prognostic factors have been proven for STS, and marginal margins have not been proven to alter the risk of locally recurrent disease or patient survival.