Aim

To identify patient, tumour or treatment factors that influence outcome in patients with radiation induced sarcoma of bone.

Dedifferentiated chondrosarcoma is a rare but highly malignant manifestation that can occasionally arise in patients with cartilage tumours. There remains uncertainty as to the best treatment for this condition and in particular whether chemotherapy may have a role in improving prognosis.

Members of EMSOS were invited to contribute data on patients, tumours, treatment and outcomes of patients with dedifferentiated chondrosarcoma.

Eight centres contributed data on 317 patients from 7 countries. The mean age was 59 (range 15 to 89) and the most common site was the femur (46%) followed by the pelvis (28%). 25% of patients presented with a pathological fracture and the most common high grade component identified was MFH. 23% had metastases at diagnosis and these patients had a median survival of 5 months. 30% of patients received chemotherapy, with 47% under 60 having chemotherapy compared with 10% over 60. One third of this group had neoadjuvant chemotherapy and the rest had adjuvant reatment. 88% had surgery with limb salvage in 80% of this group. The overall survival was 38% at 2 years and 24% at 5 years but in patients without metastases at diagnosis these figures were 44% and 28% respectively. Poor prognostic factors for survival were: metastases at diagnosis, amputation or no operation, local recurrence, age over 60 and pathological fracture at presentation. We were unable to identify any group in whom chemotherapy appeared to have a survival benefit.

Dedifferentiated chondrosarcoma carries a dismal prognosis. Although 30% of patients received chemotherapy in this study we were not able to prove that it improved survival. Early diagnosis and complete surgical excision still offer the best prognosis for this condition.

Primary malignant bone tumours frequently arise in children close to the knee, hip or shoulder. Resection of the tumour will often require excision of the epiphysis and frequently one side of the involved joint. In these children an extendable endoprosthesis is usually required to allow for maintenance of limb length equality.

We have used 180 extendable endoprostheses in 176 children since 1975. The indication for use of an extendable prosthesis was if there was more than 30mm of growth remaining in the resected bone. The age of the patients ranged from 2 to 15 and 99 were boys. The sites of the endoprostheses used were: distal femur in 91, proximal tibia in 42, proximal femur in 11, total femur in 6 and proximal or total humerus in 26. 131 of the operations were for osteosarcoma and 34 for Ewing's.

Five types of lengthening mechanism have been used. Two designs used a worm screw gear, one type used a C collar, one type a ball bearing mechanism and the latest uses a non invasive lengthening system whereby a motor inside the prosthesis is activated by an electromagnetic field.

Of the 176 patients, 59 have died and of the remainder, 89 have reached skeletal maturity. 19 patients had an amputation, 11 due to local recurrence and 8 due to infection. The risk of infection was 19% in surviving patients. Most of the skeletally mature had equal leg lengths. The average number of operations was 11 but ranged between 2 and 29. Most operations were for lengthening but younger children always needed revisions of the prosthesis. Functional scores were 77%.

Extendable endoprostheses are demanding both for the patient and the surgeon. The high complication rate should be decreased by non invasive lengthening prostheses.

All patients referred to our unit with previously untreated metastatic renal cancer were included in this review. We investigated likely prognostic factors including age, sex, site, synchronous or metachronous metastasis, stage of the disease and the type of treatment received.

From 1976 until 2004, a total of 198 patients were treated by our unit for renal metastases. 15 patients were excluded because they were referred after failure of previous treatment or only had advice. 96 patients were already known to have renal metastasis with their diagnosis having been made between 0.2 and 17 years from the diagnosis of primary cancer (mean 4 years). 33 patients presented to us with a pathological fracture and were found to have renal cancer. A total of 54 patients had multiple metastases and 129 had a solitary metastasis. The cumulative survival from the time of diagnosis of the bone metastasis is 70 percent at 1 year, 40% at 3 years and 18% at 5 years. In patients with a solitary metastasis, the overall survival was 74% at 1 year and 45% at 3 years, whereas in patients with multiple metastases it was 55% at 1 year and 22% at 3 years. (p=0.02) In patients with a solitary metastasis treated by excision of the metastasis, the survival at 1 year was 86% as compared to 38% for those that were treated with just a local procedure. Cox multivariate analysis shows that survival was better in those with solitary metachronus metastasis who underwent a radical procedure.

Introduction: Soft tissue tumours are common in the foot however few are malignant. Synovial Sarcoma of the foot is the most common soft tissue sarcoma to present in the foot (~50%). 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.

Method: The clinical presentation, radiological features and treatment of 33 patients with histologically proven synovial sarcoma of the foot were reviewed along with outcomes in terms of local control, metastases and survival.

Results: 33 patients with synovial sarcoma were treated at out unit over a 25 year period, 22 in the last 10 years. The average duration of symptoms was 125 weeks. The age range at presentation was between 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%.

Discussion: 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.

Conclusion: Earlier aggressive investigation of patients with foot swelling or pain may influence their long term prognosis.

Background: There is currently no standard follow up protocol for patients who have been diagnosed with and treated for high-grade osteosarcoma. We therefore investigated the possibility of creating a risk based follow-up protocol for patients with primary osteosarcomas.

Methods: 313 patients diagnosed with primary osteosar-comas were studied. The identified risk factors for local recurrence included poor necrosis, inadequate margins and high risk tumour site in the bone. The risk factors for metastases were poor necrosis, inadequate margins, extracompartmental stage and tumour size ≥5cm.

Results: The risk of local recurrence and/or metastases within three years of diagnosis increases as the number of risk factors increase. Patients were grouped according to their number of risk factors. The cumulative risk of metastases for patients with 0, 1, 2, 3 and 4 risk factors is 0%, 12%, 21%, 54% and 60% respectively (p=< 0.0001). Risk of local recurrence for patients with 0, 1, 2 and 3 risk factors is 5%, 14%, 25% and 20% respectively (p=0.0025).

Conclusion: Our investigation shows that by grouping patients together according to their number of identified risk factors, it is possible to identify groups of patients that are most at risk. This information can be used to design an evidence based follow up protocol which would have important implications for clinical practice.

Background: 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, endo-prosthetic 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.

Patients: 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%).

Methods: Medical records were evaluated for information on local recurrence, metastases, complications and functional outcome using the Toronto Extremity Salvage Score (TESS).

Results: 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 one of the earlier cases had problems with metacarpal stems cutting out and joint subluxation. The two earlier cases have since died at 205 (parosteal osteosarcoma) and 189 months (GCT) respectively of other disease.

Conclusions: 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 post-operative functional results and complication rates when a biological solution or preservation of the wrist joint is not indicated.

Background & Methods: We reviewed the treatment of 311 patients with primary osteosarcoma treated at a specialist centre between 1995 and 2004. Of this number we identified 8 patients (2.5%) who had undergone inappropriate surgical treatment otherwise known as a ‘whoops’ procedure. These patients were studied to determine the impact of the whoops operations on their subsequent treatment and prognosis.

Results: The whoops procedure consisted of arthroscopic surgery in 3 patients, open reduction and internal fixation of pathological fractures in 2 patients, excision in 2 patients and curettage in 1 patient. The patients who had a whoops procedure were three times more likely to be male, older (median age 25 versus 16 years), had tumours in the lower extremities and were more likely to have amputation as primary local treatment (40% versus 25%). the rates of local recurrence and survival were similar in those with whoops procedures and without.

Conclusion: Whoops procedure is associated with increased risk of amputation and more extensive procedures but does not appear to affect the prognosis of the patient.

Non-invasive expandable prostheses for limb salvage tumour surgery were first used in 2002. These implants allow ongoing lengthening of the operated limb to maintain limb-length equality and function while avoiding unnecessary repeat surgeries and the phenomenon of anniversary operations.

A large series of skeletally immature patients have been treated with these implants at the two leading orthopaedic oncology centres in England (Royal National Orthopaedic Hospital, Stanmore, and Royal Orthopaedic Hospital, Birmingham).

An up to date review of these patients has been made, documenting the relevant diagnoses, sites of tumour and types of implant used. 74 patients were assessed, with an age range of 7 – 16 years and follow up range of 4 – 88 months.

We identified five problems with lengthening. One was due to soft tissue restriction which resolved following excision of the hindering tissue. Another was due to autoclaving of the prosthesis prior to insertion and this patient, along with two others, all had successful further surgery to replace the gearbox. Another six patients required mechanism revision when the prosthesis had reached its maximal length. Complications included one fracture of the prosthesis that was revised successfully and six cases of metalwork infection (two of which were present prior to insertion of the implant and three of which were treated successfully with silver-coated implants). There were no cases of aseptic loosening.

Overall satisfaction was high with the patients avoiding operative lengthening and tolerating the non-invasive lengthenings well. Combined with satisfactory survivorship and functional outcome, we commend its use in the immature population of long bone tumour cases.

Introduction: Giant cell tumor (GCT) is a benign but locally aggressive tumor that primarily affects the epiphyses of long bones of young adults. Pulmonary metastases in giant cell tumor are rare. We report our experience of treating pulmonary metastatic GCT of bone over the last 24 years between 1984–2007.

Methods: A retrospective review of patients’ records and oncology database of patients with metastatic GCT

Results: We had 471 patients with GCT of bone out of which 7 patients developed pulmonary metastases (1.48%). Six patients following diagnosis and initial treatment and one with pulmonary metastases present at the diagnosis. There were 4 males and 3 females aged between 23 to 40 years (median, 27 years). All patients had GCT around the knee (distal femur/proximal tibia). All patients eventually required Endoprosthetic Replacement apart from one who was treated with curettage only. The time of pulmonary metastases from initial diagnosis was 16–92 months (median, 44.6 months). All patients who developed metastases in the postoperative period had thoracotomy for excision of the pulmonary metastases. Two patients received chemotherapy for control of the local recurrence. At an average follow up of 151 months (27–304 months), all patients were alive

Discussion: Pulmonary metastases have been reported as 1% to 9% in GCT. Because of its rarity, very little is known about the long-term outcome and the best treatment for the pulmonary lesions. The mortality rates recorded for patients with pulmonary metastatic GCT range from 0% to 37%. In our series the mortality rate was 0% and metastases 1.48%. It seems that surgical resection of pulmonary metastases gave excellent rate of survival.

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.

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.

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.

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.

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.

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.

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: 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.

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 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.

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: 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.

The definition of a ‘safe’ tumour margin remains controversial. Enneking’s original definition of intralesional, marginal, wide and radical margins was based on a study of just 40 patients of whom only 12 had limb salvage surgery. Since that time thre have been numerous attempts to try and define tumour margins more clearly based on anatomical structures and distances of the tumour from the cut edge. Whilst all can agree on what is a radical margin (usually an amputation) and what is an intralesional margin (tumour exposed) there remains a lack of clear definition of what comprises a wide margin and what is a marginal margin. As an example three large tumour units were asked to assess their margins for adequacy. While the rated of adequate excision varied from 49% to 70%, all 3 units had near identical local recurrence rates of 17–20%. The rate of local recurrence is also modified by adjuvant therapy. A suggestion for an International study assessing tumour margins is made, investigating both anatomical and biological factors.

Aim: To identify prognostic factors for patients with sarcomas.

Method: A retrospective analysis of all patients with newly diagnosed referred to a specialist musculoskeletal tumour centre, with a minimum 5 year follow up. Factors affecting survival and recurrence were identified.

Results: There were 1175 patients with soft tissue sarcomas and the most common diagnoses were liposarcoma (22%) followed by mfh (12%) and leiomyosarcoma (11%). 55% were high grade, 25% intermediate and 20% low grade. The most common site was the thigh (44%). 9% had metastases at diagnosis and of these only 10% survived 5 years. Prognostic factors were analyzed in the remaining 1075 patients. The 5 year disease specific survival was 87% for low grade, 66% for intermediate grade and 48% for high grade sarcomas. Prognostic factors (p< 0.05) on univariate analysis were grade, depth, size, age, sex, wide margins of excision, limb salvage (vs amputation), having had a previous excision (whoops). Many of these factors however were inter-related and on multivariate analysis it was found that the following were still significant: Grade, size, depth, age and buttock location (all p< 0.0005). There were 2320 bone tumours (954 with osteosarcoma, 454 with chondrosarcoma and 463 with Ewings). Prognostic factors for all tumours were size, good response to chemotherapy and limb salvage (as opposed to amputation)

Conclusion: This study has confirmed the generally held factors for prognosis of sarcomas. It is largely tumour factors, not treatment factors which dictate outcome. Of all the factors leading to a poor prognosis, size is the only one which can appreciably be altered – by earlier diagnosis.

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.

Introduction: Current practice in the UK is to perform clinical follow up on patients with soft tissue sarcoma (STS) and only to request cross sectional imaging if there is clinical suspicion. We aimed to assess the effectiveness of this policy by investigating the patients who developed a local recurrence (LR) after previous treatment for STS and whether the size and extent at diagnosis of LR affected their subsequent management

Method: We reviewed the clinical records and imaging of all patients with diagnosed LR of STS to investigate the site and size of the LR and its management.

Results: 35 patients were diagnosed with LR over a 3 year time period. The average time from previous definitive treatment was almost 19 months. The suspicion was made by the patient in 18 patients, by a doctor in 16 and a district nurse in 1. The patients had MRI to confirm the diagnosis followed by biopsy or excision biopsy. 31 of the tumours were high grade, 2 low and 2 were not excised. The size at time of LR was 5.6 cm (range 0.5–15). 12 of the patients had concomitant metastases. 16 were treated by local excision and 5 by amputation whilst 14 patients had palliation. It was felt that with earlier diagnosis the clinical management would have been different in:

those 7 whose LR was inoperable due to size,

Discussion: Earlier diagnosis could have led to simpler surgery in a third of the affected patients. This study raises the question as to whether a more aggressive policy of follow up with regular imaging (either MRI or ultrasound) could detect LR earlier and lead to improved outcomes.

We reviewed the treatment and clinical outcome of 32 consecutive patients with Ewing’s sarcoma who presented with or developed pathological fracture after biopsy between 1984 and 2004. The minimum follow-up was 18 months. The mean age at diagnosis was 20 years (5 – 51). There were 18 males and 14 females. All patients were newly diagnosed and had localized disease at the time of diagnosis. 21 patients presented with pathological fracture while 11 patients developed fracture during the course of chemotherapy. The femur was the most common location in 15 patients.

All the patients had chemotherapy according to the protocol current at the time of treatment. 6 patients had radiotherapy alone while 26 patients underwent surgical excision and reconstruction. Of the patients who had surgery, 7 patients had adjuvant radiotherapy. Fracture healing was the norm after pre-operative chemotherapy. Surgical margins were wide in 17 patients, marginal in 4 and intralesional in 3 patients.

Local recurrence developed in one patient (3%). Metastases occurred in 12 patients (37%). At the time of review 16 patients were free of disease, 3 were alive with disease and 13 patients had died of disease. The cumulative 5 year metastases free and overall survival in all the patients was 58% and 61 % respectively and similar to patients with Ewing’s sarcoma without fracture treated at our centre. The prognosis of patients who presented with fracture was exactly similar to those who developed fracture in the course of treatment.

We conclude that limb preserving surgery is perfectly safe in patients with Ewing’s sarcoma who have associated pathological fracture and survival is not in any way compromised. Survival of patients who present with fracture is similar to those who develop fracture in the course of treatment. The exact role of adjuvant radiotherapy in these patients needs to be clarified.

We have investigated whether improvements in design have altered outcome for patients undergoing endoprosthetic replacement of the distal femur following tumour resection.

Survival of the implant and ‘servicing’ procedures has been documented using a prospective database and review of the implant design records and case records.

A total of 335 patients underwent a distal femoral replacement with 162 having a fixed hinge design and 173 a rotating hinge. The median age of the patients was 24 years (range 13–82yrs). With a minimum follow up of 5 years and a maximum of 30 years, 192 patients remain alive with a median follow up of 11 years. The risk of revision for any reason was 17% at 5 years, 33% at 10 years and 58% at 20 years. Aseptic loosening was the most common reason for revision in the fixed hinge knees whilst infection and stem fracture were the most common reason in the rotating hinges. The risk of revision for aseptic loosening in the fixed hinges was 35% at ten years but appears to have been abolished by the use of a the rotating hinge knee with a hydroxyapatite collar. The overall risk of revision for any reason was halved by use of the rotating hinge.

Improvements in design of distal femoral replacements have significantly decreased the risk of revision surgery but infection remains a serious problem for all patients. We believe that a cemented, rotating hinge prosthesis with a hydroxyapatite collar offers the best chance of long term prosthesis survival.

Aim: To identify tumour and treatment factors significant for both local control and survival for patients with chondrosarcoma of the pelvis.

Method: The features of all patients with non metastatic chondrosarcoma of the pelvis treated at a tertiary treatment centre between 1971 and 2001 with more than 2 years of follow-up were analyzed.

Results: There were 106 patients with a median age of 44. There were equal numbers of male and female patients in the group. The median size of the tumours was 12cm. 47 tumours were grade 1, 37 were grade 2 and 22 were grade 3. Treatment involved hindquarter amputation in 33 and excision with or without reconstruction in 73. Clear margins (wide or better) were achieved in 34 cases. The excision was marginal in 30 cases and intralesional or contaminated in 37. Local recurrence arose in 39 patients and was related to adequate margins of excision (p=0.03) and grade (p=0.01). Overall survival was 72% at 5 years, 56% at 10 years and 46% at 15 years. Survival was strongly related to grade (p=0.08) but survival beyond 5 years was most strongly related to the adequacy of the excision margins.

Conclusion: Tumour grade is the most important prognostic factor for chondrosarcoma of the pelvis but the ability to obtain clear margins of excision influences both local control and the prospects for long term survival.

We reviewed the treatment and clinical outcome of 32 consecutive patients with Ewing’s sarcoma who presented with or developed pathological fracture after biopsy between 1984 and 2004. The minimum follow-up was 18 months. The mean age at diagnosis was 20 years (5 – 51). There were 18 males and 14 females. All patients were newly diagnosed and had localized disease at the time of diagnosis. 21 patients presented with pathological fracture while 11 patients developed fracture during the course of chemotherapy. The femur was the most common location in 15 patients.

All the patients had chemotherapy according to the protocol current at the time of treatment. 7 patients had radiotherapy alone while 25 patients underwent surgical excision and reconstruction. Of the patients who had surgery, 7 patients had adjuvant radiotherapy. Fracture healing was the norm after pre-operative chemotherapy. Surgical margins were wide in 17 patients, marginal in 4 and intralesional in 3 patients.

Local recurrence developed in one patient (3%). Metastases occurred in 12 patients (37%). At the time of review 16 patients were free of disease, 3 were alive with disease and 13 patients had died of disease. The cumulative 5 year metastases free and overall survival in all the patients was 58% and 61 % respectively and similar to patients with Ewing’s sarcoma without fracture treated at our centre. The prognosis of patients who presented with fracture was exactly similar to those who developed fracture in the course of treatment.

We conclude that limb preserving surgery is perfectly safe in patients with Ewing’s sarcoma who have associated pathological fracture and survival is not in any way compromised. Survival of patients who present with fracture is similar to those who develop fracture in the course of treatment. The exact role of adjuvant radiotherapy in these patients needs to be clarified.

Synovial sarcoma is a morphologically well-defined neoplasm that most commonly occurs in soft tissue accounting for 5% to 10 % of all soft tissue sarcomas. We reviewed 156 patients with synovial sarcoma of soft tissues treated at a supra-regional centre to determine survival and prognostic factors.

There were 77men and 79 women with mean age at presentation of 38 years (3 to 84). Follow-up periods ranged from 3 to 494 months (median 43 months). Tumor was located in lower extremities in 111patients, upper extremities in 34 patients, and trunk and pelvis in 11 patients. Overall survival was 66% at 5 years and 48% at 10 years. The 5 and 10 year survival for the 23 patients who had metastases at the time of diagnosis was 13% and 0% respectively compared to 75% and 54% for those without metastases at diagnosis. Local recurrence occurred in 18 patients (13%). The significant prognostic factors for survival included presence of metastases at diagnosis and development of local recurrence. Tumour size and depth, age of patients and use of chemotherapy did not significantly influence survival.

We conclude that the clinical factors which influence survival of patients with synovial sarcoma are different from those of soft tissue sarcomas in general. Biological factors may better predict prognostic survival than the usual clinical factors.

Aim: To identify patient, tumour or treatment factors that influence outcome in patients with radiation induced sarcoma of bone.

Method: A retrospective review of an oncology database supplemented by referral back to original records.

Results: We identified 42 patients who presented to our Unit over a 25 year period with a new sarcoma of bone following previous radiotherapy. The age of the patients at presentation ranged from 10 to 84 years of age (mean: 17 years) and the time interval from previous radiotherapy ranged from 4 to 50 years (median: 14 yrs; mean: 17 years). The median dose of radiotherapy given had been 50 Gy but there was no correlation of radiation dose with time to development of sarcoma. The pelvis was the most common site for development of sarcoma (14 cases) but breast cancer was the most common primary tumour (8 cases). 9 of the patients had metastases at the time of diagnosis of the sarcoma. Osteosacoma was the most common diagnosis (30). Treatment was by surgery and chemotherapy when indicated and 30 of the patients had treatment with curative intent. The survival rate was 41% at 5 years for those treated with curative intent but in those treated palliatively median survival was only 6 months and all had died by one year. The only factor found to be significant for survival was the ability to completely resect the tumour, thus limb sarcomas had a better prognosis (66% survival at 5 years) than central ones (12%)(p=0.009).

Conclusion: Radiation induced sarcoma is a rare complication of radiotherapy. Both surgical and oncological treatment is likely to be compromised by previous treatment the patient has received. Despite this 40% of patients will survive more than 5 years with aggressive modern treatment.

Purpose: To study the results of treatment of symptomatic non-union with endoprosthesis at the Royal Orthopaedic Hospital

Methods: Between 1987 & 2005, 17patients were treated with massive endoprosthesis for non-union. We performed a retrospective review of these case notes

Results: Mean age at diagnosis was 63years (range 36–86). Location of non-union was distal femur in 9, proximal femur in 4, proximal humerus in 2, proximal tibia in 1, distal humerus in 1. The majority of the patients had received prior multiple operations before endoprosthetic surgery.

Four patients had obvious infection confirmed by histology and/or microbiology prior to surgery. Endoprosthetic Reconstruction was performed as a 1 stage procedure in 13 and as a 2 stage in 4.

Complications occurred in 5 patients. These included recurrence of infection in 1, persistent pain in 1, aseptic loosening in 1, periprosthetic fracture in 1 and a non ST myocardial infarction in 1. At the last follow-up, (mean 5years, range 1–18years) majority of patients achieved good range of motion and good mobility.

Conclusion: We conclude that endoprosthetic replacement is a reasonable option for treatment of end-stage non-union in carefully selected patients. Adequate mobility and function can be achieved in majority of patients following such treatment

Purpose of the study: To analyze survival and prognostic factors in a series of patients treated for chondrosarcoma of the pelvis.

Material and methods: The series included 106 patients (53 women and 53 men) treated for non-metastatic chondrosarcoma of the pelvis. Minimum follow-up was two years. Mean age at diagnosis was 44 years. Tumors were grade 1 (n=47), grade 2 (n=37), grade 3 (n=22). Conservative surgery was performed in 73 patients (resection with or without reconstruction) and interilio-abdominal disarticulation for 33.

Results: Resection margins were sufficient for 34 patients (wide or radical resection), marginal for 35, and intratumoral or malignant for 37. Local recurrence was noted in 39 patients (37%). Prognostic factors affecting local recurrence were: quality of resection (p=0.03), grade (p=0.01). Overall survival at 5, 10 and 15 years were 72, 56, and 46% respectively. Survival was strongly correlated with grade (p=0.08) and survival after five years was also correlated with resection margins.

Conclusions: In this series, tumor grade was the most important prognostic factor for patients with chondrosarcoma of the pelvis but achieving satisfactory resection with wide margins also has a significant effect on prognosis for local recurrence and long-term survival.