Aims

The existing clinical guidelines do not describe a clear indication for adjuvant radiotherapy (RT) in the treatment of superficial soft tissue sarcomas (STSs). We aimed to determine the efficacy of adjuvant RT for superficial STSs.

Aim

We present the long-term surgical outcomes, complications, implant survival and causes of implant failure in patients treated with the modified Harrington procedure using antegrade large diameter pins.

Aims

The purpose of this study was to report the long-term results of extendable endoprostheses of the humerus in children after the resection of a bone sarcoma.

Aims

The aim of this study was to present the long-term surgical outcomes, complications, implant survival, and causes of implant failure in patients treated with the modified Harrington procedure using antegrade large diameter pins.

Aims

The purpose of this study was to review a large cohort of patients and further assess the correlation between the histological response to chemotherapy in patients with Ewing’s sarcoma with the overall (OS) and event-free survival (EFS).

Bone sarcomas are rare cancers and orthopaedic surgeons come across them infrequently, sometimes unexpectedly during surgical procedures. We investigated the outcomes of patients who underwent a surgical procedure where sarcomas were found unexpectedly and were subsequently referred to our unit for treatment. We identified 95 patients (44 intra-lesional excisions, 35 fracture fixations, 16 joint replacements) with mean age of 48 years (11 to 83); 60% were males (n = 57). Local recurrence arose in 40% who underwent limb salvage surgery versus 12% who had an amputation. Despite achieving local control, overall survival was worse for patients treated with amputation rather than limb salvage (54% vs 75% five-year survival). Factors that negatively influenced survival were invasive primary surgery (fracture fixation, joint replacement), a delay of greater than two months until referral to our oncology service, and high-grade tumours. Survival in these circumstances depends mostly on factors that are determined prior to definitive treatment by a tertiary orthopaedic oncology unit. Limb salvage in this group of patients is associated with a higher rate of inadequate marginal surgery and, consequently, higher local recurrence rates than amputation, but should still be attempted whenever possible, as local control is not the primary determinant of survival.

Cite this article: Bone Joint J 2014;96-B:665–72.

A clinical evaluation of the effect of MRI scan to bring about a change in surgical management of elderly patients who present with hip fracture with no history of trauma or a suspicious looking lesion on x-rays. Many of these patients present with or without history of previous malignancy or bone disorder.

We evaluated that if the delay in treatment within 36 hours as per national guide lines is justified to benefit patients.

Aim

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

Introduction

The role of adjuvants in curettage for giant cell tumours (GCT) is still controversial. Our aim was to determine if adjuvant cementation lowers local recurrence (LR) rates for GCTs treated with curettage.

Aim

To investigate the effectiveness of silver coated prostheses in preventing periprosthetic infection in a high-risk group.

Introduction

The aim of this study is to evaluate the functional and oncological outcome of extracorporeally irradiated autografts as a method of pelvic reconstruction after internal hemipelvectomy.

Osteosarcomas represent a heterogeneous group of primary bone tumours that affect predominantly the long bones of patients in the first two decades of life. We aim to describe the secondary effects of a poor response (⋋90% necrosis) to chemotherapy on the effectivity of other treatment outcomes, local recurrence and survival rates.

182 cases of osteosarcoma with necrosis of less than 90% and no metastases at diagnosis have been seen at our institution over 24 years. There were 60 amputations. 122 patients underwent limb salvage, with 105 marginal margins and 17 contaminated. There was no difference in size or location between the two groups. In the 122 patients with LSS, 21 had adjuvant radiotherapy and 101 did not. In the entirety of patients with ⋋90% necrosis, survival was 64% at 2 years and 37% at 5 years. When LSS Marginal resections were compared with amputation there was a significant (P=0.006) difference in survival. LSS with a marginal margin had a 25% risk of LR. In these patients there was 25% survival, whereas the absence of a local recurrence, conferred a benefit of a 40% survival XRT was used in 21 of the 122 who underwent limb salvage. The decision to use XRT was made by the local oncologist at the treating unit. There was a 24% rate of recurrence in the XRT group and 25% with no XRT.

These data demonstrated that patients who had a poor response to chemotherapy and underwent an amputation faired poorly when compared to patients with LSS. There is a selection bias in patients selected to undergo amputation. Additionally, patients who underwent amputation had a lower rate of local recurrence, but still had a poorer survival when compared to LSS.

Introduction

Myxofibrosarcoma is described by WHO as comprising of a spectrum of malignant fibroblastic lesions with variably myxoid stoma, pleomorphism and with a distinctively curvilinear vascular pattern. They are reported to be one of the commonest sarcomas of elderly patients. It has previously been reported to have a high rate of locally recurrent disease (50-60%). The aim of the study was to investigate the ROH series of tumours to determine prognostic factors for survival and local recurrence.

The aim was to identify when primary amputation was used as primary treatment and to describe outcomes in patients managed with modern chemotherapy. A detailed review of the electronic patient records was undertaken. Statistical analysis was performed with univariate analysis using Kaplan-Meier curves and Chi² testing, whilst multivariate analysis was performed using Cox regression analysis.

There were 354 osteosarcomas. 93 patients presented with metastases and 192 subsequently developed metastases at a mean of 46 months. Amputation was performed as the primary surgical treatment in 101 patients. Endoprosthetic reconstruction was used in 253 patients. Amputation was performed as a secondary procedure on 15 patients.

The 5 and 10 year survival data for all patients, including those with metastatic disease were 60% and 60% for amputation with good chemotherapy response (>89% necrosis), 65% and 63% for limb salvage and good response, 21% and 21% for amputation and poor response (⋋90% necrosis) and 51% and 30% for limb salvage with poor response.

Local recurrence occurred both with amputation (10.8%) or limb salvage (9%), with no significant differences between the two.

Univariate analysis demonstrated that the extent of response to chemotherapy induced necrosis significantly affected survival, whether the patient had an amputation or not.

Whether or not amputation or LSS was used in the surgical management of patients, local recurrence rates where similar between the two groups. Further assessment of chemotherapy-induced necrosis is a key factor in determining subsequent limb salvage or amputation management strategies.

Chondrosarcoma of bone is a surgical disease and excision with wide margins is the optimum treatment. Sometimes the size or location of the tumour at the time of diagnosis mean that only a marginal excision can be achieved. The effect of the margin of excision on outcome is investigated.

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. 87 patients were assessed, with an age range of 5 to 17 years and follow up range of up to 88 months.

Primary diagnosis was osteosarcoma, followed by Ewing's sarcoma. We implanted distal femoral, proximal femoral, total femoral and proximal tibial prostheses. All implants involving the knee joint used a rotating hinge knee. 6 implants reached maximum length and were revised. 8 implants had issues with lengthening but only 4 of these were identified as being due to failure of the lengthening mechanism and were revised successfully. Deep infection was limited to 5% of patients.

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.

Aim

To identify factors leading to the success or failure of extracorporeal irradiation and re-implantation of bone (ECIR).

Aim

To determine the overall survival of patients with Pelvic Ewing's Sarcoma treated in our unit and to identify prognostic factors in pelvic primaries that could be used to select patients who would most likely benefit from high intensity treatment.

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.

Since1986 we have monitored the actual diagnosis of all cases referred to our Unit with the diagnosis of ‘possible primary malignant bone tumour’. We have excluded all patients referred with a known diagnosis of either a benign condition or known to have bone metastases. In most cases the suspected diagnosis was based on X-rays alone, sometimes supported by further imaging.

Neoadjuvant chemotherapy for osteosarcoma improves outcomes for the majority, but if the chemotherapy does not work then the dilemma often arises as to whether to do limb salvage with a marginal (or worse) margin of excision or to do an amputation. If limb salvage is carried out with a close margin, does post operative radiotherapy make any difference? This study aims to address these questions.

The outcome for patients with Ewing's sarcoma recurrence is poor. Local recurrences occur in 8%-25%of these patients. The aim of the study was to analyze the patients who had a local recurrence to identify factors predicting the local recurrence and if it could be prevented

Introduction

The pelvis has always been a difficult area for surgeons, with high complication rates from surgery and the perception of poor oncological outcomes. The aim of the study was to look at the surgical and oncological outcomes of pelvic tumours treated at our centre.

Solitary plasmacytomas in the appendicular skeleton are rare monoclonal expansions of plasmacytoid cells. They are two main hazards; local destruction of bone with resultant loss of function and possible fracture, and progression to Myeloma.

Between February 1988 and July 2005 seven patients (4 male, 3 female) were treated for solitary plasmacytoma with surgical resection and endoprosthetic reconstruction. The median age was 46.7 (35-75). The site was: distal humerus (2), proximal humerus (2) proximal femur (2) proximal tibia (1). Three patients had sustained a pathological fracture. Five patients had received pre-operative radiotherapy and three received post-operative radiotherapy. Mean follow-up is 8.6 years. Two cases became infected at 2 and 5 years post-operatively and have had revisions of their endoprosthesis. Both remain functional at 18 and 15 years. No patient has suffered a local recurrence. Two patients have progressed to multiple Myeloma but no patients have died. Literature review shows that the progression of solitary bone plasmacytoma to Myeloma is around 53% despite radiotherapy, in an average period of 2-4 years. With resection and endoprosthetic reconstruction, the progression in this series has been 28% despite an average follow up of 8.6 years.

Although the numbers are small, due to the rarity of the condition, surgical resection and endoprosthetic reconstruction reduces disease progression than radiotherapy alone. This produces far superior results compared to the intramedullary nailing of the long bones for this condition. Endoprosthetic reconstruction after resection should be given consideration in cases of solitary plasmacytoma of the appendicular skeleton when there is extensive bone destruction present. The optimal timing of local radiotherapy to be combined with surgery is still to be established.

Between 1966 and 2001, 1254 patients underwent excision of a bone tumour with endoprosthetic replacement. All patients who had radiotherapy were identified. Their clinical details were retrieved from their records.

A total of 63 patients (5%) had received adjunctive radiotherapy, 29 pre-operatively and 34 post-operatively. The mean post-operative Musculoskeletal Tumor Society scores of irradiated patients were significantly lower (log-rank test, p = 0.009). The infection rate in the group who had not been irradiated was 9.8% (117 of 1191), compared with 20.7% (6 of 29) in those who had pre-operative radiotherapy and 35.3% (12 of 34) in those who radiotherapy post-operatively. The infection-free survival rate at ten years was 85.5% for patients without radiotherapy, 74.1% for those who had pre-operative radiotherapy and 44.8% for those who had post-operative radiotherapy (log-rank test, p < 0.001). The ten-year limb salvage rate was 89% for those who did not have radiotherapy and 76% for those who did (log-rank test, p = 0.02).

Radiotherapy increased the risk of revision (log-rank test, p = 0.015). A total of ten amputations were necessary to control infection, of which nine were successful. Radiotherapy may be necessary for the treatment of a bone sarcoma but increases the risk of deep infection for which amputation may be the only solution.

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

Survival of the implant and ‘servicing’ procedures have 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 with most of the latter group having a hydroxyapatite collar at the bone prosthesis junction. The median age of the patients was 24 years (range 13-82 yrs). 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, 34% at 10 years and 58% at 20 years. One in ten patients developed an infection and 42% of these patients eventually required an amputation. 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 32% at ten years compared with nil for the rotating hinge knees with a hydroxyapatite collar. The overall risk of revision for any reason was halved by use of the rotating hinge, and for patients older than 40 years at time of implant.

EPRs are the treatment of choice following resection of tumours. These have been used for 39 years in our institution. There has been concern regarding the long term survival of endoprosthesis; this study investigates the fate of the reconstruction.

Chondroblastomas arise in the epiphyseal area of bones. In the femoral head this can cause considerable difficulty in obtaining access as the epiphysis is entirely intra-articular.

We have reviewed management and outcome of 10 patients with chondroblastoma of the femoral head to identify outcome and complications. The mean age was 14 years and all presented with pain (frequently in the knee) and a limp. All were diagnosed on plain Xray and MRI. Five younger children were treated by curettage by a lateral approach up the femoral neck (to try and minimise damage to the epiphysis) and five by a direct approach through the joint.

Two of the five patients with a lateral approach developed local recurrence whilst none of the direct approaches did. Both local recurrences were cured with a direct curettage. One patient developed overlengthening of the leg by 1cm but there was no case of growth arrest or osteoarthritis.

We recommend a direct approach to the lesion whenever possible to give the best chance of cure with a low risk of complications.

Introduction

Despite the advances in adjuvant chemotherapy and surgical techniques, the diagnosis of a bone tumour still carries with it a significant risk of mortality. This study investigates factors affecting survival, in patients treated for malignant tumours of bone using Endoprosthetic replacement (EPR).

Aim

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

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.

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.

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.

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.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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