Aims. Surgical limb sparing for knee-bearing paediatric bone sarcoma is considered to have a clinically significant influence on postoperative function due to complications and leg-length discrepancies. However, researchers have not fully evaluated the long-term postoperative functional outcomes. Therefore, in this study, we aimed to elucidate the risk factors and long-term functional prognosis associated with paediatric limb-sparing surgery. Methods. We reviewed 40 patients aged under 14 years who underwent limb-sparing surgery for knee bone sarcoma (15 cases in the proximal tibia and 25 in the distal femur) between January 2000 and December 2013, and were followed up for a minimum of five years. A total of 35 patients underwent reconstruction using artificial materials, and five underwent biological reconstruction. We evaluated the patients’ postoperative complications, survival rate of reconstruction material, and limb, limb function, and leg-length discrepancy at the final follow-up, as well as the risk factors for each. Results. Complications were observed in 55% (22/40) of patients. The limb survival and reconstruction material rates at five and ten years were 95% and 91%, and 88% and 66%, respectively. Infection was the only risk factor in both survivals (p < 0.001, p = 0.019). In the 35 patients with limb preservation, the median International Society of Limb Salvage (ISOLS) score at the final follow-up was 80 (47% to 97%). Younger age (p = 0.027) and complications (p = 0.005) were poor prognostic factors. A negative correlation was found between age and leg-length discrepancy (R = −0.426; p = 0.011). The ISOLS scores were significantly lower in patients with a leg-length discrepancy of more than 5 cm (p = 0.005). Conclusion. Young age and complications were linked to an unfavourable functional prognosis. Leg-length correction was insufficient, especially in very young children, resulting in decreased function of the affected limb. Limb-sparing surgery for these children remains a considerable challenge. Cite this article: Bone Jt Open 2024;5(10):868–878

Aims. Surgery is often indicated in patients with metastatic bone disease (MBD) to improve pain and maximize function. Few studies are available which report on clinically meaningful outcomes such as quality of life, function, and pain relief after surgery for MBD. This is the published protocol for the Bone Metastasis Audit — Patient Reported Outcomes (BoMA-PRO) multicentre MBD study. The primary objective is to ascertain patient-reported quality of life at three to 24 months post-surgery for MBD. Methods. This will be a prospective, longitudinal study across six UK orthopaedic centres powered to identify the influence of ten patient variables on quality of life at three months after surgery for MBD. Adult patients managed for bone metastases will be screened by their treating consultant and posted out participant materials. If they opt in to participate, they will receive questionnaire packs at regular intervals from three to 24 months post-surgery and their electronic records will be screened until death or five years from recruitment. The primary outcome is quality of life as measured by the European Organisation for Research and the Treatment of Cancer Quality of Life questionnaire (EORTC-QLQ) C30 questionnaire. The protocol has been approved by the Newcastle & North Tyneside 2 Research Ethics Committee (REC ref 19/NE/0303) and the study is funded by the Royal College of Physicians and Surgeons of Glasgow (RCPSG) and the Association for Cancer Surgery (BASO-ACS). Discussion. This will be the first powered study internationally to investigate patient-reported outcomes after orthopaedic treatment for bone metastases. We will assess quality of life, function, and pain relief at three to 24 months post-surgery and identify which patient variables are significantly associated with a good outcome after MBD treatment. Cite this article: Bone Jt Open 2021;2(2):79–85

Aims

Limb salvage surgery (LSS) is the primary treatment option for primary bone malignancy. It involves the removal of bone and tissue, followed by reconstruction with endoprosthetic replacements (EPRs) to prevent amputation. Trabecular metal (TM) collars have been developed to encourage bone ingrowth (osseointegration (OI)) into EPRs. The primary aim of this study was to assess whether OI occurs when TM collars are used in EPRs for tumour.

Aims

This study aimed to analyze the accuracy and errors associated with 3D-printed, patient-specific resection guides (3DP-PSRGs) used for bone tumour resection.

Aims

The aim of this study was to investigate the safety and efficacy of 3D-printed modular prostheses in patients who underwent joint-sparing limb salvage surgery (JSLSS) for malignant femoral diaphyseal bone tumours.

Aims

For rare cases when a tumour infiltrates into the hip joint, extra-articular resection is required to obtain a safe margin. Endoprosthetic reconstruction following tumour resection can effectively ensure local control and improve postoperative function. However, maximizing bone preservation without compromising surgical margin remains a challenge for surgeons due to the complexity of the procedure. The purpose of the current study was to report clinical outcomes of patients who underwent extra-articular resection of the hip joint using a custom-made osteotomy guide and 3D-printed endoprosthesis.

Aims

Internal hemipelvectomy without reconstruction of the pelvis is a viable treatment for pelvic sarcoma; however, the time it takes to return to excellent function is quite variable. Some patients require greater time and rehabilitation than others. To determine if psoas muscle recovery is associated with changes in ambulatory function, we retrospectively evaluated psoas muscle size and limb-length discrepancy (LLD) before and after treatment and their correlation with objective functional outcomes.

Aims

We first sought to compare survival for patients treated surgically for solitary and multiple metastases in the appendicular skeleton, and second, to explore the role of complete and incomplete resection (R0 and R1/R2) in patients with a solitary bony metastasis in the appendicular skeleton.

Aims

Due to their radiolucency and favourable mechanical properties, carbon fibre nails may be a preferable alternative to titanium nails for oncology patients. We aim to compare the surgical characteristics and short-term results of patients who underwent intramedullary fixation with either a titanium or carbon fibre nail for pathological long-bone fracture.

Aims

Cell-free DNA (cfDNA) and circulating tumour DNA (ctDNA) are used for prognostication and monitoring in patients with carcinomas, but their utility is unclear in sarcomas. The objectives of this pilot study were to explore the prognostic significance of cfDNA and investigate whether tumour-specific alterations can be detected in the circulation of sarcoma patients.

Aims

Rotating-hinge knee prostheses are commonly used to reconstruct the distal femur after resection of a tumour, despite the projected long-term burden of reoperation due to complications. Few studies have examined the factors that influence their failure and none, to our knowledge, have used competing risk models to do so. The purpose of this study was to determine the risk factors for failure of a rotating-hinge knee distal femoral arthroplasty using the Fine-Gray competing risk model.

Aim

There is insufficient evidence to support bony reconstruction of the pubis after a type III internal hemipelvectomy (resection of all or part of the pubis). In this study, we compared surgical complications, postoperative pain, and functional outcome in a series of patients who had undergone a type III internal hemipelvectomy with or without bony reconstruction.

Aims

Bone metastasis ultimately occurs due to a complex multistep process, during which the interactions between cancer cells and bone microenvironment play important roles. Prior to colonization of the bone, cancer cells must succeed through a series of steps that will allow them to gain migratory and invasive properties; epithelial-to-mesenchymal transition (EMT) is known to be integral here. The aim of this study was to determine the effects of G protein subunit alpha Q (GNAQ) on the mechanisms underlying bone metastasis through EMT pathway.

Aims

The aim of this study was to assess orthopaedic oncologic patient morbidity resulting from COVID-19 related institutional delays and surgical shutdowns during the first wave of the pandemic in New York, USA.

Aims

Limb salvage for pelvic sarcomas involving the acetabulum is a major surgical challenge. There remains no consensus about what is the optimum type of reconstruction after resection of the tumour. The aim of this study was to evaluate the surgical outcomes in these patients according to the methods of periacetabular reconstruction.

Aims

Although chondrosarcomas (CSs) display true malignant features, including local recurrence (LR) and metastases, their behaviour in the hands and feet is thought to differ from that in other parts of the axial and appendicular skeleton by having a lower metastatic potential. The purpose of this study was to investigate the disease-specific and surgical factors that affect the local and systemic prognosis of CS of the hands and feet.

Aims

Aneurysmal bone cysts (ABCs) are locally aggressive lesions typically found in the long bones of children and adolescents. A variety of management strategies have been reported to be effective in the treatment of these lesions. The purpose of this review was to assess the effectiveness of current strategies for the management of primary ABCs of the long bones.

We evaluated the diagnostic accuracy of fine-needle aspiration biopsy in a prospective study of 300 patients with previously undiagnosed bone lesions. Patients with suspected local recurrence of a primary bone tumour or a metastatic lesion of a previously diagnosed malignancy were excluded. Fine-needle aspiration biopsy was performed under radiological control as an outpatient procedure. The series was grouped into three major categories: 1) benign bone lesions including infections; 2) primary malignant bone tumours; and 3) metastases including lymphomas and myelomas. We compared the cytological diagnosis with the final diagnosis as assessed by histological examination and/or the clinical and radiological features. Material considered conclusive for cytological diagnosis was obtained from 251 of the 300 patients. Of the 49 failures, there were 24 aspirates with insufficient cellular yield and 25 in which a diagnosis could not be made although the cytological material was adequate in quantity. Most of the inconclusive aspirates (36/49) were obtained from benign bone lesions. The diagnosis was correct in 239 (95%) of the 251 cases providing adequate cytological material. There were eight (3%) falsely benign diagnoses, one (0.3%) falsely malignant, and three cases in which we were unable to differentiate between sarcoma and a metastasis. Chondrosarcoma (2/12) gave the greatest diagnostic difficulty and Ewing’s sarcoma the least (0/9). There were no decisive errors of treatment. All falsely benign or malignant diagnoses were questioned, and led to open biopsy since they did not correlate with the clinical and radiological features. Our study suggests that fine-needle aspiration biopsy is a valid option for the diagnosis of bone tumours. It is a simple outpatient procedure which gives sufficient cytological material for the correct diagnosis in 80% of cases. As with histological analysis of material from open biopsy, the cytological assessment must agree with the clinical and radiological findings

Atypical cartilaginous tumours are usually treated by curettage. The purpose of this study was to show that radiofrequency ablation was an effective alternative treatment. . We enrolled 20 patients (two male, 18 female, mean age 56 years (36 to 72) in a proof-of-principle study. After inclusion, biopsy and radiofrequency ablation were performed, followed three months later by curettage and adjuvant phenolisation. The primary endpoint was the proportional necrosis in the retrieved material. Secondary endpoints were correlation with the findings on gadolinium enhanced MRI, functional outcome and complications. Our results show that 95% to 100% necrosis was obtained in 14 of the 20 patients. MRI had a 91% sensitivity and 67% specificity for detecting residual tumour after curettage. The mean functional outcome (MSTS) score six weeks after radiofrequency ablation was 27.1 (23 to 30) compared with 18.1 (12 to 25) after curettage (p < 0.001). No complications occurred after ablation, while two patients developed a pathological fracture after curettage. We have shown that radiofrequency ablation is capable of completely eradicating cartilaginous tumour cells in selective cases. MRI has a 91% sensitivity for detecting any residual tumour. Radiofrequency ablation can be performed on an outpatient basis allowing a rapid return to normal activities. If it can be made more effective, it has the potential to provide better local control, while improving functional outcome. Cite this article: Bone Joint J 2014;96-B:1540–5

Aims

Accurate estimations of the risk of fracture due to metastatic bone disease in the femur is essential in order to avoid both under-treatment and over-treatment of patients with an impending pathological fracture. The purpose of the current retrospective in vivo study was to use CT-based finite element analyses (CTFEA) to identify a clear quantitative differentiating factor between patients who are at imminent risk of fracturing their femur and those who are not, and to identify the exact location of maximal weakness where the fracture is most likely to occur.

Aims

Iliac wing (Type I) and iliosacral (Type I/IV) pelvic resections for a primary bone tumour create a large segmental defect in the pelvic ring. The management of this defect is controversial as the surgeon may choose to reconstruct it or not. When no reconstruction is undertaken, the residual ilium collapses back onto the remaining sacrum forming an iliosacral pseudarthrosis. The aim of this study was to evaluate the long-term oncological outcome, complications, and functional outcome after pelvic resection without reconstruction.

Aims. The purpose of this study was to develop a prognostic model for predicting survival of patients undergoing surgery owing to metastatic bone disease (MBD) in the appendicular skeleton. Methods. We included a historical cohort of 130 consecutive patients (mean age 64 years, 30 to 85; 76 females/54 males) who underwent joint arthroplasty surgery (140 procedures) owing to MBD in the appendicular skeleton during the period between January 2003 and December 2008. Primary cancer, pre-operative haemoglobin, fracture versus impending fracture, Karnofsky score, visceral metastases, multiple bony metastases and American Society of Anaesthesiologist’s score were included into a series of logistic regression models. The outcome was the survival status at three, six and 12 months respectively. Results were internally validated based on 1000 cross-validations and reported as time-dependent area under the receiver-operating characteristic curves (AUC) for predictions of outcome. . Results. The predictive scores obtained showed AUC values of 79.1% (95% confidence intervals (CI) 65.6 to 89.6), 80.9% (95% CI 70.3 to 90.84) and 85.1% (95% CI 73.5 to 93.9) at three, six and 12 months. . Discussion. In conclusion, we have presented and internally validated a model for predicting survival after surgery owing to MBD in the appendicular skeleton. The model is the first, to our knowledge, built solely on material from patients who only had surgery in the appendicular skeleton. Take home message: Applying this prognostic model will help determine whether the patients’ anticipated survival makes it reasonable to subject them to extensive reconstructive surgery for which there may be an extended period of rehabilitation. Cite this article: Bone Joint J 2016;98-B:271–7

Aims

The purpose of this study was to describe the effect of histological grade on disease-specific survival in patients with chondrosarcoma.

Aims

We retrospectively report our experience of managing 30 patients with a primary malignant tumour of the distal tibia; 25 were treated by limb salvage surgery and five by amputation. We compared the clinical outcomes of following the use of different methods of reconstruction.

Aims

The aim of this paper was to investigate the prognostic factors for local recurrence in patients with pathological fracture through giant cell tumours of bone (GCTB).

Aims

Adjuvant treatment after intralesional curettage for atypical cartilaginous tumours (ACTs) of long bones is widely accepted for extending surgical margins. However, evaluating the isolated effect of adjuvant treatment is difficult, and it is unclear whether not using such adjuvants provides poor oncological outcomes. Hence, we analyzed whether intralesional curettage without cryosurgery or chemical adjuvants provides poor oncological outcomes in patients with an ACT.

Aims

Pelvic reconstruction after the resection of a tumour around the acetabulum is a challenging procedure due to the complex anatomy and biomechanics. Several pelvic endoprostheses have been introduced, but the rates of complication remain high. Our aim was to review the use of a stemmed acetabular pedestal cup in the management of these patients.

Objectives

As tumours of bone and soft tissue are rare, multicentre prospective collaboration is essential for meaningful research and evidence-based advances in patient care. The aim of this study was to identify barriers and facilitators encountered in large-scale collaborative research by orthopaedic oncological surgeons involved or interested in prospective multicentre collaboration.

Aims

Few studies dealing with chondrosarcoma of the pelvis are currently available. Different data about the overall survival and prognostic factors have been published but without a detailed analysis of surgery-related complications. We aimed to analyse the outcome of a series of pelvic chondrosarcomas treated at a single institution, with particular attention to the prognostic factors. Based on a competing risk model, our objective was to identify risk factors for the development of complications.

Aims

To assess complications and failure mechanisms of osteoarticular allograft reconstructions for primary bone tumours.

Aims

The aim of this study was to identify any progression between benign osteofibrous dysplasia (OFD), OFD-like adamantinoma and malignant adamantinoma, and to investigate the rates of local recurrence, metastases and survival, in order to develop treatment algorithms for each.

Aims

Massive endoprostheses rely on extra-cortical bone bridging (ECBB) to enhance fixation. The aim of this study was to investigate the role of selective laser sintered (SLS) porous collars in augmenting the osseointegration of these prostheses.

Extracorporeal irradiation of an excised tumour-bearing segment of bone followed by its re-implantation is a technique used in bone sarcoma surgery for limb salvage when the bone is of reasonable quality. There is no agreement among previous studies about the dose of irradiation to be given: up to 300 Gy have been used.

We investigated the influence of extracorporeal irradiation on the elastic and viscoelastic properties of bone. Bone was harvested from mature cattle and subdivided into 13 groups: 12 were exposed to increasing levels of irradiation: one was not and was used as a control. The specimens, once irradiated, underwent mechanical testing in saline at 37°C.

The mechanical properties of each group, including Young’s modulus, storage modulus and loss modulus, were determined experimentally and compared with the control group.

There were insignificant changes in all of these mechanical properties with an increasing level of irradiation.

We conclude that the overall mechanical effect of high levels of extracorporeal irradiation (300 Gy) on bone is negligible. Consequently the dose can be maximised to reduce the risk of local tumour recurrence.

Cite this article: Bone Joint J 2015;97-B:1152–6.

Aims

The aim of this study was to establish what happens to patients in the long term after endoprosthetic replacement for a primary malignant tumour of bone.

Objective

Clinical studies of patients with bone sarcomas have been challenged by insufficient numbers at individual centres to draw valid conclusions. Our objective was to assess the feasibility of conducting a definitive multi-centre randomised controlled trial (RCT) to determine whether a five-day regimen of post-operative antibiotics, in comparison to a 24-hour regimen, decreases surgical site infections in patients undergoing endoprosthetic reconstruction for lower extremity primary bone tumours.

To date, all surgical techniques used for reconstruction of the pelvic ring following supra-acetabular tumour resection produce high complication rates. We evaluated the clinical, oncological and functional outcomes of a cohort of 35 patients (15 men and 20 women), including 21 Ewing’s sarcomas, six chondrosarcomas, three sarcomas not otherwise specified, one osteosarcoma, two osseous malignant fibrous histiocytomas, one synovial cell sarcoma and one metastasis. The mean age of the patients was 31 years (8 to 79) and the latest follow-up was carried out at a mean of 46 months (1.9 to 139.5) post-operatively.

We undertook a functional reconstruction of the pelvic ring using polyaxial screws and titanium rods. In 31 patients (89%) the construct was encased in antibiotic-impregnated polymethylmethacrylate. Preservation of the extremities was possible for all patients. The survival rate at three years was 93.9% (95% confidence interval (CI) 77.9 to 98.4), at five years it was 82.4% (95% CI 57.6 to 93.4). For the 21 patients with Ewing’s sarcoma it was 95.2% (95% CI 70.7 to 99.3) and 81.5% (95% CI 52.0 to 93.8), respectively. Wound healing problems were observed in eight patients, deep infection in five and clinically asymptomatic breakage of the screws in six. The five-year implant survival was 93.3% (95% CI 57.8 to 95.7). Patients were mobilised at a mean of 3.5 weeks (1 to 7) post-operatively. A post-operative neurological defect occurred in 12 patients. The mean Musculoskeletal Tumor Society score at last available follow-up was 21.2 (10 to 27).

This reconstruction technique is characterised by simple and oncologically appropriate applicability, achieving high primary stability that allows early mobilisation, good functional results and relatively low complication rates.

Cite this article: Bone Joint J 2013;95-B:1410–16.

Previously, we showed that case-specific non-linear finite element (FE) models are better at predicting the load to failure of metastatic femora than experienced clinicians. In this study we improved our FE modelling and increased the number of femora and characteristics of the lesions. We retested the robustness of the FE predictions and assessed why clinicians have difficulty in estimating the load to failure of metastatic femora. A total of 20 femora with and without artificial metastases were mechanically loaded until failure. These experiments were simulated using case-specific FE models. Six clinicians ranked the femora on load to failure and reported their ranking strategies. The experimental load to failure for intact and metastatic femora was well predicted by the FE models (R² = 0.90 and R² = 0.93, respectively). Ranking metastatic femora on load to failure was well performed by the FE models (τ = 0.87), but not by the clinicians (0.11 < τ < 0.42). Both the FE models and the clinicians allowed for the characteristics of the lesions, but only the FE models incorporated the initial bone strength, which is essential for accurately predicting the risk of fracture. Accurate prediction of the risk of fracture should be made possible for clinicians by further developing FE models.

In this case study, we describe the clinical presentation and treatment of 36 patients with periosteal chondrosarcoma collected over a 59-year period by the archive of the Netherlands Committee on Bone Tumours. The demographics, clinical presentation, radiological features, treatment and follow-up are presented with the size, location, the histological grading of the tumour and the survival.

We found a slight predominance of men (61%), and a predilection for the distal femur (33%) and proximal humerus (33%). The metaphysis was the most common site (47%) and the most common presentation was with pain (44%). Half the tumours were classified histologically as grade 1. Pulmonary metastases were reported in one patient after an intra-lesional resection. A second patient died from local recurrence and possible pulmonary and skin metastases after an incomplete resection.

It is clearly important to make the diagnosis appropriately because an incomplete resection may result in local recurrence and metastatic spread. Staging for metastatic disease is recommended in grade II or III lesions.

These patients should be managed with a contrast-enhanced MRI of the tumour and histological confirmation by biopsy, followed by en-bloc excision.

Cite this article: Bone Joint J 2014;96-B:823–8.

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.

We retrospectively reviewed the outcomes of 33 consecutive patients who had undergone an extra-articular, total or partial scapulectomy for a malignant tumour of the shoulder girdle between 1 July 2001 and 30 September 2013. Of these, 26 had tumours which originated in the scapula or the adjacent soft tissue and underwent a classic Tikhoff–Linberg procedure, while seven with tumours arising from the proximal humerus were treated with a modified Tikhoff-Linberg operation. We used a Ligament Advanced Reinforcement System for soft-tissue reconstruction in nine patients, but not in the other 24.

The mean Musculoskeletal Tumor Society score (MSTS) was 17.6 (95% confidence interval (CI) 15.9 to 19.4); 17.6 (95% CI 15.5 to 19.6) after the classic Tikhoff–Linberg procedure and 18.1 (95% CI 13.8 to 22.3) after the modified Tikhoff–Linberg procedure. Patients who had undergone a LARS soft-tissue reconstruction had a mean score of 18.6 (95% (CI) 13.9 to 22.4) compared with 17.2 (95% CI 15.5 to 19.0) for those who did not.

The Tikhoff–Linberg procedure is a useful method for wide resection of a malignant tumour of the shoulder girdle which helps to preserve hand and elbow function. The method of soft-tissue reconstruction has no effect on functional outcome.

Cite this article: Bone Joint J 2014;96-B:684–90.

We report our early experience with the use of a new prosthesis, the Modular Hemipelvic Prosthesis II, for reconstruction of the hemipelvis after resection of a primary malignant peri-acetabular tumour involving the sacroiliac joint.

We retrospectively reviewed the outcome of 17 patients who had undergone resection of a pelvic tumour and reconstruction with this prosthesis between July 2002 and July 2010.

One patient had a type I+II+III+IV resection (ilium + peri-acetabulum + pubis/ischium + sacrum) and 16 had a type I+II+IV resection (ilium + acetabulum + sacrum). The outcome was assessed at a mean follow-up of 33 months (15 to 59). One patient was alive with disease, 11 were alive without disease and five had died of disease. The overall five-year survival rate was 62.4%. Six patients had a local recurrence. The mean Musculoskeletal Tumour Society score was 58% (33 to 77). Deep infection occurred in two patients, problems with wound healing in five and dislocation in one.

For patients with a primary malignant peri-acetabular sarcoma involving the sacroiliac joint, we believe that this new prosthesis is a viable option for reconstruction of the bony defect left following resection of the tumour. It results in a satisfactory functional outcome with an acceptable rate of complications.

Cite this article: Bone Joint J 2014;96-B:399–405.

The aims of this study were to evaluate the incidence of local argyria in patients with silver-coated megaprostheses and to identify a possible association between argyria and elevated levels of silver both locally and in the blood. Between 2004 and 2011, 32 megaprostheses with silver coatings were implanted in 20 female and 12 male patients following revision arthroplasty for infection or resection of a malignant tumour, and the levels of silver locally in drains and seromas and in the blood were determined. The mean age of the patients was 46 years (10 to 81); one patient died in the immediate post-operative period and was excluded.

Seven patients (23%) developed local argyria after a median of 25.7 months (interquartile range 2 to 44.5). Patients with and without local argyria had comparable levels of silver in the blood and aspiration fluids. The length of the implant did not influence the development of local argyria. Patients with clinical evidence of local argyria had no neurological symptoms and no evidence of renal or hepatic failure. Thus, we conclude that the short-term surveillance of blood silver levels in these patients is not required.

Cite this article: Bone Joint J 2013;95-B:988–92.

Giant cell tumours (GCTs) of the small bones of the hands and feet are rare. Small case series have been published but there is no consensus about ideal treatment. We performed a systematic review, initially screening 775 titles, and included 12 papers comprising 91 patients with GCT of the small bones of the hands and feet. The rate of recurrence across these publications was found to be 72% (18 of 25) in those treated with isolated curettage, 13% (2 of 15) in those treated with curettage plus adjuvants, 15% (6 of 41) in those treated by resection and 10% (1 of 10) in those treated by amputation.

We then retrospectively analysed 30 patients treated for GCT of the small bones of the hands and feet between 1987 and 2010 in five specialised centres. The primary treatment was curettage in six, curettage with adjuvants (phenol or liquid nitrogen with or without polymethylmethacrylate (PMMA)) in 18 and resection in six. We evaluated the rate of complications and recurrence as well as the factors that influenced their functional outcome.

At a mean follow-up of 7.9 years (2 to 26) the rate of recurrence was 50% (n = 3) in those patients treated with isolated curettage, 22% (n = 4) in those treated with curettage plus adjuvants and 17% (n = 1) in those treated with resection (p = 0.404). The only complication was pain in one patient, which resolved after surgical removal of remnants of PMMA. We could not identify any individual factors associated with a higher rate of complications or recurrence. The mean post-operative Musculoskeletal Tumor Society scores were slightly higher after intra-lesional treatment including isolated curettage and curettage plus adjuvants (29 (20 to 30)) compared with resection (25 (15 to 30)) (p = 0.091). Repeated curettage with adjuvants eventually resulted in the cure for all patients and is therefore a reasonable treatment for both primary and recurrent GCT of the small bones of the hands and feet.

Cite this article: Bone Joint J 2013;95-B:838–45.

Rarely, the extent of a malignant bone tumour may necessitate resection of the complete humerus to achieve adequate oncological clearance. We present our experience with reconstruction in such cases using a total humeral endoprosthesis (THER) in 20 patients (12 male and eight female) with a mean age of 22 years (6 to 59). We assessed the complications, the oncological and functional outcomes and implant survival. Surgery was performed between June 2001 and October 2009. The diagnosis included osteosarcoma in nine, Ewing’s sarcoma in eight and chondrosarcoma in three. One patient was lost to follow-up. The mean follow-up was 41 months (10 to 120) for all patients and 56 months (25 to 120) in survivors. There were five local recurrences (26.3%) and 11 patients were alive at time of last follow-up, with overall survival for all patients being 52% (95% confidence interval (CI) 23.8 to 74) at five years. The mean Musculoskeletal Tumor Society score for the survivors was 22 (73%; 16 to 23). The implant survival was 95% (95% CI 69.5 to 99.3) at five years.

The use of a THER in the treatment of malignant tumours of bone is oncologically safe; it gives consistent and predictable results with low rates of complication.

Pathological fractures in children can occur as a result of a variety of conditions, ranging from metabolic diseases and infection to tumours. Fractures through benign and malignant bone tumours should be recognised and managed appropriately by the treating orthopaedic surgeon. The most common benign bone tumours that cause pathological fractures in children are unicameral bone cysts, aneurysmal bone cysts, non-ossifying fibromas and fibrous dysplasia. Although pathological fractures through a primary bone malignancy are rare, these should be recognised quickly in order to achieve better outcomes. A thorough history, physical examination and review of plain radiographs are crucial to determine the cause and guide treatment. In most benign cases the fracture will heal and the lesion can be addressed at the time of the fracture, or after the fracture is healed. A step-wise and multidisciplinary approach is necessary in caring for paediatric patients with malignancies. Pathological fractures do not have to be treated by amputation; these fractures can heal and limb salvage can be performed when indicated.

The current indications for functional restoration of extension of the knee following quadriceps resection or loss require reappraisal. The contribution of pedicled and free functional muscle transfer is likely to be over-emphasised in many studies, with good functional outcomes predominantly reported only in the context of cases with residual quadriceps function. In cases with total quadriceps resection or loss, all forms of reconstruction perform poorly. Furthermore, in smaller resections with loss of two or fewer components of the quadriceps, minimal impairment of function occurs in the absence of functional reconstruction, suggesting that functional restoration may not be warranted. Thus there is a paradox in the current approach to quadriceps reconstruction, in that small resections are likely to be over-treated and large resections remain under-treated.

This review suggests a shift is required in the approach and rationale for reconstructing functional extension of the knee after quadriceps resection or loss. A classification based on current evidence is suggested that emphasises more clearly the indications and rationale for functional transfers.

We retrospectively reviewed 101 consecutive patients with 114 femoral tumours treated by massive bone allograft at our institution between 1986 and 2005. There were 49 females and 52 males with a mean age of 20 years (4 to 74). At a median follow-up of 9.3 years (2 to 19.8), 36 reconstructions (31.5%) had failed. The allograft itself failed in 27 reconstructions (24%).

Mechanical complications such as delayed union, fracture and failure of fixation were studied. The most adverse factor on the outcome was the use of intramedullary nails, followed by post-operative chemotherapy, resection length > 17 cm and age > 18 years at the time of intervention. The simultaneous use of a vascularised fibular graft to protect the allograft from mechanical complications improved the outcome, but the use of intramedullary cementing was not as successful.

In order to improve the strength of the reconstruction and to advance the biology of host–graft integration, we suggest avoiding the use of intramedullary nails and titanium plates, but instead using stainless steel plates, as these gave better results. The use of a supplementary vascularised fibular graft should be strongly considered in adult patients with resection > 17 cm and in those who require post-operative chemotherapy.

We analysed the outcome of patients with primary non-metastatic diaphyseal sarcomas who had en bloc resection with preservation of the adjoining joints and reconstruction with re-implantation of sterilised tumour bone after extracorporeal radiation (50 Gy). Between March 2005 and September 2009, 32 patients (16 Ewing’s sarcoma and 16 osteogenic sarcoma) with a mean age of 15 years (2 to 35) underwent this procedure. The femur was the most common site in 17 patients, followed by the tibia in 11, humerus in three and ulna in one. The mean resected length of bone was 19 cm (10 to 26). A total of 31 patients were available at a mean follow-up of 34 months (12 to 74). The mean time to union for all osteotomy sites was 7.3 months (3 to 28): metaphyseal osteotomy sites united quicker than diaphyseal osteotomy sites (5.8 months (3 to 10) versus 9.5 months (4 to 28)). There were three local recurrences, all in soft-tissue away from irradiated graft. At the time of final follow-up, 19 patients were free of disease, one was alive with disease and 11 had died of disease. The mean Musculoskeletal Tumor Society Score for 29 patients evaluated at the last follow-up was 26 (9 to 30).

Extracorporeal irradiation is an oncologically safe and inexpensive technique for limb salvage in diaphyseal sarcomas and has good functional results.

Endoprosthetic reconstruction following resection of 31 tumours of the proximal femur in 30 patients was performed using a Wagner SL femoral revision stem. The mean follow-up was 25.6 months (0.6 to 130.0). Of the 28 patients with a metastasis, 27 died within a mean follow-up period of 18.1 months (0.6 to 56.3) after the operation, and the remaining patient was excluded from the study 44.4 months post-operatively when the stem was removed. The two patients with primary bone tumours were still alive at the latest follow-up of 81.0 and 130.0 months, respectively. One stem only was removed for suspected low-grade infection 44.4 months post-operatively. The worst-case survival rate with removal of the stem for any cause and/or loss to follow-up was 80.0% (95% confidence interval 44.9 to 100) at 130.0 months. The mean Karnofsky index increased from 44.2% (20% to 70%) pre-operatively to 59.7% (0% to 100%) post-operatively, and the mean Merle d’Aubigné score improved from 4.5 (0 to 15) to 12.0 (0 to 18). The mean post-operative Musculoskeletal Tumour Society score was 62.4% (3.3% to 100%).

The Wagner SL femoral revision stem offers an alternative to special tumour prostheses for the treatment of primary and secondary tumours of the proximal femur. The mid-term results are very promising, but long-term experience is necessary.

There are eight reported cases in the literature of osteosarcomas secreting β-hCG. Our primary aim was to investigate the rate of β-hCG expression in osteosarcoma and attempt to understand the characteristics of osteosarcomas that secrete β-hCG. We reviewed 37 histopathology slides (14 biopsies and 23 surgical specimens) from 32 patients with osteosarcoma. The slides were retrospectively stained for β-hCG expression. Patient and tumour characteristics, including age, gender, tumour location, subtype, proportion of necrosis, presence of metastases and recurrence were recorded. A total of five of the 32 tumours were found to be positive for β-hCG expression (one strongly and four weakly). This incidence of this expression was found in tumours with poor histological response to neoadjuvant chemotherapy.

The use of β-hCG expression as a diagnostic, prognostic or follow-up marker is questionable and needs further investigation with a larger sample size.

In skeletally immature patients, resection of bone tumours and reconstruction of the lower limb often results in leg-length discrepancy. The Stanmore non-invasive extendible endoprosthesis, which uses electromagnetic induction, allows post-operative lengthening without anaesthesia. Between 2002 and 2009, 55 children with a mean age of 11.4 years (5 to 16) underwent reconstruction with this prosthesis; ten patients (18.2%) died of disseminated disease and one child underwent amputation due to infection. We reviewed 44 patients after a mean follow-up of 41.2 months (22 to 104). The mean Musculoskeletal Tumor Society score was 24.7 (8 to 30) and the Toronto Extremity Salvage score was 92.3% (55.2% to 99.0%). There was no local recurrence of tumour. Complications developed in 16 patients (29.1%) and ten (18.2%) underwent revision.

The mean length gained per patient was 38.6 mm (3.5 to 161.5), requiring a mean of 11.3 extensions (1 to 40), and ten component exchanges were performed in nine patients (16.4%) after attaining the maximum lengthening capacity of the implant. There were 11 patients (20%) who were skeletally mature at follow-up, ten of whom had equal leg lengths and nine had a full range of movement of the hip and knee.

This is the largest reported series using non-invasive extendible endoprostheses after excision of primary bone tumours in skeletally immature patients. The technique produces a good functional outcome, with prevention of limb-length discrepancy at skeletal maturity.

We report our experience of treating 17 patients with benign lesions of the proximal femur with non-vascularised, autologous fibular strut grafts, without osteosynthesis. The mean age of the patients at presentation was 16.5 years (5 to 33) and they were followed up for a mean of 2.9 years (0.4 to 19.5). Histological diagnoses included simple bone cyst, fibrous dysplasia, aneurysmal bone cysts and giant cell tumour. Local recurrence occurred in two patients (11.7%) and superficial wound infection, chronic hip pain and deep venous thrombosis occurred in three. Pathological fracture did not occur in any patient following the procedure.

We conclude that non-vascularised fibular strut grafts are a safe and satisfactory method of treating benign lesions of the proximal femur.

We have developed a hollow perforated cannulated screw. One or more of these was implanted percutaneously in 11 patients with an osteolytic metastasis in the femoral neck and multiple metastases elsewhere. They were supplemented by one or two additional standard 6.5 mm cannulated screws in nine patients. Polymethylmethacrylate bone cement was injected through the screw into the neck of the femur using small syringes, as in vertebroplasty. The mean amount of cement injected was 23.2 ml (17 to 30). Radiotherapy was started on the fourth post-operative day and chemotherapy, on average, was resumed a day later.

Good structural stability and satisfactory relief from pain were achieved in all the patients. This technique may be useful in the palliation of metastases in the femoral neck.

Between 1996 and 2003, 16 patients (nine female, seven male) were treated for a primary bone sarcoma of the femur by wide local excision of the tumour, extracorporeal irradiation and re-implantation. An additional vascularised fibular graft was used in 13 patients (81%). All patients were free from disease when reviewed at a minimum of two years postoperatively (mean 49.7 months (24 to 96).

There were no cases of infection. Primary union was achieved after a median of nine months (interquartile range 7 to 11). Five host-donor junctions (16%) united only after a second procedure. Primary union recurred faster at metaphyseal junctions (94% (15) at a median of 7.5 months (interquartile range 4 to 12)) than at diaphyseal junctions (75% (12) at a median of 11.1 months (interquartile range 5 to 18)).

Post-operatively, the median Musculoskeletal Tumour Society score was 85% (interquartile range 75 to 96) and the median Toronto Extremity Salvage score 94% (interquartile range 82 to 99). The Mankin score gave a good or excellent result in 14 patients (88%).

The range of movement of the knee was significantly worse when the extracorporeally irradiated autografts were fixed by plates rather than by nails (p = 0.035).

A total of 16 (62%) of the junctions of the vascularised fibular grafts underwent hypertrophy, indicating union and loading.

Extracorporeal irradiation autografting with supplementary vascularised fibular grafting is a promising biological alternative for intercalary reconstruction after wide resection of malignant bone tumours of the femur.

The use of allografts for the treatment of bone tumours in children is limited by nonunion and the difficulty of finding a suitable graft. Furthermore, appositional growth can’t be expected of an allograft.

We used an overlapping allograft in 11 children, with a mean age of ten years (4 to 15), with a mean follow-up of 24.1 months (20 to 33). There were five intercalary and six intra-articular resections, and the tumours were in the femur in six cases and the humerus in five. Rates of union, times to union, remodelling patterns and allograft-associated complications were evaluated.

No allograft was removed due to a complication. Of the 16 junctional sites, 15 (94%) showed union at a mean of 3.1 months (2 to 5). Remodelling between host and allograft was seen at 14 junctions at a mean of five months (4 to 7). The mean Musculoskeletal Tumor Society score was 26.5 of 30 (88.3%). One case of nonunion and another with screw protrusion required re-operation.

Overlapping allografts have the potential to shorten time to union, decrease rates of nonunion and have positive appositional growth effect.

We report positive and negative factors associated with the most commonly-used methods of reconstruction after pathological fracture of the proximal femur. The study was based on 142 patients treated surgically for 146 metastatic lesions between 1996 and 2003. The local rate of failure was 10.3% (15 of 146). Of 37 operations involving osteosynthetic devices, six failed (16.2%) compared with nine (8.3%) in 109 operations involving endoprostheses. Of nine cases of prosthetic failure, four were due to periprosthetic fractures and three to recurrent dislocation. In the osteosynthesis group, three (13.6%) of 22 reconstruction nails failed. The two-year risk of re-operation after any type of osteosynthesis was 0.35 compared with 0.18 after any type of endoprosthetic reconstruction (p = 0.07). Endoprosthetic reconstructions are preferable to the use of reconstruction nails and other osteosynthetic devices for the treatment of metastatic lesions in the proximal third of the femur.

Disarticulation of the hip in patients with high-grade tumours in the upper thigh results in significant morbidity. In patients with no disease of the proximal soft tissue a femoral stump may be preserved, leaving a fulcrum for movement and weight-bearing. We reviewed nine patients in whom the oncological decision would normally be to disarticulate, but who were treated by implantation of an endoprosthesis in order to create a functioning femoral stump. The surgery was undertaken for chondrosarcoma in four patients, pleomorphic sarcoma in three, osteosarcoma in one and fibrous dysplasia in one. At follow-up at a mean of 80 months (34 to 132), seven patients were alive and free from disease, one had died from lung metastases and another from a myocardial infarction. The mean functional outcome assessment was 50 (musculoskeletal tumor society), 50 and 60 (physical and mental Short-form 36 scores).

Implantation of an endoprosthesis into the stump in carefully selected patients allows fitting of an above-knee prosthesis and improves wellbeing and the functional outcome.

We treated 50 patients with bony malignancy by en-bloc resection, extracorporeal irradiation with 50 Gy and re-implantation of the bone segment.

The mean survivor follow-up was 38 months (12 to 92) when 42 patients were alive and without disease. There were four recurrences. The functional results were good according to the Mankin score (17 excellent, 13 good, nine fair, three failures), the Musculoskeletal Tumour Society score (mean 77) and the Toronto Extremity Salvage score (mean 81). There was solid union, but bone resorption was seen in some cases. The dose of radiation was lethal to all cells and produced a dead autograft of perfect fit.

Extracorporeal irradiation is a useful technique for limb salvage when there is reasonable residual bone stock. It allows effective re-attachment of tendons and produces a lasting biological reconstruction. There should be no risk of local recurrence or of radiotherapy-induced malignancy in the replanted bone.

We evaluated the long-term outcome of patients with an osteosarcoma who had undergone prior manipulative therapy, a popular treatment in Asia, and investigated its effects on several prognostic factors. Of the 134 patients in this study, 70 (52%) patients had manipulative therapy and 64 (48%) did not. The age, location, and size of tumour were not significantly different between the groups. The five-year overall survival rate was 58% and 92% in the groups with and without manipulative therapy (p = 0.004). Both the primary and overall rates of lung metastasis were significantly higher in the manipulative group (primary: 32% vs 3%, p = 0.003; overall lung metastasis rate: 51.4% vs 18.8%, p < 0.001). Patients who had manipulative therapy had higher local recurrence rates in comparison to patients who did not (29% vs 6%, p = 0.011). The prognosis for patients with osteosarcoma who had manipulative therapy was significantly poorer than those who had not. Manipulative therapy was an independent factor for survival.

This form of therapy may serve as a mechanism to accelerate the spread of tumour cells, and therefore must be avoided in order to improve the outcome for patients with an osteosarcoma.

We describe a retrospective review of 38 cases of reconstruction following resection of the metaphysiodiaphysis of the lower limb for malignant bone tumours using free vascularised fibular grafts. The mean follow-up was for 7.6 years (0.4 to 18.4). The mean Musculoskeletal Tumor Society score was 27.2 (20 to 30). The score was significantly higher when the graft was carried out in a one-stage procedure after resection of the tumour rather than in two stages. Bony union was achieved in 89% of the cases. The overall mean time to union was 1.7 years (0.2 to 10.3).

Free vascularised fibular transfer is a major operation with frequent, but preventable, complications which allows salvage of the limb with satisfactory functional results.

We identified 42 patients who presented to our unit over a 27-year period with a secondary radiation-induced sarcoma of bone. We reviewed patient, tumour and treatment factors to identify those that affected outcome. The mean age of the patients at presentation was 45.6 years (10 to 84) and the mean latent interval between radiotherapy and diagnosis of the sarcoma was 17 years (4 to 50). The median dose of radiotherapy given was estimated at 50 Gy (mean 49; 20 to 66). There was no correlation between radiation dose and the time to development of a sarcoma. The pelvis was the most commonly affected site (14 patients (33%)). Breast cancer was the most common primary tumour (eight patients; 19%). Metastases were present at diagnosis of the sarcoma in nine patients (21.4%). Osteosarcoma was the most common diagnosis and occurred in 30 cases (71.4%). Treatment was by surgery and chemotherapy when indicated: 30 patients (71.4%) were treated with the intention to cure. The survival rate was 41% at five years for those treated with the intention to cure but in those treated palliatively the mean survival was only 8.8 months (2 to 22), and all had died by two years. The only factor found to be significant for survival was the ability to completely resect the tumour. Limb sarcomas had a better prognosis (66% survival at five years) than central ones (12% survival at five years) (p = 0.009).

Radiation-induced sarcoma is a rare complication of radiotherapy. Both surgical and oncological treatment is likely to be compromised by the treatment received previously by the patient.

Bone loss secondary to primary or metastatic lesions of the proximal humerus remains a challenging surgical problem. Options include preservation of the joint with stabilisation using internal fixation or resection of the tumour with prosthetic replacement. Resection of the proximal humerus often includes the greater tuberosity and adjacent diaphysis, which may result in poor function secondary to loss of the rotator cuff and/or deltoid function. Preservation of the joint with internal fixation may reduce the time in hospital and peri-operative morbidity compared with joint replacement, and result in a better functional outcome. We included 32 patients with pathological fractures of the proximal humerus in this study. Functional and radiological assessments were performed. At a mean follow-up of 17.6 months (8 to 61) there was no radiological evidence of failure of fixation. The mean revised musculoskeletal Tumour Society functional score was 94.6% (86% to 99%). There was recurrent tumour requiring further surgery in four patients (12.5%). Of the 22 patients who were employed prior to presentation all returned to work without restrictions.

The use of a locking plate combined with augmentation with cement extends the indications for salvage of the proximal humerus with good function in patients with pathological and impending pathological fractures.

We reviewed 25 patients who had undergone resection of a primary bone sarcoma which extended to within 5 cm of the knee with reconstruction by a combination of a free vascularised fibular graft and a massive allograft bone shell. The distal femur was affected in four patients and the proximal tibia in 21. Their mean age at the time of operation was 19.7 years (5 to 52) and the mean follow-up period 140 months (28 to 213). Three vascularised transfers failed. The mean time to union of the fibula was 5.6 months (3 to 10) and of the allograft 19.6 months (10 to 34). Full weight-bearing was allowed at a mean of 21.4 months (14 to 36). The mean functional score at final follow-up was 27.4 (18 to 30) using a modfied 30-point Musculoskeletal Tumour Society rating system. The overall limb-salvage rate was 88%. The results of our study suggest that the combined use of a vascularised fibular graft and allograft is of value as a limb-salvage procedure for intercalary reconstruction after resection of bone tumours around the knee, especially in skeletally immature patients.

We analysed the influence of the timing of surgery (< 48 hours, group 1, 21 patients vs > 48 hours, group 2, 14 patients) on the neurological outcome and restoration of mobility in 35 incomplete tetra- and paraplegic patients with metastatic spinal-cord compression.

Pain and neurological symptoms were assessed using the American Spinal Injury Association impairment scale. More improvement was found in group 1 than in group 2 when comparing the pre-operative findings with those both immediately post-operatively (p = 0.021) and those at follow-up at four to six weeks (p = 0.010). In group 1 the number of pre-operatively mobile patients increased from 17 (81%) to 19 patients (90%) whereas the number of mobile patients in group 2 changed from nine (64%) to ten (71%).

These results suggest that early surgical treatment in patients with metastatic spinal-cord compression gives a better neurological outcome even in a palliative situation.

We review the treatment of pelvic Ewing’s sarcoma by the implantation of extracorporeally-irradiated (ECI) autografts and compare the outcome with that of other reported methods.

We treated 13 patients with ECI autografts between 1994 and 2004. There were seven males and six females with a median age of 15.7 years (interquartile range (IQR) 12.2 to 21.7). At a median follow-up of five years (IQR 1.8 to 7.4), the disease-free survival was 69% overall, and 75% if one patient with local recurrence after initial treatment elsewhere was excluded. Four patients died from distant metastases at a mean of 17 months (13 to 23). There were three complications which required operative intervention; one was a deep infection which required removal of the graft. The functional results gave a mean Musculoskeletal Tumor Society score of 85% (60% to 97%), a mean Toronto extremity salvage score of 86% (69% to 100%) and a mean Harris hip score of 92 (67 to 100).

We conclude that ECI grafting is a suitable form of treatment for localised and resectable pelvic Ewing’s sarcoma.

Deep-seated lipomas are often atypical histologically and are considered by some to have a high risk of recurrence after excision. We reviewed 215 deep-seated lipomas of the extremities and trunk wall with reference to histology, cytogenetics, clinical features and local recurrence. We classified tumours with atypical features and/or ring chromosomes as atypical lipomas. These were more common in men, larger than ordinary lipomas and more often located in the upper leg. The annual incidence was estimated as ten per million inhabitants and the ratio of atypical to ordinary lipomas was 1:3. In total, six tumours (3%), recurred locally after a median of eight years (1 to 16); of these, four were classified as atypical.

The low recurrence rate of deep-seated lipomas of the extremity or trunk wall, irrespective of histological subtype, implies that if surgery is indicated, the tumour may be shelled out, that atypical lipomas in these locations do not deserve the designation well-differentiated liposarcoma, and that routine review after surgery is not required.

We investigated whether our policy of routine re-excision of the tumour bed after an unplanned excision of a soft-tissue sarcoma was justified.

Between April 1982 and December 2005, 2201 patients were referred to our hospital with the diagnosis of soft-tissue sarcoma, of whom 402 (18%) had undergone an unplanned excision elsewhere. A total of 363 (16.5%) were included in this study. Each patient was routinely restaged and the original histology was reviewed. Re-excision was undertaken in 316 (87%). We analysed the patient, tumour and treatment factors in relation to local control, metastasis and overall survival.

Residual tumour was found in 188 patients (59%). There was thus no residual disease in 128 patients of whom 10% (13) went on to develop a local recurrence. In 149 patients (47%), the re-excision specimen contained residual tumour, but it had been widely excised. Local recurrence occurred in 30 of these patients (20%). In 39 patients (12%), residual tumour was present in a marginal resection specimen. Of these, 46% (18) developed a local recurrence. A final positive margin in a high-grade tumour had a 60% risk of local recurrence even with post-operative radiotherapy.

Metastases developed in 24% (86). The overall survival was 77% at five years. Survival was related to the grade of the tumour and the finding of residual tumour at the time of re-excision.

We concluded that our policy of routine re-excision after unplanned excision of soft-tissue sarcoma was justified in view of the high risk of finding residual tumour.

Despite local treatment with systemic chemotherapy in Ewing’s sarcoma family tumours (ESFT), patients with detectable metastases at presentation have a markedly worse prognosis than those with apparently localised disease. We investigated the clinical, pathological and laboratory differences in 888 patients with ESFT, 702 with localised disease and 186 with overt metastases at presentation, seen at our institution between 1983 and 2006.

Multivariate analyses showed that location in the pelvis, a high level of serum lactic dehydrogenase, the presence of fever and a short interval between the onset of symptoms and diagnosis were indicative of metastatic disease. The rate of overt metastases at presentation was 10% without these four risk factors, 22.7% with one, 31.4% with two, and 50% for those with three or four factors. We concluded that in ESFT the site, the serum level of lactic dehydrogenase, fever, and the interval between the onset of symptoms and diagnosis are indicators of tumours having a particularly aggressive metastatic behaviour.

We present a retrospective study of patients suffering from a variety of benign tumours in whom external fixators were used to treat deformity and limb-length discrepancy, and for the reconstruction of bone defects. A total of 43 limbs in 31 patients (12 male and 19 female) with a mean age of 14 years (2 to 54) were treated.

The diagnosis was Ollier’s disease in 12 limbs, fibrous dysplasia in 11, osteochondroma in eight, giant cell tumour in five, osteofibrous dysplasia in five and non-ossifying fibroma in two. The lesions were treated in the tibia in 19 limbs, in the femur in 16, and in the forearm in eight. The Ilizarov frame was used in 25 limbs, the Taylor Spatial Frame in seven, the Orthofix fixator in six, the Monotube in four and the Heidelberg fixator in one. The mean follow-up was 72 months (22 to 221).

The mean external fixation period was 168 days (71 to 352). The mean external fixation index was 42 days/cm (22.2 to 102.0) in the 22 patients who required limb lengthening. The mean correction angle for those with angular deformity was 23° (7° to 45°).

At final follow-up all patients had returned to normal activities. Four patients required a second operation for recurrent deformity of further limb lengthening. Local recurrence occurred in one patient, requiring further surgery.

We evaluated the construct validity of the Musculoskeletal Tumour Society rating scale (Enneking score) as a functional measure for patients with sarcoma involving the upper limb. We compared the Enneking score by examining the correlation between two patient-derived outcome measures, the Disability of the Arm, Shoulder, and Hand (DASH) questionnaire and the Medical Outcomes Study Short Form-36 (SF-36) as indicators of functional status in 40 patients with malignant or aggressive benign bone and soft-tissue tumours of the upper limb who had undergone surgical treatment.

The frequency distributions were similar among the three scoring systems. As for the validity, Spearman’s rank correlation coefficient of the Enneking score to the DASH questionnaire was −0.79 and that of the Enneking to the SF-36 subscales ranged from 0.38 to 0.60. Despite being a measure from the surgeon’s perspective, the Enneking score was shown to be a valid indicator of physical disability in patients with malignant or aggressive benign tumours of the upper limb and reflected their opinion.

The most appropriate protocol for the biopsy of musculoskeletal tumours is controversial, with some authors advocating CT-guided core biopsy. At our hospital the initial biopsies of most musculoskeletal tumours has been by operative core biopsy with evaluation by frozen section which determines whether diagnostic tissue has been obtained and, if possible, gives the definitive diagnosis. In order to determine the accuracy and cost-effectiveness of this protocol we have undertaken a retrospective audit of biopsies of musculoskeletal tumours performed over a period of two years.

A total of 104 patients had biopsies according to this regime. All gave the diagnosis apart from one minor error which did not alter the management of the patient. There was no requirement for re-biopsy. This protocol was more labour-intensive and 38% more costly than CT-guided core biopsy (AU$1804 vs AU$1308). However, the accuracy and avoidance of the anxiety associated with repeat biopsy outweighed these disadvantages.

Between December 1995 and March 2003, 38 adult patients with intermediate or high-grade liposarcoma in a limb were treated by limb-sparing surgery and post-operative radiotherapy. The ten-year local recurrence-free survival was 83%, the ten-year metastasis-free survival 61%, the ten-year disease-free survival 51% and the ten-year overall survival 67%. Analysis of failure and success showed no association with the age of the patients, gender, the location of the primary tumour, the type of liposarcoma and the quality of resection.

Our results indicate that liposarcoma may recur even ten years after the end of definitive therapy and may spread to unexpected sites as for soft-tissue sarcoma.

Between 1986 and 2002, 42 patients with synchronous multifocal osteosarcoma were treated with two different protocols of neoadjuvant chemotherapy. When feasible, the primary and secondary tumours were excised as a combined procedure.

After initial chemotherapy 26 patients were excluded from simultaneous excision of all their secondary bone lesions as their disease was too advanced. In 12 patients only isolated excision of the primary lesion was possible. For 16 patients simultaneous operations were conducted to excise the primary and secondary lesions. This involved two supplementary sites in 15 patients and four additional sites in one patient. Of these, 15 attained remission but 12 relapsed and died (11 within two years). Three patients remained disease-free at five, six and 17 years. The histological response to pre-operative chemotherapy of the primary and secondary lesions was concordant in 13 of the 16 patients who underwent simultaneous operations at more than one site.

The prognosis for synchronous multifocal osteosarcoma remains poor despite combined chemotherapy and surgery. The homogeneous histological responses in a large proportion of the primary and secondary lesions implies that synchronous multifocal osteosarcoma tumours are not multicentric in origin, but probably represent bone-to-bone metastases from a single tumour.

We report our early experience with the use of a non-invasive distal femoral expandable endoprosthesis in seven skeletally immature patients with osteosarcoma of the distal femur. The patients had a mean age of 12.1 years (9 to 15) at the time of surgery. The prosthesis was lengthened at appropriate intervals in outpatient clinics, without anaesthesia, using the principle of electromagnetic induction. The patients were functionally evaluated using the Musculoskeletal Tumour Society scoring system. The mean follow-up was 20.2 months (14 to 30). The prostheses were lengthened by a mean of 25 mm (4.25 to 55) and maintained a mean knee flexion of 110° (100° to 120°). The mean Musculoskeletal Tumour Society score was 68% (11 to 29). Complications developed in two patients; one developed a flexion deformity of 25° at the knee joint, which was subsequently overcome and one died of disseminated disease. The early results from patients treated with this device have been encouraging. The implant avoids multiple surgical procedures, general anaesthesia and assists in maintaining leg-length equality.