Aims

Osteoarticular reconstruction of the distal femur in childhood has the advantage of preserving the tibial physis. However, due to the small size of the distal femur, matching the host bone with an osteoarticular allograft is challenging. In this study, we compared the outcomes and complications of a resurfaced allograft-prosthesis composite (rAPC) with those of an osteoarticular allograft to reconstruct the distal femur in children.

Aims. The purpose of this study was to investigate the potential for achieving local and systemic control after local recurrence of a chondrosarcoma of bone. Patients and Methods. A total of 126 patients with local recurrence (LR) of chondrosarcoma (CS) of the pelvis or a limb bone were identified from a prospectively maintained database, between 1990 and 2015 at the Royal Orthopaedic Hospital, Birmingham, United Kingdom. There were 44 female patients (35%) and 82 male patients (65%) with a mean age at the time of LR of 56 years (13 to 96). The 126 patients represented 24.3% of the total number of patients with a primary CS (519) who had been treated during this period. Clinical data collected at the time of primary tumour and LR included the site (appendicular, extremity, or pelvis); primary and LR tumour size (in centimetres); type of operation at the time of primary or LR (limb-salvage or amputation); surgical margin achieved at resection of the primary tumour and the LR; grade of the primary tumour and the LR; gender; age; and oncological outcomes, including local recurrence-free survival and disease-specific survival. A minimum two years’ follow-up and complete histopathology records were available for all patients included in the study. Results. For patients without metastases prior to or at the time of local recurrence, the disease-specific survival after local recurrence was 62.5% and 45.5% at one and five years, respectively. After univariable analysis, significant factors predicting disease-specific survival were grade (p < 0.001) and surgical margin (p = 0.044). After multivariable analysis, grade, increasing age at the time of diagnosis of local recurrence, and a greater time interval from primary surgery to local recurrence were significant factors for disease-specific survival. A secondary local recurrence was seen in 26% of patients. Wide margins were a good predictor of local recurrence-free survival for subsequent recurrences after univariable analysis when compared with intralesional margins (p = 0.002) but marginal margins did not reach statistical significance when compared with intralesional margins (p = 0.084). Conclusion. In cases of local recurrence of a chondrosarcoma of bone, we have shown that if the tumour is non-metastatic at re-staging, an increase in disease-specific survival and in local recurrence-free survival is achievable, but only by resection of the local recurrence with a wide margin. Cite this article: Bone Joint J 2019;101-B:266–271

Aims. Children treated for osteosarcoma around the knee often have a substantial leg-length discrepancy at skeletal maturity. The aim of this study was to investigate the results of staged skeletal reconstruction after a leg lengthening procedure using an external fixator in these patients. Patients and Methods. We reviewed 11 patients who underwent staged reconstruction with either an arthroplasty (n = 6) or an arthrodesis (n = 5). A control group of 11 patients who had undergone wide excision and concurrent reconstruction with an arthroplasty were matched for gender, location, and size of tumour. We investigated the change in leg-length discrepancy, function as assessed by the Musculoskeletal Tumor Society Scale (MSTS) score and complications. Results. A mean 5.2 cm (1.7 to 8.9) of lengthening was achieved. The mean MSTS scores significantly improved after staged reconstruction (p = 0.003) but were still worse than those of the control group (p = 0.049). However, the MSTS scores of the arthroplasty subgroup were comparable with those of the controls, although the extensor lag was greater and the range of movement was less. The patient group experienced more complications, but all of these resolved. Conclusion. Approximately 5 cm of lengthening and significant functional improvement can be achieved by staged reconstruction and lengthening, without major complications. Although it has limitations, this method of treatment seems to be a satisfactory surgical option for growing children with a significant leg-length discrepancy after excision of an osteosarcoma around the knee. Cite this article: Bone Joint J 2017;99-B:401–8

Aims. Instability of the hip is the most common mode of failure after reconstruction with a proximal femoral arthroplasty (PFA) using an endoprosthesis after excision of a tumour. Small studies report improved stability with capsular repair of the hip and other techniques, but these have not been investigated in a large series of patients. The aim of this study was to evaluate variables associated with the patient and the operation that affect post-operative stability. We hypothesised an association between capsular repair and stability. Patients and Methods. In a retrospective cohort study, we identified 527 adult patients who were treated with a PFA for tumours. Our data included demographics, the pathological diagnosis, the amount of resection of the abductor muscles, the techniques of reconstruction and the characteristics of the implant. We used regression analysis to compare patients with and without post-operative instability. Results. A total of 20 patients out of 527 (4%) had instability which presented at a mean of 35 days (3 to 131) post-operatively. Capsular repair was not associated with a reduced rate of instability. Bivariate analysis showed that a posterolateral surgical approach (odds ratio (OR) 0.11, 95% confidence interval (CI) 0.02 to 0.86) and the type of implant (p = 0.046) had a significant association with reduced instability; age > 60 years predicted instability (OR 3.17, 95% CI 1.00 to 9.98). Multivariate analysis showed age > 60 years (OR 5.09, 95% CI 1.23 to 21.07), female gender (OR 1.73, 95% CI 1.04 to 2.89), a malignant primary bone tumour (OR 2.04, 95% CI 1.06 to 3.95), and benign condition (OR 5.56, 95% CI 1.35 to 22.90), but not metastatic disease or soft-tissue tumours, predicted instability, while a posterolateral approach (OR 0.09, 95% CI 0.01 to 0.53) was protective against instability. No instability occurred when a synthetic graft was used in 70 patients. Conclusion. Stability of the hip after PFA is influenced by variables associated with the patient, the pathology, the surgical technique and the implant. We did not find an association between capsular repair and improved stability. Extension of the tumour often dictates surgical technique; however, our results indicate that PFA using a posterolateral approach with a hemiarthroplasty and synthetic augment for soft-tissue repair confers the lowest risk of instability. Patients who are elderly, female, or with a primary benign or malignant bone tumour should be counselled about an increased risk of instability. Cite this article: Bone Joint J 2017;99-B:531–7

The aim of this study was to evaluate the prognostic and therapeutic factors which influence the oncological outcome of parosteal osteosarcoma. A total of 80 patients with a primary parosteal osteosarcoma were included in this retrospective study. There were 51 females and 29 males with a mean age of 29.9 years (11 to 78). The mean follow-up was 11.2 years (1 to 40). Overall survival was 91.8% at five years and 87.8% at ten years. Local recurrence occurred in 14 (17.5%) patients and was associated with intralesional surgery and a large volume of tumour. On histological examination, 80% of the local recurrences were dedifferentiated high-grade tumours. A total of 12 (14.8%) patients developed pulmonary metastases, of whom half had either a dedifferentiated tumour or a local recurrence. Female gender and young age were good prognostic factors. Local recurrence was a poor prognostic factor for survival. Medullary involvement or the use of chemotherapy had no impact on survival. The main goal in treating a parosteal osteosarcoma must be to achieve a wide surgical margin, as inadequate margins are associated with local recurrence. Local recurrence has a significant negative effect on survival, as 80% of the local recurrences are high-grade dedifferentiated tumours, and half of these patients develop metastases. The role of chemotherapy in the treatment of parosteal osteosarcoma is not as obvious as it is in the treatment of conventional osteosarcoma. The mainstay of treatment is wide local excision. Cite this article: Bone Joint J 2015;97-B:1698–1703

Avascular necrosis (AVN) is a serious complication of high-dose chemotherapy for haematological malignancy in childhood. In order to describe its incidence and main risk factors and to evaluate the current treatment options, we reviewed 105 children with a mean age of 8.25 years (1 to 17.8) who had acute lymphoblastic or acute myeloid leukaemia, or a non-Hodgkin’s lymphoma. Overall, eight children (7.6%) developed AVN after a mean of 16.8 months (8 to 49). There were four boys and four girls with a mean age of 14.4 years (9.8 to 16.8) and a total of 18 involved sites, 12 of which were in the femoral head. All these children were aged > nine years (p < 0.001). All had received steroid treatment with a mean cumulative dose of prednisone of 5967 mg (4425 to 9599) compared with a mean of 3943 mg (0 to 18 585) for patients without AVN (p = 0.005). No difference existed between genders and no thrombophilic disorders were identified. Their initial treatment included 11 core decompressions and two bipolar hip replacements. Later, two salvage osteotomies were done and three patients (four hips) eventually needed a total joint replacement. We conclude that AVN mostly affects the weight-bearing epiphyses. Its risk increases with age and higher steroid doses. These high-risk patients may benefit from early screening for AVN. Cite this article: Bone Joint J 2013;95-B:1708–13

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

We undertook a prospective study to evaluate the prognostic significance of the serum levels of vascular endothelial growth factor (VEGF) in predicting the survival of patients with osteosarcoma. The levels were measured by an enzyme-linked immunosorbent assay in 15 patients with osteosarcoma before commencing treatment. The patients were divided into two groups, with a high or a low serum VEGF level, and the incidence of metastases and overall survival rate were compared. No significant relationship was observed between the serum VEGF levels and gender, age, the size of the tumour or the response to pre-operative chemotherapy. Patients with a serum VEGF > 1000 pg/ml had significantly worse survival than those with a level < 1000 pg/ml (p = 0.002). The serum VEGF level may be useful in predicting the prognosis for survival in patients with osteosarcoma

Aims

There is a lack of evidence about the risk factors for local recurrence of a giant cell tumour (GCT) of the sacrum treated with nerve-sparing surgery, probably because of the rarity of the disease. This study aimed to answer two questions: first, what is the rate of local recurrence of sacral GCT treated with nerve-sparing surgery and second, what are the risk factors for its local recurrence?

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

We reviewed retrospectively the results in 211 consecutive patients who had undergone limb salvage for bone neoplasia with endoprosthetic reconstruction of the proximal femur (96), distal femur (78), proximal tibia (30) and total femur (7). Their mean age was 50 years (11 to 86) and the mean follow-up period was 37.3 months (1 to 204). A total of 35 (16.6%) prostheses failed. Overall, implant survival was 78% (95% confidence interval (CI) 0.29 to 0.54) at five years, 60% (95% CI 0.93 to 2.35) at ten years and 60% (95% CI 1.27 to 3.88) at 15 years. Survivorship of the limb was 97.6% (95% CI 1.73 to 3.35) at ten years. The gender, age, diagnosis and location of the tumour were not prognostic variables for failure. Modular endoprosthetic replacement in the lower limb is a durable long-term reconstructive option, with the implants generally outlasting the patient

The management of spinal metastases is palliative and aimed at improving quality of life at an acceptable risk. This population study uses administrative databases and measures survivorship and complication rates after surgery for spinal metastases. The effects of various potential predictor variables were evaluated. We identified 987 patients with a median survival for all types of cancer of 227 days. The one and three-month mortality was 9% and 29%, respectively. Increasing age, male gender and primary lung cancer were significant risk factors for death within 30 days of surgery. A preoperative neurological deficit contributed a 19% increase in mortality and a 71% increase in the risk of postoperative wound infection. We found an overall major complication rate of 27%. This information will provide patients, families and clinicians with objective data which will help in the choice of treatment and the understanding of the surgical risk and outcome

We performed a prospective genotype-phenotype study using molecular screening and clinical assessment to compare the severity of disease and the risk of sarcoma in 172 individuals (78 families) with hereditary multiple exostoses. We calculated the severity of disease including stature, number of exostoses, number of surgical procedures that were necessary, deformity and functional parameters and used molecular techniques to identify the genetic mutations in affected individuals. Each arm of the genotype-phenotype study was blind to the outcome of the other. Mutations EXT1 and EXT2 were almost equally common, and were identified in 83% of individuals. Non-parametric statistical tests were used. There was a wide variation in the severity of disease. Children under ten years of age had fewer exostoses, consistent with the known age-related penetrance of this condition. The severity of the disease did not differ significantly with gender and was very variable within any given family. The sites of mutation affected the severity of disease with patients with EXT1 mutations having a significantly worse condition than those with EXT2 mutations in three of five parameters of severity (stature, deformity and functional parameters). A single sarcoma developed in an EXT2 mutation carrier, compared with seven in EXT1 mutation carriers. There was no evidence that sarcomas arose more commonly in families in whom the disease was more severe. The sarcoma risk in EXT1 carriers is similar to the risk of breast cancer in an older population subjected to breast-screening, suggesting that a role for regular screening in patients with hereditary multiple exostoses is justifiable

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 study was to investigate the local recurrence rate at an extended follow-up in patients following navigated resection of primary pelvic and sacral tumours.

Aims

The aim of this study was to evaluate health-related quality of life (HRQoL) and joint function in tenosynovial giant cell tumour (TGCT) patients before and after surgical treatment.

Aims

The aims of this study were to evaluate the efficacy of preoperative denosumab in achieving prospectively decided intention of therapy in operable giant cell tumour of bone (GCTB) patients, and to document local recurrence-free survival (LRFS).

Aims

The primary aim of this study was to determine the effect of the duration of symptoms (DOS) prior to diagnosis on the overall survival in patients with a primary bone sarcoma.

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

The aim of this study was to evaluate the surgical management and outcome of patients with an acral soft-tissue sarcoma of the hand or foot.

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The aim of this study was to assess the current trends in the estimation of survival and the preferred forms of treatment of pathological fractures among national and international general and oncological orthopaedic surgeons, and to explore whether improvements in the management of these patients could be identified in this way.

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

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The aims of this study were to evaluate the long-term outcome of surgery for bone or soft-tissue metastases from renal cell carcinoma (RCC) and to determine factors that affect prognosis.

Aims

Intra-articular ⁹⁰Yttrium (⁹⁰Y) is an adjunct to surgical treatment by synovectomy for patients with diffuse-type tenosynovial giant-cell tumour (dtTGCT) of the knee, with variable success rates. Clinical information is, however, sparse and its value remains unclear. We investigated the long-term outcome of patients who underwent synovectomy with and without adjuvant treatment with ⁹⁰Yttrium.

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

The aim of this study was to analyse a group of patients with non-metastatic Ewing’s sarcoma at presentation and identify prognostic factors affecting the development of local recurrence, in order to assess the role of radiotherapy.

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The sacrum is frequently invaded by a pelvic tumour. The aim of this study was to review our experience of treating this group of patients and to identify the feasibility of a new surgical classification in the management of these tumours.

Aims

The use of a noninvasive growing endoprosthesis in the management of primary bone tumours in children is well established. However, the efficacy of such a prosthesis in those requiring a revision procedure has yet to be established. The aim of this series was to present our results using extendable prostheses for the revision of previous endoprostheses.

Aims

After intercalary resection of a bone tumour from the femur, reconstruction with a vascularized fibular graft (VFG) and massive allograft is considered a reliable method of treatment. However, little is known about the long-term outcome of this procedure. The aims of this study were to determine whether the morbidity of this procedure was comparable to that of other reconstructive techniques, if it was possible to achieve a satisfactory functional result, and whether biological reconstruction with a VFG and massive allograft could achieve a durable, long-lasting reconstruction.

Aims

The aim of the study was to compare measures of the quality of life (QOL) after resection of a chordoma of the mobile spine with the national averages in the United States and to assess which factors influenced the QOL, symptoms of anxiety and depression, and coping with pain post-operatively in 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

The aims of this study were to analyse the long-term outcome of vascularised fibular graft (VFG) reconstruction after tumour resection and to evaluate the usefulness of the method.

Aims

This study aims to assess first, whether mutations in the epidermal growth factor receptor (EGFR) and Kirsten rat sarcoma (kRAS) genes are associated with overall survival (OS) in patients who present with symptomatic bone metastases from non-small cell lung cancer (NSCLC) and secondly, whether mutation status should be incorporated into prognostic models that are used when deciding on the appropriate palliative treatment for symptomatic bone metastases.

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

Intercalary allografts following resection of a primary diaphyseal tumour have high rates of complications and failures. At our institution intercalary allografts are augmented with intramedullary cement and fixed using compression plating. Our aim was to evaluate their long-term outcomes.

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.

Aims

Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically presenting as painless slowly growing masses in the extremities. Locally infiltrative growth means that the risk of local recurrence is high. We reviewed our experience to make recommendations about resection strategies and the role of the multidisciplinary team in the management of these tumours.

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The purpose of this study was to determine if clinical and radiological surveillance of cartilage tumours with low biological activity is appropriate.

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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).

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The aim of the study was to investigate the controversial issue of whether the pelvic ring should be reconstructed following resection of the sarcomas of the ilium.

Aims

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

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The purpose of the study was to investigate whether closed intramedullary (IM) nailing with percutaneous cement augmentation is better than conventional closed nailing at relieving pain and suppressing tumours in patients with metastases of the femur and humerus.

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The aims of this retrospective study were to report the feasibility of using 3D-printing technology for patients with a pelvic tumour who underwent reconstruction.

Aims

The purpose of this retrospective study was to differentiate between the MRI features of normal post-operative change and those of residual or recurrent disease after intralesional treatment of an atypical cartilage tumour (ACT)/grade I chondrosarcoma.

The aim of this study was to determine whether the high-sensitivity modified Glasgow prognostic score (Hs-mGPS) could predict the disease-specific survival and oncological outcome in adult patients with non-metastatic soft-tissue sarcoma before treatment. A total of 139 patients treated between 2001 and 2012 were retrospectively reviewed. The Hs-mGPS varied between 0 and 2. Patients with a score of 2 had a poorer disease-specific survival than patients with a score of 0 (p < 0.001). The estimated five-year rate of disease-specific survival for those with a score of 2 was 0%, compared with 85.4% (95% CI 77.3 to 93.5) for those with a score of 0. Those with a score of 2 also had a poorer disease-specific survival than those with a score of 1 (75.3%, 95% CI 55.8 to 94.8; p < 0.001). Patients with a score of 2 also had a poorer event-free rate than those with a score of 0 (p < 0.001). Those with a score of 2 also had a poorer event-free survival than did those with a score of 1 (p = 0.03). A multivariate analysis showed that the Hs-mGPS remained an independent predictor of survival and recurrence. The Hs-mGPS could be a useful prognostic marker in patients with a soft-tissue sarcoma.

Cite this article: Bone Joint J 2015; 97-B:847–52.

Aims

Osteosarcoma of the pelvis is a particularly difficult tumour to treat as it often presents late, may be of considerable size and/or associated with metastases when it presents, and is frequently chondroid in origin and resistant to chemotherapy.

The aim of this study was to review our experience of managing this group of patients and to identify features predictive of a poor outcome.

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.

Deformity of the proximal femur in fibrous dysplasia leads to deviation of the mechanical axis of the hip, which may lead to the development of secondary osteoarthritis (OA). This study investigated the prevalence and predisposing factors for the development of OA in patients with fibrous dysplasia of the proximal femur. We reviewed the records of 209 patients from our institutional database with fibrous dysplasia of the proximal femur, investigating possible predisposing factors including patient demographics, the extent of the coxa vara deformity, the presence of peri-articular disease, and the overall burden of skeletal disease. Of the 209 patients, 24 (12%) had radiological evidence of OA in the ipsilateral hip. The prevalence was significantly higher in patients with polyostotic fibrous dysplasia compared with those with monostotic disease (p < 0.001). In a subgroup analysis of patients with polyostotic disease, the extent of deformity (quantified using the neck–shaft angle), and the presence of peri-articular disease (whether in the head of the femur or the acetabulum) were significant predictors of osteoarthritis (neck–shaft angle likelihood ratio (LR) = 0.847 per 1° increase, p = 0.004; presence of lesion in the head of the femur LR = 9.947, p = 0.027; presence of lesion in the acetabulum LR = 11.231, p = 0.014).

Our data suggest that patients with polyostotic fibrous dysplasia have a high risk of developing secondary OA of the hips. This risk is higher in patients with peri-articular disease, and those with a more severe deformity of proximal femur.

Cite this article: Bone Joint J 2015;97-B:1007–11.

Objectives

Our objective was to predict the knee extension strength and post-operative function in quadriceps resection for soft-tissue sarcoma of the thigh.

Aims

Surgical intervention in patients with bone metastases from breast cancer is dependent on the estimated survival of the patient. The purpose of this paper was to identify factors that would predict survival so that specific decisions could be made in terms of surgical (or non-surgical) management.

Vascularised fibular grafts (VFGs ) are a valuable surgical technique in limb salvage after resection of a tumour. The primary objective of this multicentre study was to assess the risk factors for failure and complications for using a VFG after resection of a tumour.

The study involved 74 consecutive patients (45 men and 29 women with mean age of 23 years (1 to 64) from four tertiary centres for orthopaedic oncology who underwent reconstruction using a VFG after resection of a tumour between 1996 and 2011. There were 52 primary and 22 secondary reconstructions. The mean follow-up was 77 months (10 to 195).

In all, 69 patients (93%) had successful limb salvage; all of these united and 65 (88%) showed hypertrophy of the graft. The mean time to union differed between those involving the upper (28 weeks; 12 to 96) and lower limbs (44 weeks; 12 to 250). Fracture occurred in 11 (15%), and nonunion in 14 (19%) patients.

In 35 patients (47%) at least one complication arose, with a greater proportion in lower limb reconstructions, non-bridging osteosynthesis, and in children. These complications resulted in revision surgery in 26 patients (35%).

VFG is a successful and durable technique for reconstruction of a defect in bone after resection of a tumour, but is accompanied by a significant risk of complications, that often require revision surgery. Union was not markedly influenced by the need for chemo- or radiotherapy, but should not be expected during chemotherapy. Therefore, restricted weight-bearing within this period is advocated.

Cite this article: Bone Joint J 2015;97-B:853–61.

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.

Monostotic fibrous dysplasia of the proximal femur has a variable clinical course, despite its reported limited tendency to progress.

We investigated the natural history and predisposing factors for progression of dysplasia in a group of 76 patients with a mean follow-up of 8.5 years (2.0 to 15.2). Of these, 31 (41%) presented with an asymptomatic incidental lesion while 45 (59%) presented with pain or a pathological fracture. A group of 23 patients (30%) underwent early operative treatment for pain (19: 25%) or pathological fracture (4: 5%).

Of the 53 patients who were initially treated non-operatively, 45 (85%) remained asymptomatic but eight (15%) needed surgery because of pain or fracture. The progression-free survival of the observation group was 81% (sd 6.4%) at five-years follow-up. An initial presentation of pain (p < 0.001), a limp (p < 0.001), radiological evidence of microfracture (p = 0.001) and younger age (< 17 years) (p = 0.016) were significant predisposing factors for disease progression.

The risk of experiencing pain or pathological fracture is considerable in monostotic fibrous dysplasia of the proximal femur. Patients presenting with pain, a limp or radiological evidence of microfracture have a high chance of needing surgical treatment.

Cite this article: Bone Joint J 2014;96-B:673–6.

The purpose of this study was to evaluate the long-term outcome of patients with a sacral chordoma and the surgical management of locally recurrent disease.

Between October 1990 and August 2013 we operated on 54 consecutive patients with a sacral chordoma. There were 34 men and 20 women with a mean age of 60 years (25 to 86). The mean maximum diameter of the tumour was 9.3 cm (3 to 20).

The mean follow-up was 7.8 years (2 months to 23.4 years). The disease-specific survival was 82% at five years, 57% at ten years and 45% at 15 years. The local recurrence-free survival was 49% at five years, 37% at ten years and 20% at 15 years. Local recurrence occurred in 30 patients (56%) at a mean of 3.8 years (3 months to 13 years) post-operatively.

Survival after the treatment of recurrence was 89% at two years, 56% at five years and 19% at ten years. Of nine patients who had complete resection of a recurrence, one died after 72 months and eight remain disease-free. Incomplete resection of recurrent disease resulted in a survival of 54% at two years and 36% at five years.

For 12 patients with a local recurrence who were treated palliatively, survival was 81% at two years and 31% at five years.

A wide margin of resection gave the best chance of long-term survival and complete resection of local recurrence the best chance of control of disease.

Cite this article: Bone Joint J 2015;97-B:711–15.

The aim of this study was to define the incidence of venous thromboembolism (VTE) and risk factors for the development of deep-vein thrombosis (DVT) after the resection of a musculoskeletal tumour. A total of 94 patients who underwent resection of a musculoskeletal tumour between January 2003 and December 2005 were prospectively studied. There were 42 men and 52 women with a mean age of 54.4 years (18 to 86). All patients wore intermittent pneumatic compression devices and graduated compression stockings. Ultrasound examination of the lower limbs was conducted to screen for DVT between the fifth and ninth post-operative days.

DVT was detected in 21 patients (22%). Of these, two were symptomatic (2%). One patient (1%) had a fatal pulmonary embolism. Patients aged ≥ 70 years had an increased risk of DVT (p = 0.004).

The overall incidence of DVT (both symptomatic and asymptomatic) after resection of a musculoskeletal tumour with mechanical prophylaxis was high. It seems that both mechanical and anticoagulant prophylaxis is needed to prevent VTE in patients who have undergone the resection of a musculoskeletal tumour.

Cite this article: Bone Joint J 2013;95-B:1280–4.

We determined the efficacy of a devitalised autograft (n = 13) and allograft (n = 16) cortical strut bone graft combined with long-stem endoprosthetic reconstruction in the treatment of massive tumours of the lower limb. A total of 29 patients (18 men:11 women, mean age 20.1 years (12 to 45) with a ratio of length of resection to that of the whole prosthesis of > 50% were treated between May 2003 and May 2012. The mean follow-up was 47 months (15 to 132). The stem of the prosthesis was introduced through bone graft struts filled with cement, then cemented into the residual bone. Bone healing was achieved in 23 patients (86%). The mean Musculoskeletal Tumour Society functional score was 85% (57 to 97). The five-year survival rate of the endoprostheses was 81% (95% confidence intervals 67.3 to 92.3). The mean length of devitalised autografts and allografts was 8.6 cm (5 to 15), which increased the ratio of the the length of the stem of the prosthesis to that of the whole length of the prosthesis from a theoretical 35% to an actual 55%.

Cortical strut bone grafting and long-stem endoprosthetic reconstruction is an option for treating massive segmental defects following resection of a tumour in the lower limb. Patients can regain good function with a low incidence of aseptic loosening. The strut graft and the residual bone together serve as a satisfactory bony environment for a revision prosthesis, if required, once union is achieved.

Cite this article: Bone Joint J 2015;97-B:544–9.

We retrospectively reviewed 30 patients with a diffuse-type giant-cell tumour (Dt-GCT) (previously known as pigmented villonodular synovitis) around the knee in order to assess the influence of the type of surgery on the functional outcome and quality of life (QOL). Between 1980 and 2001, 15 of these tumours had been treated primarily at our tertiary referral centre and 15 had been referred from elsewhere with recurrent lesions.

The mean follow-up was 64 months (24 to 393). Functional outcome and QOL were assessed with range of movement and the Knee injury and Osteoarthritis Outcome Score (KOOS), the Musculoskeletal Tumour Society (MSTS) score, the Toronto Extremity Salvage Score (TESS) and the SF-36 questionnaire. There was recurrence in four of 14 patients treated initially by open synovectomy. Local control was achieved after a second operation in 13 of 14 (93%). Recurrence occurred in 15 of 16 patients treated initially by arthroscopic synovectomy. These patients underwent a mean of 1.8 arthroscopies (one to eight) before open synovectomy. This achieved local control in 8 of 15 (53%) after the first synovectomy and in 12 of 15 (80%) after two. The functional outcome and QOL of patients who had undergone primary arthroscopic synovectomy and its attendant subsequent surgical procedures were compared with those who had had a primary open synovectomy using the following measures: range of movement (114º versus 127º; p = 0.03); KOOS (48 versus 71; p = 0.003); MSTS (19 versus 24; p = 0.02); TESS (75 versus 86; p = 0.03); and SF-36 (62 versus 80; p = 0.01).

Those who had undergone open synovectomy needed fewer subsequent operations. Most patients who had been referred with a recurrence had undergone an initial arthroscopic synovectomy followed by multiple further synovectomies. At the final follow-up of eight years (2 to 32), these patients had impaired function and QOL compared with those who had undergone open synovectomy initially.

We conclude that the natural history of Dt-GCT in patients who are treated by arthroscopic synovectomy has an unfavourable outcome, and that primary open synovectomy should be undertaken to prevent recurrence or residual disease.

Cite this article: Bone Joint J 2014; 96-B:1111–18.

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.

We report our experience of using a computer navigation system to aid resection of malignant musculoskeletal tumours of the pelvis and limbs and, where appropriate, their subsequent reconstruction. We also highlight circumstances in which navigation should be used with caution.

We resected a musculoskeletal tumour from 18 patients (15 male, three female, mean age of 30 years (13 to 75) using commercially available computer navigation software (Orthomap 3D) and assessed its impact on the accuracy of our surgery. Of nine pelvic tumours, three had a biological reconstruction with extracorporeal irradiation, four underwent endoprosthetic replacement (EPR) and two required no bony reconstruction. There were eight tumours of the bones of the limbs. Four diaphyseal tumours underwent biological reconstruction. Two patients with a sarcoma of the proximal femur and two with a sarcoma of the proximal humerus underwent extra-articular resection and, where appropriate, EPR. One soft-tissue sarcoma of the adductor compartment which involved the femur was resected and reconstructed using an EPR. Computer navigation was used to aid reconstruction in eight patients.

Histological examination of the resected specimens revealed tumour-free margins in all patients. Post-operative radiographs and CT showed that the resection and reconstruction had been carried out as planned in all patients where navigation was used. In two patients, computer navigation had to be abandoned and the operation was completed under CT and radiological control.

The use of computer navigation in musculoskeletal oncology allows accurate identification of the local anatomy and can define the extent of the tumour and proposed resection margins. Furthermore, it helps in reconstruction of limb length, rotation and overall alignment after resection of an appendicular tumour.

Cite this article: Bone Joint J 2015;97-B:258–64.

Resection of malignant bony tumours of the pelvis creates large bone and soft-tissue defects, and is frequently associated with complications such as wound dehiscence and deep infection. We present the results of six patients in whom a rectus abdominis myocutaneous (RAM) flap was used following resection of a malignant tumour of the pelvis. Bony reconstruction was performed using a constrained hip tumour prosthesis in three patients, vascularised fibular graft in two and frozen autograft in one. At a mean follow-up of 63 months (16 to 115), no patients had a problem with the wound.

Immediate reconstruction using a RAM flap may be used after resection of a malignant tumour of the pelvis to provide an adequate volume of tissue to eliminate the dead space, cover the exposed bone or implants with well-vascularised soft tissue and to reduce the risk of complications.

Cite this article: Bone Joint J 2014;96-B:270–3.

A poor response to chemotherapy (≤ 90% necrosis) for osteosarcomas leads to poorer survival and an increased risk of local recurrence, particularly if there is a close margin of excision. We evaluated whether amputation confers any survival benefit over limb salvage surgery (LSS) with narrow margins in patients who respond poorly to chemotherapy.

We only analysed patients with an osteosarcoma of the limb, a poor response to chemotherapy and close margins on LSS (marginal/intralesional) or primary amputation: 360 patients (36 LSS (intralesional margins), 197 LSS (marginal margins) and 127 amputations) were included. Local recurrence developed in 13 (36%) following LSS with intralesional margins, and 39 (20%) following LSS with marginal margins. There was no local recurrence in patients who underwent amputation. The five-year survival for all patients was 41% (95% confidence interval (CI) 35 to 46), but for those treated by LSS with marginal margins was 46.2% (95% CI 38 to 53), 36.3% (95% CI 27 to 45) for those treated by amputation, and 28% (95 CI 14 to 44) for those treated by LSS with intralesional margins. Patients who had LSS and then developed local recurrence as a first event had the same survival as those who had primary amputation without local recurrence. Prophylactic adjuvant radiotherapy was used in 40 patients but had no discernible effect in preventing local recurrence.

Although amputation offered better local control, it conferred no clear survival benefit over LSS with marginal margins in these patients with a poor overall prognosis.

Cite this article: Bone Joint J 2015;97-B:115–20.

Cancellous allograft bone chips are commonly used in the reconstruction of defects in bone after removal of benign tumours. We investigated the MRI features of grafted bone chips and their change over time, and compared them with those with recurrent tumour. We retrospectively reviewed 66 post-operative MRIs from 34 patients who had undergone curettage and grafting with cancellous bone chips to fill the defect after excision of a tumour. All grafts showed consistent features at least six months after grafting: homogeneous intermediate or low signal intensities with or without scattered hyperintense foci (speckled hyperintensities) on T1 images; high signal intensities with scattered hypointense foci (speckled hypointensities) on T2 images, and peripheral rim enhancement with or without central heterogeneous enhancements on enhanced images. Incorporation of the graft occurred from the periphery to the centre, and was completed within three years. Recurrent lesions consistently showed the same signal intensities as those of pre-operative MRIs of the primary lesions. There were four misdiagnoses, three of which were chondroid tumours.

We identified typical MRI features and clarified the incorporation process of grafted cancellous allograft bone chips. The most important characteristics of recurrent tumours were that they showed the same signal intensities as the primary tumours. It might sometimes be difficult to differentiate grafted cancellous allograft bone chips from a recurrent chondroid tumour.

Cite this article: Bone Joint J 2015;97-B:121–8.

In patients with a tumour affecting the distal ulna it is difficult to preserve the function of the wrist following extensive local resection. We report the outcome of 12 patients (nine female, three male) who underwent excision of the distal ulna without local soft-tissue reconstruction. In six patients, an aggressive benign tumour was present and six had a malignant tumour. At a mean follow-up of 64 months (15 to 132) the mean Musculoskeletal Tumour score was 64% (40% to 93%) and the mean DASH score was 35 (10 to 80). The radiological appearances were satisfactory in most patients. Local recurrence occurred in one patient with benign disease and two with malignant disease. The functional outcome was thus satisfactory at a mean follow-up in excess of five years, with a relatively low rate of complications. The authors conclude that complex reconstructive soft-tissue procedures may not be needed in these patients.

Cite this article: Bone Joint J 2014;96-B:1392–5.

The aim of this study was to determine whether the level of circulating C-reactive protein (CRP) before treatment predicted overall disease-specific survival and local tumour control in patients with a sarcoma of bone.

We retrospectively reviewed 318 patients who presented with a primary sarcoma of bone between 2003 and 2010. Those who presented with metastases and/or local recurrence were excluded.

Elevated CRP levels were seen in 84 patients before treatment; these patients had a poorer disease-specific survival (57% at five years) than patients with a normal CRP (79% at five years) (p < 0.0001). They were also less likely to be free of recurrence (71% at five years) than patients with a normal CRP (79% at five years) (p = 0.04). Multivariate analysis showed the pre-operative CRP level to be an independent predictor of survival and local control. Patients with a Ewing’s sarcoma or chondrosarcoma who had an elevated CRP before their treatment started had a significantly poorer disease-specific survival than patients with a normal CRP (p = 0.02 and p < 0.0001, respectively). Patients with a conventional osteosarcoma and a raised CRP were at an increased risk of poorer local control.

We recommend that CRP levels are measured routinely in patients with a suspected sarcoma of bone as a further prognostic indicator of survival.

Cite this article: Bone Joint J 2013;95-B:411–18.

The aim of this study was to evaluate the functional and oncological outcome of extracorporeally irradiated autografts used to reconstruct the pelvis after a P1/2 internal hemipelvectomy.

The study included 18 patients with a primary malignant bone tumour of the pelvis. There were 13 males and five females with a mean age of 24.8 years (8 to 62). Of these, seven had an osteogenic sarcoma, six a Ewing’s sarcoma, and five a chondrosarcoma. At a mean follow-up of 51.6 months (4 to 185), nine patients had died with metastatic disease while nine were free from disease. Local recurrence occurred in three patients all of whom eventually died of their disease. Deep infection occurred in three patients and required removal of their graft in two while the third underwent a hindquarter amputation for extensive flap necrosis.

The mean Musculoskeletal Tumor Society functional score of the 16 patients who could be followed-up for at least 12 months was 77% (50 to 90). Those 15 patients who completed the Toronto Extremity Salvage Score questionnaire had a mean score of 71% (53 to 85).

Extracorporeal irradiation and re-implantation of bone is a valid method of reconstruction after an internal hemipelvectomy. It has an acceptable morbidity and a functional outcome that compares favourably with other available reconstructive techniques.

Cite this article: Bone Joint J 2014;96-B:1404–10

It is important to be able to identify patients with an increased risk of venous thromboembolism (VTE) in order to minimise the risk of an event. We investigated the incidence and risk factors for post-operative VTE in 168 consecutive patients with a malignancy of the lower limb. The period of study included ten months before and 12 months after the introduction of chemical thromboprophylaxis. All data about the potential risk factors were identified and classified into three groups (patient-, surgery- and tumour-related). The outcome measure was a thromboembolic event within 90 days of surgery.

Of the 168 patients, eight (4.8%) had a confirmed symptomatic deep-vein thrombosis and one (0.6%) a fatal pulmonary embolism. Of the 28 variables tested, age > 60 years, higher American Society of Anesthesiologists grade and metastatic tumour were independent risk factors for VTE. The overall rate of symptomatic VTE was not significantly different between patients who received chemical thromboprophylaxis and those who did not. Knowledge of these risk factors may be of value in improving the surgical outcome of patients with a malignancy of the lower limb.

Cite this article: Bone Joint J 2013;95-B:558–62.

Resection of a primary sarcoma of the diaphysis of a long bone creates a large defect. The biological options for reconstruction include the use of a vascularised and non-vascularised fibular autograft.

The purpose of the present study was to compare these methods of reconstruction.

Between 1985 and 2007, 53 patients (26 male and 27 female) underwent biological reconstruction of a diaphyseal defect after resection of a primary sarcoma. Their mean age was 20.7 years (3.6 to 62.4). Of these, 26 (49 %) had a vascularised and 27 (51 %) a non-vascularised fibular autograft. Either method could have been used for any patient in the study. The mean follow-up was 52 months (12 to 259). Oncological, surgical and functional outcome were evaluated. Kaplan–Meier analysis was performed for graft survival with major complication as the end point.

At final follow-up, eight patients had died of disease. Primary union was achieved in 40 patients (75%); 22 (42%) with a vascularised fibular autograft and 18 (34%) a non-vascularised (p = 0.167). A total of 32 patients (60%) required revision surgery. Kaplan–Meier analysis revealed a mean survival without complication of 36 months (0.06 to 107.3, sd 9) for the vascularised group and 88 months (0.33 to 163.9, sd 16) for the non-vascularised group (p = 0.035).

Both groups seem to be reliable biological methods of reconstructing a diaphyseal bone defect. Vascularised autografts require more revisions mainly due to problems with wound healing in distal sites of tumour, such as the foot.

Cite this article: Bone Joint J 2014;96-B:1258–63.

Giant cell tumour is the most common aggressive benign tumour of the musculoskeletal system and has a high rate of local recurrence. When it occurs in proximity to the hip, reconstruction of the joint is a challenge. Options for reconstruction after wide resection include the use of a megaprosthesis or an allograft-prosthesis composite. We performed a clinical and radiological study to evaluate the functional results of a proximal femoral allograft-prosthesis composite in the treatment of proximal femoral giant cell tumour after wide resection. This was an observational study, between 2006 and 2012, of 18 patients with a mean age of 32 years (28 to 42) and a mean follow-up of 54 months (18 to 79). We achieved excellent outcomes using Harris Hip Score in 13 patients and a good outcome in five. All allografts united. There were no complications such as infection, failure, fracture or resorption of the graft, or recurrent tumour. Resection and reconstruction of giant cell tumours with proximal femoral allograft–prosthesis composite is a better option than using a prosthesis considering preservation of bone stock and excellent restoration of function.

A good result requires demanding bone banking techniques, effective measures to prevent infection and stability at the allograft-host junction.

Cite this article: Bone Joint J 2014; 96-B:1106–10.

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.

The aim of this study was to assess a specific protocol for the treatment of patients with a parosteal osteosarcoma of the distal femur with limb salvage involving hemicortical resection and reconstruction using recycled pasteurised autograft and internal fixation. Between January 2000 and January 2010, 13 patients with a mean age of 26.5 years (17 to 39) underwent this procedure. All the tumours were staged according to Enneking’s criteria: there were eight stage IA tumours and five stage IB tumours. The mean follow-up was 101.6 months (58 to 142), and mean post-operative Musculoskeletal Tumour Society functional score was 88.6% (80% to 100%) at the final follow-up. All the patients had achieved bony union; the mean time to union was 11.2 months (6 to 18). Local recurrence occurred in one patient 27 months post-operatively. No patient had a pulmonary metastasis.

A hemicortical procedure for the treatment of a parosteal osteosarcoma is safe and effective. Precise pre-operative planning using MRI is essential in order to define the margins of resection. Although it is a technically demanding procedure, gratifying results make it worthwhile for selected patients.

Cite this article: Bone Joint J 2013;95-B:1275–9.

The pre-operative differentiation between enchondroma, low-grade chondrosarcoma and high-grade chondrosarcoma remains a diagnostic challenge. We reviewed the accuracy and safety of the radiological grading of cartilaginous tumours through the assessment of, first, pre-operative radiological and post-operative histological agreement, and second the rate of recurrence in lesions confirmed as high-grade on histology. We performed a retrospective review of major long bone cartilaginous tumours managed by curettage as low grade between 2001 and 2012. A total of 53 patients with a mean age of 47.6 years (8 to 71) were included. There were 23 men and 30 women. The tumours involved the femur (n = 20), humerus (n = 18), tibia (n = 9), fibula (n = 3), radius (n = 2) and ulna (n = 1). Pre-operative diagnoses resulted from multidisciplinary consensus following radiological review alone for 35 tumours, or with the addition of pre-operative image guided needle biopsy for 18. The histologically confirmed diagnosis was enchondroma for two (3.7%), low-grade chondrosarcoma for 49 (92.6%) and high-grade chondrosarcoma for two (3.7%). Three patients with a low-grade tumour developed a local recurrence at a mean of 15 months (12 to 17) post-operatively. A single high-grade recurrence (grade II) was treated with tibial diaphyseal replacement. The overall recurrence rate was 7.5% at a mean follow-up of 4.7 years (1.2 to 12.3). Cartilaginous tumours identified as low-grade on pre-operative imaging with or without additional image-guided needle biopsy can safely be managed as low-grade without pre-operative histological diagnosis. A few tumours may demonstrate high-grade features histologically, but the rates of recurrence are not affected.

Cite this article: Bone Joint J 2014; 96-B:1098–105.

We evaluated the risk of late relapse and further outcome in patients with soft-tissue sarcomas who were alive and event-free more than five years after initial treatment. From our database we identified 1912 patients with these pathologies treated between 1980 and 2006. Of these 1912 patients, 603 were alive and event-free more than five years after initial treatment and we retrospectively reviewed them. The mean age of this group was 48 years (4 to 94) and 340 were men. The mean follow-up was 106 months (60 to 336). Of the original cohort, 582 (97%) were alive at final follow-up. The disease-specific survival was 96.4% (95% confidence interval (CI) 94.4 to 98.3) at ten years and 92.9% (95% CI 89 to 96.8) at 15 years. The rate of late relapse was 6.3% (38 of 603). The ten- and 15-year event-free rates were 93.2% (95% CI 90.8 to 95.7) and 86.1% (95% CI 80.2 to 92.1), respectively. Multivariate analysis showed that tumour size and tumour grade remained independent predictors of events. In spite of further treatment, 19 of the 38 patients died of sarcoma. The three- and five-year survival rates after the late relapse were 56.2% (95% CI 39.5 to 73.3) and 43.2% (95% CI 24.7 to 61.7), respectively, with a median survival time of 46 months. Patients with soft-tissue sarcoma, especially if large, require long-term follow-up, especially as they have moderate potential to have their disease controlled.

Cite this article: Bone Joint J 2013;95-B:1139–43.

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.

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.

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.

In 1999, we developed a technique for biological reconstruction after excision of a bone tumour, which involved using autografts of the bone containing the tumour treated with liquid nitrogen. We have previously reported the use of this technique in 28 patients at a mean follow up of 27 months (10 to 54).

In this study, we included 72 patients who underwent reconstruction using this technique. A total of 33 patients died and three were lost to follow-up, at a mean of 23 months (2 to 56) post-operatively, leaving 36 patients available for a assessment at a mean of 101 months 16 to 163) post-operatively. The methods of reconstruction included an osteo-articular graft in 16, an intercalary in 13 and, a composite graft with prosthesis in seven.

Post-operative function was excellent in 26 patients (72.2%), good in seven (19.4%), and fair in three (8.3%) according to the functional evaluation system of Enneking. No recurrent tumour occurred within the grafts. The autografts survived in 29 patients (80.6%), and the rates of survival at five and ten years were 86.1% and 80.6 %, respectively. Seven of 16 osteo-articular grafts (44%) failed because of fracture or infection, but all the composite and intercalary grafts survived.

The long-term outcomes of frozen autografting, particularly using composite and intercalary grafts, are satisfactory and thus represent a good method of treatment for patients with a sarcoma of bone or soft tissue.

Cite this article: Bone Joint J 2014;96-B:555–61.

The purpose of this study was to assess whether the use of a joint-sparing technique such as curettage and grafting was successful in eradicating giant cell tumours of the proximal femur, or whether an alternative strategy was more appropriate.

Between 1974 and 2012, 24 patients with a giant cell tumour of the proximal femur were treated primarily at our hospital. Treatment was either joint sparing or joint replacing. Joint-sparing treatment was undertaken in ten patients by curettage with or without adjunctive bone graft. Joint replacement was by total hip replacement in nine patients and endoprosthetic replacement in five. All 11 patients who presented with a pathological fracture were treated by replacement.

Local recurrence occurred in five patients (21%): two were treated by hip replacement, three by curettage and none with an endoprosthesis. Of the ten patients treated initially by curettage, six had a successful outcome without local recurrence and required no further surgery. Three eventually needed a hip replacement for local recurrence and one an endoprosthetic replacement for mechanical failure. Thus 18 patients had the affected joint replaced and only six (25%) retained their native joint. Overall, 60% of patients without a pathological fracture who were treated with curettage had a successful outcome.

Cite this article: Bone Joint J 2014;96-B:127–31.

We investigated the functional outcome in patients who underwent reverse shoulder replacement (RSR) after removal of a tumour of the proximal humerus. A total of 16 patients (ten women and six men) underwent this procedure between 1998 and 2011 in our hospital. Five patients died and one was lost to follow-up. Ten patients were available for review at a mean follow-up of 46 months (12 to 136). Eight patients had a primary and two patients a secondary bone tumour.

At final follow up the mean range of active movement was: abduction 78° (30° to 150°); flexion 98° (45° to 180°); external rotation 32° (10° to 60°); internal rotation 51° (10° to 80°). The mean Musculoskeletal Tumor Society score was 77% (60% to 90%) and the mean Toronto Extremity Salvage Score was 70% (30% to 91%). Two patients had a superficial infection and one had a deep infection and underwent a two-stage revision procedure. In two patients there was loosening of the RSR; one dislocated twice. All patients had some degree of atrophy or pseudo-atrophy of the deltoid muscle.

Use of a RSR in patients with a tumour of the proximal humerus gives acceptable results.

Cite this article: Bone Joint J 2013;95-B:1551–5.

We welcome letters to the Editor concerning articles that have recently been published. Such letters will be subject to the usual stages of selection and editing; where appropriate the authors of the original article will be offered the opportunity to reply.

We evaluated the clinical results and complications after extra-articular resection of the distal femur and/or proximal tibia and reconstruction with a tumour endoprosthesis (MUTARS) in 59 patients (mean age 33 years (11 to 74)) with malignant bone or soft-tissue tumours. According to a Kaplan–Meier analysis, limb survival was 76% (95% confidence interval (CI) 64.1 to 88.5) after a mean follow-up of 4.7 years (one month to 17 years). Peri-prosthetic infection was the most common indication for subsequent amputation (eight patients). Survival of the prosthesis without revision was 48% (95% CI 34.8 to 62.0) at two years and 25% (95% CI 11.1 to 39.9) at five years post-operatively. Failure of the prosthesis was due to deep infection in 22 patients (37%), aseptic loosening in ten patients (17%), and peri-prosthetic fracture in six patients (10%). Wear of the bearings made a minor revision necessary in 12 patients (20%). The mean Musculoskeletal Tumor Society score was 23 (10 to 29). An extensor lag > 10° was noted in ten patients (17%).

These results suggest that limb salvage after extra-articular resection with a tumour prosthesis can achieve good functional results in most patients, although the rates of complications and subsequent amputation are higher than in patients treated with intra-articular resection.

Cite this article: Bone Joint J 2013;95-B:1425–31.

We investigated the clinical outcome of internal fixation for pathological fracture of the femur after primary excision of a soft-tissue sarcoma that had been treated with adjuvant radiotherapy.

A review of our database identified 22 radiation-induced fractures of the femur in 22 patients (seven men, 15 women). We noted the mechanism of injury, fracture pattern and any complications after internal fixation, including nonunion, hardware failure, secondary fracture or deep infection.

The mean age of the patients at primary excision of the tumour was 58.3 years (39 to 86). The mean time from primary excision to fracture was 73.2 months (2 to 195). The mean follow-up after fracture fixation was 65.9 months (12 to 205). Complications occurred in 19 patients (86%). Nonunion developed in 18 patients (82%), of whom 11 had a radiological nonunion at 12 months, five a nonunion and hardware failure and two an infected nonunion. One patient developed a second radiation-associated fracture of the femur after internal fixation and union of the initial fracture. A total of 13 patients (59%) underwent 24 revision operations.

Internal fixation of a pathological fracture of the femur after radiotherapy for a soft-tissue sarcoma has an extremely high rate of complication and requires specialist attention.

Cite this article: Bone Joint J 2013;95-B:1144–8.

We hypothesised that the use of computer navigation-assisted surgery for pelvic and sacral tumours would reduce the risk of an intralesional margin. We reviewed 31 patients (18 men and 13 women) with a mean age of 52.9 years (13.5 to 77.2) in whom computer navigation-assisted surgery had been carried out for a bone tumour of the pelvis or sacrum. There were 23 primary malignant bone tumours, four metastatic tumours and four locally advanced primary tumours of the rectum. The registration error when using computer navigation was <  1 mm in each case. There were no complications related to the navigation, which allowed the preservation of sacral nerve roots (n = 13), resection of otherwise inoperable disease (n = 4) and the avoidance of hindquarter amputation (n = 3). The intralesional resection rate for primary tumours of the pelvis and sacrum was 8.7% (n = 2): clear bone resection margins were achieved in all cases. At a mean follow-up of 13.1 months (3 to 34) three patients (13%) had developed a local recurrence. The mean time alive from diagnosis was 16.8 months (4 to 48).

Computer navigation-assisted surgery is safe and has reduced our intralesional resection rate for primary tumours of the pelvis and sacrum. We recommend this technique as being worthy of further consideration for this group of patients.

Cite this article: Bone Joint J 2013;95-B:1417–24.

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.

The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing’s sarcoma. We identified 300 patients with new primary Ewing’s sarcoma diagnosed between 1980 and 2005 from the centres’ local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing’s sarcomas. Although patients with skeletal Ewing’s were younger (mean age 16.8 years) than those with extra-skeletal Ewing’s sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had surgery. There was no difference in the overall survival of patients with skeletal (64%) and extra-skeletal Ewing’s sarcoma (61%) (p = 0.85), and this was also the case when both groups were split by whether they had metastases or not.

This large series has shown that the oncological outcomes of Ewing’s sarcoma are related to tumour characteristics and patient age, and not determined by whether they arise in bone or soft tissue.

Segmental resection of malignant bone disease in the femoral diaphysis with subsequent limb reconstruction is a major undertaking. This is a retrospective review of 23 patients who had undergone limb salvage by endoprosthetic replacement of the femoral diaphysis for a primary bone tumour between 1989 and 2005.

There were 16 males and seven females, with a mean age of 41.3 years (10 to 68). The mean overall follow-up was for 97 months (3 to 240), and 120 months (42 to 240) for the living patients. The cumulative patient survival was 77% (95% confidence interval 63% to 95%) at ten years. Survival of the implant, with failure of the endoprosthesis as an endpoint, was 85% at five years and 68% (95% confidence interval 42% to 92%) at ten years. The revision rate was 22% and the overall rate of re-operation was 26%. Complications included deep infection (4%), breakage of the prosthesis (8%), periprosthetic fracture (4%), aseptic loosening (4%), local recurrence (4%) and metastases (17%). The 16 patients who retained their diaphyseal endoprosthesis had a mean Musculoskeletal Tumour Society score of 87% (67% to 93%). They were all able to comfortably perform most activities of daily living.

Femoral diaphyseal endoprosthetic replacement is a viable option for reconstruction following segmental resection of malignant bone disease. It allows immediate weight-bearing, is associated with a good long-term functional outcome, has an acceptable complication and revision rate and, most importantly, does not appear to compromise patient survival.

We evaluated the long-term functional outcome in 118 patients treated for osteosarcoma or Ewing’s sarcoma in the extremities a minimum of five years after treatment. We also examined if impaired function influenced their quality of life and ability to work.

The function was evaluated according to the Musculoskeletal Tumor Society (MSTS) score and the Toronto Extremity Salvage Score (TESS). Quality of life was assessed by using the Short Form-36 (SF-36).

The mean age at follow-up was 31 years (15 to 57) and the mean follow-up was for 13 years (6 to 22). A total of 67 patients (57%) initially had limb-sparing surgery, but four had a secondary amputation. The median MSTS score was 70% (17% to 100%) and the median TESS was 89% (43% to 100%). The amputees had a significantly lower MSTS score than those with limb-sparing surgery (p < 0.001), but there was no difference for the TESS. Tumour localisation above knee level resulted in significantly lower MSTS scores and TESS (p = 0.003 and p = 0.02, respectively).

There were no significant differences in quality of life between amputees and those with limb-sparing surgery except in physical functioning. Of the patients 11% (13) did not work or study. In multivariate analysis, amputation, tumour location above the knee and having muscular pain were associated with low physical function.

We conclude that most of the bone tumour survivors managed well after adjustment to their physical limitations. A total of 105 are able to work and have an overall good quality of life.

We evaluated the oncological and functional outcome of 18 patients, whose malignant bone tumours were excised with the assistance of navigation, and who were followed up for more than three years. There were 11 men and seven women, with a mean age of 31.8 years (10 to 57). There were ten operations on the pelvic ring and eight joint-preserving limb salvage procedures. The resection margins were free of tumour in all specimens. The tumours, which were stage IIB in all patients, included osteosarcoma, high-grade chondrosarcoma, Ewing’s sarcoma, malignant fibrous histiocytoma of bone, and adamantinoma. The overall three-year survival rate of the 18 patients was 88.9% (95% confidence interval (CI) 75.4 to 100). The three-year survival rate of the patients with pelvic malignancy was 80.0% (95% CI 55.3 to 100), and of the patients with metaphyseal malignancy was 100%. The event-free survival was 66.7% (95% CI 44.9 to 88.5). Local recurrence occurred in two patients, both of whom had a pelvic malignancy. The mean Musculoskeletal Tumor Society functional score was 26.9 points at a mean follow-up of 48.2 months (22 to 79).

We suggest that navigation can be helpful during surgery for musculoskeletal tumours; it can maximise the accuracy of resection and minimise the unnecessary sacrifice of normal tissue by providing precise intra-operative three-dimensional radiological information.

The purpose of this study was to assess the outcome of 15 patients (mean age 13.6 years (7 to 25)) with a primary sarcoma of the tibial diaphysis who had undergone excision of the affected segment that was then irradiated (90 Gy) and reimplanted with an ipsilateral vascularised fibular graft within it.

The mean follow-up was 57 months (22 to 99). The mean time to full weight-bearing was 23 weeks (9 to 57) and to complete radiological union 42.1 weeks (33 to 55). Of the 15 patients, seven required a further operation, four to obtain skin cover. The mean Musculoskeletal Society Tumor Society functional score at final follow-up was 27 out of 30 once union was complete. The functional results were comparable with those of allograft reconstruction and had a similar rate of complication.

We believe this to be a satisfactory method of biological reconstruction of the tibial diaphysis in selected patients.

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.

Skeletal metastases from hepatocellular carcinoma are highly destructive vascular lesions which severely reduce the quality of life. Pre-existing liver cirrhosis presents unique challenges during the surgical management of such lesions. We carried out a retrospective study of 42 patients who had been managed surgically for skeletal metastases from hepatocellular carcinoma affecting the appendicular skeleton between January 2000 and December 2006. There were 38 men and four women with a mean age of 60.2 years (46 to 77). Surgery for a pathological fracture was undertaken in 30 patients and because of a high risk of fracture in 12. An intralesional surgical margin was achieved in 36 and a wide margin in six. Factors influencing survival were determined by univariate and multivariate analyses.

The survival rates at one, two and three years after surgery were 42.2%, 25.8% and 19.8%, respectively. The median survival time was ten months (95% confidence interval 6.29 to 13.71). The number of skeletal metastases and the Child-Pugh grade were identified as independent prognostic factors by Cox regression analysis. The method of management of the hepatocellular carcinoma, its status in the liver, the surgical margin for skeletal metastases, the presence of a pathological fracture and adjuvant radiotherapy were not found to be significantly related to the survival of the patient, which was affected by hepatic function, as represented by the Child-Pugh grade.

Giant cell tumours (GCT) of the synovium and tendon sheath can be classified into two forms: localised (giant cell tumour of the tendon sheath, or nodular tenosynovitis) and diffuse (diffuse-type giant cell tumour or pigmented villonodular synovitis). The former principally affects the small joints. It presents as a solitary slow-growing tumour with a characteristic appearance on MRI and is treated by surgical excision. There is a significant risk of multiple recurrences with aggressive diffuse disease. A multidisciplinary approach with dedicated MRI, histological assessment and planned surgery with either adjuvant radiotherapy or systemic targeted therapy is required to improve outcomes in recurrent and refractory diffuse-type GCT.

Although arthroscopic synovectomy through several portals has been advocated as an alternative to arthrotomy, there is a significant risk of inadequate excision and recurrence, particularly in the posterior compartment of the knee. For local disease partial arthroscopic synovectomy may be sufficient, at the risk of recurrence. For both local and diffuse intra-articular disease open surgery is advised for recurrent disease. Marginal excision with focal disease will suffice, not dissimilar to the treatment of GCT of tendon sheath. For recurrent and extra-articular soft-tissue disease adjuvant therapy, including intra-articular radioactive colloid or moderate-dose external beam radiotherapy, should be considered.

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.

We treated 98 patients with peri-acetabular tumours by resection and reconstruction with a custom-made pelvic endoprosthesis. The overall survival of the patients was 67% at five years, 54% at ten years and 51% at 30 years. One or more complications occurred in 58.1% of patients (54), of which infection was the most common, affecting 30% (28 patients). The rate of local recurrence was 31% (29 patients) after a mean follow-up of 71 months (11 to 147). Dislocation occurred in 20% of patients (19). Before 1996 the rate was 40.5% (17 patients) but this was reduced to 3.9% (two patients) with the introduction of a larger femoral head. There were six cases of palsy of the femoral nerve with recovery in only two. Revision or excision arthroplasty was performed in 23.7% of patients (22), principally for uncontrolled infection or aseptic loosening. Higher rates of death, infection and revision occurred in men.

This method of treatment is still associated with high morbidity. Patients should be carefully selected and informed of this pre-operatively.

We retrospectively reviewed 71 histopathologically-confirmed bone and soft-tissue metastases of unknown origin at presentation. In order to identify the site of the primary tumour all 71 cases were examined with conventional procedures, including CT, serum tumour markers, a plain radiograph, ultrasound examination and endoscopic examinations, and 24 of the 71 cases underwent 2-deoxy-2-[F-18] fluoro-D-glucose positron emission tomography (FDG-PET). This detected multiple bone metastases in nine patients and the primary site in 12 of the 24 cases; conventional studies revealed 16 primary tumours. There was no significant difference in sensitivity between FDG-PET and conventional studies.

The mean maximal standardised uptake value of the metastatic tumours was significantly higher than that of the primary tumours, which is likely to explain why FDG-PET did not provide better results. It was not superior to conventional procedures in the search for the primary site of bone and soft-tissue metastases; however, it seemed to be useful in the staging of malignancy.

We have investigated the significance of the method of treatment on the oncological and functional outcomes and on the complications in 184 patients with soft-tissue sarcomas of the adductor compartment managed at three international centres. The overall survival at five years was 65% and was related to the grade at diagnosis and the size of the tumour. There was no difference in overall survival between the three centres. There was, however, a significant difference in local control with a rate of 28% in Centre 1 compared with 10% in Centre 2 and 5% in Centre 3.

The overall mean functional score using the Toronto Extremity Salvage Score in 70 patients was 77% but was significantly worse in patients with wound complications or high-grade tumours. The scores were not affected by the timing of radiotherapy or the use of muscle flaps.

This large series of soft-tissue sarcomas of the adductor compartment has shown that factors influencing survival do not vary across the international boundaries studied, but that methods of treatment affect complications, local recurrence and function.

We identified eight patients of 2900 with a primary malignant bone tumour who had coexisting neurofibromatosis type 1. This was a much higher incidence than would be expected by chance. The patients had a mean age of 22.4 years (9 to 54): five were male. Two patients subsequently developed a second bone sarcoma, one of which was radiation induced. Four of the primary tumours were osteosarcomas, four were spindle-cell sarcomas and one a Ewing’s sarcoma. All the patients were treated with chemotherapy and surgery: six of the eight appear to be cured.

This study suggests a possible relationship between neurofibromatosis type 1 and the development of a bone sarcoma, the increased risk being estimated at eight times that of the normal population. We recommend that further research into this possible link should be considered.

We investigated the eventual diagnosis in patients referred to a tertiary centre with a possible diagnosis of a primary bone malignancy.

We reviewed our database from between 1986 and 2010, during which time 5922 patients referred with a suspicious bone lesion had a confirmed diagnosis. This included bone sarcoma in 2205 patients (37%), benign bone tumour in 1309 (22%), orthopaedic conditions in 992 (17%), metastatic disease in 533 (9%), infection in 289 (5%) and haematological disease in 303 (5%). There was a similar frequency of all diagnoses at different ages except for metastatic disease. Only 0.6% of patients (17 of 2913) under the age of 35 years had metastatic disease compared with 17.1% (516 of 3009) of those over 35 years (p < 0.0001). Of the 17 patients under 35 years with metastatic disease, only four presented with an isolated lesion, had no past history of cancer and were systematically well.

Patients under the age of 35 years should have suitable focal imaging (plain radiography, CT or MRI) and simple systemic studies (blood tests and chest radiography). Reduction of the time to biopsy can be achieved by avoiding an unnecessary investigation for a primary tumour to rule out metastatic disease.

We have investigated the oncological outcome of 63 patients with soft-tissue sarcomas of the hand managed at three major centres in the United Kingdom. There were 44 males and 19 females with a mean age of 45 years (11 to 92). The three most common diagnoses were synovial sarcoma, clear cell sarcoma and epithelioid sarcoma. Local excision was carried out in 45 patients (71%) and amputation in 18 (29%). All those treated by amputation had a wide margin of excision but this was only achieved in 58% of those treated by local excision. The risk of local recurrence was 6% in those treated by amputation compared with 42% for those who underwent attempted limb salvage. An inadequate margin of excision resulted in a 12 times greater risk of local recurrence when compared with those in whom a wide margin of excision had been achieved. We were unable to demonstrate any role for radiotherapy in decreasing the risk of local recurrence when there was an inadequate margin of excision. Patients with an inadequate margin of excision had a much higher risk of both local recurrence and metastasis than those with wide margins. The overall survival rate at five years was 87% and was related to the grade and size of the tumour and to the surgical margin.

We have shown that a clear margin of excision is essential to achieve local control of a soft-tissue sarcoma in the hand and that failure to achieve this results in a high risk of both local recurrence and metastastic disease.