Aims. The preoperative grading of chondrosarcomas of bone that accurately predicts surgical management is difficult for surgeons, radiologists, and pathologists. There are often discrepancies in grade between the initial biopsy and the final histology. Recent advances in the use of imaging methods have shown promise in the ability to predict the final grade. The most important clinical distinction is between grade 1 chondrosarcomas, which are amenable to curettage, and resection-grade chondrosarcomas (grade 2 and 3) which require en bloc resection. The aim of this study was to evaluate the use of a Radiological Aggressiveness Score (RAS) to predict the grade of primary chondrosarcomas in long bones and thus to guide management. Methods. A total of 113 patients with a primary chondrosarcoma of a long bone presenting between January 2001 and December 2021 were identified on retrospective review of a single oncology centre’s prospectively collected database. The nine-parameter RAS included variables from radiographs and MRI scans. The best cut-off of parameters to predict the final grade of chondrosarcoma after resection was determined using a receiver operating characteristic curve (ROC), and this was correlated with the biopsy grade. Results. A RAS of ≥ four parameters was 97.9% sensitive and 90.5% specific in predicting resection-grade chondrosarcoma based on a ROC cut-off derived using the Youden index. Cronbach’s α of 0.897 was derived as the interclass correlation for scoring the lesions by four blinded reviewers who were surgeons. Concordance between resection-grade lesions predicted from the RAS and ROC cut-off with the final grade after resection was 96.46%. Concordance between the biopsy grade and the final grade was 63.8%. However, when the patients were analyzed based on surgical management, the initial biopsy was able to differentiate low-grade from resection-grade chondrosarcomas in 82.9% of biopsies. Conclusion. These findings suggest that the RAS is an accurate method for guiding the surgical management of patients with these tumours, particularly when the initial biopsy results are discordant with the clinical presentation. Cite this article: Bone Joint J 2023;105-B(7):808–814

Aims. Iliosacral sarcoma resections have been shown to have high rates of local recurrence (LR) and poor overall survival. There is also no universal classification for the resection of pelvic sarcomas invading the sacrum. This study proposes a novel classification system and analyzes the survival and risk of recurrence, when using this system. Methods. This is a retrospective analysis of 151 patients (with median follow-up in survivors of 44 months (interquartile range 12 to 77)) who underwent hemipelvectomy with iliosacral resection at a single centre between 2007 and 2019. The proposed classification differentiates the extent of iliosacral resection and defines types S1 to S6 (S1 resection medial and parallel to the sacroiliac joint, S2 resection through the ipsilateral sacral lateral mass to the neuroforamina, S3 resection through the ipsilateral neuroforamina, S4 resection through ipsilateral the spinal canal, and S5 and S6 contralateral sacral resections). Descriptive statistics and the chi-squared test were used for categorical variables, and the Kaplan-Meier survival analysis were performed. Results. Resections were S1 in 25/151 patients (17%), S2 in 70/151 (46%), S3 in 33/151 (22%), S4 in 77/151 (11%), S5 in 4/151 (3%), and S6 in 2/151 (1%). An internal hemipelvectomy was performed in 113/151 patients (75%), and 38/151 patients (25%) had an external hemipelvectomy. The predominant types of sarcoma were high-grade osteosarcoma in 48/151 patients (32%), chondrosarcoma in 41/151 (27%), Ewing sarcoma in 33/151 (22%), pleomorphic sarcoma in 17/151 (11%), and others in 2/151 (8%). LR was found in 24/151 patients (15%) with S3, S5, with S6 resections showing the highest rate of LR (p = 0.038). Overall, 19/151 patients (16%) had evidence of metastastic disease at the time of surgery and these patients showed poorer survival when compared to patients with no metastasis. Conclusion. The proposed classification can help to report and compare different surgical and reconstructive approaches in these difficult cases who are still have a considerable risk of LR. Cite this article: Bone Joint J 2022;104-B(2):290–296

Aims. Accurate estimations of the risk of fracture due to metastatic bone disease in the femur is essential in order to avoid both under-treatment and over-treatment of patients with an impending pathological fracture. The purpose of the current retrospective in vivo study was to use CT-based finite element analyses (CTFEA) to identify a clear quantitative differentiating factor between patients who are at imminent risk of fracturing their femur and those who are not, and to identify the exact location of maximal weakness where the fracture is most likely to occur. Methods. Data were collected on 82 patients with femoral metastatic bone disease, 41 of whom did not undergo prophylactic fixation. A total of 15 had a pathological fracture within six months following the CT scan, and 26 were fracture-free during the five months following the scan. The Mirels score and strain fold ratio (SFR) based on CTFEA was computed for all patients. A SFR value of 1.48 was used as the threshold for a pathological fracture. The sensitivity, specificity, positive, and negative predicted values for Mirels score and SFR predictions were computed for nine patients who fractured and 24 who did not, as well as a comparison of areas under the receiver operating characteristic curves (AUC of the ROC curves). Results. The sensitivity of SFR was 100% compared with 88% for the Mirels score, and the specificity of SFR was 67% compared with 38% for the Mirels score. The AUC was 0.905 for SFR compared with 0.578 for the Mirels score (p = 0.008). Conclusion. All the patients who sustained a pathological fracture of the femur had an SFR of > 1.48. CTFEA was far better at predicting the risk of fracture and its location accurately compared with the Mirels score. CTFEA is quick and automated and can be incorporated into the protocol of CT scanners. Cite this article: Bone Joint J 2020;102-B(5):638–645

Aims

The present study investigated receptor activator of nuclear factor kappa-Β ligand (RANKL), osteoprotegerin (OPG), and Runt-related transcription factor 2 (RUNX2) gene expressions in giant cell tumour of bone (GCTB) patients in relationship with tumour recurrence. We also aimed to investigate the influence of CpG methylation on the transcriptional levels of RANKL and OPG.

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The aim of this study was to identify factors associated with five-year cancer-related mortality in patients with limb and trunk soft-tissue sarcoma (STS) and develop and validate machine learning algorithms in order to predict five-year cancer-related mortality in these patients.

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Clear cell sarcoma (CCS) of soft-tissue is a rare melanocytic subtype of mesenchymal malignancy. The aim of this study was to investigate the clinical and therapeutic factors associated with increased survival, stratified by clinical stage, in order to determine the optimal treatment.

Aims

Low-grade central osteosarcoma (LGCOS), a rare type of osteosarcoma, often has misleading radiological and pathological features that overlap with those of other bone tumours, thereby complicating diagnosis and treatment. We aimed to analyze the clinical, radiological, and pathological features of patients with LGCOS, with a focus on diagnosis, treatment, and outcomes.

Aims. 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. Materials and Methods. All members of the Dutch Orthopaedic Society (DOS) and the European Musculoskeletal Oncology Society (EMSOS) were invited to complete a web-based questionnaire containing 12 cases. Results. A total of 96 of 948 members of the DOS (10.1%; groups 1 and 2) and 33 of 182 members of the EMSOS (18%; group 3) replied. The estimation of survival was accurate by more than 50% of all three groups, if the expected survival was short (< 3 months) or long (> 12 months). General orthopaedic surgeons preferred using an intramedullary nail for fractures of the humerus and femur, irrespective of the expected survival or the origin of primary tumour or the location of the fracture. Oncological orthopaedic surgeons recommended prosthetic reconstruction in patients with a long expected survival. Conclusion. Identifying patients who require centralized care, as opposed to those who can be adequately treated in a regional centre, can improve the management of patients with pathological fractures. This differentiation should be based on the expected survival, the type and extent of the tumour, and the location of the fracture. Cite this article: Bone Joint J 2018;100-B:1392–8

Aims

Due to its indolent clinical behaviour, the treatment paradigm of atypical cartilaginous tumours (ACTs) in the long bones is slowly shifting from intralesional resection (curettage) and local adjuvants, towards active surveillance through wait-and-scan follow-up. In this retrospective cohort study performed in a tertiary referral centre, we studied the natural behaviour of ACT lesions by active surveillance with MRI. Clinical symptoms were not considered in the surveillance programme.

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Venous tumour thrombus (VTT) is a rare finding in osteosarcoma. Despite the high rate of VTT in osteosarcoma of the pelvis, there are very few descriptions of VTT associated with extrapelvic primary osteosarcoma. We therefore sought to describe the prevalence and presenting features of VTT in osteosarcoma of both the pelvis and the limbs.

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The aim of this study was to report the patterns of symptoms and insufficiency fractures in patients with tumour-induced osteomalacia (TIO) to allow the early diagnosis of this rare condition.

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

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

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

Aims

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

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

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

Aims

Local recurrence remains a challenging and common problem following curettage and joint-sparing surgery for giant cell tumour of bone (GCTB). We previously reported a 15% local recurrence rate at a median follow-up of 30 months in 20 patients with high-risk GCTB treated with neoadjuvant Denosumab. The aim of this study was to determine if this initial favourable outcome following the use of Denosumab was maintained with longer follow-up.

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To assess the correlation between the histological response to preoperative chemotherapy and event-free survival (EFS) or overall survival (OS) in patients with high-grade localized osteosarcoma.

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Our aim was to develop and validate nomograms that would predict the cumulative incidence of sarcoma-specific death (CISSD) and disease progression (CIDP) in patients with localized high-grade primary central and dedifferentiated chondrosarcoma.

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To investigate the benefits of denosumab in combination with nerve-sparing surgery for treatment of sacral giant cell tumours (GCTs).

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The early mortality in patients with hip fractures from bony metastases is unknown. The objectives of this study were to quantify 30- and 90-day mortality in patients with proximal femoral metastases, and to create a mortality prediction tool based on biomarkers associated with early death.

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The aim of this study was to report the outcomes of patients who underwent definitive surgery for secondary chondrosarcomas arising from osteochondromas.

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The purpose of this study was to investigate the potential for achieving local and systemic control after local recurrence of a chondrosarcoma of bone

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

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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.

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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 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.

Objectives

The diagnosis of surgical site infection following endoprosthetic reconstruction for bone tumours is frequently a subjective diagnosis. Large clinical trials use blinded Central Adjudication Committees (CACs) to minimise the variability and bias associated with assessing a clinical outcome. The aim of this study was to determine the level of inter-rater and intra-rater agreement in the diagnosis of surgical site infection in the context of a clinical trial.

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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.

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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.

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.

Atypical cartilaginous tumours are usually treated by curettage. The purpose of this study was to show that radiofrequency ablation was an effective alternative treatment.

We enrolled 20 patients (two male, 18 female, mean age 56 years (36 to 72) in a proof-of-principle study. After inclusion, biopsy and radiofrequency ablation were performed, followed three months later by curettage and adjuvant phenolisation. The primary endpoint was the proportional necrosis in the retrieved material. Secondary endpoints were correlation with the findings on gadolinium enhanced MRI, functional outcome and complications.

Our results show that 95% to 100% necrosis was obtained in 14 of the 20 patients. MRI had a 91% sensitivity and 67% specificity for detecting residual tumour after curettage. The mean functional outcome (MSTS) score six weeks after radiofrequency ablation was 27.1 (23 to 30) compared with 18.1 (12 to 25) after curettage (p < 0.001). No complications occurred after ablation, while two patients developed a pathological fracture after curettage.

We have shown that radiofrequency ablation is capable of completely eradicating cartilaginous tumour cells in selective cases. MRI has a 91% sensitivity for detecting any residual tumour. Radiofrequency ablation can be performed on an outpatient basis allowing a rapid return to normal activities. If it can be made more effective, it has the potential to provide better local control, while improving functional outcome.

Cite this article: Bone Joint J 2014;96-B:1540–5.

Previous classification systems of failure of limb salvage focused primarily on endoprosthetic failures and lacked sufficient depth for the effective study of the causes of failure. In order to address these inadequacies, the International Society of Limb Salvage (ISOLS) formed a committee to recommend revisions of the previous systems. The purpose of this study was to report on their recommendations. The modifications were prepared using an earlier, evidence-based model with subclassification based on the existing medical literature. Subclassification for all five primary types of failure of limb salvage following endoprosthetic reconstruction were formulated and a complementary system was derived for the failure of biological reconstruction. An additional classification of failure in paediatric patients was also described.

Limb salvage surgery presents a complex array of potential mechanisms of failure, and a complete and precise classification of types of failure is required. Earlier classification systems lacked specificity, and the evidence-based system outlined here is designed to correct these weaknesses and to provide a means of reporting failures of limb salvage in order to allow the interpretation of outcome following reconstructive surgery.

Cite this article: Bone Joint J 2014;96-B:1436–40.

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 describe 22 cases of bizarre parosteal osteochondromatous proliferation, or Nora’s lesion. These are surface-based osteocartilaginous lesions typically affecting the hands and feet. All patients were identified from the records of a regional bone tumour unit and were treated between 1985 and 2009. Nine lesions involved the metacarpals, seven the metatarsals, one originated from a sesamoid bone of the foot and five from long bones (radius, ulna, tibia, and femur in two). The mean age of the patients was 31.8 years (6 to 66), with 14 men and eight women. Diagnosis was based on the radiological and histological features. The initial surgical treatment was excision in 21 cases and amputation of a toe in one. The mean follow-up was for 32 months (12 to 162). Recurrence occurred in six patients (27.3%), with a mean time to recurrence of 49 months (10 to 120). Two of the eight patients with complete resection margins developed a recurrence (25.0%), compared with four of 14 with a marginal or incomplete resection (28.6%).

Given the potential surgical morbidity inherent in resection, our data suggest that there may be a role for a relatively tissue-conserving approach to the excision of these lesions.

Four patients who developed malignant synovial tumours are described; one with chondromatosis developed a synovial chondrosarcoma and three with pigmented villonodular synovitis developed malignant change. The relevant literature is discussed.

We investigated the use of hypoxia-inducible factor (HIF) proteins as prognostic markers in chondrosarcoma and the relationship of HIF to the biological characteristics of cartilage tumours. The expression of HIF-1α, HIF-2α, proliferating cell nuclear antigen (PCNA) and microvessel density (MVD) were measured immunohistochemically in 29 specimens of cartilage tumour.

There was no HIF-1α and HIF-2α staining in any of the nine benign cartilage tumours. In 20 specimens of chondrosarcoma, the rate of HIF-1α and HIF-2α expression was 40% and 25%, respectively. The tumour size (≥ 8 cm), histological grade (grade 2 and grade 3) surgical margin (marginal and intralesional) and HIF-1α expression (positive) correlated significantly with a shorter disease-free survival. There was a significant association between HIF-1α and the MVD and a strong trend towards a correlation between HIF-1α and the PCNA index or histological grade.

Our findings suggest that HIF-1α protein may be a useful objective marker in the assessment of the prognosis in chondrosarcoma, since it plays an important role in tumour angiogenesis and cell proliferation.

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

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

We aimed to identify the incidence, outcome and prognostic factors associated with spindle cell sarcomas of bone (SCSB). We studied 196 patients with a primary non-metastatic tumour treated with the intent to cure. The results were compared with those of osteosarcoma patients treated at our hospital during the same period. The overall incidence of SCSB was 7.8% of all patients with a primary bone sarcoma. The five- and ten-year survival rates were 67.0% and 60.0%, respectively, which were better than those of patients with osteosarcoma treated over the same period. All histological subtypes had similar outcomes. On univariate analysis, factors that were significantly associated with decreased survival were age > 40 years, size > 8 cm, the presence of a pathological fracture, amputation, involved margins and a poor response to pre-operative chemotherapy. Multivariate analyses showed that age > 65 years, amputation and involved margins were all statistically significant prognostic factors. Involved margins and poor response to pre-operative chemotherapy were associated with an increased risk of local recurrence. SCSB has a better prognosis than osteosarcoma when matched for age. Most prognostic factors for osteosarcoma also seem to apply to SCSB. Patients with SCSB should be treated in the same way as patients of the same age with osteosarcoma.

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 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.

Pathological fractures due to metastasis with destruction of the acetabulum and central dislocation of the hip present a difficult surgical challenge. We describe a series using a single technique in which a stable and long-lasting reconstruction was obtained using standard primary hip replacement implants augmented by strong, fully-threaded steel rods with cement and steel mesh, where required.

Between 1997 and 2006, 19 patients with a mean age of 66 years (48 to 83) were treated using a modified Harrington technique. Acetabular destruction was graded as Harrington class II in six cases and class III in 13.

Reconstruction was achieved using three 6.5 mm rods inserted through a separate incision in the iliac crest followed by augmentation with cement and a conventional cemented Charnley or Exeter primary hip replacement. There were no peri-operative deaths. At the final follow-up (mean 25 months (5 to 110)) one rod had fractured and one construct required revision. Of the 18 patients who did not require revision, 13 had died. The mean time to death was 16 months (5 to 55). The mean follow-up of the five survivors was 31 months (18 to 47). There were no cases of dislocation, deep infection or injury to a nerve, the blood vessels or the bladder.

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

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

We have investigated whether improvements in design have altered the outcome for patients undergoing endoprosthetic replacement of the proximal tibia following resection of a tumour. Survival of the implant and ‘servicing’ procedures have been documented using a prospective database.

A total of 194 patients underwent a proximal tibial replacement, with 95 having a fixed-hinge design and 99 a rotating-hinge with a hydroxyapatite collar; their median age was 21.5 years (10 to 74). At a mean follow-up of 14.7 years (5 to 29), 115 patients remain alive. The risk of revision for any reason in the fixed-hinge group was 32% at five years, 61% at ten years and 75% at 15 and 20 years, and in the rotating-hinge group 12% at five years, 25% at ten years and 30% at 15 years. Aseptic loosening was the most common reason for revision in the fixed-hinge knees, fracture of the implant in the early design of rotating hinges and infection in the current version. The risk of revision for aseptic loosening in the fixed-hinge knees was 46% at ten years. This was reduced to 3% in the rotating-hinge knee with a hydroxyapatite collar. The cemented, rotating hinge design currently offers the best chance of long-term survival of the prosthesis.

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

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

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