Background:The study aimed to analyse the demographic, clinical, and histological features of patients with a malignant primary bone tumour of the femur presenting with a pathological fracture. Methods: Eighty-six patients with primary malignant bone tumours of the femur presenting with pathological fracture were identified from a unique national database that contains original radiographs, casenotes and histology for all patients diagnosed with a primary bone tumour since september 1936 to the present. Demographic data, presenting features, tumour location, histological diagnosis, treatment, local recurrence, metastasis and survival data were gathered. Results: The median age was 63 years (range 4 to 87 years) and 47% were men. Forty-two percent of patients presented with a history of trauma. Forty percent of lesions were in the proximal femur, 34% in the diaphysis and 26% in the distal femur. The most common histological diagnoses were osteosarcoma (13 patients), Paget’s sarcoma (12 patients), myeloma (11 patients), chondrosarcoma and lymphoma (9 patients each). Other diagnoses were fibrosarcoma, Ewing’s sarcoma, spindle cell sarcoma, reticulum cell sarcoma, malignant fibrous hystiocytoma, and malignant giant cell tumour. The local recurrence rate was 31%. The median survival was 12 months (95% confidence interval 6 to 18 months). Overall 5 and 10-year survival were 22.4% and 17.4% respectively. Specifically for osteosarcoma, chondrosarcoma and Paget’s sarcoma the five year survival rates were 15.4%, 11.1% and 19.0% respectively. Those in whom the age at the time of presentation was over 60 years had a significantly worse prognosis (log rank 13.4, p< 0.001). Conclusion: Pathological fracture as a presenting symptom of primary malignant bone tumours is associated with a poor prognosis in nearly all tumour types studied. The prognosis is worse in those who are over 60 years at the time of presentation

Purpose: Replacing the entire femur for primary bone tumour is exceptional. Prostheses used in this series were custom-made by Link using the Endo Klinik (Hambourg) model. These prostheses have adjustable ante-version and a hinge type knee with rotation. Patients and Results: Case n° 1. A 15-year-old boy, grade 2A osteosarcoma in the diaphyseal zone of the lower femur. Rosen chemotherapy. En bloc resection. Total femur prosthesis sleeved onto the trochanteric mass left in place. Excellent response:100%. Excellent functional result: mountain climbing! Current status: recurrence-free, metastasis-free at 16 years follow-up. Case n° 2. Adult osteosarcoma. 68-year-old woman with pulmonary metastasis at diagnosis. Indication due to fracture to mid third of femur. Excellent immediate result. Nine-month survival in very satisfactory condition. Case n° 3. 72-year-old woman treated one year earlier for T1N0M0 breast cancer. Metastatic image in the trochanter. Treatment by curettage and THA. Histology reported chondrosarcoma. Scintigraphy showed uptake in gluteus medius and the lower part of the femur. En bloc resection of entire femur and gluteus medius. Total femur replacement with prosthesis sleeved onto an allograft. Complete resection. Current status: recurrence-free, metastasis-free at five years. No limitation on walking distance. Walks with cane due to moderate limp. Case n° 4. Adult osteosarcoma (32 years). Low-grade tumour (1B) occupying the entire femur. Rizzoli Institute chemotherapy protocol. Resection and total femur replacement with prosthesis sleeved onto an allograf. Complete resection. Poor response. Excellent functional result, persistence of minimal limp. Active tumour with pulmonary metastasis at one year and death at 1.5 yers. Case n° 5. Grade 2B osseous leiomyosarcoma in the diaphyseal zone of the lower femur in a 37-year-old woman. Neoadjuvant chemotherapy followed by total femur resection. Excellent responder:100%. Excellent functional outcome at short follow-up (1 year). Discussion: Total femur replacement with a prostheses sleeved on an allograft allows reinsertion of the gluteus medius and the psoas. This method is reliable and avoids major instability. Complications in this small number of patients were rare and were not serious. The functional results have been excellent and appear to depend primarily on the importance of muscle resection required to achieve tumour resection

Aims. The ulna is an extremely rare location for primary bone tumours of the elbow in paediatrics. Although several reconstruction options are available, the optimal reconstruction method is still unknown due to the rarity of proximal ulna tumours. In this study, we report the outcomes of osteoarticular ulna allograft for the reconstruction of proximal ulna tumours. Methods. Medical profiles of 13 patients, who between March 2004 and November 2021 underwent osteoarticular ulna allograft reconstruction after the resection of the proximal ulna tumour, were retrospectively reviewed. The outcomes were measured clinically by the assessment of elbow range of motion (ROM), stability, and function, and radiologically by the assessment of allograft-host junction union, recurrence, and joint degeneration. The elbow function was assessed objectively by the Musculoskeletal Tumor Society (MSTS) score and subjectively by the Toronto Extremity Salvage Score (TESS) and Mayo Elbow Performance Score (MEPS) questionnaire. Results. The mean follow-up of patients was 60.3 months (SD 28.5). The mean elbow flexion-extension ROM was 95.8° (SD 21). The mean MSTS of the patients was 84.4 (SD 8.2), the mean TESS was 83.8 (SD 6.7), and the mean MEPS was 79.2 (SD 11.5). All the patients had radiological union at the osteotomy site. Symptomatic osteoarthritic change was observed in three patients (23%), one of whom ended up with elbow joint fusion. Two patients (15.4%) had recurrence during the follow-up period. Surgical complications included two allograft fractures, two plate fractures, three medial instabilities, and two infections. Conclusion. Osteoarticular ulna allograft reconstruction provides acceptable functional outcomes. Despite a high rate of complications, it is still a valuable reconstruction method, particularly in skeletally immature patients who need their distal humerus physis for the rest of hand growth. Cite this article: Bone Jt Open 2024;5(9):749–757

Aims. The proximal tibia (PT) is the anatomical site most frequently affected by primary bone tumours after the distal femur. Reconstruction of the PT remains challenging because of the poor soft-tissue cover and the need to reconstruct the extensor mechanism. Reconstructive techniques include implantation of massive endoprosthesis (megaprosthesis), osteoarticular allografts (OAs), or allograft-prosthesis composites (APCs). Methods. This was a retrospective analysis of clinical data relating to patients who underwent proximal tibial arthroplasty in our regional bone tumour centre from 2010 to 2018. Results. A total of 76 patients fulfilled the inclusion criteria and were included in the study. Mean age at surgery was 43.2 years (12 to 86 (SD 21)). The mean follow-up period was 60.1 months (5.4 to 353). In total 21 failures were identified, giving an overall failure rate of 27.6%. Prosthesis survival at five years was 75.5%, and at ten years was 59%. At last follow-up, mean knee flexion was 89.8° (SD 36°) with a mean extensor lag of 18.1° (SD 24°). In univariate analysis, factors associated with better survival of the prosthesis were a malignant or metastatic cancer diagnosis (versus benign), with a five- and ten-year survival of 78.9% and 65.7% versus 37.5% (p = 0.045), while in-hospital length of stay longer than nine days was also associated with better prognosis with five- and ten-year survival rates at 84% and 84% versus 60% and 16% (p < 0.001). In multivariate analysis, only in-hospital length of stay was associated with longer survival (hazard ratio (HR) 0.23, 95% confidence interval (CI) 0.08 to 0.66). Conclusion. We have shown that proximal tibial arthroplasty with endoprosthesis is a safe and reliable method for reconstruction in patients treated for orthopaedic oncological conditions. Either modular or custom implants in this series performed well. Cite this article: Bone Jt Open 2022;3(9):733–740

The purpose of this study was to evaluate the functional and oncological outcome of recycled autograft reconstruction after a wide excision for primary malignant bone tumor around the hip.

From 1998 to 2015, 67 patients with a primary malignant bone tumor involving proximal femur or periacetabular zone (P2) were included. There were 36 males and 31 females with a mean age of 34 years (13 to 58). Of these, 29 patients had grade I or II chondrosarcoma, 28 high-grade osteosarcoma, 6 Ewing's sarcoma and 4 undifferentiated pleomorphic sarcoma. Enneking stage of all 67 patients was stage II. Of the resection classification, proximal femur resection was performed in 29 patients, P1+P2 in 15, P2+P3 in 14, P1+P2+P3 in 4, P2 only in 4, and P2+proximal femur in 1. Extracorporeally irradiated recycled autograft and liquid nitrogen frozen autograft were performed in 44 and 23 patients, respectively.

At a mean follow-up of 98 months (10 to 239), 48 patients (72%) were continuously disease-free, 12 (18%) died of disease and 7 (10%) were alive with disease. The tumors of these patients who had died of disease were usually located in pelvic bones (10/12). Of these 37 patients with pelvic tumors, 7 patients (22%) had local recurrence, four of them received external hemipelvectomy. Other complications included hip dislocation in 2 patients, nerve injury in 2 and deep infection in 2. However, the above complications were rarely occurred in the patients with proximal femur reconstruction. The mean Musculoskeletal Tumor Society functional score were 77% (53 to 93).

Recycled autograft reconstruction for primary malignant bone tumor around the hip is a valid method with acceptable morbidity and a favorable functional outcome.

Primary malignant bone tumours are a scarce entity with limited population-based data from developing countries. The aim of the study is to investigate the frequency and anatomical distribution of primary malignant bone tumours in a local South African population. This will be an epidemiological retrospective study. Data will be used of patients that were diagnosed with primary malignant bone tumours over a period of nine years spanning from 1 January 2014 to 31 December 2022. This data will be received from private and government laboratories. Data to be considered are type of primary malignant bone tumours diagnosed, incidence of primary malignant bone tumours over a period of nine years and the most common anatomical sites of primary malignant bone tumours. The rationale behind our study is to assess the frequency of different primary malignant bone tumours in another geographic area of South Africa and to compare these findings to local and international literature. With a projected increase in diagnosis of primary malignant bone tumours in developing countries it is important to have more available data about primary malignant bone tumours from these areas to have a better understanding of these conditions and to understand the impact of the burden they impose on healthcare systems so that management of these conditions can also be improved. Preliminary results show that 23.83% of primary malignant bone tumours occurred in the age group 0–24 years of age, 49.22% in the 25–59 age group and 26.95% in the 60+ age group. The most common tumour that occurred was chondrosarcoma (49.21%) followed by osteosarcoma (41.80%) then Ewing's sarcoma (4,69%) and lastly chordoma (4.30%). From the 256 samples that met the inclusion criteria the five most common anatomical sites were distal femur (63), proximal tibia (41), proximal humerus (38), pelvis (34) and proximal femur (20)

En bloc resection for primary bone tumours and isolated metastasis are complex surgeries associated with a high rate of adverse events (AEs). The primary objective of this study was to explore the relationship between frailty/sarcopenia and major perioperative AEs following en bloc resection for primary bone tumours or isolated metastases of the spine. Secondary objectives were to report the prevalence and distribution of frailty and sarcopenia, and determine the relationship between these factors and length of stay (LOS), unplanned reoperation, and 1-year postoperative mortality in this population. This is a retrospective study of prospectively collected data from a single quaternary care referral center consisting of patients undergoing an elective en bloc resection for a primary bone tumour or an isolated spinal metastasis between January 1st, 2009 and February 28th, 2020. Frailty was calculated with the modified frailty index (mFI) and spine tumour frailty index (STFI). Sarcopenia, determined by the total psoas area (TPA) vertebral body (VB) ratio (TPA/VB), was measured at L3 and L4. Regression analysis produced ORs, IRRs, and HRs that quantified the association between frailty/sarcopenia and major perioperative AEs, LOS, unplanned reoperation and 1-year postoperative mortality. One hundred twelve patients met the inclusion criteria. Using the mFI, five patients (5%) were frail (mFI ³ 0.21), while the STFI identified 21 patients (19%) as frail (STFI ³ 2). The mean CT ratios were 1.45 (SD 0.05) and 1.81 (SD 0.06) at L3 and L4 respectively. Unadjusted analysis demonstrated that sarcopenia and frailty were not significant predictors of major perioperative AEs, LOS or unplanned reoperation. Sarcopenia defined by the CT L3 TPA/VB and CT L4 TPA/VB ratios significantly predicted 1-year mortality (HR of 0.32 per one unit increase, 95% CI 0.11-0.93, p=0.04 vs. HR of 0.28 per one unit increase, 95% CI 0.11-0.69, p=0.01) following unadjusted analysis. Frailty defined by an STFI score ≥ 2 predicted 1-year postoperative mortality (OR of 2.10, 95% CI 1.02-4.30, p=0.04). The mFI was not predictive of any clinical outcome in patients undergoing en bloc resection for primary bone tumours or isolated metastases of the spine. Sarcopenia defined by the CT L3 TPA/VB and L4 TPA/VB and frailty assessed with the STFI predicted 1-year postoperative mortality on univariate analysis but not major perioperative AEs, LOS or reoperation. Further investigation with a larger cohort is needed to identify the optimal measure for assessing frailty and sarcopenia in this spine population

Primary bone tumours of the clavicle are rare. Currently the existing literature is limited to a single case series and case reports or cases. Information regarding the patient's demographics and tumour types is therefore limited. The aim of this study was to investigate the and also suggest a management protocol for suspected primary bone tumours of the clavicle. We retrospectively reviewed the Scottish Bone Tumour Register from January 1971 to January 2012 and included all primary bone tumours of the clavicle. We identified only sixteen primary bone tumours over forty one year's highlighting the rarity of these tumours. There were ten benign and six malignant tumours with a mean age of 32 years (Range 4 to 66). The average presentation to orthopaedics after onset of symptoms was two months with five patients presenting following a pathological fracture. Malignant tumour types identified were consistent with previous literature with two cases of Ewing's sarcoma and osteosarcoma and a single case of osteosarcoma post radiotherapy and a single case of chondrosarcoma. Benign tumours were treated effectively with intralesional procedures. Malignant tumours were treated with wide local excision and subtotal or total clavicle excision. We suggest an investigatory and treatment protocol for patients with a suspected primary bone tumour of the clavicle. This is the largest series of primary bone tumours of the clavicle in the literature

Primary bone tumours of the talus are rare. Currently the existing literature is limited to a single case series and case reports or cases described in series of foot tumours. Information regarding the patient's demographics and tumour types is therefore limited. The aim of this study was to investigate these questions and also suggest a management protocol for suspected primary bone tumours of the talus. We retrospectively reviewed the Scottish Bone Tumour Register from January 1954 to May 2010 and included all primary bone tumours of the talus. We identified only twenty three bone tumours over fifty six years highlighting the rarity of these tumours. There were twenty benign and three malignant tumours with a mean age of twenty eight years. A delay in presentation was common with a mean time from onset of symptoms to diagnosis of ten months. Tumour types identified were consistent with previous literature. We identified cases of desmoplastic fibroma and intraosseous lipodystrophy described for the first time. We suggest an investigatory and treatment protocol for patients with a suspected primary bone tumour of the talus. This is the largest series of primary bone tumours of the talus in the literature

Patients with peripheral primary bone tumours are often identified and referred at an early stage to a regional tumour service according to established guidelines. In patients with primary bone tumours of the spine, however, the definitive management or outcome of such patients is being prejudiced by preliminary intervention from non-specialist services prior to their referral. Objective: To audit the standards of management of patients with primary bone tumours of the spine referred to a regional tumour service. Retrospective review of case notes and radiology. Subjects: Patients with primary bone tumour of the spine managed at the Orthopaedic Spine Unit with the Regional Bone Tumour Service in Newcastle Upon Tyne Hospitals NHS Trust. Referral to tumour service, prior intervention, operative treatment, survival, factors affecting definitive management. 31 of 39 (16 benign, 23 malignant) patients were initially referred from primary care to services other than the regional tumour service, most commonly neurosurgery (11/39) and paediatric oncology (4/39). Seven of 39 of these patients had undergone interventions prior to their referral to the tumour service, which may have negatively impacted their definitive management or curative surgery. These tumours present complex issues regarding their definitive management to optimise outcome. Closer links between departments are required to enable the multidisciplinary management of primary bone tumours of the spine. Prior surgical intervention may compromise cure. Those involved in their management should be encouraged to liaise with their regional bone tumour service to improve outcome

Purpose of the study. To review the primary bone tumours of the spine treated at our unit. Description of methods. Retrospective review of folders and x-rays of all the patients with primary bone tumours of the spine treated at our unit between 2005 and 2012. All haematological tumours were excluded. Summary of results. We treated 15 cases during this period. The median age at presentation was 36 years (8–65). There was a significant delay from onset of symptoms to diagnosis in most cases (median 7 months). Histological diagnoses included:. -Benign tumours.  Active. Hemangioma. 3. Osteoid osteoma. 1. Eosinophilic granuloma. 1.  Aggressive. Osteoblastoma. 1. Giant cell tumours. 2. Aneurysmal bone cysts. 4. -Malignant tumours.  Osteosarcomas. 2.  Leiomyosarcoma of bone. 1. A variety of definitive surgical methods were utilised. Seven patients had a debulking or intralesional resection of the tumour. Eight patients had an attempted marginal excision. This was achieved through anterior surgery only in 1 case, posterior only surgery in 6 cases and combination anterior and posterior surgery in 8 cases. The anterior and posterior surgery was performed in a single sitting in 5 cases and in a staged fashion in 3 cases. Adjuvant radiotherapy and chemotherapy were used where indicated. Three cases presented with significant neurological impairment. Of these 2 made a significant recovery. There were no cases of neurological deterioration following surgery. All 3 patients with malignant tumours died in the follow up period. We had 1 case of hardware failure due to chronic sepsis. Conclusion. Primary bone tumours of the spine are associated with a significant delay in diagnosis. Surgical treatment options and adjuvant therapy should be tailor made for each case depending on the diagnosis. Acceptable results with minimal complications can be achieved with this approach

The use of routine sampling for histological analysis during revision hip replacement has been standard practice in our unit for many years. It is used to identify the presence of inflammatory processes that may represent peri-prosthetic infection. This study follows up on a smaller study in the same unit in 2019 where an initial 152 cases were scrutinised. In this follow up study we examined 1,361 consecutive patients over a 16-year period whom had undergone revision hip replacement in a tertiary orthopaedic centre for any reason excluding primary bone tumour or malignant metastasis. All patients had tissue sampling for histopathological analysis performed by consultant histopathologists with a specialist interest in musculoskeletal pathology. The presence of bacteria in greater than 50% of samples sent for microbiological analysis in each patient was used as the gold standard diagnostic comparator for infection. This was then compared with the histology report for each patient. After excluding 219 patients with incomplete data and 1 sample rejection, 1,141 cases were examined. Microbiology confirmed infection in 132 cases (prevalence of infection 11.04%) and histopathology analysis suggested infection in 171 cases. Only 64 cases with confirmed infection in more than 50% of microbiology samples had concurrent diagnosis of infection on histological analysis (5.60% of total; PPV 51.20%). Furthermore, microbiology analysis confirmed infection in 62 cases where histological analysis failed to identify infection (5.43% of total; False negative rate 49.21%). Overall, histopathology analysis was seen to have a good specificity of 93.99% but poor sensitivity of 50.79%. We believe that this is the largest series in the literature and is somewhat unique in that all histology analysis was performed by consultant histopathologists with specialist interest in musculoskeletal pathology. Based on the costs incurred by this additional investigation our experience does not support routine sampling for histological analysis in revision hip arthroplasty. This is a substantial paradigm shift from current practice among revision arthroplasty surgeons in the United Kingdom but would equate to a substantial cost saving

Abstract. Background. Schwannomas are slow-growing, benign tumours normally originating from the Schwann cells of the nerve sheath. Intraosseous schwannoma accounts for 0.175% of primary bone tumours and extremely rare especially outside the axial skeleton. Monoclonal gammopathy has been associated with soft tissue schwannomas but never with the intraosseous variety. Presenting problem. A 55-year-old woman with a background of monoclonal gammopathy of undetermined significance (MGUS) presented with a 2-year history of right thigh pain. CT scan showed a well defined, lytic lesion with a thin peripheral rim of sclerosis in the midshaft of the femur. MRI displayed a hyperintense, well marginated and homogenous lesion. Definitive diagnosis was made based on the classical histopathological appearance of schwannoma. Clinical management. We managed our patient with local curettage and prophylactic cephalomedullary nailing on the basis of a high mirel score. Discussion. Intraosseous schwannomas are poorly understood but most commonly reported in middle-aged women. Radiologically, their differential diagnosis includes malignant bone tumours, solitary bone cysts, aneurysmal bone cysts and giant cell tumours. As a result, they are usually diagnosed incidentally on histology. Although malignant transformation is possible in soft tissue schwannomas, all intraosseous schwannomas reported to date have been benign. This case demonstrates the importance of suspecting intraosseous schwannoma as a differential diagnosis for lytic bone lesions to avoid the overtreatment of patients. We also highlight monoclonal gammopathy of undetermined significance as a potential risk factor for a poorly understood disease and make recommendations about the appropriate management of these lesions. Declaration of Interest. (b) declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported:I declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project

INTRODUCTION. We conducted a review of the Leeds Regional Bone Tumour Registry for primary bone tumours of the spine since establishment in 1958 until year 2000. AIM. To analyze the incidence of primary tumours of the spine and to record the site of occurrence, sex distribution, survival and pathology of these tumours. Summary of the Background Data. Primary tumours of the spine are particularly rare, accounting for between 4% and 13% of published series of primary bone tumours. METHOD. The Leeds Bone Tumour Registry was reviewed and a total of 2750 cases of bone tumours and tumour-like cases were analyzed. Consultants in orthopaedic surgery, neurosurgery, oncology and pathology in North and West Yorkshire and Humberside contribute to the Registry. RESULTS. Primary bone tumours of the osseous spine constitute only 126 of the 2,750 cases (4.6%). Chordoma was the most frequent tumour in the cervical and sacral regions, while the most common diagnosis overall was multiple myeloma and plasmacytoma. Osteosarcoma ranked third. The mean age of presentation was 42 years and pain was the most common presenting symptom, occurring in 95% of malignant and 76% of benign tumours. Neurological involvement occurred in 52% of malignant tumours and usually meant a poor prognosis,. CONCLUSIONS. The establishment of Bone Tumour Registries is the only way that sufficient data on large numbers of these rare tumours can be accumulated to provide a valuable and otherwise unavailable source of information for research, education and clinical follow-up

Purpose: We conducted a review of the Leeds Regional Bone Tumour Registry for primary bone tumours of the axial skeleton since establishment in 1958 until year 2000 to analyze the incidence of primary tumours of the axial skeleton and to record their site of occurrence, sex distribution, survival and pathology. Method: Primary tumours of the axial skeleton are particularly rare, accounting for between 4% and 13% of published series of primary bone tumours. The Leeds Bone Tumour Registry was reviewed and a total of 2750 cases of bone tumours and tumour-like cases were analyzed. Consultants in orthopaedic surgery, neurosurgery, oncology and pathology in North and West Yorkshire and Humberside contribute to the Registry. Results: Primary bone tumours of the axial skeleton constitute only 126 of the 2,750 cases (4.6%). Chordoma was the most frequent tumour in the cervical and sacral regions, while the most common diagnosis overall was myeloma. Osteosarcoma ranked third. Mean age of presentation was 42 years. Pain was the most common presenting symptom, occurring in 95% of malignant and 76% of benign tumours. Neurological involvement occurred in 52% of malignant tumours and usually meant a poor prognosis,. Conclusions: The establishment of Bone Tumour Registries is the only way that sufficient data on large numbers of these rare tumours can be accumulated to provide a valuable and otherwise unavailable source of information for research, education and clinical follow-up

The October 2012 Oncology Roundup. 360. looks at: the causes of primary bone tumours; adjuvant chemotherapy in the longer term; vascularised fibular grafts to salvage massive femoral allografts; a new look at old risks; reconstruction with excised irradiated bone; predicting chemosensitivity in osteosarcoma ; and chemotherapy, osteoporosis and the risk of fracture

Osteosarcoma is the most common primary bone tumour worldwide. This disease presents a formidable challenge to the orthopaedic surgeon, with a mortality rate of 30 per cent, even after surgical clearance. Aberrant Wnt signalling has been implicated in the pathogenesis osteoblastic tumours. The objective of this study is 2 fold- to investigate if osteosarcoma does indeed demonstrate aberrant Wnt signaling, and if so, does osteosarcoma respond to a novel Wnt inhibitor(ETC159). This can potentially lead to the development of a new adjuvant treatment modality for osteosarcoma. A novel Wnt signaling pathway protein antibody (YJ5) was used in immunihistochemistry staining of clinical osteosarcoma samples. A Wnt high osteosarcoma cell line(SJSA-1) was then implanted subcutaneously in a mouse model. These mice were treated with a novel PORCN inhibitor, ETC 159 for a period of 4 weeks in a two-arm randomised control study. The results of treatment were evaulated by clinical outcome parameters as well as immunohstochemistry. 100 per cent of clinical osteosarcoma samples demonstrated increased WLS expression and Wnt protein expression. SJSA-1 showed no significant decrease in tumour volume after 30 days of drug treatment (3070 SD 625 mm3 vs 3480 SD 433 mm3 p= 0.605 and 2060 SD 209 vs 1677 SD 213 mm3 p=0.219 respectively). Significantly, SJSA-1 demonstrated increased tumour necrosis in the treatment arm(30–60 percent increase across all samples p < 0 .005) Treated tumours also demonstrated markedly less angiogenesis compared to the non treatment arm. Osteosarcoma demonstrates aberrant Wnt signaling in a large percentage of cases. The use of a novel PORCN inhibitor ETC 159 for the treatment of Osteosarcoma has a marked effect on tumour necrosis. Our results suggest that ETC159 may cause tumour necrosis by inhibiting angiogenesis within the tumour. Further evaluation and understanding of the mechanism of Wnt singaling in regulating tumour pathogenesis may hold the potential for developing a curative therapeutic drug for this deadly disease

Introduction: Only 4–13 % of all spine tumours are primary bone tumours. We report on 180 cases of primary malignant bone tumours of the spine from the Scottish Bone Tumour Registry. The aim of the study was to analyse the incidence, demography, pathology and survival patterns of primary malignant bone tumours of the vertebral column. Materials and Methods: All of the data in the Scottish Bone Tumour Registry is compiled prospectively. This report is based on a retrospective review of all the data from 180 cases of primary malignant bone tumours (excluding metastatic lesions). Results: Of 4,301 registry cases 4.1% were primary malignant lesions (n=180). Seventy two percent of all spinal tumours were primary malignant neoplasms. There were 22 cervical, 72 thoracic, 45 lumbar and 41 sacrococcygeal lesions. There was a male preponderance (103 males & 77 females). The mean age at presentation was 54 years (range, 4–86 years). The top two ranked tumours were myeloma (42) and chordoma (41). Ewing’ sarcoma (15), Leukaemik-Lymphomatous lesions (13), conventional osteosarcoma (10) and Paget’s sarcoma (9) followed thereafter. The predominant presenting symptom was pain. Pathological fracture occurred in 7 patients. The operative treatment consisted of curettage (21), excision (51) and resection (9) with supplemented bone grafting (13). Adjuvant chemo (=61) and radiotherapy (=131) was also used in selective cases. Thirty patients were alive with no evidence of disease at a mean 5 year follow-up. Six were alive with persistent primary disease and/or local recurrence and/or metastases at the time of review. Eighty four patients died with persistent primary disease, 30 patients died of metastatic disease, 9 due to local recurrence and 17 of unrelated causes. Conclusions: Only 4.1% of the musculoskeletal tumours were spinal: 40% involved the thoracic spine. Mean age at presentation was 54 years. Myeloma, chordoma, chondrosarcoma and Ewing’ sarcoma were the most common pathologies. Myeloma predominated: osteosarcoma was much less common in our series compared to previous reports. Early diagnoses resulted into improved outcome

Introduction. The pelvis has always been a difficult area for surgeons, with high complication rates from surgery and the perception of poor oncological outcomes. The aim of the study was to look at the surgical and oncological outcomes of pelvic tumours treated at our centre. Methods. From the 3100 primary bone tumours seen at the ROH. Information was retrieved on 539 patients seen with a primary bone tumour of the pelvis. The demographic details, oncological and surgical outcomes were reviewed. Results. The mean age at presentation (39 yrs old) and duration of symptoms prior to diagnosis (30 weeks) were both significantly greater than the appendicular skeleton. The volume at resection was 3.8 times greater than the appendicular skeleton. The rates of wide surgical margins and response to chemotherapy were significantly poorer (p<0.05) than the appendicular skeleton resulting in rates of local recurrence (27% vs 12%, p<0.05) and overall survival that were poorer that the appendicular skeleton (51% vs 31% 10 yr survival, p<0.05). The dilemma of treating pelvic ewings will be discussed together with the subanalysis of the EICESS 92/EUROEWING 99 trials in relation to pelvic ewings sarcoma. Conclusions. Despite poorer oncological outcomes surgical resection of pelvic tumours can deliver good oncological and surgical results but has high complication rates compared to tumours of the appendicular skeleton

Aim. To identify patterns in referral and the management pathway of patients with primary bone tumours of the spine referred to the Orthopaedic Spine Unit in order to recommend ways to improve the service. Methods and Results. A retrospective notes and imaging review to evaluate the referral pathway undertaken by patients ending up in the orthopaedic spine unit over a 5 year period according to the recommendations for primary bone tumours. Significant events leading to potential improvement in outcomes were assessed. Recommendations for improvements are suggested. None of the 38 patients evaluated were referred within two weeks of presentation, and only 6 were referred directly to the bone tumour service. Almost half (15/32) of the patients who had an indirect referral pathway had a prior intervention. Five of these had non-surgical, while 10 had surgical interventions outside the tumour centre before their referral. Of these, seven had malignant tumours. Conclusion. In order to optimise outcome, patients with potentially malignant primary tumours of the spine should be referred directly to tumour services. Prior procedures should be limited to biopsy procedures and discussed with the tumour service before this is undertaken having appropriate investigation and imaging available. Guidelines for this should be directed at streamlining the referral pathway and encouraging communication between stakeholders. Further research should assess the impact of patient-related delay in presentation contributes to overall delay in referral to tumour service; how early radiological assessment may augment timely referral; and how indirect referral and prior intervention affect patient outcomes