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The Bone & Joint Journal
Vol. 102-B, Issue 6 | Pages 779 - 787
1 Jun 2020
Gupta S Griffin AM Gundle K Kafchinski L Zarnett O Ferguson PC Wunder J

Aims. Iliac wing (Type I) and iliosacral (Type I/IV) pelvic resections for a primary bone tumour create a large segmental defect in the pelvic ring. The management of this defect is controversial as the surgeon may choose to reconstruct it or not. When no reconstruction is undertaken, the residual ilium collapses back onto the remaining sacrum forming an iliosacral pseudarthrosis. The aim of this study was to evaluate the long-term oncological outcome, complications, and functional outcome after pelvic resection without reconstruction. Methods. Between 1989 and 2015, 32 patients underwent a Type I or Type I/IV pelvic resection without reconstruction for a primary bone tumour. There were 21 men and 11 women with a mean age of 35 years (15 to 85). The most common diagnosis was chondrosarcoma (50%, n = 16). Local recurrence-free, metastasis-free, and overall survival were assessed using the Kaplan-Meier method. Patient function was evaluated using the Musculoskeletal Tumour Society (MSTS) and Toronto Extremity Salvage Score (TESS). Results. At a mean follow-up of 159 months (1 to 207), 23 patients were alive without disease, one was alive with lung metastases, one was alive following local recurrence, four were dead of disease, and three had died from other causes. The overall ten-year survival was 77%. There was only one (3%) local recurrence, which occurred at 26 months. There were 18 complications in 17 patients; 13 wound healing complications/infections, three fractures, one pulmonary embolism, and one dislocation of the hip. Most complications occurred early. The mean functional scores were 21.1 (SD 8.1) for MSTS-87, 67.3 (SD 23.9) for MSTS-93 and 76.2 (SD 20.6) for TESS. Conclusion. Patients requiring Type I or Type I/IV pelvic resections can expect a good oncological outcome and a high rate of local control. Complications are generally acute in nature and are easily manageable. These patients achieved a good functional outcome without the need for bony reconstruction. Cite this article: Bone Joint J 2020;102-B(6):779–787


Orthopaedic Proceedings
Vol. 90-B, Issue SUPP_II | Pages 400 - 400
1 Jul 2008
Godley K Watts A Robb J
Full Access

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


The Journal of Bone & Joint Surgery British Volume
Vol. 91-B, Issue 10 | Pages 1378 - 1382
1 Oct 2009
Shekkeris AS Hanna SA Sewell MD Spiegelberg BGI Aston WJS Blunn GW Cannon SR Briggs TWR

Endoprosthetic replacement of the distal tibia and ankle joint for a primary bone tumour is a rarely attempted and technically challenging procedure. We report the outcome of six patients treated between 1981 and 2007. There were four males and two females, with a mean age of 43.5 years (15 to 75), and a mean follow-up of 9.6 years (1 to 27). No patient developed a local recurrence or metastasis. Two of the six went on to have a below-knee amputation for persistent infection after a mean 16 months (1 to 31). The four patients who retained their endoprosthesis had a mean musculoskeletal tumour society score of 70% and a mean Toronto extremity salvage score of 71%. All were pain free and able to perform most activities of daily living in comfort. A custom-made endoprosthetic replacement of the distal tibia and ankle joint is a viable treatment option for carefully selected patients with a primary bone tumour. Patients should, however, be informed of the risk of infection and the potential need for amputation if this cannot be controlled


The Journal of Bone & Joint Surgery British Volume
Vol. 82-B, Issue 1 | Pages 50 - 54
1 Jan 2000
Saifuddin A Mitchell R Burnett SJD Sandison A Pringle JAS

Needle biopsy is an established technique for the histological diagnosis of bone tumours, usually guided by fluoroscopy or CT. Surface lesions and aggressive tumours which have extended through the cortex are also amenable to imaging with ultrasound (US). We have assessed the diagnostic accuracy of US-guided Trucut needle biopsy in a consecutive series of patients referred to a Bone Tumour Unit with suspected primary bone tumours. Of 144 patients (83 men, 61 women; mean age 34.7 years) referred over a period of two years, 63 were considered suitable for US-guided biopsy. This was based on the presence of a relatively large extraosseous component, seen typically in osteosarcoma and malignant round-cell tumours. The results of needle biopsy were compared with those of surgical biopsy. The diagnostic accuracy was 98.4%, with only a single failed biopsy. Thus, in a selected group of patients, US is a very reliable technique of guidance for percutaneous needle biopsy of bone tumours


The Journal of Bone & Joint Surgery British Volume
Vol. 92-B, Issue 6 | Pages 867 - 874
1 Jun 2010
Hanna SA Sewell MD Aston WJS Pollock RC Skinner JA Cannon SR Briggs TWR

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


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_I | Pages 66 - 66
1 Jan 2004
Perrin M Fraisse J Cuisenier J
Full Access

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


The Journal of Bone & Joint Surgery British Volume
Vol. 91-B, Issue 10 | Pages 1373 - 1377
1 Oct 2009
Spiegelberg BGI Sewell MD Aston WJS Blunn GW Pollock R Cannon SR Briggs TWR

This paper describes the preliminary results of a proximal tibial endoprosthesis which spares the knee joint and enables retention of the natural articulation by replacing part of the tibial metaphysis and diaphysis. In eight patients who had a primary malignant bone tumour of the proximal tibia, the distal stem, which had a hydroxyapatite-coated collar to improve fixation, was cemented into the medullary canal. The proximal end had hydroxyapatite-coated extracortical plates which were secured to the remaining proximal tibial metaphysis using cortical screws. The mean age of the patients at operation was 28.9 years (8 to 43) and the mean follow-up was for 35 months (4 to 48). The mean Musculoskeletal Tumour Society score was 79% (57% to 90%), the mean Oxford Knee score was 40 points of 48 (36 to 46) and the mean knee flexion was 112° (100° to 120°). In one patient, revision to a below-knee amputation through the prosthesis was required because of recurrence of the tumour. Another patient sustained a periprosthetic fracture which healed with a painful malunion. This was revised to a further endoprosthesis which replaced the knee. In the remaining six patients the prosthesis allowed preservation of the knee joint with good function and no early evidence of loosening. Further follow-up is required to assess the longevity of these prostheses


The Bone & Joint Journal
Vol. 103-B, Issue 6 | Pages 1155 - 1159
1 Jun 2021
Jamshidi K Zandrahimi F Bagherifard A Mohammadi F Mirzaei A

Aim

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

Methods

In a retrospective cohort study, 32 patients who had undergone a type III hemipelvectomy with or without allograft reconstruction (n = 15 and n = 17, respectively) were reviewed. The mean follow-up was 6.7 years (SD 3.8) for patients in the reconstruction group and 6.1 years (SD 4.0) for patients in the non-reconstruction group. Functional outcome was evaluated using the Musculoskeletal Tumor Society (MSTS) scoring system and the level of postoperative pain with a visual analogue scale (VAS).


The Bone & Joint Journal
Vol. 102-B, Issue 4 | Pages 524 - 529
1 Apr 2020
Jamshidi K Mirkazemi M Gharedaghi M Izanloo A Mohammadpour M Pisoudeh K Bagherifard A Mirzaei A

Aims

The consensus is that bipolar hemiarthroplasty (BHA) in allograft-prosthesis composite (APC) reconstruction of the proximal femur following primary tumour resection provides more stability than total hip arthroplasty (THA). However, no comparative study has been performed. In this study, we have compared the outcome and complication rates of these two methods.

Methods

In a retrospective study, 57 patients who underwent APC reconstruction of proximal femur following the primary tumour resection, either using BHA (29) or THA (28), were included. Functional outcome was assessed using the Musculoskeletal Tumour Society (MSTS) scoring system and Harris Hip Score (HHS). Postoperative complications of the two techniques were also compared.


The Journal of Bone & Joint Surgery British Volume
Vol. 94-B, Issue 9 | Pages 1277 - 1281
1 Sep 2012
Puri A Gulia A

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

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


Bone & Joint 360
Vol. 13, Issue 1 | Pages 26 - 29
1 Feb 2024

The February 2024 Shoulder & Elbow Roundup. 360. looks at: Does indomethacin prevent heterotopic ossification following elbow fracture fixation?; Arthroscopic capsular shift in atraumatic shoulder joint instability; Ultrasound-guided lavage with corticosteroid injection versus sham; Combined surgical and exercise-based interventions following primary traumatic anterior shoulder dislocation: a systematic review and meta-analysis; Are vascularized fibula autografts a long-lasting reconstruction after intercalary resection of the humerus for primary bone tumours?; Anatomical versus reverse total shoulder arthroplasty with limited forward elevation; Tension band or plate fixation for simple displaced olecranon fractures?; Is long-term follow-up and monitoring in shoulder and elbow arthroplasty needed?


Bone & Joint 360
Vol. 13, Issue 4 | Pages 35 - 37
2 Aug 2024

The August 2024 Oncology Roundup. 360. looks at: What factors are associated with osteoarthritis after cementation for benign aggressive bone tumour of the knee joint: a systematic review and meta-analysis; Recycled bone grafts treated with extracorporeal irradiation or liquid nitrogen freezing after malignant tumour resection; Intercalary resection of the tibia for primary bone tumours: are vascularized fibula autografts with or without allografts a durable reconstruction?; 3D-printed modular prostheses for the reconstruction of intercalary bone defects after joint-sparing limb salvage surgery for femoral diaphyseal tumours; Factors influencing the outcome of patients with primary Ewing’s sarcoma of the sacrum; The significance of surveillance imaging in children with Ewing’s sarcoma and osteosarcoma; Resection margin and soft-tissue sarcomas of the extremities treated with limb-sparing surgery and postoperative radiotherapy


Bone & Joint Open
Vol. 5, Issue 9 | Pages 749 - 757
12 Sep 2024
Hajialiloo Sami S Kargar Shooroki K Ammar W Nahvizadeh S Mohammadi M Dehghani R Toloue B

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


Bone & Joint Open
Vol. 3, Issue 9 | Pages 733 - 740
21 Sep 2022
Sacchetti F Aston W Pollock R Gikas P Cuomo P Gerrand C

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 Journal of Bone & Joint Surgery British Volume
Vol. 73-B, Issue 5 | Pages 762 - 769
1 Sep 1991
Roberts P Chan D Grimer R Sneath R Scales J

Over a 16-year period, 135 custom-made distal femoral prostheses, based on a fully constrained Stanmore-type knee replacement, were used in the treatment of primary malignant or aggressive benign tumours. Survivorship analysis showed a cumulative success rate of 72% at five years and 64% at seven years. Intact prostheses in 91% of the surviving patients gave good or excellent functional results. Deep infection was the major complication, occurring in 6.8% of cases; clinical aseptic loosening occurred in 6.0%. Revision surgery was carried out for loosening and infection, and the early results are encouraging. We conclude that prosthetic replacement of the distal femur can meet the objectives of limb salvage surgery.


The Bone & Joint Journal
Vol. 103-B, Issue 10 | Pages 1633 - 1640
1 Oct 2021
Lex JR Evans S Parry MC Jeys L Stevenson JD

Aims. Proximal femoral endoprosthetic replacements (PFEPRs) are the most common reconstruction option for osseous defects following primary and metastatic tumour resection. This study aimed to compare the rate of implant failure between PFEPRs with monopolar and bipolar hemiarthroplasties and acetabular arthroplasties, and determine the optimum articulation for revision PFEPRs. Methods. This is a retrospective review of 233 patients who underwent PFEPR. The mean age was 54.7 years (SD 18.2), and 99 (42.5%) were male. There were 90 patients with primary bone tumours (38.6%), 122 with metastatic bone disease (52.4%), and 21 with haematological malignancy (9.0%). A total of 128 patients had monopolar (54.9%), 74 had bipolar hemiarthroplasty heads (31.8%), and 31 underwent acetabular arthroplasty (13.3%). Results. At a mean 74.4 months follow-up, the overall revision rate was 15.0%. Primary malignancy (p < 0.001) and age < 50 years (p < 0.001) were risk factors for revision. The risks of death and implant failure were similar in patients with primary disease (p = 0.872), but the risk of death was significantly greater for patients who had metastatic bone disease (p < 0.001). Acetabular-related implant failures comprised 74.3% of revisions; however, no difference between hemiarthroplasty or arthroplasty groups (p = 0.209), or between monopolar or bipolar hemiarthroplasties (p = 0.307), was observed. There was greater radiological wear in patients with longer follow-up and primary bone malignancy. Re-revision rates following a revision PFEPR was 34.3%, with dual-mobility bearings having the lowest rate of instability and re-revision (15.4%). Conclusion. Hemiarthroplasty and arthroplasty PFEPRs carry the same risk of revision in the medium term, and is primarily due to acetabular complications. There is no difference in revision rates or erosion between monopolar and bipolar hemiarthroplasties. The main causes of failure were acetabular wear in the hemiarthroplasty group and instability in the arthroplasty group. These risks should be balanced and patient prognosis considered when contemplating the bearing choice. Dual-mobility, constrained bearings, or large diameter heads (> 32 mm) are recommended in all revision PFEPRs. Cite this article: Bone Joint J 2021;103-B(10):1633–1640


The Journal of Bone & Joint Surgery British Volume
Vol. 70-B, Issue 3 | Pages 348 - 353
1 May 1988
Uyttendaele D De Schryver A Claessens H Roels H Berkvens P Mondelaers W

En-bloc resection, extracorporeal irradiation and re-implantation of the irradiated bone have been used to treat 15 patients suffering from primary malignant tumours of bone or cartilage and two with benign lesions. This treatment is an alternative to replacement by prosthesis or allograft bridging techniques. After a mean follow-up of over five years results are encouraging, despite some complications and the relatively long period before weight-bearing is allowed.


The Bone & Joint Journal
Vol. 99-B, Issue 9 | Pages 1244 - 1249
1 Sep 2017
Cladière-Nassif V Bourdet C Audard V Babinet A Anract P Biau D

Aims. Resection of the proximal humerus for the primary malignant bone tumour sometimes requires en bloc resection of the deltoid. However, there is no information in the literature which helps a surgeon decide whether to preserve the deltoid or not. The aim of this study was to determine whether retaining the deltoid at the time of resection would increase the rate of local recurrence. We also sought to identify the variables that persuade expert surgeons to choose a deltoid sparing rather than deltoid resecting procedure. Patients and Methods. We reviewed 45 patients who had undergone resection of a primary malignant tumour of the proximal humerus. There were 29 in the deltoid sparing group and 16 in the deltoid resecting group. Imaging studies were reviewed to assess tumour extension and soft-tissue involvement. The presence of a fat rim separating the tumour from the deltoid on MRI was particularly noted. The cumulative probability of local recurrence was calculated in a competing risk scenario. Results. There was no significant difference (adjusted p = 0.89) in the cumulative probability of local recurrence between the deltoid sparing (7%, 95% confidence interval (CI) 1 to 20) and the deltoid resecting group (26%, 95% CI 8 to 50). Patients were more likely to be selected for a deltoid sparing procedure if they presented with a small tumour (p = 0.0064) with less bone involvement (p = 0.032) and a continuous fat rim on MRI (p = 0.002) and if the axillary nerve could be identified (p = 0.037). Conclusion. A deltoid sparing procedure can provide good local control after resection of the proximal humerus for a primary malignant bone tumour. A smaller tumour, the presence of a continuous fat rim and the identification of the axillary nerve on pre-operative MRI will persuade surgeons to opt for a deltoid resecting procedure. Cite this article: Bone Joint J 2017;99-B:1244–9


The Bone & Joint Journal
Vol. 106-B, Issue 1 | Pages 6 - 8
1 Jan 2024
Stevenson J Cool P Ashford R

Cite this article: Bone Joint J 2024;106-B(1):6–8.


The Bone & Joint Journal
Vol. 106-B, Issue 1 | Pages 86 - 92
1 Jan 2024
Scholte CHJ Dorleijn DMJ Krijvenaar DT van de Sande MAJ van Langevelde K

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.

Methods

The aim of this study was to see whether active surveillance is safe regarding malignant degeneration and local progression. In total, 117 patients were evaluated with MRI assessing growth, cortical destruction, endosteal scalloping, periosteal reaction, relation to the cortex, and perilesional bone marrow oedema. Patients received up to six follow-up scans.


Bone & Joint 360
Vol. 11, Issue 1 | Pages 41 - 43
1 Feb 2022


Orthopaedic Proceedings
Vol. 100-B, Issue SUPP_11 | Pages 40 - 40
1 Aug 2018
Chen W
Full Access

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.


Orthopaedic Proceedings
Vol. 105-B, Issue SUPP_15 | Pages 37 - 37
7 Nov 2023
du Preez J le Roux T Meijer J
Full Access

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)


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_13 | Pages 52 - 52
1 Dec 2022
Moskven E Lasry O Singh S Flexman A Fisher C Street J Boyd M Ailon T Dvorak M Kwon B Paquette S Dea N Charest-Morin R
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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


Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_30 | Pages 34 - 34
1 Aug 2013
Bell S Mohammed F Mullen M Mahendra A
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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


The Bone & Joint Journal
Vol. 105-B, Issue 12 | Pages 1314 - 1320
1 Dec 2023
Broida SE Sullivan MH Barlow JD Morrey M Scorianz M Wagner ER Sanchez-Sotelo J Rose PS Houdek MT

Aims. The scapula is a rare site for a primary bone tumour. Only a small number of series have studied patient outcomes after treatment. Previous studies have shown a high rate of recurrence, with functional outcomes determined by the preservation of the glenohumeral joint and deltoid. The purpose of the current study was to report the outcome of patients who had undergone tumour resection that included the scapula. Methods. We reviewed 61 patients (37 male, 24 female; mean age 42 years (SD 19)) who had undergone resection of the scapula. The most common resection was type 2 (n = 34) according to the Tikhoff-Linberg classification, or type S1A (n = 35) on the Enneking classification. Results. The ten-year disease-specific survival was 76%. High tumour grade (hazard ratio (HR) 4.27; p = 0.016) and a total resection of the scapula (HR 3.84; p = 0.015) were associated with worse survival. The ten-year metastasis-free and local recurrence-free survivals were 82% and 86%, respectively. Total scapular resection (HR 6.29; p = 0.004) was associated with metastatic disease and positive margins were associated with local recurrence (HR 12.86; p = 0.001). At final follow-up, the mean shoulder forward elevation and external rotation were 79° (SD 62°) and 27° (SD 25°), respectively. The most recent functional outcomes evaluated included the mean Musculoskeletal Tumor Society Score (76% (SD 17%)), the American Shoulder and Elbow Score (73% (SD 20%)), and the Simple Shoulder Test (7 (SD 3)). Preservation of the glenoid (p = 0.001) and scapular spine (p < 0.001) improved clinical outcomes; interestingly, preservation of the scapular spine without the glenoid improved outcomes (p < 0.001) compared to preservation of the glenoid alone (p = 0.05). Conclusion. Resection of the scapula is a major undertaking with an oncological outcome related to tumour grade, and a functional outcome associated with the status of the scapular spine and glenoid. Positive resection margins are associated with local recurrence. Cite this article: Bone Joint J 2023;105-B(12):1314–1320


The Journal of Bone & Joint Surgery British Volume
Vol. 91-B, Issue 1 | Pages 108 - 112
1 Jan 2009
Chandrasekar CR Grimer RJ Carter SR Tillman RM Abudu A Buckley L

Endoprosthetic replacement of the proximal femur may be required to treat primary bone tumours or destructive metastases either with impending or established pathological fracture. Modular prostheses are available off the shelf and can be adapted to most reconstructive situations for this purpose. We have assessed the clinical and functional outcome of using the METS (Stanmore Implants Worldwide) modular tumour prosthesis to reconstruct the proximal femur in 100 consecutive patients between 2001 and 2006. We compared the results with the published series for patients managed with modular and custom-made endoprosthetic replacements for the same conditions. There were 52 males and 48 females with a mean age of 56.3 years (16 to 84) and a mean follow-up of 24.6 months (0 to 60). In 65 patients the procedure was undertaken for metastases, in 25 for a primary bone tumour, and in ten for other malignant conditions. A total of 46 patients presented with a pathological fracture, and 19 presented with failed fixation of a previous pathological fracture. The overall patient survival was 63.6% at one year and 23.1% at five years, and was significantly better for patients with a primary bone tumour than for those with metastatic tumour (82.3% vs 53.3%, respectively at one year (p = 0.003)). There were six early dislocations of which five could be treated by closed reduction. No patient needed revision surgery for dislocation. Revision surgery was required by six (6%) patients, five for pain caused by acetabular wear and one for tumour progression. Amputation was needed in four patients for local recurrence or infection. The estimated five-year implant survival with revision as the endpoint was 90.7%. The mean Toronto Extremity Salvage score was 61% (51% to 95%). The implant survival and complications resulting from the use of the modular system were comparable to the published series of both custom-made and other modular proximal femoral implants. We conclude that at intermediate follow-up the modular tumour prosthesis for proximal femur replacement provides versatility, a low incidence of implant-related complications and acceptable function for patients with metastatic tumours, pathological fractures and failed fixation of the proximal femur. It also functions as well as a custom-made endoprosthetic replacement


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXVIII | Pages 21 - 21
1 Jun 2012
Bell S Young P Mahendra A
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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


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_X | Pages 125 - 125
1 Apr 2012
Oliver W Khan A Fender D Gibson M
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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


Orthopaedic Proceedings
Vol. 103-B, Issue SUPP_10 | Pages 36 - 36
1 Aug 2021
Holland T Capella S Lee M Sumathi V Davis E
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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


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_13 | Pages 30 - 30
1 Sep 2014
Laubscher M Held M Dunn RN
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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


Orthopaedic Proceedings
Vol. 103-B, Issue SUPP_2 | Pages 2 - 2
1 Mar 2021
McAleese T Clesham K Moloney D Hughes A Faheem N Merghani K
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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


Orthopaedic Proceedings
Vol. 91-B, Issue SUPP_I | Pages 136 - 136
1 Mar 2009
Kelley S Ashford R Rao A Dickson R
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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


Orthopaedic Proceedings
Vol. 90-B, Issue SUPP_II | Pages 403 - 403
1 Jul 2008
Kelley S Ashford R Rao A Dickson R
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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


Orthopaedic Proceedings
Vol. 102-B, Issue SUPP_6 | Pages 89 - 89
1 Jul 2020
Chua K
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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


Bone & Joint 360
Vol. 1, Issue 5 | Pages 26 - 28
1 Oct 2012

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


The Bone & Joint Journal
Vol. 100-B, Issue 3 | Pages 370 - 377
1 Mar 2018
Gilg MM Gaston CL Jeys L Abudu A Tillman RM Stevenson JD Grimer RJ Parry MC

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. Patients and Methods. All patients who had a noninvasive growing endoprosthesis inserted at the time of a revision procedure were identified from our database. A total of 21 patients (seven female patients, 14 male) with a mean age of 20.4 years (10 to 41) at the time of revision were included. The indications for revision were mechanical failure, trauma or infection with a residual leg-length discrepancy. The mean follow-up was 70 months (17 to 128). The mean shortening prior to revision was 44 mm (10 to 100). Lengthening was performed in all but one patient with a mean lengthening of 51 mm (5 to 140). Results. The mean residual leg length discrepancy at final follow-up of 15 mm (1 to 35). Two patients developed a deep periprosthetic infection, of whom one required amputation to eradicate the infection; the other required two-stage revision. Implant survival according to Henderson criteria was 86% at two years and 72% at five years. When considering revision for any cause (including revision of the growing prosthesis to a non-growing prosthesis), revision-free implant survival was 75% at two years, but reduced to 55% at five years. Conclusion. Our experience indicates that revision surgery using a noninvasive growing endoprosthesis is a successful option for improving leg length discrepancy and should be considered in patients with significant leg-length discrepancy requiring a revision procedure. Cite this article: Bone Joint J 2018;100-B:370–7


Orthopaedic Proceedings
Vol. 91-B, Issue SUPP_III | Pages 486 - 487
1 Sep 2009
Sharma H Reid R Reece A
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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


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XIV | Pages 39 - 39
1 Apr 2012
Jeys L Grimer R Carter S Tillman R Abudu S
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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


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXVI | Pages 67 - 67
1 Jun 2012
Khan AL Oliver WM Fender D Gibson MJ
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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


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXX | Pages 26 - 26
1 Jul 2012
Sankar B Refaie R Murray S Gerrand C
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Introduction. We aimed to determine the extent of osseous integration of the hydroxyapatite collars of tumour endoprostheses implanted in our unit. Methods. We identified 57 patients who had massive endoprostheses implanted over the last six years and reviewed clinical records and x-rays. There were 20 proximal femoral, 23 distal femoral, 6 proximal tibial, 8 proximal humeral and 1 distal humeral replacements. Patients fell into three groups: 1.Primary bone tumours, 2. Metastatic bone tumours and 3. Non-tumour indications. Results. There were 32 patients in Group 1. Mean age 32(10-71) years. Mean follow up was 22 (12-60) months. Osseous integration of the collars was noted in 19 patients (60%). Eight patients had no bony ongrowth on the collars. Nine patients underwent revision, seven for aseptic loosening and one for infection. There were 23 patients in Group 2. Mean age 72(45-83) years. The primary tumour was lung in four and renal in three. One patient had bilateral surgery. Mean follow up was 6(4-9) months. One prosthesis required revision for aseptic loosening in spite of showing good growth over the collar. True osseous integration of the collar occurred only in one patient. There were two patients in Group 3: one with a distal femoral fracture non union who demonstrated good integration of the collar. The other had a revision for infected total hip replacement with no osseous integration. Discussion. Hydroxyapatite coated collars may help integration of massive endoprostheses, but ongrowth is not always seen. Integration is best after resection of primary bone tumours. Their role in metastatic tumour surgery is questionable


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXIX | Pages 112 - 112
1 Sep 2012
Chakravarthy J Jeys L
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The distal humerus represents 1% of all primary bone tumours. Endoprosthetic replacement can potentially improve function and provide good pain relief. We present out experience with the custom made Stanmore elbow endoprosthesis used after resection of malignant tumours of the distal humerus. Between 1970–2009 we carried out 19 endoprosthetic replacments for malignant tumours of the distal humerus. 10 were a result of metastasis and 9 were primary bone tumours. 7 patients had a pathological fracture as their first presentation and 3 had pathological fractures after the diagnosis was made. 11 patients died between 3 months to 16 year following surgery. The mean survival of the patient group was 7.1 years (range 3 months to 37 years). 4 patients underwent a revision EPR and one patient underwent two revision EPR's due to loosening. Two patients underwent maintenance procedures (rebushing) due to wear of the poly bushing. We have had no revisions since the design of hte implant was changed to a floppy hinge design. One patient underwent an above elbow amputation four years after surgery due to local recurrence. There were no early post operative infections. One patient developed a sinus requiring multiple wound explorations, one year after insertion of the endoprosthesis for a sarcoma. This patient was infection free till the time of death 3 years later. There were no nerve palsies, periprosthetic fractures or wound problems. The mean TES score was 72% (59–78%) in the surviving patients at review. As the majority of the patients were implanted for metastatic disease the initial reliablity and low complication rate of the procedure, in our series, confirms that this is a suitable reconstruction for patients in significant metastatic pain from a destructive lesion of the distal humerus, rapidly restoring function and relieving pain in a predictable manner


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXXIX | Pages 113 - 113
1 Sep 2012
Sankar B Refaie R Murray S Gerrand C
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Introduction. Aseptic loosening is the most common mode of failure of massive endoprostheses. Introduction of Hydroxyapatite coated collars have reduced the incidence of aseptic loosening. However bone growth is not always seen on these collars. Objectives. The aims of our study were to determine the extent of osseous integration of Hydroxyapatite coated collars, attempt a grading system for bone growth and to determine the effect of diagnosis, surgical technique and adjuvant therapy on bone growth. Methods. We reviewed the records and radiographs of 58 patients who had a massive endoprosthesis implanted by two surgeons in our unit over the last five years. Revision surgeries were recorded separately. Bone growth was graded 1–4 based on appearance in antero-posterior and lateral radiographs. Results. Three groups were identified. Group 1-Resections for primary bone tumours (33 patients), Group 2-resections for metastatic bone disease (22 patients) and Group 3- Resections for non tumour indications (3 patients). Overall, 60% of patients had grade 1, 12% had grade 2, 19% had grade 3 and 9% had grade 4 osteointegration. Grade 3 or 4 Collar osteointegration was found in 37% of patients in Group 1, 9% in group 2 and 67% in group 3. 5% of patients with grade 1 integration, 100% patients with grade 2 integration and none of the patients with grade 3 or 4 integration underwent revision for aseptic loosening. Appearance or widening of a gap between the resected bone end and the collar indicated loosening and impending revision. Proximal humeral replacements had the lowest rate of osteointegration (12%). Adjuvant therapy did not affect osteointegration. Conclusion. Osteointegration of collars is seen more often after resection of primary bone tumours. The role of collars in metastatic tumour surgery is questionable. Our radiographic grading system of bone growth predicted aseptic loosening


The Journal of Bone & Joint Surgery British Volume
Vol. 78-B, Issue 4 | Pages 652 - 657
1 Jul 1996
Abudu A Carter SR Grimer RJ

Improvement in the life expectancy of patients with primary bone tumours has led to increased emphasis on limb salvage and preservation of function. Between 1979 and 1994 we used custom-made endoprostheses in 18 patients to reconstruct diaphyseal defects after excision of primary bone tumours. The mean age at operation was 26 years (9 to 64) and the median follow-up 65 months (6 to 188). Fifteen patients have survived and are free from local or metastatic disease. Local recurrence developed in one patient. Using the modified Enneking functional scoring system, 77% of the patients achieved 80% or more of their premorbid functional capability. Mechanical loosening, limb shortening and secondary osteoarthritis were the main complications. There were no infections. We conclude that diaphyseal endoprostheses offer a good clinical and functional outcome in the lower limb


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_III | Pages 90 - 90
1 Feb 2012
Stokes O Al-Hakim W Park D Unwin P Blunn G Pollock R Skinner J Cannon S Briggs T
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Background. Endoprosthetic reconstruction is an established method of treatment for primary bone tumours in children. Traditionally these were implanted with cemented intramedullary fixation. Hydroxyapatite collars at the shoulder of the implant are now standard on all extremity endoprostheses, but older cases were implanted without collars. Uncemented intramedullary fixation with hydroxyapatite collars has also been used in an attempt to reduce the incidence of problems such as aseptic loosening. Currently there are various indications that dictate which method is used. Aims. To establish long term survivorship of cemented versus uncemented endoprosthesis in paediatric patients with primary bone tumours. Methods. This was a retrospective study of 441 endoprostheses implanted in 367 consecutive patients aged 18 years or less, between 1973 and 2005. This included the use of case notes, hospital databases and a radiological review. Information obtained included patient demographics, indications for surgery, anatomical distribution and type of implants, complications and survivorship. Results. Mean age was 13.9 (range 3 - 38). 210 patients were male, 157 were female. There were 364 primaries and 77 revision implants. 161 extendable and 280 definitive prostheses. 282 patients had osteosarcoma, 54 had Ewing's sarcoma and 28 had other diagnoses. Commonest sites included 197 distal femoral replacements, 85 proximal tibial implants and 57 were in the upper limb. Kaplan-Meier survival analysis was used to compare anatomical sites and method of fixation. Upper limb implants had the best long term survival. Failure rates for distal femoral replacements were compared for cemented fixation (21.7% due to aseptic loosening) with cement plus hydroxyapatite collars (3.1%) and uncemented implants with hydroxyapatite collars (6.2%). Conclusions. In the distal femur cemented fixation with hydroxyapatite collars gave the best survivorship in definitive primary prostheses. Uncemented fixation with hydroxyapatite collars gave the best survivorship in extendable prostheses. Cemented fixation without hydroxyapatite gave the worst survivorship


The Journal of Bone & Joint Surgery British Volume
Vol. 93-B, Issue 5 | Pages 684 - 688
1 May 2011
Fisher NE Patton JT Grimer RJ Porter D Jeys L Tillman RM Abudu A Carter SR

Endoprosthetic replacement of the pelvis is one of the most challenging types of limb-salvage surgery, with a high rate of complications. In an attempt to reduce this and build greater versatility into the reconstruction process, a new type of pelvic endoprosthesis was developed in 2003, based on the old McKee-Farrar prosthesis. This study reviews the outcomes in 27 patients who had an ice-cream cone pelvic prosthesis inserted at two different specialist bone tumour centres in the United Kingdom over the past six years. The indications for treatment included primary bone tumours in 19 patients and metastatic disease in two, and six implants were inserted following failure of a previous pelvic reconstruction. Most of the patients had a P2+P3 resection as classified by Enneking, and most had resection of the ilium above the sciatic notch. The mean age of the patients at operation was 49 years (13 to 81). Complications occurred in ten patients (37.0%), of which dislocation was the most common, affecting four patients (14.8%). A total of three patients (11.1%) developed a deep infection around the prosthesis but all were successfully controlled by early intervention and two patients (7.4%) developed a local recurrence, at the same time as widespread metastases appeared. In one patient the prosthesis was removed for severe pain. This method of treatment is still associated with high morbidity, but early results are promising. Complications are diminishing with increasing experience


The Journal of Bone & Joint Surgery British Volume
Vol. 90-B, Issue 4 | Pages 484 - 487
1 Apr 2008
Watts AC Teoh K Evans T Beggs I Robb J Porter D

This study reports the experience of one treatment centre with routine surveillance MRI following excision of musculoskeletal sarcoma. The case notes, MRI and histology reports for 57 patients were reviewed. The primary outcome was local tumour recurrence detected on either surveillance MRI in asymptomatic patients, or interval MRI in patients with clinical concern. A total of 47 patients had a diagnosis of soft-tissue sarcoma and ten of a primary bone tumour. A total of 13 patients (22%) had local recurrence. Nine were identified on a surveillance scan, and four by interval scans. The cost of surveillance is estimated to be £4414 per recurrence detected if low-grade tumours with clear resection margins are excluded. Surveillance scanning has a role in the early detection of local recurrence of bone and soft-tissue sarcoma


The Journal of Bone & Joint Surgery British Volume
Vol. 92-B, Issue 10 | Pages 1435 - 1441
1 Oct 2010
Bischel OE Böhm PM

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


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_I | Pages 65 - 66
1 Jan 2004
Fiorenza F Kulkarni A Grimer R Carter S Tillman R Charissoux J Pynsent P
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Purpose: Primary bone tumours situated at the distal end of the humerus are exceptional and observed in only 1% of the cases. Reconstruction at this level is often difficult due to the wide bone resection. Choosing a massive prosthesis is an interesting method allowing correct carcinological and functional results. We report a series of ten patients who underwent reconstruction of the lower end of the humerus between 1970 and 2001 with a massive prosthesis after tumour resection. Material and methods: This series included four men and six women, mean age 51 years (15–76). Eight patients had primary bone tumours and two had secondary bone lesions. Mean follow-up was 79 months (9–372). The custom-made constrained hinge prosthesis composed of chromium-cobalt and titanium was cemented. Regular clinical and radiological follow-up data were recorded. The Henneking score and the Toronto Extremity Survival Score (TESS) were used to assess functional outcome. Results: Three patients underwent revision for aseptic loosening of the humeral component at 48, 56 and 366 months with problem of polyethylene insert wear for two of them. There were no infctions or local recurrences and no secondary amputations. The posterior or anterolateral approach was used for humeral resection (mean 153 mm, 63–160 mm). Postoperatively, three patients developed transient palsy (one radial and two ulnar) which regressed. Four patients died from metastasis and had a satsifactory prosthesis result at time of death. The mean TESS was 73% (29–93%) at last follow-up. Discussion: Although this is a small series, the long-term results appear to indicate that reconstruction of the lower extremity of the humerus with a massive prosthesis is a satisfactory option for this rare tumour localisation


The Bone & Joint Journal
Vol. 99-B, Issue 4 | Pages 522 - 530
1 Apr 2017
Bus MPA van de Sande MAJ Taminiau AHM Dijkstra PDS

Aims. To assess complications and failure mechanisms of osteoarticular allograft reconstructions for primary bone tumours. Patients and Methods. We retrospectively evaluated 38 patients (28 men, 74%) who were treated at our institution with osteoarticular allograft reconstruction between 1989 and 2010. Median age was 19 years (interquartile range 14 to 32). Median follow-up was 19.5 years (95% confidence interval (CI) 13.0 to 26.1) when 26 patients (68%) were alive. In addition, we systematically searched the literature for clinical studies on osteoarticular allografts, finding 31 studies suitable for analysis. Results of papers that reported on one site exclusively were pooled for comparison. Results. A total of 20 patients (53%) experienced graft failure, including 15 due to mechanical complications (39%) and three (9%) due to infection. In the systematic review, 514 reconstructions were analysed (distal femur, n = 184, 36%; proximal tibia, n = 136, 26%; distal radius, n = 99, 19%; proximal humerus, n = 95, 18%). Overall rates of failure, fracture and infection were 27%, 20%, and 10% respectively. With the distal femur as the reference, fractures were more common in the humerus (odds ratio (OR) 4.1, 95% CI 2.2 to 7.7) and tibia (OR 2.2, 95% CI 1.3 to 4.4); infections occurred more often in the tibia (OR 2.2, 95% CI 1.3 to 4.4) and less often in the radius (OR 0.1, 95% CI 0.0 to 0.8). Conclusion. Osteoarticular allograft reconstructions are associated with high rates of mechanical complications. Although comparative studies with alternative techniques are scarce, the risk of mechanical failure in our opinion does not justify routine employment of osteoarticular allografts for reconstruction of large joints after tumour resection. Cite this article: Bone Joint J 2017;99-B:522–30