Tumours of the sacrum are difficult to manage. The sacrum provides the structural connection between the torso and lower half of the body and is subject to both axial and rotational forces. Thus, tumours or their treatment can compromise the stability of the spinopelvic junction. Additionally, nerves responsible for lower limb motor groups as well as bowel, bladder, and sexual function traverse or abut the sacrum. Preservation or sacrifice of these nerves in the treatment of sacral tumours has profound implications on the function and quality of life of the patient. This annotation will discuss current treatment protocols for sacral tumours. Cite this article: Bone Joint J 2022;104-B(12):1284–1291

Aims. Treatment of high-grade limb bone sarcoma that invades a joint requires en bloc extra-articular excision. MRI can demonstrate joint invasion but is frequently inconclusive, and its predictive value is unknown. We evaluated the diagnostic accuracy of direct and indirect radiological signs of intra-articular tumour extension and the performance characteristics of MRI findings of intra-articular tumour extension. Methods. We performed a retrospective case-control study of patients who underwent extra-articular excision for sarcoma of the knee, hip, or shoulder from 1 June 2000 to 1 November 2020. Radiologists blinded to the pathology results evaluated preoperative MRI for three direct signs of joint invasion (capsular disruption, cortical breach, cartilage invasion) and indirect signs (e.g. joint effusion, synovial thickening). The discriminatory ability of MRI to detect intra-articular tumour extension was determined by receiver operating characteristic analysis. Results. Overall, 49 patients underwent extra-articular excision. The area under the curve (AUC) ranged from 0.65 to 0.76 for direct signs of joint invasion, and was 0.83 for all three combined. In all, 26 patients had only one to two direct signs of invasion, representing an equivocal result. In these patients, the AUC was 0.63 for joint effusion and 0.85 for synovial thickening. When direct signs and synovial thickening were combined, the AUC was 0.89. Conclusion. MRI provides excellent discrimination for determining intra-articular tumour extension when multiple direct signs of invasion are present. When MRI results are equivocal, assessment of synovial thickening increases MRI’s discriminatory ability to predict intra-articular joint extension. These results should be interpreted in the context of the study’s limitations. The inclusion of only extra-articular excisions enriched the sample for true positive cases. Direct signs likely varied with tumour histology and location. A larger, prospective study of periarticular bone sarcomas with spatial correlation of histological and radiological findings is needed to validate these results before their adoption in clinical practice. Cite this article: Bone Jt Open 2024;5(10):944–952

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 aim of this study was to determine the prevalence and impact of tourniquet use in patients undergoing limb salvage surgery with endoprosthetic reconstruction for a tumour around the knee. Methods. We retrieved data from the Prophylactic Antibiotic Regimens in Tumor Surgery (PARITY) trial; specifically, differences in baseline characteristics, surgical details, and postoperative functional outcomes between patients who had undergone surgery under tourniquet and those who had not. A linear regression model was created to evaluate the impact of tourniquet use on postoperative Toronto Extremity Salvage Scores (TESSs) while controlling for confounding variables. A negative-binomial regression model was constructed to explore predictors of postoperative length of stay (LOS). Results. Of the 604 patients enrolled in the PARITY trial, 421 had tumours around the knee joint, of whom 225 (53%) underwent surgery under tourniquet. The tourniquet group was younger (p = 0.014), more likely to undergo surgery for a tumour of the tibia, and had shorter operating times by a mean of 50 minutes (95% confidence interval 30 to 72; p < 0.001). The adjusted linear regression model found that the use of a tourniquet, a shorter operating time, and a higher baseline TESS independently predicted better function at both three- and six-month follow-up. The negative-binomial regression model showed that tourniquet use, shorter operating time, younger age, and intraoperative tranexamic acid administration independently predicted a shorter LOS in hospital. Conclusion. The results of this study show that in patients undergoing resection of a tumour around the knee and endoprosthetic reconstruction, the use of an intraoperative tourniquet is associated with a shorter operating time, a reduced length of stay in hospital, and a better early functional outcome. Cite this article: Bone Joint J 2022;104-B(10):1168–1173

Aims. The aim of this study was to investigate the feasibility of application of a 3D-printed megaprosthesis with hemiarthroplasty design for defects of the distal humerus or proximal ulna following tumour resection. Methods. From June 2018 to January 2020, 13 patients with aggressive or malignant tumours involving the distal humerus (n = 8) or proximal ulna (n = 5) were treated by en bloc resection and reconstruction with a 3D-printed megaprosthesis with hemiarthroplasty, designed in our centre. In this paper, we summarize the baseline and operative data, oncological outcome, complication profiles, and functional status of these patients. Results. Preparation of the prosthesis was a mean of 8.0 days (SD 1.5), during which time no patients experienced tumour progression. The mean operating time and intraoperative blood loss were 158.1 minutes (SD 67.6) and 176.9 ml (SD 187.8), respectively. All of the prostheses were implanted successfully. During a mean follow-up of 25.7 months (SD 7.8), no patients died, but four had complications (two superficial wound problems, one temporary palsy of radial nerve, and one dislocation). No aseptic loosening, structural failure, infection, heterotopic ossification, or degenerative arthritis was seen in this study. The mean flexion of the elbow was 119.6° (SD 15.9°) and the mean extension lag was 11.9° (SD 13.8°). The mean Musculoskeletal Tumor Society 93 score and Mayo Elbow Performance Score were 28.4 (SD 0.9) and 97.7 (SD 4.4), respectively. Conclusion. The custom-made, 3D-printed megaprosthesis with hemiarthroplasty is a feasible option for functional reconstruction after resection of a tumour in the distal humerus or proximal ulna. Cite this article: Bone Joint J 2022;104-B(6):747–757

In patients with a tumour affecting the distal ulna it is difficult to preserve the function of the wrist following extensive local resection. We report the outcome of 12 patients (nine female, three male) who underwent excision of the distal ulna without local soft-tissue reconstruction. In six patients, an aggressive benign tumour was present and six had a malignant tumour. At a mean follow-up of 64 months (15 to 132) the mean Musculoskeletal Tumour score was 64% (40% to 93%) and the mean DASH score was 35 (10 to 80). The radiological appearances were satisfactory in most patients. Local recurrence occurred in one patient with benign disease and two with malignant disease. The functional outcome was thus satisfactory at a mean follow-up in excess of five years, with a relatively low rate of complications. The authors conclude that complex reconstructive soft-tissue procedures may not be needed in these patients. Cite this article: Bone Joint J 2014;96-B:1392–5

The purpose of this study was to assess the outcome of 15 patients (mean age 13.6 years (7 to 25)) with a primary sarcoma of the tibial diaphysis who had undergone excision of the affected segment that was then irradiated (90 Gy) and reimplanted with an ipsilateral vascularised fibular graft within it. The mean follow-up was 57 months (22 to 99). The mean time to full weight-bearing was 23 weeks (9 to 57) and to complete radiological union 42.1 weeks (33 to 55). Of the 15 patients, seven required a further operation, four to obtain skin cover. The mean Musculoskeletal Society Tumor Society functional score at final follow-up was 27 out of 30 once union was complete. The functional results were comparable with those of allograft reconstruction and had a similar rate of complication. We believe this to be a satisfactory method of biological reconstruction of the tibial diaphysis in selected patients

Aims

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

Aims. The aim of this study was to determine the rate of indocyanine green (ICG) staining of bone and soft-tissue tumours, as well as the stability and accuracy of ICG fluorescence imaging in detecting tumour residuals during surgery for bone and soft-tissue tumours. Methods. ICG fluorescence imaging was performed during surgery in 34 patients with bone and soft-tissue tumours. ICG was administered intravenously at a dose of 2 mg/kg over a period of 60 minutes on the day prior to surgery. The tumour stain rate and signal-to-background ratio of each tumour were post hoc analyzed. After tumour resection, the tumour bed was scanned to locate sites with fluorescence residuals, which were subsequently inspected and biopsied. Results. The overall tumour stain rate was 88% (30/34 patients), and specific stain rates included 90% for osteosarcomas and 92% for giant cell tumours. For malignant tumours, the overall stain rate was 94%, while it was 82% for benign tumours. The ICG tumour stain was not influenced by different pathologies, such as malignant versus benign pathology, the reception (or lack thereof) of neoadjuvant chemotherapies, the length of time between drug administration and surgery, the number of doses of denosumab for patients with giant cell tumours, or the tumour response to neoadjuvant chemotherapy. The overall accuracy rate of successfully predicting tumour residuals using fluorescence was 49% (23/47 pieces of tissue). The accuracy rate after en bloc resection was significantly lower than that after piecemeal resection (16% vs 71%; p < 0.001). Conclusion. A high percentage of bone and soft-tissue tumours can be stained by ICG and the tumour staining with ICG was stable. This approach can be used in both benign and malignant tumours, regardless of whether neoadjuvant chemotherapy is adopted. The technique is also useful to detect tumour residuals in the wound, especially in patients undergoing piecemeal resection. Cite this article: Bone Joint J 2023;105-B(5):551–558

Aims. Tenosynovial giant cell tumour (TGCT) is a rare benign tumour of the musculoskeletal system. Surgical management is fraught with challenges due to high recurrence rates. The aim of this study was to describe surgical treatment and evaluate surgical outcomes of TGCT at an Australian tertiary referral centre for musculoskeletal tumours and to identify factors affecting recurrence rates. Methods. A prospective database of all patients with TGCT surgically managed by two orthopaedic oncology surgeons was reviewed. All cases irrespective of previous treatment were included and patients without follow-up were excluded. Pertinent tumour characteristics and surgical outcomes were collected for analysis. Results. There were 111 total cases included in the study; 71 (64%) were female, the mean age was 36 years (SD 13.6), and the knee (n = 64; 57.7%) was the most commonly affected joint. In all, 60 patients (54.1%) had diffuse-type (D-TGCT) disease, and 94 patients (84.7%) presented therapy-naïve as "primary cases" (PC). The overall recurrence rate was 46.8% for TGCT. There was a statistically significant difference in recurrence rates between D-TGCT and localized disease (75.0% vs 13.7%, relative risk (RR) 3.40, 95% confidence interval (CI) 2.17 to 5.34; p < 0.001), and for those who were referred in the ”revision cases” (RC) group compared to the PC group (82.4% vs 48.9%, RR 1.68, 95% CI 1.24 to 2.28; p = 0.011). Age, sex, tumour volume, and mean duration of symptoms were not associated with recurrence (p > 0.05). Conclusion. Recurrence rates remain high even at a tertiary referral hospital. Highest rates are seen in D-TGCT and “revision cases”. Due to the risks of recurrence, the complexity of surgery, and the need for adjuvant therapy, this paper further supports the management of TGCT in a tertiary referral multi-disciplinary orthopaedic oncology service. Cite this article: Bone Jt Open 2023;4(11):846–852

Aims. Giant cell tumour of bone (GCTB) is a locally aggressive lesion that is difficult to treat as salvaging the joint can be associated with a high rate of local recurrence (LR). We evaluated the risk factors for tumour relapse after treatment of a GCTB of the limbs. Methods. A total of 354 consecutive patients with a GCTB underwent joint salvage by curettage and reconstruction with bone graft and/or cement or en bloc resection. Patient, tumour, and treatment factors were analyzed for their impact on LR. Patients treated with denosumab were excluded. Results. There were 53 LRs (15%) at a mean 30.5 months (5 to 116). LR was higher after curettage (18.4%) than after resection (4.6%; p = 0.008). Neither pathological fracture (p = 0.240), Campanacci grade (p = 0.734), soft-tissue extension (p = 0.297), or tumour size (p = 0.872) affected the risk of recurrence. Joint salvage was possible in 74% of patients overall (262/354), and 98% after curettage alone (262/267). Of 49 patients with LR after curettage, 44 (90%) underwent repeated curettage and joint salvage. For patients treated by curettage, only age less than 30 years (p = 0.042) and location in the distal radius (p = 0.043) predicted higher LR. The rate of LR did not differ whether cement or bone graft was used (p = 0.753), but may have been reduced by the use of hydrogen peroxide (p = 0.069). Complications occurred in 15.3% of cases (54/354) and did not differ by treatment. Conclusion. Most patients with a GCTB can undergo successful joint salvage by aggressive curettage, even in the presence of a soft-tissue mass, pathological fracture, or a large lesion, with an 18.4% risk of local recurrence. However, 90% of local relapses after curettage can be treated by repeat joint salvage. Maximizing joint salvage is important to optimize long-term function since most patients with a GCTB are young adults. Younger patients and those with distal radius tumours treated with joint-sparing procedures have a higher rate of local relapse and may require more aggressive treatment and closer follow-up. Cite this article: Bone Joint J 2023;105-B(5):559–567

Desmoid tumours are a rare fibroblastic proliferation of monoclonal origin, arising in deep soft-tissues. Histologically, they are characterized by locally aggressive behaviour and an inability to metastasize, and clinically by a heterogeneous and unpredictable course. Desmoid tumours can occur in any anatomical site, but commonly arise in the limbs. Despite their benign nature, they can be extremely disabling and sometimes life-threatening, causing severe pain and functional limitations. Their surgical management is complex and challenging, due to uncertainties surrounding the biological and clinical behaviour, rarity, and limited available literature. Resection has been the first-line approach for patients with a desmoid tumour but, during the last few decades, a shift towards a more conservative approach has occurred, with an initial ‘wait and see’ policy. Many medical and regional forms of treatment are also available for the management of this condition, and others have recently emerged with promising results. However, many areas of controversy remain, and further studies and global collaboration are needed to obtain prospective and randomized data, in order to develop an appropriate shared stepwise approach. Cite this article: Bone Joint J 2023;105-B(7):729–734

Aims. Custom triflange acetabular components (CTACs) play an important role in reconstructive orthopaedic surgery, particularly in revision total hip arthroplasty (rTHA) and pelvic tumour resection procedures. Accurate CTAC positioning is essential to successful surgical outcomes. While prior studies have explored CTAC positioning in rTHA, research focusing on tumour cases and implant flange positioning precision remains limited. Additionally, the impact of intraoperative navigation on positioning accuracy warrants further investigation. This study assesses CTAC positioning accuracy in tumour resection and rTHA cases, focusing on the differences between preoperative planning and postoperative implant positions. Methods. A multicentre observational cohort study in Australia between February 2017 and March 2021 included consecutive patients undergoing acetabular reconstruction with CTACs in rTHA (Paprosky 3A/3B defects) or tumour resection (including Enneking P2 peri-acetabular area). Of 103 eligible patients (104 hips), 34 patients (35 hips) were analyzed. Results. CTAC positioning was generally accurate, with minor deviations in cup inclination (mean 2.7°; SD 2.84°), anteversion (mean 3.6°; SD 5.04°), and rotation (mean 2.1°; SD 2.47°). Deviation of the hip centre of rotation (COR) showed a mean vector length of 5.9 mm (SD 7.24). Flange positions showed small deviations, with the ischial flange exhibiting the largest deviation (mean vector length of 7.0 mm; SD 8.65). Overall, 83% of the implants were accurately positioned, with 17% exceeding malpositioning thresholds. CTACs used in tumour resections exhibited higher positioning accuracy than rTHA cases, with significant differences in inclination (1.5° for tumour vs 3.4° for rTHA) and rotation (1.3° for tumour vs 2.4° for rTHA). The use of intraoperative navigation appeared to enhance positioning accuracy, but this did not reach statistical significance. Conclusion. This study demonstrates favourable CTAC positioning accuracy, with potential for improved accuracy through intraoperative navigation. Further research is needed to understand the implications of positioning accuracy on implant performance and long-term survival. Cite this article: Bone Jt Open 2024;5(4):260–268

Aims. Venous tumour thrombus (VTT) is a rare finding in osteosarcoma. Despite the high rate of VTT in osteosarcoma of the pelvis, there are very few descriptions of VTT associated with extrapelvic primary osteosarcoma. We therefore sought to describe the prevalence and presenting features of VTT in osteosarcoma of both the pelvis and the limbs. Methods. Records from a single institution were retrospectively reviewed for 308 patients with osteosarcoma of the pelvis or limb treated between January 2000 and December 2022. Primary lesions were located in an upper limb (n = 40), lower limb (n = 198), or pelvis (n = 70). Preoperative imaging and operative reports were reviewed to identify patients with thrombi in proximity to their primary lesion. Imaging and histopathology were used to determine presence of tumour within the thrombus. Results. Tumours abutted the blood vessels in 131 patients (43%) and encased the vessels in 30 (10%). Any form of venous thrombus was identified in 31 patients (10%). Overall, 21 of these thrombi were determined to be involved with the tumour based on imaging (n = 9) or histopathology (n = 12). The rate of VTT was 25% for pelvic osteosarcoma and 1.7% for limb osteosarcoma. The most common imaging features associated with histopathologically proven VTT were enhancement with contrast (n = 12; 100%), venous enlargement (n = 10; 83%), vessel encasement (n = 8; 66%), and visible intraluminal osteoid matrix (n = 6; 50%). Disease-specific survival (DSS) for patients with VTT was 95% at 12 months (95% CI 0.87 to 1.00), 50% at three years (95% CI 0.31 to 0.80), and 31% at five years (95% CI 0.14 to 0.71). VTT was associated with worse DSS (hazard ratio 2.3 (95% CI 1.11 to 4.84). Conclusion. VTT is rare with osteosarcoma and occurs more commonly in the pelvis than the limbs. Imaging features suggestive of VTT include enhancement with contrast, venous dilation, and vessel encasement. VTT portends a worse prognosis for patients with osteosarcoma, with a similar survivability to metastatic disease. Cite this article: Bone Joint J 2024;106-B(8):865–870

Aims. We performed a systematic literature review to define features of patients, treatment, and biological behaviour of multicentric giant cell tumour (GCT) of bone. Methods. The search terms used in combination were “multicentric”, “giant cell tumour”, and “bone”. Exclusion criteria were: reports lacking data, with only an abstract; papers not reporting data on multicentric GCT; and papers on multicentric GCT associated with other diseases. Additionally, we report three patients treated under our care. Results. A total of 52 papers reporting on 104 patients were included in the analysis, with our addition of three patients. Multicentric GCT affected predominantly young people at a mean age of 22 years (10 to 62), manifesting commonly as metachronous tumours. The mean interval between the first and subsequent lesions was seven years (six months to 27 years). Synchronous lesions were observed in one-third of the patients. Surgery was curettage in 63% of cases (163 lesions); resections or amputation were less frequent. Systemic treatments were used in 10% (n = 14) of patients. Local recurrence and distant metastases were common. Conclusion. Multicentric GCT is rare, biologically aggressive, and its course is unpredictable. Patients with GCT should be followed indefinitely, and referred promptly if new symptoms, particularly pain, emerge. Denosumab can have an important role in the treatment. Cite this article: Bone Joint J 2022;104-B(12):1352–1361

Aims. The present study investigated receptor activator of nuclear factor kappa-Β ligand (RANKL), osteoprotegerin (OPG), and Runt-related transcription factor 2 (RUNX2) gene expressions in giant cell tumour of bone (GCTB) patients in relationship with tumour recurrence. We also aimed to investigate the influence of CpG methylation on the transcriptional levels of RANKL and OPG. Methods. A total of 32 GCTB tissue samples were analyzed, and the expression of RANKL, OPG, and RUNX2 was evaluated by quantitative polymerase chain reaction (qPCR). The methylation status of RANKL and OPG was also evaluated by quantitative methylation-specific polymerase chain reaction (qMSP). Results. We found that RANKL and RUNX2 gene expression was upregulated more in recurrent than in non-recurrent GCTB tissues, while OPG gene expression was downregulated more in recurrent than in non-recurrent GCTB tissues. Additionally, we proved that changes in DNA methylation contribute to upregulating the expression of RANKL and downregulating the expression of OPG, which are critical for bone homeostasis and GCTB development. Conclusion. Our results suggest that the overexpression of RANKL/RUNX2 and the lower expression of OPG are associated with recurrence in GCTB patients. Cite this article: Bone Joint Res 2024;13(2):84–91

Aims. The aim of this study was to investigate the safety and efficacy of 3D-printed modular prostheses in patients who underwent joint-sparing limb salvage surgery (JSLSS) for malignant femoral diaphyseal bone tumours. Methods. We retrospectively reviewed 17 patients (13 males and four females) with femoral diaphyseal tumours who underwent JSLSS in our hospital. Results. In all, 17 patients with locally aggressive bone tumours (Enneking stage IIB) located in the femoral shaft underwent JSLSS and reconstruction with 3D-printed modular prostheses between January 2020 and June 2022. The median surgical time was 153 minutes (interquartile range (IQR) 117 to 248), and the median estimated blood loss was 200ml (IQR 125 to 400). Osteosarcoma was the most common pathological type (n = 12; 70.6%). The mean osteotomy length was 197.53 mm (SD 12.34), and the median follow-up was 25 months (IQR 19 to 38). Two patients experienced local recurrence and three developed distant metastases. Postoperative complications included wound infection in one patient and screw loosening in another, both of which were treated successfully with revision surgery. The median Musculoskeletal Tumor Society score at the final follow-up was 28 (IQR 27 to 28). Conclusion. The 3D-printed modular prosthesis is a reliable and feasible reconstruction option for patients with malignant femoral diaphyseal tumours. It helps to improve the limb salvage rate, restore limb function, and achieve better short-term effectiveness. Cite this article: Bone Jt Open 2024;5(4):317–323

Aims. For rare cases when a tumour infiltrates into the hip joint, extra-articular resection is required to obtain a safe margin. Endoprosthetic reconstruction following tumour resection can effectively ensure local control and improve postoperative function. However, maximizing bone preservation without compromising surgical margin remains a challenge for surgeons due to the complexity of the procedure. The purpose of the current study was to report clinical outcomes of patients who underwent extra-articular resection of the hip joint using a custom-made osteotomy guide and 3D-printed endoprosthesis. Methods. We reviewed 15 patients over a five-year period (January 2017 to December 2022) who had undergone extra-articular resection of the hip joint due to malignant tumour using a custom-made osteotomy guide and 3D-printed endoprosthesis. Each of the 15 patients had a single lesion, with six originating from the acetabulum side and nine from the proximal femur. All patients had their posterior column preserved according to the surgical plan. Results. Postoperative pathological assessment revealed a negative surgical margin was achieved in all patients. At final follow-up, 13.3% (2/15) died and no recurrence occurred. The overall survival was 81.7% at five years. None of the patients showed any signs of aseptic loosening, and no wound healing issues were observed. In total, 20% (3/15) developed complications, with two cases of early hip dislocation and one case of deep infection. The cumulative incidence of mechanical and non-mechanical failure in this series was 13.7% and 9.3%, respectively, at five years. In this cohort, the mean time to full weightbearing was 5.89 (SD 0.92) weeks and the mean Musculoskeletal Tumor Society score was 24.1 (SD 4.4). Conclusion. For patients with a hip joint tumour who met the inclusion criteria and were deemed suitable for posterior column preservation, a custom-made osteotomy guide combined with 3D-printed endoprosthesis is worth performing when treating patients who require extra-articular resection of the hip joint, as it can achieve adequate margin for local control, maximize bone preservation to maintain pelvic ring integrity, reduce the risk of complications by simplifying the surgical procedure, and allow for more precise reconstruction for better function. Cite this article: Bone Jt Open 2024;5(11):1027–1036

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. Results. At the time of the first follow-up MRI, 8% of the lesions showed growth (n = 9), 86% remained stable (101), and 6% decreased in size (n = 7). During the third follow-up, with a mean follow-up time of 60 months (SD 23), 24 patients were scanned, of whom 13% had lesions that had grown and 13% lesions that had decreased in size. After 96 months (SD 37), at the sixth follow-up MRI, 100% of the lesions remained stable. None of the lesions showed malignant progression and although some lesions grew in size (mean 1 mm (SD 0.8)), no malignant progression occurred. Conclusion. We conclude that active surveillance with MRI is safe for ACTs in the long bones in the short- and mid-term follow-up. Cite this article: Bone Joint J 2024;106-B(1):86–92

Aims. Cell-free DNA (cfDNA) and circulating tumour DNA (ctDNA) are used for prognostication and monitoring in patients with carcinomas, but their utility is unclear in sarcomas. The objectives of this pilot study were to explore the prognostic significance of cfDNA and investigate whether tumour-specific alterations can be detected in the circulation of sarcoma patients. Methods. Matched tumour and blood were collected from 64 sarcoma patients (n = 70 samples) prior to resection of the primary tumour (n = 57) or disease recurrence (n = 7). DNA was isolated from plasma, quantified, and analyzed for cfDNA. A subset of cases (n = 6) underwent whole exome sequencing to identify tumour-specific alterations used to detect ctDNA using digital droplet polymerase chain reaction (ddPCR). Results. Cell-free was present in 69 of 70 samples above 0.5 ng/ml. Improved disease-free survival was found for patients with lower cfDNA levels (90% vs 48% at one-year for ≤ 6 ng/ml and > 6 ng/ml, respectively; p = 0.005). Digital droplet PCR was performed as a pilot study and mutant alleles were detectable at 0.5% to 2.5% of the wild type genome, and at a level of 0.25 ng tumour DNA. Tumour-specific alterations (ctDNA) were found in five of six cases. Conclusion. This work demonstrates the feasibility and potential utility of cfDNA and ctDNA as biomarkers for bone and soft-tissue sarcomas, despite the lack of recurrent genomic alterations. A larger study is required to validate these findings. Cite this article: Bone Joint Res 2021;10(9):602–610

Aims. To evaluate the perioperative complications associated with total en bloc spondylectomy (TES) in patients with spinal tumours, based on the extent and level of tumour resection. Methods. In total, 307 patients who underwent TES in a single centre were reviewed retrospectively. There were 164 male and 143 female patients with a mean age at the time of surgery of 52.9 years (SD 13.3). A total of 225 patients were operated on for spinal metastases, 34 for a malignant primary tumour, 41 for an aggressive benign tumour, and seven with a primary of unknown origin. The main lesion was located in the thoracic spine in 213, and in the lumbar spine in 94 patients. There were 97 patients who underwent TES for more than two consecutive vertebrae. Results. Major and minor perioperative complications were observed in 122 (39.7%) and 84 (27.4%) patients respectively. The breakdown of complications was as follows: bleeding more than 2,000 ml in 60 (19.5%) patients, hardware failure in 82 (26.7%), neurological in 46 (15.0%), surgical site infection in 23 (7.5%), wound dehiscence in 16 (5.2%), cerebrospinal fluid leakage in 45 (14.7%), respiratory in 52 (16.9%), cardiovascular in 11 (3.6%), digestive in 19 (6.2%)/ The mortality within two months of surgery was four (1.3%). The total number of complications per operation were 1.01 (SD 1.0) in the single vertebral resection group and 1.56 (SD 1.2) in the group with more than two vertebral resections. Cardiovascular and respiratory complications, along with hardware failure were statistically higher in the group who had more than two vertebrae resected. Also, in this group the amount of bleeding in patients with a lumbar lesion or respiratory complication in patients with a thoracic lesion, were statistically higher. Multivariate analysis showed that using a combined anterior and posterior approach, when more than two vertebral resections were significant independent factors. Conclusion. The characteristics of perioperative complications after TES were different depending on the extent and level of the tumour resection. In addition to preoperative clinical and pathological factors, it is therefore important to consider these factors in patients who undergo en bloc resection for spinal tumours. Cite this article: Bone Joint J 2021;103-B(5):976–983

Aims. Tenosynovial giant cell tumour (TGCT) is one of the most common soft-tissue tumours of the foot and ankle and can behave in a locally aggressive manner. Tumour control can be difficult, despite the various methods of treatment available. Since treatment guidelines are lacking, the aim of this study was to review the multidisciplinary management by presenting the largest series of TGCT of the foot and ankle to date from two specialized sarcoma centres. Methods. The Oxford Tumour Registry and the Leiden University Medical Centre Sarcoma Registry were retrospectively reviewed for patients with histologically proven foot and ankle TGCT diagnosed between January 2002 and August 2019. Results. A total of 84 patients were included. There were 39 men and 45 women with a mean age at primary treatment of 38.3 years (9 to 72). The median follow-up was 46.5 months (interquartile range (IQR) 21.3 to 82.3). Localized-type TGCT (n = 15) predominantly affected forefoot, whereas diffuse-type TGCT (Dt-TGCT) (n = 9) tended to panarticular involvement. TGCT was not included in the radiological differential diagnosis in 20% (n = 15/75). Most patients had open rather than arthroscopic surgery (76 vs 17). The highest recurrence rates were seen with Dt-TGCT (61%; n = 23/38), panarticular involvement (83%; n = 5/8), and after arthroscopy (47%; n = 8/17). Three (4%) fusions were carried out for osteochondral destruction by Dt-TGCT. There were 14 (16%) patients with Dt-TGCT who underwent systemic treatment, mostly in refractory cases (79%; n = 11). TGCT initially decreased or stabilized in 12 patients (86%), but progressed in five (36%) during follow-up; all five underwent subsequent surgery. Side effects were reported in 12 patients (86%). Conclusion. We recommend open surgical excision as the primary treatment for TGCT of the foot and ankle, particularly in patients with Dt-TGCT with extra-articular involvement. Severe osteochondral destruction may justify salvage procedures, although these are not often undertaken. Systemic treatment is indicated for unresectable or refractory cases. However, side effects are commonly experienced, and relapses may occur once treatment has ceased. Cite this article: Bone Joint J 2021;103-B(4):788–794

Aims. The aim of this study was to report the results of three forms of reconstruction for patients with a ditsl tibial bone tumour: an intercalary resection and reconstruction, an osteoarticular reconstruction, and arthrodesis of the ankle. Methods. A total of 73 patients with a median age of 19 years (interquartile range (IQR) 14 to 36) were included in this retrospective, multicentre study. Results. Reconstructions included intercalary resection in 17 patients, osteoarticular reconstruction in 11, and ankle arthrodesis in 45. The median follow-up was 77 months (IQR 35 to 130). Local recurrence occurred in eight patients after a median of 14 months (IQR 9 to 36), without a correlation with adequacy of margins or reconstructive technique. Major complications included fracture of the graft in ten patients, nonunion of the proximal osteotomy in seven, and infection in five. In the osteoarticular group, three of 11 patients developed radiological evidence of severe osteoarthritis, but only one was symptomatic and required conversion to ankle arthrodesis. Functional evaluation showed higher values of the Musculoskeletal Tumour Society (MSTS) and American Orthopaedic Foot and Ankle Society (AOFAS) scores in the intercalary group compared with the others. Conclusion. Preservation of the epiphysis in patients with a distal tibial bone tumour is a safe and effective form of limb-sparing treatment. It requires rigorous preoperative planning after accurate analysis of the imaging. When joint-sparing resection is not indicated, ankle arthrodesis, either isolated tibiotalar or combined tibiotalar and subtalar arthrodesis, should be preferred over osteoarticular reconstruction. Cite this article: Bone Joint J 2020;102-B(11):1567–1573

Giant cell tumours (GCT) of the synovium and tendon sheath can be classified into two forms: localised (giant cell tumour of the tendon sheath, or nodular tenosynovitis) and diffuse (diffuse-type giant cell tumour or pigmented villonodular synovitis). The former principally affects the small joints. It presents as a solitary slow-growing tumour with a characteristic appearance on MRI and is treated by surgical excision. There is a significant risk of multiple recurrences with aggressive diffuse disease. A multidisciplinary approach with dedicated MRI, histological assessment and planned surgery with either adjuvant radiotherapy or systemic targeted therapy is required to improve outcomes in recurrent and refractory diffuse-type GCT. Although arthroscopic synovectomy through several portals has been advocated as an alternative to arthrotomy, there is a significant risk of inadequate excision and recurrence, particularly in the posterior compartment of the knee. For local disease partial arthroscopic synovectomy may be sufficient, at the risk of recurrence. For both local and diffuse intra-articular disease open surgery is advised for recurrent disease. Marginal excision with focal disease will suffice, not dissimilar to the treatment of GCT of tendon sheath. For recurrent and extra-articular soft-tissue disease adjuvant therapy, including intra-articular radioactive colloid or moderate-dose external beam radiotherapy, should be considered

Aims. Dislocation of the hip remains a major complication after periacetabular tumour resection and endoprosthetic reconstruction. The position of the acetabular component is an important modifiable factor for surgeons in determining the risk of postoperative dislocation. We investigated the significance of horizontal, vertical, and sagittal displacement of the hip centre of rotation (COR) on postoperative dislocation using a CT-based 3D model, as well as other potential risk factors for dislocation. Methods. A total of 122 patients who underwent reconstruction following resection of periacetabular tumour between January 2011 and January 2020 were studied. The risk factors for dislocation were investigated with univariate and multivariate logistic regression analysis on patient-specific, resection-specific, and reconstruction-specific variables. Results. The dislocation rate was 13.9% (n = 17). The hip COR was found to be significantly shifted anteriorly and inferiorly in most patients in the dislocation group compared with the non-dislocation group. Three independent risk factors were found to be related to dislocation: resection of gluteus medius (odds ratio (OR) 3.68 (95% confidence interval (CI) 1.24 to 19.70); p = 0.039), vertical shift of COR > 18 mm (OR 24.8 (95% CI 6.23 to 128.00); p = 0.001), and sagittal shift of COR > 20 mm (OR 6.22 (95% CI 1.33 to 32.2); p = 0.026). Conclusion. Among the 17 patients who dislocated, 70.3% (n = 12) were anterior dislocations. Three independent risk factors were identified, suggesting the importance of proper restoration of the COR and the role of the gluteus medius in maintaining hip joint stability. Cite this article: Bone Joint J 2022;104-B(10):1180–1188

Aims. Rotating-hinge knee prostheses are commonly used to reconstruct the distal femur after resection of a tumour, despite the projected long-term burden of reoperation due to complications. Few studies have examined the factors that influence their failure and none, to our knowledge, have used competing risk models to do so. The purpose of this study was to determine the risk factors for failure of a rotating-hinge knee distal femoral arthroplasty using the Fine-Gray competing risk model. Methods. We retrospectively reviewed 209 consecutive patients who, between 1991 and 2016, had undergone resection of the distal femur for tumour and reconstruction using a rotating-hinge knee prosthesis. The study endpoint was failure of the prosthesis, defined as removal of the femoral component, the tibial component, or the bone-implant fixation; major revision (exchange of the femoral component, tibial component, or the bone-implant fixation); or amputation. Results. Multivariate Fine-Gray regression analyses revealed different hazards for each Henderson failure mode: percentage of femoral resection (p = 0.001) and extent of quadriceps muscle resection (p = 0.005) for overall prosthetic failure; extent of quadriceps muscle resection (p = 0.002) and fixation of femoral component (p = 0.011) for type 2 failure (aseptic loosening); age (p = 0.009) and percentage of femoral resection (p = 0.019) for type 3 failure (mechanical failure); and type of joint resection (p = 0.037) for type 4 (infection) were independent predictors. A bone stem ratio of > 2.5 reliably predicted aseptic loosening. Conclusion. We identified independent risk factors for overall and cause-specific prosthetic failure after rotating-hinge knee distal femoral arthroplasty using a competing risk Fine-Gray model. A bone stem ratio > 2.5 reliably predicts aseptic loosening. An accurate knowledge of the risks of distal femoral arthroplasty after resection for tumour assists surgical planning and managing patient expectations. Cite this article: Bone Joint J 2021;103-B(8):1405–1413

Aims. Giant cell tumours (GCTs) of the proximal femur are rare, and there is no consensus about the best method of filling the defect left by curettage. In this study, we compared the outcome of using a fibular strut allograft and bone cement to reconstruct the bone defect after extended curettage of a GCT of the proximal femur. Methods. In a retrospective study, we reviewed 26 patients with a GCT of the proximal femur in whom the bone defect had been filled with either a fibular strut allograft (n = 12) or bone cement (n = 14). Their demographic details and oncological and nononcological complications were retrieved from their medical records. Limb function was assessed using the Musculoskeletal Tumor Society (MSTS) score. Results. Mean follow-up was 116 months (SD 59.2; 48 to 240) for the fibular strut allograft group and 113 months (SD 43.7; 60 to 192) for the bone cement group (p = 0.391). The rate of recurrence was not significantly different between the two groups (25% vs 21.4%). The rate of nononcological complications was 16.7% in the strut allograft group and 42.8% in the bone cement group. Degenerative joint disease was the most frequent nononcological complication in the cement group. The mean MSTS score of the patients was 92.4% (SD 11.5%; 73.3% to 100.0%) in the fibular strut allograft group and 74.2% (SD 10.5%; 66.7% to 96.7%) in the bone cement group (p < 0.001). Conclusion. Given the similar rate of recurrence and a lower rate of nononcological complications, fibular strut grafting could be recommended as a method of reconstructing the bone defect left by curettage of a GCT of the proximal femur. Cite this article: Bone Joint J 2022;104-B(2):297–301

Aims. The risk of postoperative complications after resection of soft-tissue sarcoma in the medial thigh is higher than in other locations. This study investigated whether a vessel sealing system (VSS) could help reduce the risk of postoperative complications after wide resection of soft-tissue sarcoma in the medial thigh. Methods. Of 285 patients who underwent wide resection for soft-tissue sarcoma between 2014 and 2021 at our institution, 78 patients with tumours in the medial thigh were extracted from our database. Information on clinicopathological characteristics, preoperative treatment, surgical treatment (use of VSS, blood loss volume, operating time), and postoperative course (complications, postoperative haemoglobin changes, total drainage volume, and drainage and hospitalization durations) were obtained from medical records. We statistically compared clinical outcomes between patients whose surgery did or did not use VSS (VSS and non-VSS groups, respectively). Results. There were 24 patients in the VSS group and 54 in the non-VSS group. There were no significant differences between the two groups in terms of clinicopathological background. The total drainage volume in the VSS group was significantly less than that in the non-VSS group (1,176 ml vs 3,114 ml; p = 0.018). Moreover, the drainage and hospitalization durations were significantly shorter in the VSS group compared to the non-VSS group (p = 0.017 and p = 0.024, respectively). Conclusion. Our results suggest that use of VSS can help reduce the risk of postoperative complications after wide resection of soft-tissue sarcoma in the medial thigh. Cite this article: Bone Jt Open 2023;4(6):442–446

Aims

To evaluate mid-to long-term patient-reported outcome measures (PROMs) of endoprosthetic reconstruction after resection of malignant tumours arising around the knee, and to investigate the risk factors for unfavourable PROMs.

Aims. Giant cell tumour of bone (GCTB) treatment changed since the introduction of denosumab from purely surgical towards a multidisciplinary approach, with recent concerns of higher recurrence rates after denosumab. We evaluated oncological, surgical, and functional outcomes for distal radius GCTB, with a critically appraised systematic literature review. Methods. We included 76 patients with distal radius GCTB in three sarcoma centres (1990 to 2019). Median follow-up was 8.8 years (2 to 23). Seven patients underwent curettage, 38 curettage with adjuvants, and 31 resection; 20 had denosumab. Results. Recurrence rate was 71% (5/7) after curettage, 32% (12/38) after curettage with adjuvants, and 6% (2/31) after resection. Median time to recurrence was 17 months (4 to 77). Recurrences were treated with curettage with adjuvants (11), resection (six), or curettage (two). Overall, 84% (38/45) was cured after one to thee intralesional procedures. Seven patients had 12 months neoadjuvant denosumab (5 to 15) and sixmonths adjuvant denosumab; two recurred (29%). Twelve patients had six months neoadjuvant denosumab (4 to 10); five recurred (42%). Two had pulmonary metastases (2.6%), both stable after denosumab. Complication rate was 18% (14/76, with 11 requiring surgery). At follow-up, median MusculoSkeletal Tumour Society score was 28 (18 to 30), median Short Form-36 Health Survey was 86 (41 to 95), and median Disability of Arm, Shoulder, and Hand was 7.8 (0 to 58). Conclusion. Distal radius GCTB treatment might deviate from general GCTB treatment because of complexity of wrist anatomy and function. Novel insights on surgical treatment are presented in this multicentre study and systematic review. Intralesional surgery resulted in high recurrence-rate for distal radius GCTB, also with additional denosumab. The large majority of patients however, were cured after repeated curettage. Cite this article: Bone Jt Open 2022;3(7):515–528

Aims. There is a lack of evidence about the risk factors for local recurrence of a giant cell tumour (GCT) of the sacrum treated with nerve-sparing surgery, probably because of the rarity of the disease. This study aimed to answer two questions: first, what is the rate of local recurrence of sacral GCT treated with nerve-sparing surgery and second, what are the risk factors for its local recurrence?. Methods. A total of 114 patients with a sacral GCT who underwent nerve-sparing surgery at our hospital between July 2005 and August 2017 were reviewed. The rate of local recurrence was determined, and Kaplan-Meier survival analysis carried out to evaluate the mean recurrence-free survival. Possible risks factors including demographics, tumour characteristics, adjuvant therapy, operation, and laboratory indices were analyzed using univariate analysis. Variables with p < 0.100 in the univariate analysis were further considered in a multivariate Cox regression analysis to identify the risk factors. Results. The rate of local recurrence of sacral GCT treated with nerve-sparing surgery was 28.95% (33/114). Multivariate Cox regression analysis showed that large tumour size (> 8.80 cm) (hazard ratio (HR) 3.16; 95% confidence interval (CI) 1.27 to 7.87; p = 0.014), high neutrophil-to-lymphocyte ratio (NLR) (> 2.09) (HR 3.13; 95% CI 1.28 to 7.62; p = 0.012), involvement of a sacroiliac joint (HR 3.09; 95% CI 1.06 to 9.04; p = 0.039), and massive intraoperative blood loss (> 1,550 ml) (HR 2.47; 95% CI 1.14 to 5.36; p = 0.022) were independent risk factors for local recurrence. Conclusion. Patients with a sacral GCT who undergo nerve-sparing surgery have a local recurrence rate of 29%. Large tumour size, high NLR, involvement of a sacroiliac joint, and massive intraoperative blood loss are independent risk factors. Cite this article: Bone Joint J 2020;102-B(10):1392–1398

Aims. There is an increased risk of dislocation of the hip after the resection of a periacetabular tumour and endoprosthetic reconstruction of the defect in the hemipelvis. The aim of this study was to determine the rate and timing of dislocation and to identify its risk factors. Methods. To determine the dislocation rate, we conducted a retrospective single-institution study of 441 patients with a periacetabular tumour who had undergone a standard modular hemipelvic endoprosthetic reconstruction between 2003 and 2019. After excluding ineligible patients, 420 patients were enrolled. Patient-specific, resection-specific, and reconstruction-specific variables were studied using univariate and multivariate analyses. Results. The dislocation rate was 9.3% (n = 41). Dislocation was most likely to occur in the first three months after surgery. Four independent risk factors were found, one of which was older age at operation (p = 0.039). The odds ratios (ORs) of those aged ≥ 60 years and 30 to 60 years were 8.50 and 4.64, respectively, compared with those aged < 30 years. The other three risk factors were resection of gluteus maximus (p = 0.010, OR = 5.8), vertical shift of the centre of rotation (COR) of the hip by ≥ 20 mm (p = 0.008, OR = 3.60), and a type I+II+III pelvic resection (p = 0.014, OR = 3.04). Conclusion. Hemipelvic endoprosthetic reconstruction after resection of a periacetabular tumour has a dislocation rate of 9.3% (n = 41). Patients are most likely to dislocate in the first three months after surgery. The risk is increased for older patients (especially those aged > 60 years) and for those with gluteus maximus resection, vertical shift of the COR ≥ 20 mm, and a type I+II+III pelvic resection. Cite this article: Bone Joint J 2021;103-B(2):382–390

Aims. Local recurrence remains a challenging and common problem following curettage and joint-sparing surgery for giant cell tumour of bone (GCTB). We previously reported a 15% local recurrence rate at a median follow-up of 30 months in 20 patients with high-risk GCTB treated with neoadjuvant Denosumab. The aim of this study was to determine if this initial favourable outcome following the use of Denosumab was maintained with longer follow-up. Methods. Patients with GCTB of the limb considered high-risk for unsuccessful joint salvage, due to minimal periarticular and subchondral bone, large soft tissue mass, or pathological fracture, were treated with Denosumab followed by extended intralesional curettage with the goal of preserving the joint surface. Patients were followed for local recurrence, metastasis, and secondary sarcoma. Results. A total of 25 patients with a mean age of 33.8 years (18 to 67) with high-risk GCTB received median six cycles of Denosumab before surgery. Tumours occurred most commonly around the knee (17/25, 68%). The median follow-up was 57 months (interquartile range (IQR) 13 to 88). The joint was salvaged in 23 patients (92%). Two required knee arthroplasty due to intra-articular fracture and arthritis. Local recurrence developed in 11 patients (44%) at a mean of 32.5 months (3 to 75) following surgery, of whom four underwent repeat curettage and joint salvage. One patient developed secondary osteosarcoma and another benign GCT lung metastases. Conclusion. The use of Denosumab for joint salvage was associated with a higher than expected rate of local recurrence at 44%. Neoadjuvant Denosumab for joint-sparing procedures should be considered with caution in light of these results. Cite this article: Bone Joint J 2021;103-B(1):184–191

Aims. The aim of this paper was to investigate the prognostic factors for local recurrence in patients with pathological fracture through giant cell tumours of bone (GCTB). Patients and Methods. A total of 107 patients presenting with fractures through GCTB treated at our institution (Royal Orthopaedic Hospital, Birmingham, United Kingdom) between 1995 and 2016 were retrospectively studied. Of these patients, 57 were female (53%) and 50 were male (47%).The mean age at diagnosis was 33 years (14 to 86). A univariate analysis was performed, followed by multivariate analysis to identify risk factors based on the treatment and clinical characteristics. Results. The initial surgical treatment was curettage with or without adjuvants in 55 patients (51%), en bloc resection with or without reconstruction in 45 patients (42%), and neoadjuvant denosumab, followed by resection (n = 3, 3%) or curettage (n = 4, 4%). The choice of treatment depended on tumour location, Campanacci tumour staging, intra-articular involvement, and fracture displacement. Neoadjuvant denosumab was used only in fractures through Campanacci stage 3 tumours. Local recurrence occurred in 28 patients (25%). Surgery more than six weeks after the fracture did not affect the risk of recurrence in any of the groups. In Campanacci stage 3 tumours not treated with denosumab, en bloc resection had lower local recurrences (13%), compared with curettage (39%). In tumours classified as Campanacci 2, intralesional curettage and en bloc resections had similar recurrence rates (21% and 24%, respectively). After univariate analysis, the type of surgical intervention, location, and the use of denosumab were independent factors predicting local recurrence. Further surgery was required 33% more often after intralesional curettage in comparison with resections (mean 1.59, 0 to 5 vs 1.06, 0 to 3 operations). All patients treated with denosumab followed by intralesional curettage developed local recurrence. Conclusion. In patients with pathological fractures through GCTB not treated with denosumab, en bloc resection offers lower risks of local recurrence in tumours classified as Campanacci stage 3. Curettage or resections are both similar options in terms of the risk of local recurrence for tumours classified as Campanacci stage 2. The benefits of denosumab followed by intralesional curettage in these patients still remains unclear

Aims. We analyzed the long-term outcomes of patients observed over ten years after resection en bloc and reconstruction with extracorporeal irradiated autografts. Patients and Methods. This retrospective study included 27 patients who underwent resection en bloc and reimplantation of an extracorporeal irradiated autograft. The mean patient age and follow-up period were 31.7 years (9 to 59) and 16.6 years (10.3 to 24.3), respectively. The most common diagnosis was osteosarcoma (n = 10), followed by chondrosarcoma (n = 6). The femur (n = 13) was the most frequently involved site, followed by the tibia (n = 7). There were inlay grafts in five patients, intercalary grafts in 15 patients, and osteoarticular grafts in seven patients. Functional outcome was evaluated with the Musculoskeletal Tumor Society (MSTS) scoring system. Results. There were no recurrences in the irradiated autograft and the autograft survived in 24 patients (88.9%). Major complications included nonunion (n = 9), subchondral bone collapse (n = 4), and deep infection (n = 4). Although 34 revision procedures were performed, 25 (73.5%) and four (11.8%) of these were performed less than five years and ten years after the initial surgery, respectively. The mean MSTS score at the last follow-up was 84.3% (33% to 100%). Conclusion. Considering long-term outcomes, extracorporeal irradiated autograft is an effective method of reconstruction for malignant musculoskeletal tumours. Cite this article: Bone Joint J 2019;101-B:1151–1159

Aims. The aim of this study was to explore the prognostic factors for postoperative neurological recovery and survival in patients with complete paralysis due to neoplastic epidural spinal cord compression. Patients and Methods. The medical records of 135 patients with complete paralysis due to neoplastic cord compression were retrospectively reviewed. Potential factors including the timing of surgery, muscular tone, and tumour characteristics were analyzed in relation to neurological recovery using logistical regression analysis. The association between neurological recovery and survival was analyzed using a Cox model. A nomogram was formulated to predict recovery. Results. A total of 52 patients (38.5%) achieved American Spinal Injury Association Impairment Scale (AIS) D or E recovery postoperatively. The timing of surgery (p = 0.003) was found to be significant in univariate analysis. In multivariate analysis, surgery within one week was associated with better neurological recovery than surgery within three weeks (p = 0.002), with a trend towards being associated with a better neurological recovery than surgery within one to two weeks (p = 0.597) and two to three weeks (p = 0.055). Age (p = 0.039) and muscle tone (p = 0.018) were also significant predictors. In Cox regression analysis, good neurological recovery (p = 0.004), benign tumours (p = 0.039), and primary tumours (p = 0.005) were associated with longer survival. Calibration graphs showed that the nomogram did well with an ideal model. The bootstrap-corrected C-index for neurological recovery was 0.72. Conclusion. In patients with complete paralysis due to neoplastic spinal cord compression, whose treatment is delayed for more than 48 hours from the onset of symptoms, surgery within one week is still beneficial. Surgery undertaken at this time may still offer neurological recovery and longer survival. The identification of the association between these factors and neurological recovery may help guide treatment for these patients. Cite this article: Bone Joint J 2019;101-B:872–879

Aims. Aseptic loosening is a major cause of failure in cemented endoprosthetic reconstructions. This paper presents the long-term outcomes of a custom-designed cross-pin fixation construct designed to minimize rotational stress and subsequent aseptic loosening in selected patients. The paper will also examine the long-term survivorship and modes of failure when using this technique. Patients and Methods. A review of 658 consecutive, prospectively collected cemented endoprosthetic reconstructions for oncological diagnoses at a single centre between 1980 and 2017 was performed. A total of 51 patients were identified with 56 endoprosthetic implants with cross-pin fixation, 21 of which were implanted following primary resection of tumour. Locations included distal femoral (n = 36), proximal femoral (n = 7), intercalary (n = 6), proximal humeral (n = 3), proximal tibial (n = 3), and distal humeral (n = 1). Results. The median follow-up was 132 months (interquartile range (IQR) 44 to 189). In all, 20 stems required revision: eight for infection, five for structural failure, five for aseptic loosening, and two for tumour progression. Mechanical survivorship at five, ten, and 15 years was 84%, 78%, and 78%, respectively. Mechanical failure rate varied by location, with no mechanical failures of proximal femoral constructs and distal femoral survivorship of 82%, 77%, and 77% at five, ten, and 15 years. The survivorship of primary constructs at five years was 74%, with no failure after 40 months, while the survivorship for revision constructs was 89%, 80%, and 80% at five, ten, and 15 years. . Conclusion. The rate of mechanical survivorship in our series is similar to those reported for other methods of reconstruction for short diaphyseal segments, such as compressive osseointegration. The mechanical failure rate differed by location, while there was no substantial difference in long-term survival between primary and revision reconstructions. Overall, custom cross-pin fixation is a viable option for endoprosthetic reconstruction of short metaphyseal segments with an acceptable rate of mechanical failure. Cite this article: Bone Joint J 2019;101-B:724–731

Aims. To investigate the benefits of denosumab in combination with nerve-sparing surgery for treatment of sacral giant cell tumours (GCTs). Methods. This is a retrospective cohort study of patients with GCT who presented between January 2011 and July 2017. Intralesional curettage was performed and patients treated from 2015 to 2017 also received denosumab therapy. The patients were divided into three groups: Cohort 1: control group (n = 36); cohort 2: adjuvant denosumab group (n = 9); and cohort 3: neo- and adjuvant-denosumab group (n = 17). Results. There were 68 patients within the study period. Six patients were lost to follow-up. The mean follow-up was 47.7 months (SD 23.2). Preoperative denosumab was found to reduce intraoperative haemorrhage and was associated with shorter operating time for tumour volume > 200 cm. 3. A total of 17 patients (27.4%) developed local recurrence. The locoregional control rate was 77.8% (7/9) and 87.5% (14/16) respectively for cohorts 2 and 3, in comparison to 66.7% (24/36) of the control group. The recurrence-free survival (RFS) rate was significantly higher for adjuvant denosumab group versus those without adjuvant denosumab during the first two years: 100% vs 83.8% at one year and 95.0% vs 70.3% at two years. No significant difference was found for the three-year RFS rate. Conclusion. Preoperative denosumab therapy was found to reduce intraoperative haemorrhage and was associated with shorter operating times. Adjuvant denosumab was useful to prevent early recurrence during the first two years after surgery. Cite this article: Bone Joint J 2020;102-B(2):177–185

Aims. Due to the complex anatomy of the pelvis, limb-sparing resections of pelvic tumours achieving adequate surgical margins, can often be difficult. The advent of computer navigation has improved the precision of resection of these lesions, though there is little evidence comparing resection with or without the assistance of navigation. Our aim was to evaluate the efficacy of navigation-assisted surgery for the resection of pelvic bone tumours involving the posterior ilium and sacrum. . Patients and Methods. Using our prospectively updated institutional database, we conducted a retrospective case control study of 21 patients who underwent resection of the posterior ilium and sacrum, for the treatment of a primary sarcoma of bone, between 1987 and 2015. The resection was performed with the assistance of navigation in nine patients and without navigation in 12. We assessed the accuracy of navigation-assisted surgery, as defined by the surgical margin and how this affects the rate of local recurrence, the disease-free survival and the effects on peri-and post-operative morbidity. . Results. The mean age of the patients was 36.4 years (15 to 66). The mean size of the tumour was 10.9 cm. In the navigation-assisted group, the margin was wide in two patients (16.7%), marginal in six (66.7%) and wide-contaminated in one (11.1%) with no intralesional margin. In the non-navigated-assisted group; the margin was wide in two patients (16.7%), marginal in five (41.7%), intralesional in three (25.0%) and wide-contaminated in two (16.7%). Local recurrence occurred in two patients in the navigation-assisted group (22.2%) and six in the non-navigation-assisted group (50.0%). The disease-free survival was significantly better when operated with navigation-assistance (p = 0.048). The blood loss and operating time were less in the navigated-assisted group, as was the risk of a foot drop post-operatively. Conclusion . The introduction of navigation-assisted surgery for the resection of tumours of the posterior ilium and sacrum has increased the safety for the patients and allows for a better oncological outcome. . Cite this article: Bone Joint J 2017;99-B:261–6

Objectives. To assess the accuracy of patient-specific instruments (PSIs) versus standard manual technique and the precision of computer-assisted planning and PSI-guided osteotomies in pelvic tumour resection. Methods. CT scans were obtained from five female cadaveric pelvises. Five osteotomies were designed using Mimics software: sacroiliac, biplanar supra-acetabular, two parallel iliopubic and ischial. For cases of the left hemipelvis, PSIs were designed to guide standard oscillating saw osteotomies and later manufactured using 3D printing. Osteotomies were performed using the standard manual technique in cases of the right hemipelvis. Post-resection CT scans were quantitatively analysed. Student’s t-test and Mann–Whitney U test were used. Results. Compared with the manual technique, PSI-guided osteotomies improved accuracy by a mean 9.6 mm (p < 0.008) in the sacroiliac osteotomies, 6.2 mm (p < 0.008) and 5.8 mm (p < 0.032) in the biplanar supra-acetabular, 3 mm (p < 0.016) in the ischial and 2.2 mm (p < 0.032) and 2.6 mm (p < 0.008) in the parallel iliopubic osteotomies, with a mean linear deviation of 4.9 mm (p < 0.001) for all osteotomies. Of the manual osteotomies, 53% (n = 16) had a linear deviation > 5 mm and 27% (n = 8) were > 10 mm. In the PSI cases, deviations were 10% (n = 3) and 0 % (n = 0), respectively. For angular deviation from pre-operative plans, we observed a mean improvement of 7.06° (p < 0.001) in pitch and 2.94° (p < 0.001) in roll, comparing PSI and the standard manual technique. Conclusion. In an experimental study, computer-assisted planning and PSIs improved accuracy in pelvic tumour resections, bringing osteotomy results closer to the parameters set in pre-operative planning, as compared with standard manual techniques. Cite this article: A. Sallent, M. Vicente, M. M. Reverté, A. Lopez, A. Rodríguez-Baeza, M. Pérez-Domínguez, R. Velez. How 3D patient-specific instruments improve accuracy of pelvic bone tumour resection in a cadaveric study. Bone Joint Res 2017;6:577–583. DOI: 10.1302/2046-3758.610.BJR-2017-0094.R1

The purpose of this study was to assess whether the use of a joint-sparing technique such as curettage and grafting was successful in eradicating giant cell tumours of the proximal femur, or whether an alternative strategy was more appropriate. Between 1974 and 2012, 24 patients with a giant cell tumour of the proximal femur were treated primarily at our hospital. Treatment was either joint sparing or joint replacing. Joint-sparing treatment was undertaken in ten patients by curettage with or without adjunctive bone graft. Joint replacement was by total hip replacement in nine patients and endoprosthetic replacement in five. All 11 patients who presented with a pathological fracture were treated by replacement. Local recurrence occurred in five patients (21%): two were treated by hip replacement, three by curettage and none with an endoprosthesis. Of the ten patients treated initially by curettage, six had a successful outcome without local recurrence and required no further surgery. Three eventually needed a hip replacement for local recurrence and one an endoprosthetic replacement for mechanical failure. Thus 18 patients had the affected joint replaced and only six (25%) retained their native joint. Overall, 60% of patients without a pathological fracture who were treated with curettage had a successful outcome. Cite this article: Bone Joint J 2014;96-B:127–31

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

Aims. Dislocation rates are reportedly lower in patients requiring proximal femoral hemiarthroplasty than for patients undergoing hip arthroplasty for neoplasia. Without acetabular replacement, pain due to acetabular wear necessitating revision surgery has been described. We aimed to determine whether wear of the native acetabulum following hemiarthroplasty necessitates revision surgery with secondary replacement of the acetabulum after proximal femoral replacement (PFR) for tumour reconstruction. Patients and Methods. We reviewed 100 consecutive PFRs performed between January 2003 and January 2013 without acetabular resurfacing. The procedure was undertaken in 74 patients with metastases, for a primary bone tumour in 20 and for myeloma in six. There were 48 male and 52 female patients, with a mean age of 61.4 years (19 to 85) and median follow-up of two years (interquartile range (IQR) 0.5 to 3.7 years). In total, 52 patients presented with a pathological fracture and six presented with failed fixation of a previously instrumented pathological fracture. Results. All patients underwent reconstruction with either a unipolar (n = 64) or bipolar (n = 36) articulation. There were no dislocations and no acetabular resurfacings. Articular wear was graded using the criteria of Baker et al from 0 to 3, where by 0 is normal; grade 1 represents a narrowing of articular cartilage and no bone erosion; grade 2 represents acetabular bone erosion and early migration; and grade 3 represents protrusio acetabuli. Of the 49 patients with radiological follow-up greater than one year, six demonstrated grade 1 acetabular wear and two demonstrated grade 2 acetabular wear. The remainder demonstrated no radiographic evidence of wear. Median medial migration was 0.3 mm (IQR -0.2 to 0.7) and superior migration was 0.3 mm (IQR -0.2 to 0.6). No relationship between unipolar versus bipolar articulations and wear was evident. Conclusion. Hemiarthroplasty PFRs for tumour reconstruction eliminate joint instability and, in the short to medium term, do not lead to native acetabular wear necessitating later acetabular resurfacing. Cite this article: Bone Joint J 2018;100B:101–8

Giant-cell tumour of the synovium is known to affect the fingers or toes of adults. It has seldom been described in the spine and rarely in the thoracic vertebrae or in a child. The lesions of giant-cell tumour of the synovium have a classical radiological appearance, but require a high index of suspicion for correct recognition. Unlike giant-cell tumour of the synovium at other well-known sites, spinal lesions lack the characteristic papillary architecture, thereby raising other diagnostic possibilities. We describe a giant-cell tumour of the synovium of the left facet joint of a thoracic vertebra in a nine-year-old girl. The tumour was treated successfully by surgical excision

Peri-acetabular tumour resections and their subsequent reconstruction are among the most challenging procedures in orthopaedic oncology. Despite the fact that a number of different pelvic endoprostheses have been introduced, rates of complication remain high and long-term results are mostly lacking. . In this retrospective study, we aimed to evaluate the outcome of reconstructing a peri-acetabular defect with a pedestal cup endoprosthesis after a type 2 or type 2/3 internal hemipelvectomy. A total of 19 patients (11M:8F) with a mean age of 48 years (14 to 72) were included, most of whom had been treated for a primary bone tumour (n = 16) between 2003 and 2009. After a mean follow-up of 39 months (28 days to 8.7 years) seven patients had died. After a mean follow-up of 7.9 years (4.3 to 10.5), 12 patients were alive, of whom 11 were disease-free. Complications occurred in 15 patients. Three had recurrent dislocations and three experienced aseptic loosening. There were no mechanical failures. Infection occurred in nine patients, six of whom required removal of the prosthesis. Two patients underwent hindquarter amputation for local recurrence. The implant survival rate at five years was 50% for all reasons, and 61% for non-oncological reasons. The mean Musculoskeletal Tumor Society score at final follow-up was 49% (13 to 87). Based on these poor results, we advise caution if using the pedestal cup for reconstruction of a peri-acetabular tumour resection. Cite this article: Bone Joint J 2014;96-B:1706–12

Various chemicals are commonly used as adjuvant treatment to surgery for giant-cell tumour (GCT) of bone. The comparative effect of these solutions on the cells of GCT is not known. In this study we evaluated the cytotoxic effect of sterile water, 95% ethanol, 5% phenol, 3% hydrogen peroxide (H. 2. O. 2. ) and 50% zinc chloride (ZnCI. 2. ) on GCT monolayer tumour cultures which were established from six patients. The DNA content, the metabolic activity and the viability of the cultured samples of tumour cells were assessed at various times up to 120 hours after their exposure to these solutions. Equal cytotoxicity to the GCT monolayer culture was observed for 95% ethanol, 5% phenol, 3% H. 2. O. 2. and 50% ZnCI. 2. The treated samples showed significant reductions in DNA content and metabolic activity 24 hours after treatment and this was sustained for up to 120 hours. The samples treated with sterile water showed an initial decline in DNA content and viability 24 hours after treatment, but the surviving cells were viable and had proliferated. No multinucleated cell formation was seen in these cultures. These results suggest that the use of chemical adjuvants other than water could help improve local control in the treatment of GCT of bone

Total en bloc spondylectomy (TES) is the total resection of a vertebra containing a tumour. Many authors have investigated patient-reported outcomes after routine spinal surgery and surgery for tumours in general. However, this is the first report of patient-reported outcomes, including health-related quality of life (HRQoL) and satisfaction, after en bloc vertebral resection for a spinal tumour. Of the 54 patients who underwent TES for a primary tumour between 1993 and 2010, 19 died and four were lost to follow-up. In January 2012, a questionnaire was sent to the 31 surviving patients. This included the short form-36 to assess HRQoL and questions about the current condition of their disease, activities of daily living (ADL) and surgery. The response rate was high at 83.9% (26/31 patients). We found that most patients were satisfied and maintained good performance of their ADLs. . The mental health status and social roles of the HRQoL scores were nearly equivalent to those of healthy individuals, regardless of the time since surgery. There was significant impairment of physical health in the early post-operative years, but this usually returned to normal approximately three years after surgery. Cite this article: Bone Joint J 2014;96-B:1693–8

The aim of this study was to define the incidence of venous thromboembolism (VTE) and risk factors for the development of deep-vein thrombosis (DVT) after the resection of a musculoskeletal tumour. A total of 94 patients who underwent resection of a musculoskeletal tumour between January 2003 and December 2005 were prospectively studied. There were 42 men and 52 women with a mean age of 54.4 years (18 to 86). All patients wore intermittent pneumatic compression devices and graduated compression stockings. Ultrasound examination of the lower limbs was conducted to screen for DVT between the fifth and ninth post-operative days. DVT was detected in 21 patients (22%). Of these, two were symptomatic (2%). One patient (1%) had a fatal pulmonary embolism. Patients aged ≥ 70 years had an increased risk of DVT (p = 0.004). The overall incidence of DVT (both symptomatic and asymptomatic) after resection of a musculoskeletal tumour with mechanical prophylaxis was high. It seems that both mechanical and anticoagulant prophylaxis is needed to prevent VTE in patients who have undergone the resection of a musculoskeletal tumour. Cite this article: Bone Joint J 2013;95-B:1280–4

We reviewed 20 patients who had undergone a Coonrad-Morrey total elbow arthroplasty after resection of a primary or metastatic tumour from the elbow or distal humerus between 1980 and 2002. Eighteen patients underwent reconstruction for palliative treatment with restoration of function after intralesional surgery and two after excision of a primary bone tumour. The mean follow-up was 30 months (1 to 192). Five patients (25%) were alive at the final follow-up; 14 (70%) had died of their disease and one of unrelated causes. Local control was achieved in 15 patients (75%). The mean Mayo Elbow Performance Score improved from 22 (5 to 45) to 75 points (55 to 95). Four reconstructions (20%) failed and required revision. Seven patients (35%) had early complications, the most frequent being nerve injury (25%). There were no infections or wound complications although 18 patients (90%) had radiotherapy, chemotherapy or both. The Coonrad-Morrey total elbow arthroplasty provides good relief from pain and a good functional outcome after resection of tumours of the elbow. The rates of complications involving local recurrence of tumour (25%) and nerve injury (25%) are of concern

Despite local treatment with systemic chemotherapy in Ewing’s sarcoma family tumours (ESFT), patients with detectable metastases at presentation have a markedly worse prognosis than those with apparently localised disease. We investigated the clinical, pathological and laboratory differences in 888 patients with ESFT, 702 with localised disease and 186 with overt metastases at presentation, seen at our institution between 1983 and 2006. Multivariate analyses showed that location in the pelvis, a high level of serum lactic dehydrogenase, the presence of fever and a short interval between the onset of symptoms and diagnosis were indicative of metastatic disease. The rate of overt metastases at presentation was 10% without these four risk factors, 22.7% with one, 31.4% with two, and 50% for those with three or four factors. We concluded that in ESFT the site, the serum level of lactic dehydrogenase, fever, and the interval between the onset of symptoms and diagnosis are indicators of tumours having a particularly aggressive metastatic behaviour

Patients who have limb amputation for musculoskeletal tumours are a rare group of cancer survivors. This was a prospective cross-sectional survey of patients from five specialist centres for sarcoma surgery in England. Physical function, pain and quality of life (QOL) outcomes were collected after lower extremity amputation for bone or soft-tissue tumours to evaluate the survivorship experience and inform service provision. Of 250 patients, 105 (42%) responded between September 2012 and June 2013. From these, completed questionnaires were received from 100 patients with a mean age of 53.6 years (19 to 91). In total 60 (62%) were male and 37 (38%) were female (three not specified). The diagnosis was primary bone sarcoma in 63 and soft-tissue tumour in 37. A total of 20 tumours were located in the hip or pelvis, 31 above the knee, 32 between the knee and ankle and 17 in the ankle or foot. In total 22 had hemipelvectomy, nine hip disarticulation, 35 transfemoral amputation, one knee disarticulation, 30 transtibial amputation, two toe amputations and one rotationplasty. The Toronto Extremity Salvage Score (TESS) differed by amputation level, with poorer scores at higher levels (p < 0.001). Many reported significant pain. In addition, TESS was negatively associated with increasing age, and pain interference scores. QOL for Cancer Survivors was significantly correlated with TESS (p < 0.001). This relationship appeared driven by pain interference scores. . This unprecedented national survey confirms amputation level is linked to physical function, but not QOL or pain measures. Pain and physical function significantly impact on QOL. These results are helpful in managing the expectations of patients about treatment and addressing their complex needs. Cite this article: Bone Joint J 2015;97-B:1284–90

The use of a navigation system in musculoskeletal tumour surgery enables the integration of pre-operative CT and MRI images to generate a precise three-dimensional anatomical model of the site and the extent of the tumour. We carried out six consecutive resections of musculoskeletal tumour in five patients using an existing commercial computer navigation system. There were three women and two men with a mean age of 41 years (24 to 47). Reconstruction was performed using a tumour prosthesis in three lesions and a vascularised fibular graft in one. No reconstruction was needed in two cases. The mean follow-up was 6.9 months (3.5 to 10). The mean duration of surgery was 28 minutes (13 to 50). Examination of the resected specimens showed clear margins in all the tumour lesions and a resection that was exactly as planned

Primary giant-cell tumour of soft tissue arising in the ligamentum teres has not been previously described. We report a case of such a tumour in a 46-year-old woman. The lesion was only detected at the time of hip arthroscopy despite pre-operative MRI being performed. It was successfully excised arthroscopically with resolution of the symptoms

Giant cell tumours (GCTs) of the small bones of the hands and feet are rare. Small case series have been published but there is no consensus about ideal treatment. We performed a systematic review, initially screening 775 titles, and included 12 papers comprising 91 patients with GCT of the small bones of the hands and feet. The rate of recurrence across these publications was found to be 72% (18 of 25) in those treated with isolated curettage, 13% (2 of 15) in those treated with curettage plus adjuvants, 15% (6 of 41) in those treated by resection and 10% (1 of 10) in those treated by amputation. We then retrospectively analysed 30 patients treated for GCT of the small bones of the hands and feet between 1987 and 2010 in five specialised centres. The primary treatment was curettage in six, curettage with adjuvants (phenol or liquid nitrogen with or without polymethylmethacrylate (PMMA)) in 18 and resection in six. We evaluated the rate of complications and recurrence as well as the factors that influenced their functional outcome. At a mean follow-up of 7.9 years (2 to 26) the rate of recurrence was 50% (n = 3) in those patients treated with isolated curettage, 22% (n = 4) in those treated with curettage plus adjuvants and 17% (n = 1) in those treated with resection (p = 0.404). The only complication was pain in one patient, which resolved after surgical removal of remnants of PMMA. We could not identify any individual factors associated with a higher rate of complications or recurrence. The mean post-operative Musculoskeletal Tumor Society scores were slightly higher after intra-lesional treatment including isolated curettage and curettage plus adjuvants (29 (20 to 30)) compared with resection (25 (15 to 30)) (p = 0.091). Repeated curettage with adjuvants eventually resulted in the cure for all patients and is therefore a reasonable treatment for both primary and recurrent GCT of the small bones of the hands and feet. Cite this article: Bone Joint J 2013;95-B:838–45

We evaluated the clinical results and complications after extra-articular resection of the distal femur and/or proximal tibia and reconstruction with a tumour endoprosthesis (MUTARS) in 59 patients (mean age 33 years (11 to 74)) with malignant bone or soft-tissue tumours. According to a Kaplan–Meier analysis, limb survival was 76% (95% confidence interval (CI) 64.1 to 88.5) after a mean follow-up of 4.7 years (one month to 17 years). Peri-prosthetic infection was the most common indication for subsequent amputation (eight patients). Survival of the prosthesis without revision was 48% (95% CI 34.8 to 62.0) at two years and 25% (95% CI 11.1 to 39.9) at five years post-operatively. Failure of the prosthesis was due to deep infection in 22 patients (37%), aseptic loosening in ten patients (17%), and peri-prosthetic fracture in six patients (10%). Wear of the bearings made a minor revision necessary in 12 patients (20%). The mean Musculoskeletal Tumor Society score was 23 (10 to 29). An extensor lag > 10° was noted in ten patients (17%). These results suggest that limb salvage after extra-articular resection with a tumour prosthesis can achieve good functional results in most patients, although the rates of complications and subsequent amputation are higher than in patients treated with intra-articular resection. Cite this article: Bone Joint J 2013;95-B:1425–31

1. One hundred and seventy-nine cases of primary malignant bone tumour and giant-cell tumour seen at the Middlesex Hospital since 1925 are reviewed. Tumours arising from non-skeletal tissues in bone have been excluded. 2. The following histological classification is used. Osteosarcoma (osteoblast sarcoma): This tumour is not synonymous with osteogenic (bone-forming) sarcoma. The essential feature is the formation of osteoid tissue by malignant osteoblasts, with no intermediate matrix of cartilage or fibrous tissue. It is the most malignant bone tumour and only four of the thirty-two patients survived three years. Chondrosarcoma: These tumours are composed of cartilage, and some show secondary ossification. The behaviour of this group is related to the degree of cartilaginous differentiation. In general, compared with the osteosarcoma, it is of low-grade malignancy. More than half of the sixty-eight patients survived four years. Fibrosarcoma: The essential feature of this tumour is the production of collagen by malignant fibroblastic tumour cells. Tumours of this type invading the medullary cavity have an average prognosis between that of an osteosarcoma and a chondrosarcoma. Nine of the thirty-four patients survived three years. Spindle-cell sarcoma: These tumours are composed of spindle cells which produce no diagnostic matrix. In spite of the lack of differentiation the outlook is not hopeless. Six of the eleven patients survived for five years or more. Giant-cell tumour: This tumour is composed of a cellular stroma with diagnostic giant cells resembling osteoclasts. It is by no means a benign lesion, for half the tumours recurred after treatment and a quarter of the patients died with metastases. 3. The subdivision of primary malignant skeletal tumours into groups according to the histological pattern appears to be reflected in the behaviour of the individual tumours after treatment. The prognosis of each group has been stated in the appropriate sections

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

In developing countries locally-made low-cost prostheses are mainly used in limb-salvage surgery to alleviate the economic burden. We retrospectively collected data on 104 patients treated by limb-salvage surgery between July 1997 and July 2005. We used a locally-designed and fabricated stainless-steel endoprosthesis in each case. Oncological and functional outcomes were evaluated at a mean follow-up of 47 months (12 to 118). A total of 73 patients (70.2%) were free from disease, nine (8.7%) were alive with disease, 19 (18.2%) had died from their disease and three (2.9%) from unrelated causes. According to the Musculoskeletal Tumor Society scoring system, the mean functional score was 76.3% (SD 17.8). The five-year survival for the implant was 70.5%. There were nine cases (8.7%) of infection, seven early and two late, seven (6.7%) of breakage of the prosthesis, three (2.9%) of aseptic loosening and two (1.9%) of failure of the polyethylene bushing. Multivariate analysis showed that a proximal tibial prosthesis and a resection length of 14 cm or more were significant negative prognostic factors. Our survival rates and Musculoskeletal Tumor Society functional scores are similar to those reported in the literature. Although longer follow-up is needed to confirm our results, we believe that a low-cost custom-made endoprosthesis is a cost-effective and reliable reconstructive option for limb salvage in developing countries

Desmoid tumours are not common but have a distinct resemblance to fibrosarcomata. Their clinical appearance and progress should be recognised since failure to distinguish them from sarcomata could result in extensive and unnecessarily mutilating operations. Three cases of extra-abdominal desmoid tumours are reported. Two of the patients had tumours arising from multicentric foci in the same limb. The disappearance of the tumours in two patients after the menopause, and the variation in the tumours during the menstrual cycle in the third patient, add weight to the theories about endocrine control

Aims. Osteoarticular reconstruction of the distal femur in childhood has the advantage of preserving the tibial physis. However, due to the small size of the distal femur, matching the host bone with an osteoarticular allograft is challenging. In this study, we compared the outcomes and complications of a resurfaced allograft-prosthesis composite (rAPC) with those of an osteoarticular allograft to reconstruct the distal femur in children. Methods. A retrospective analysis of 33 skeletally immature children with a malignant tumour of the distal femur, who underwent resection and reconstruction with a rAPC (n = 15) or osteoarticular allograft (n = 18), was conducted. The median age of the patients was ten years (interquartile range (IQR) 9 to 11) in the osteoarticular allograft group and nine years (IQR 8 to 10) in the rAPC group (p = 0.781). The median follow-up of the patients was seven years (IQR 4 to 8) in the osteoarticular allograft group and six years (IQR 3 to 7) in the rAPC group (p = 0.483). Limb function was evaluated using the Musculoskeletal Tumor Society (MSTS) score. Results. At final follow-up, the knee was unstable in 9/18 patients (50%) in the osteoarticular allograft group and 2/15 patients (13%) in the rAPC group (p = 0.026). The median range of motion (ROM) of the knee was 117° (IQR 115° to 120°) in the osteoarticular allograft group and 100° (IQR 95° to 105°) in the rAPC group (p < 0.001). The median MSTS score was 25 (IQR 23 to 26) in the osteoarticular allograft group and 28 (IQR 26 to 29) in the rAPC group (p = 0.007). Osteoarthritic change was detected in 11/18 patients (61%) in the osteoarticular allograft group and in 4/15 (26%) patients in the rAPC group (p = 0.048). Conclusion. In our series, a resurfaced allograft-prosthesis composite provided better knee stability and function, with a lower rate of osteoarthritis; an osteoarticular allograft was associated with better knee ROM. Cite this article: Bone Joint J 2022;104-B(10):1174–1179

1. Fifty-five giant-cell tumours of bone are described in fifty-two patients of whom thirty-five were women. The highest incidence was in the third and fourth decades. 2. The tumours were characteristic radiologically: they all occurred in skeletally mature patients, and chiefly in the expanded extremities of long bones. 3. Histological grading was not done, the tumours being designated typical or malignant. 4. Curettage and graft, resection, radiotherapy and amputation were the basic forms of treatment. Amputation has an occasional place in primary treatment and radiotherapy should be confined to tumours in surgically inaccessible sites. 5. Resection is the method of choice for tumours in suitable sites, such as the upper end of the fibula ; careful curettage and grafting should be used to preserve the function of a large joint. 6. Recurrences within two years should be treated by limited resection without preliminary biopsy. Late recurrences must have a biopsy taken to establish the diagnosis before planning treatment

Forty-seven histologically confirmed cases of Ewing's tumour are reported, with typical age, sex and skeletal distributions. The disease-free survival curves are like those for osteosarcoma of corresponding types of bone, but with higher mortality. The two-year disease-free survival rates were 24 per cent for tumours of long bones and 5 per cent for those of other bones. Osseous metastases were most frequent, although the lung was the commonest single site involved. There were secondary deposits in lymph nodes in nine patients, possibly also in four more with mediastinal enlargement. Although initially radiosensitive, one-third of tumours recurred locally, almost always with the appearance of metastases. Neither whole bone irradiation nor high dosage alone ensures complete control of the primary tumour, and it is suggested that recently reported improved results of treatment should be attributed to adjuvant multi-drug chemotherapy. Inadequate control of the primary tumour diminishes the chance of survival and for some sites radical operation merits reconsideration

Aims. The aim of this study was to evaluate health-related quality of life (HRQoL) and joint function in tenosynovial giant cell tumour (TGCT) patients before and after surgical treatment. Patients and Methods. This prospective cohort study run in two Dutch referral centres assessed patient-reported outcome measures (PROMs; 36-Item Short-Form Health Survey (SF-36), visual analogue scale (VAS) for pain, and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC)) in 359 consecutive patients with localized- and diffuse-type TGCT of large joints. Patients with recurrent disease (n = 121) and a wait-and-see policy (n = 32) were excluded. Collected data were analyzed at specified time intervals preoperatively (baseline) and/or postoperatively up to five years. Results. A total of 206 TGCT patients, 108 localized- and 98 diffuse-type, were analyzed. Median age at diagnosis of localized- and diffuse-type was 41 years (interquartile range (IQR) 29 to 49) and 37 years (IQR 27 to 47), respectively. SF-36 analyses showed statistically significant and clinically relevant deteriorated preoperative and immediate postoperative scores compared with general Dutch population means, depending on subscale and TGCT subtype. After three to six months of follow-up, these scores improved to general population means and continued to be fairly stable over the following years. VAS scores, for both subtypes, showed no statistically significant or clinically relevant differences pre- or postoperatively. In diffuse-type patients, the improvement in median WOMAC score was statistically significant and clinically relevant preoperatively versus six to 24 months postoperatively, and remained up to five years’ follow-up. Conclusion. Patients with TGCT report a better HRQoL and joint function after surgery. Pain scores, which vary hugely between patients and in patients over time, did not improve. A disease-specific PROM would help to decipher the impact of TGCT on patients’ daily life and functioning in more detail. Cite this article: Bone Joint J 2019;101-B:272–280

Giant cell tumour is the most common aggressive benign tumour of the musculoskeletal system and has a high rate of local recurrence. When it occurs in proximity to the hip, reconstruction of the joint is a challenge. Options for reconstruction after wide resection include the use of a megaprosthesis or an allograft-prosthesis composite. We performed a clinical and radiological study to evaluate the functional results of a proximal femoral allograft-prosthesis composite in the treatment of proximal femoral giant cell tumour after wide resection. This was an observational study, between 2006 and 2012, of 18 patients with a mean age of 32 years (28 to 42) and a mean follow-up of 54 months (18 to 79). We achieved excellent outcomes using Harris Hip Score in 13 patients and a good outcome in five. All allografts united. There were no complications such as infection, failure, fracture or resorption of the graft, or recurrent tumour. Resection and reconstruction of giant cell tumours with proximal femoral allograft–prosthesis composite is a better option than using a prosthesis considering preservation of bone stock and excellent restoration of function. A good result requires demanding bone banking techniques, effective measures to prevent infection and stability at the allograft-host junction. . Cite this article: Bone Joint J 2014; 96-B:1106–10

Concomitant tumour resistance (CTR) is a unique phenomenon in which animals harbouring large primary tumours are resistant to the growth of smaller metastatic tumours by systemic angiogenic suppression. To examine this clinically, in ten patients with osteosarcoma, we investigated the effects of removal of the primary tumour on the development of pulmonary metastases, the systemic angiogenesis-inducing ability and the serum levels of several angiogenesis modulators. We found that removal of the primary tumour significantly elevated systemic angiogenesis-inducing ability in five patients who had post-operative recurrence of the tumour. Post-operative elevation of the angiogenesis-induced ability was suppressed by the addition of an angiogenic inhibitor, endostatin. Also, primary removal of the tumour decreased the serum levels of vascular endothelial growth factor and endostatin. These findings suggest, for the first time, the presence of CTR in patients with osteosarcoma for whom postoperative antiangiogenic therapy may be used to prevent the post-operative progression of micrometastases

Between June 2005 and March 2008, 14 patients with a Campanacci grade-3 giant-cell tumour of the distal radius were treated by en bloc resection and reconstruction by ulnar translocation with arthrodesis of the wrist. The mean length of radius resected was 7.9 cm (5.5 to 15). All the patients were followed to bony union and 12 were available at a mean follow-up of 26 months (10 to 49). The mean time to union was four months (3 to 7) at the ulnocarpal junction and five months (3 to 8) at the ulnoradial junction. All except one patient had an excellent range of pronation and supination. The remaining patient developed a radio-ulnar synostosis. The mean Musculoskeletal Tumor Society score was 26 (87%, range 20 to 28). Three patients had a soft-tissue recurrence, but with no bony involvement. They underwent a further excision and are currently well and free from disease. Ulnar translocation provides a local vascularised bone graft to reconstruct the defect left after excision of the distal radius for giant cell tumour. It avoids the need for a microvascular procedure while retaining rotation of the forearm and good function of the hand

Giant-cell tumour of bone (GCT) is a locally benign aggressive tumour. The use of adjuvant agents, such as phenol or liquid nitrogen has been recommended to destroy the remaining tumour cells after curettage, and filling of the defect with methylmethacrylate cement has been advocated. Between 1957 and 1992 we treated 92 patients with a GCT with 50% aqueous zinc chloride solution and bone grafting. Their mean age at the time of surgery was 31 years (15 to 59) and the mean follow-up was 11 years (5 to 31). Twelve (13%) had a local recurrence and one had a wound infection. Two developed degenerative changes around the knee. Eighty-six (93%) achieved good or excellent function. Three had moderate function, and three needed amputation. Our findings indicate that treatment with an aqueous solution of zinc chloride and reconstructive bone grafting after curettage gives good results

We reviewed nine patients at a mean period of 11 years (6 to 16) after curettage and cementing of a giant-cell tumour around the knee to determine if there were any long-term adverse effects on the cartilage. Plain radiography, MRI, delayed gadolinium-enhanced MRI of the cartilage and measurement of the serum level of cartilage oligomeric matrix protein were carried out. The functional outcome was evaluated using the Lysholm knee score. Each patient was physically active and had returned to their previous occupation. Most participated in recreational sports or exercise. The mean Lysholm knee score was 92 (83 to 100). Only one patient was found to have cartilage damage adjacent to the cement. This patient had a history of intra-articular fracture and local recurrence, leading to degenerative changes. Interpretation of the data obtained from delayed gadolinium-enhanced MRI of the cartilage was difficult, with variation in the T1 values which did not correlate with the clinical or radiological findings. We did not find it helpful in the early diagnosis of degeneration of cartilage. We also found no obvious correlation between the serum cartilage oligomeric matrix protein level and the radiological and MR findings, function, time after surgery and the age of the patient. In summary, we found no evidence that the long-term presence of cement close to the knee joint was associated with the development of degenerative osteoarthritis

Aims. Adjuvant treatment after intralesional curettage for atypical cartilaginous tumours (ACTs) of long bones is widely accepted for extending surgical margins. However, evaluating the isolated effect of adjuvant treatment is difficult, and it is unclear whether not using such adjuvants provides poor oncological outcomes. Hence, we analyzed whether intralesional curettage without cryosurgery or chemical adjuvants provides poor oncological outcomes in patients with an ACT. Patients and Methods. A total of 24 patients (nine men, 15 women) (mean age 45 years; 18 to 62) were treated for ACTs of long bones and followed up for a median of 66 months (interquartile range 50 to 84). All patients were treated with extensive manual curettage and limited burring. Bone cement and grafts were used to fill bone defects in 16 and eight patients, respectively. No chemical adjuvants or cryosurgery were used. Results. No local recurrence was detectable on plain radiographs and MRI or CT images. At the last follow-up, there were no distant metastases or disease-specific deaths. No procedure-related complications or postoperative fractures developed. Conclusion. Intralesional curettage without cryosurgery or chemical adjuvants may provide excellent oncological outcomes for patients with ACTs of long bones, without the risk of complications related to adjuvant use. Our investigation suggests thorough curettage alone is a reasonable treatment option for ACT. However, we acknowledge the limited size of our investigation warrants a multicentre collaborative study to confirm our findings. Cite this article: Bone Joint J 2018;100-B:256–61

Between 1955 and 1989 we treated 24 patients (17 women and seven men) with giant-cell tumours of the spine at the Mayo Clinic. Their mean age was 30 years and the mean follow-up time was 12.4 years. Pain was the presenting symptom in all and half had a neurological deficit. The cervical, thoracic, and lumbar spines were equally involved. The tumours recurred in five of the 14 patients treated by one-stage surgery and in five of the ten treated by two-stage surgery. Seven patients received adjuvant radiotherapy, one for the primary lesion and six for recurrent lesions. Surgical management was by curettage or en bloc excision depending on the location and the extent of the tumour. Because of the risk of sarcomatous transformation, radiation therapy should be reserved for patients with incomplete excision or for those with local recurrence

Excision of the proximal femur for tumour with prosthetic reconstruction using a bipolar femoral head places a considerable load on the unreplaced acetabulum. . We retrospectively reviewed the changes which occur around the affected hip joint by evaluating the post-operative radiographs of 65 consecutive patients who underwent proximal prosthetic arthroplasty of the femur, and in whom an acetabular component had not been used. There were 37 men and 28 women with a mean age of 57.3 years (17 to 93). Radiological assessment included the extent of degenerative change in the acetabulum, heterotopic ossification, and protrusio acetabuli. . The mean follow-up was 9.1 years (2 to 11.8). Degenerative changes in the acetabulum were seen in three patients (4.6%), Brooker grade 1 or 2 heterotopic ossification in 17 (26%) and protrusion of the prosthetic head in nine (13.8%). . A total of eight patients (12.3%) needed a revision. Five were revised to the same type of prosthesis and three (4.6%) were converted to a total hip arthroplasty. . We conclude that radiological evidence of degenerative change, heterotopic ossification and protrusion occur in a few patients who undergo prosthetic arthroplasty of the proximal femur for tumour. The limited extent of these changes and the lack of associated symptoms do not justify the routine arthroplasty of the acetabulum in these patients. . Cite this article: Bone Joint J 2015;97-B:1704–9

We retrospectively studied local recurrence of giant cell tumour in long bones following treatment with curettage and cementing in 137 patients. The median follow-up time was 60 months (3 to 166). A total of 19 patients (14%) had at least one local recurrence, the first was diagnosed at a median of 17 months (3 to 29) after treatment of the primary tumour. There were 13 patients with a total of 15 local recurrences who were successfully treated by further curettage and cementing. Two patients with a second local recurrence were consequently treated twice. At the last follow-up, at a median of 53 months (3 to 128) after the most recent operation, all patients were free from disease and had good function. We concluded that local recurrence of giant cell tumour after curettage and cementing in long bones can generally be successfully treated with further curettage and cementing, with only a minor risk of increased morbidity. This suggests that more extensive surgery for the primary tumour in an attempt to obtain wide margins is not the method of choice, since it leaves the patient with higher morbidity with no significant gain with respect to cure of the disease

We evaluated the possible induction of a systemic immune response to increase anti-tumour activity by the re-implantation of destructive tumour tissue treated by liquid nitrogen in a murine osteosarcoma (LM8) model. The tumours were randomised to treatment by excision alone or by cryotreatment after excision. Tissue from the tumour was frozen in liquid nitrogen, thawed in distilled water and then re-implanted in the same animal. In addition, some mice received an immunological response modifier of OK-432 after treatment. We measured the levels of interferon-gamma and interleukin-12 cytokines and the cytotoxicity activity of splenocytes against murine LM8 osteosarcoma cells. The number of lung and the size of abdominal metastases were also measured. Re-implantation of tumour tissue after cryotreatment activated immune responses and inhibited metastatic tumour growth. OK-432 synergistically enhanced the anti-tumour effect. Our results suggest that the treatment of malignant bone tumours by reconstruction using autografts containing tumours which have been treated by liquid nitrogen may be of clinical value

1. A parallel study has been made of fifty patients presenting with a sternomastoid tumour and fifty-two patients presenting with muscular torticollis. 2. In the birth histories of both these groups there was a high incidence of breech, forceps and primiparous births. The distribution of each was strikingly similar. 3. Sternomastoid tumours were right-sided in three-quarters of all cases and in an even higher proportion of the breech births. There was twice the expected incidence of plagiocephaly. Only one in seven proceeded to muscular torticollis, but in some of the remainder minor residua could be detected. 4. Muscular torticollis presented at any age, but one-third commenced in the first year of life. Only one in five gave a history of previous sternomastoid tumour. The contracture showed a predilection for the clavicular head, and was generally associated with some degree of facial asymmetry. 5. Nine of a combined 102 cases had a first or second degree similarly affected relative. 6. It is concluded that whatever the condition in the muscle at birth, it has three inconstant and variable sequelae. The torticollis may resolve completely; it may become clinically manifest as a tumour; or it may remain clinically latent, subsequently undergoing a variable degree of cicatrisation to produce torticollis. 7. The treatment of established torticollis by open division is described and the follow-up in thirty-six cases recorded. 8. This operation can be relied on to cure the principal deformity, but is accompanied by a number of minor cosmetic defects. Of these the most striking are tight bands apparently due to anomalous reattachment of the clavicular head, and loss of the sternomastoid column of the neck. 9. The method could not be relied on to cure facial asymmetry completely, even in the early years of life. However, there was some evidence to suggest that persistence of asymmetry was allied to persistence of other residua of the torticollis, for example fascial bands

Aims. The aims of this study were to analyse the long-term outcome of vascularised fibular graft (VFG) reconstruction after tumour resection and to evaluate the usefulness of the method. . Patients and Methods. We retrospectively reviewed 49 patients who had undergone resection of a sarcoma and reconstruction using a VFG between 1988 and 2015. Their mean follow-up was 98 months (5 to 317). Reconstruction was with an osteochondral graft (n = 13), intercalary graft (n = 12), inlay graft (n = 4), or resection arthrodesis (n = 20). We analysed the oncological and functional outcome, and the rate of bony union and complications. Results. Five- and ten-year overall survival rates were 89% and 86%, respectively. Local recurrence occurred in two patients. Eight patients developed pulmonary metastases. Bone union was achieved in 44 patients (90%). Fracture occurred in six patients (12%), infection in three (6%), and nonunion in five (10%). The mean Musculoskeletal Tumor Society (MSTS) scores were as follows: osteochondral graft 70%; intercalary graft 73%; inlay graft 89%; and resection arthrodesis 83%. Conclusion. Although associated with a relatively high rate of complications, each reconstruction method is useful, with a high rate of successful limb salvage and a good long-term functional outcome. Cite this article: Bone Joint J 2017;99-B:1237–43

We describe a method of reconstruction using tumour-bearing autograft treated by liquid nitrogen in 28 patients. The operative technique consisted of en bloc excision of the tumour, removal of soft tissue, curettage of the tumour, drilling and preparation for internal fixation or prosthetic replacement before incubation for 20 minutes in liquid nitrogen, thawing at room temperature for 15 minutes, thawing in distilled water for ten minutes, and internal fixation with an intramedullary nail, plate or composite use of prosthetic replacement. Bone graft or cement was used to augment bone strength when necessary. The limb function was rated as excellent in 20 patients (71.4%), good in three (10.7%), fair in three (10.7%), and poor in two (7.1%). At the final follow-up six patients had died at a mean of 19.8 months after the operation, while 21 remained free from disease with a mean follow-up of 28.1 months (10 to 54). One patient is alive with disease. Bony union was seen at a mean of 6.7 months after the operation in 26 patients. Complications were encountered in seven patients, including three deep infections, two fractures, and two local recurrences. All were managed successfully. Our results suggest that this is a simple and effective method of biological reconstruction

1. A case is described of three giant-cell tumours, the first in 1966 in the lower left femur, the second in 1968 in the upper right femur, the third later in 1968 in the upper left femur. 2. None of the tumours could be described as frankly malignant. 3. Despite a lapse of four years it is still not possible to decide whether the first tumour had metastasised or whether all three arose independently by multifocal origin

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

Aims. After intercalary resection of a bone tumour from the femur, reconstruction with a vascularized fibular graft (VFG) and massive allograft is considered a reliable method of treatment. However, little is known about the long-term outcome of this procedure. The aims of this study were to determine whether the morbidity of this procedure was comparable to that of other reconstructive techniques, if it was possible to achieve a satisfactory functional result, and whether biological reconstruction with a VFG and massive allograft could achieve a durable, long-lasting reconstruction. Patients and Methods. A total of 23 patients with a mean age of 16 years (five to 40) who had undergone resection of an intercalary bone tumour of the femur and reconstruction with a VFG and allograft were reviewed clinically and radiologically. The mean follow-up was 141 months (24 to 313). The mean length of the fibular graft was 18 cm (12 to 29). Full weight-bearing without a brace was allowed after a mean of 13 months (seven to 26). Results. At final follow-up, the mean Musculoskeletal Tumor Society Score of 22 evaluable patients was 94% (73 to 100). Eight major complications, five fractures (21.7%), and three nonunions (13%) were seen in seven patients (30.4%). Revision-free survival was 72.3% at five, ten, and 15 years, with fracture and nonunion needing surgery as failure endpoints. Overall survival, with removal of allograft or amputation as failure endpoints, was 94.4% at five, ten, and 15 years. Discussion. There were no complications needing surgical revision after five years had elapsed from surgery, suggesting that the mechanical strength of the implant improves with time, thereby decreasing the risk of complications. In young patients with an intercalary bone tumour of the femur, combining a VFG and massive allograft may result in a reconstruction that lasts a lifetime. Cite this article: Bone Joint J 2018;100-B:378–86

The aim of this study was to evaluate the functional and oncological outcome of extracorporeally irradiated autografts used to reconstruct the pelvis after a P1/2 internal hemipelvectomy. The study included 18 patients with a primary malignant bone tumour of the pelvis. There were 13 males and five females with a mean age of 24.8 years (8 to 62). Of these, seven had an osteogenic sarcoma, six a Ewing’s sarcoma, and five a chondrosarcoma. At a mean follow-up of 51.6 months (4 to 185), nine patients had died with metastatic disease while nine were free from disease. Local recurrence occurred in three patients all of whom eventually died of their disease. Deep infection occurred in three patients and required removal of their graft in two while the third underwent a hindquarter amputation for extensive flap necrosis. The mean Musculoskeletal Tumor Society functional score of the 16 patients who could be followed-up for at least 12 months was 77% (50 to 90). Those 15 patients who completed the Toronto Extremity Salvage Score questionnaire had a mean score of 71% (53 to 85). . Extracorporeal irradiation and re-implantation of bone is a valid method of reconstruction after an internal hemipelvectomy. It has an acceptable morbidity and a functional outcome that compares favourably with other available reconstructive techniques. Cite this article: Bone Joint J 2014;96-B:1404–10

Aims. Intercalary allografts following resection of a primary diaphyseal tumour have high rates of complications and failures. At our institution intercalary allografts are augmented with intramedullary cement and fixed using compression plating. Our aim was to evaluate their long-term outcomes. Patients and Methods. A total of 46 patients underwent reconstruction with an intercalary allograft between 1989 and 2014. The patients had a mean age of 32.8 years (14 to 77). The most common diagnoses were osteosarcoma (n = 16) and chondrosarcoma (n = 9). The location of the tumours was in the femur in 21, the tibia in 16 and the humerus in nine. Function was assessed using the Musculoskeletal Tumor Society (MSTS) scoring system and the Toronto Extremity Salvage Score (TESS). The survival of the graft and the overall survival were assessed using the Kaplan-Meier method. Results. The median follow-up was 92 months (4 to 288). The mean MSTS 87 score was 29.1 (19 to 35), the mean MSTS 93 score was 82.2 (50 to 100) and the mean TESS score was 81.2 (43 to 100). Overall survival of the allograft was 84.8%. A total of 15 patients (33%) had a complication. Five allografts were revised for complications and one for local recurrence. Conclusion. Intercalary allografts augmented with intramedullary cement and compression plate fixation provide a reliable and durable method of reconstruction after the excision of a primary diaphyseal bone tumour, with high levels of function and satisfaction. . Cite this article: Bone Joint J 2017;99-B:973–8

We reviewed 23 patients with a giant-cell tumour (GCT) of bone and histologically-proven lung metastases at a mean of 11.9 years (3 to 24.5) after the original diagnosis. The mean age of the patients at diagnosis was 27.3 years (9 to 61); the male to female ratio was 0.9:1. The most common primary site was the distal radius. The mean interval between the onset of the tumour and the detection of lung metastases was 4.1 years (0 to 24). There had been local recurrence in 19 (83%) either before or at the time of diagnosis of lung metastasis. Surgical resection alone was the preferred treatment for lung metastases in 70% and resulted in 18 patients (76%) being free from disease when last reviewed. One patient had the spontaneous regression of lung metastases and was free from recurrence 16 years later. Sixteen patients (69.7%) have survived and four (17.4%) have died from progression of the tumour. Three other deaths were not related to the tumour. Local recurrence and a primary lesion at the distal radius seem to be associated with an increased risk of lung metastases. Repeated surgical resection of lung metastases gave a high rate of survival

1. A case of glomus tumour of the subcutaneous tissues of the knee which presented with extreme pain is described. Successful removal of the tumour led to complete relief of pain and return to normal function. 2. The histological features are given. 3. The literature is briefly reviewed

Amalignant peripheral nerve-sheath tumour developed in the right S1 nerve root in a man aged 30 causing back pain and sciatica. CT and MRI revealed a destructive tumour of the sacrum invading the retroperitoneal space. The tumour was not resectable with an adequate margin. Chemotherapy, consisting of high-dose ifosfamide followed by a combination of vincristine, doxorubicin and cyclophosphamide, was given with success. Malignant peripheral nerve-sheath tumours are thought to respond weakly to chemotherapy, but the response in our patient was complete

A giant cell tumour is a primary lesion of bone of intermediate severity. Its histogenesis is unclear. In a few cases pulmonary metastases have been described. Multiple skeletal metastases in the absence of sarcomatous change have been observed. We present a case report of a 25-year-old woman with a recurrent giant cell tumour of the distal fibula. After a second recurrence and six years after the initial diagnosis, she rapidly developed multiple bony metastases. The outcome was fatal

1. A twenty-six-year-old woman was paraplegic because of a benign giant-cell tumour which had destroyed the body of the twelfth thoracic vertebra completely and the bodies of the eleventh thoracic and first lumbar vertebrae partially. The tumour had expanded into both pleural cavities and displaced the aorta forward and to the left. The extent and topography of the tumour were evaluated before operation by angiography. The function of the spinal cord had not been improved significantly by laminectomy. It was therefore decided to attempt extirpation of the tumour by removing all remaining parts of the three vertebrae involved. 2. After the removal of the tumour, only the spinal cord with the thecal sac bridged the gap between the tenth thoracic and second lumbar vertebrae. The gap was bridged with struts of cortical bone from both tibiae and with two strong plates, all secured with steel wire. The metal was removed five months later because it had become loose. Gradual shortening and angulation of the spine then occurred, together with progressive resorption of the cortical grafts. Nevertheless, the operation restored the function of the spinal cord; the patient regained ability to walk and full control of the bladder

We investigated the use of hypoxia-inducible factor (HIF) proteins as prognostic markers in chondrosarcoma and the relationship of HIF to the biological characteristics of cartilage tumours. The expression of HIF-1α, HIF-2α, proliferating cell nuclear antigen (PCNA) and microvessel density (MVD) were measured immunohistochemically in 29 specimens of cartilage tumour. There was no HIF-1α and HIF-2α staining in any of the nine benign cartilage tumours. In 20 specimens of chondrosarcoma, the rate of HIF-1α and HIF-2α expression was 40% and 25%, respectively. The tumour size (≥ 8 cm), histological grade (grade 2 and grade 3) surgical margin (marginal and intralesional) and HIF-1α expression (positive) correlated significantly with a shorter disease-free survival. There was a significant association between HIF-1α and the MVD and a strong trend towards a correlation between HIF-1α and the PCNA index or histological grade. Our findings suggest that HIF-1α protein may be a useful objective marker in the assessment of the prognosis in chondrosarcoma, since it plays an important role in tumour angiogenesis and cell proliferation

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

Aims. The purpose of this retrospective study was to differentiate between the MRI features of normal post-operative change and those of residual or recurrent disease after intralesional treatment of an atypical cartilage tumour (ACT)/grade I chondrosarcoma. Patients and Methods. We reviewed the case notes, radiology and histology of 75 patients, who had been treated for an ACT/grade I chondrosarcoma by curettage, phenolisation and bone allografting between 1994 and 2005. The first post-operative Gd-enhanced MRI scan was carried out within one year of surgery. Patients had a minimum of two scans and a mean follow-up of 72 months (13 to 169). Further surgery was undertaken in cases of suspected recurrence. Results. In 14 patients (18.6%) a second procedure was undertaken after a mean period of 59 months (8 to 114). Radio frequency ablation (RFA) was used in lesions of < 10 mm and curettage, phenolisation and bone grafting for those ≥ 10 mm. Only six of these (8% of total) had a histologically-proven recurrence. No increase in tumour grade was seen at time of recurrence. Conclusion. Based on this study, we have been able to classify the post-operative MRI appearances into four groups. These groups differ in follow-up, and have a different risk of recurrence of the lesion. Follow-up and treatment vary for the patients in each group. We present a flow diagram for the appropriate and safe follow-up for this specific group of patients. Cite this article: Bone Joint J 2016;98-B:1674–81

We evaluated the construct validity of the Musculoskeletal Tumour Society rating scale (Enneking score) as a functional measure for patients with sarcoma involving the upper limb. We compared the Enneking score by examining the correlation between two patient-derived outcome measures, the Disability of the Arm, Shoulder, and Hand (DASH) questionnaire and the Medical Outcomes Study Short Form-36 (SF-36) as indicators of functional status in 40 patients with malignant or aggressive benign bone and soft-tissue tumours of the upper limb who had undergone surgical treatment. The frequency distributions were similar among the three scoring systems. As for the validity, Spearman’s rank correlation coefficient of the Enneking score to the DASH questionnaire was −0.79 and that of the Enneking to the SF-36 subscales ranged from 0.38 to 0.60. Despite being a measure from the surgeon’s perspective, the Enneking score was shown to be a valid indicator of physical disability in patients with malignant or aggressive benign tumours of the upper limb and reflected their opinion

We hypothesised that the use of computer navigation-assisted surgery for pelvic and sacral tumours would reduce the risk of an intralesional margin. We reviewed 31 patients (18 men and 13 women) with a mean age of 52.9 years (13.5 to 77.2) in whom computer navigation-assisted surgery had been carried out for a bone tumour of the pelvis or sacrum. There were 23 primary malignant bone tumours, four metastatic tumours and four locally advanced primary tumours of the rectum. The registration error when using computer navigation was <  1 mm in each case. There were no complications related to the navigation, which allowed the preservation of sacral nerve roots (n = 13), resection of otherwise inoperable disease (n = 4) and the avoidance of hindquarter amputation (n = 3). The intralesional resection rate for primary tumours of the pelvis and sacrum was 8.7% (n = 2): clear bone resection margins were achieved in all cases. At a mean follow-up of 13.1 months (3 to 34) three patients (13%) had developed a local recurrence. The mean time alive from diagnosis was 16.8 months (4 to 48). . Computer navigation-assisted surgery is safe and has reduced our intralesional resection rate for primary tumours of the pelvis and sacrum. We recommend this technique as being worthy of further consideration for this group of patients. Cite this article: Bone Joint J 2013;95-B:1417–24

Giant-cell tumour of the tendon sheath, also called pigmented villonodular synovitis, is a benign tumour with a high incidence of recurrence. We have tried to identify risk factors for recurrence. Of the 48 patients included in the study, 14 received radiotherapy after surgery. Only two (4%) had a recurrence. This compares favourably with previously reported incidences of between 25% and 45%

1. The clinical appearance and histological structure of a myo-epithelial tumour of a sweat gland of the hand is described. 2. A review of previously published papers is given. 3. It is suggested that trauma may play a part in the causation of these tumours

In 45 patients we assessed the functional results and complications for three different reconstructive procedures after resection of primary tumors of the proximal humerus. An osteoarticular allograft was used in 11, a clavicula pro humero operation in 15 and a tumour prosthesis in 19. The glenoid was resected with the proximal humerus in 25 patients. The axillary nerve was resected in 42 patients. The complication rate was lowest after reconstruction with a tumour prosthesis. The clavicula pro humero operation resulted in the most revisions. Cumulative survival rates for all the reconstructive procedures were similar. At follow-up at two years the functional results for the three reconstructive procedures were the same with a mean functional rating of 79% (Musculoskeletal Tumor Society). Excision of the glenoid had no influence on the functional result. Our findings indicate that the use of a tumour prosthesis is the most reliable limb-salvage procedure for the proximal humerus. The clavicula pro humero is an appropriate procedure if a prosthesis cannot be used

Despite extensive experience with prosthetic replacement for the reconstruction of limbs following juxta-articular resection of tumours, there are few reports of prosthetic replacement of the distal radius. We present two cases of massive bone defects of the distal radius in which alumina ceramic prosthetic replacements were used. We evaluated the patients more than ten years after the procedure. Both patients had degenerative changes to the wrist. This, however, was not associated with pain or decreased function, and both had returned to their previous occupation after surgery. When a patient has a massive defect of the distal radius, reconstruction using a ceramic prosthesis is a reasonable alternative to using autograft. This method of treatment results in little pain, a moderate range of movement and satisfactory function

A 15-year-old girl presented with a very large desmoid tumour in her buttock; it extended into the pelvis and thigh and would have required hindquarter amputation for its removal. This was not performed and the tumour underwent spontaneous regression. Fourteen years later the patient is alive and well. The suggestion is made that in some cases a more expectant approach to treatment might be justified for what is essentially a non-malignant condition

The best method of reconstruction after resection of malignant tumours of the tibial diaphysis is unknown. In the absence of any long-term studies analysing the results of intercalary endoprosthetic replacement, we present a retrospective review of 18 patients who underwent limb salvage using a tibial diaphyseal endoprosthetic replacement following excision of a malignant bone tumour. There were ten men and eight women with a mean age of 42.5 years (16 to 76). Mean follow-up was 58.5 months (20 to 141) for all patients and 69.3 months (20 to 141) for the 12 patients still alive. Cumulative patient survival was 59% (95% confidence interval (CI) 32 to 84) at five years. Implant survival was 63% (95% CI 35 to 90) at ten years. Four patients required revision to a proximal tibial replacement at a mean follow-up of 29 months (10 to 54). Complications included metastases in five patients, aseptic loosening in four, peri-prosthetic fracture in two, infection in one and local recurrence in one. The mean Musculoskeletal Tumor Society score and the mean Toronto Extremity Salvage Score were 23 (17 to 28) and 74% (53 to 91), respectively. Although rates of complication and revision were high, custom-made tibial diaphyseal replacement following resection of malignant bone tumours enables early return to function and provides an attractive alternative to other surgical options, without apparent compromise of patient survival

Giant-cell tumours of the sacrum are difficult to treat. Surgery carries a high risk of morbidity, local recurrence and mortality. Radiation is effective in some patients, but has a risk of malignant change. We evaluated the effectiveness of serial arterial embolisation as an alternative to surgery. Five patients with giant-cell tumours of the sacrum which had been primarily treated by serial embolisation were retrospectively reviewed for changes in the size of the tumour. In four the symptoms resolved with full return of function and arrest in the growth of the tumour. They remained free from growth, recurrence, or metastases at follow-up (4 to 17 years). One patient died from metastatic disease within 18 months of the initial diagnosis

The level of bone resection for osteosarcoma depends on the pre-operative evaluation of the extent of intramedullary tumour. We compared the accuracy of magnetic resonance imaging (MRI), computerised tomography (CT), and isotope bone scanning with the actual extent of the tumour in the resected specimens from 34 patients with primary osteosarcoma of a long bone. The extent of medullary tumour was defined accurately in 23 of 24 MRI scans (96%) and 24 of 32 CT scans (75%). A flexion contracture of a joint close to the tumour was an important cause for inaccurate measurements from both MRI and CT scans. Isotope bone scanning was inaccurate: its role is now confined to detecting skeletal metastases and skip lesions