Extra-articular hip resection with maintenance of pelvic continuity in malignant tumours of the proximal femur with articular invasion.

INTRODUCTION: Extra-articular hip resection may be necessary in cases of malignant tumour of the pelvic bone or of the proximal femur invading the hip joint. When the tumour is in the proximal femur, it is possible to resect the acetabulum en bloc by performing a periacetabular osteotomy, but this creates a discontinuity in the pelvic ring with difficult reconstruction and diminished function. Several techniques described recently seek to be as sparing as possible on the pelvic bone by preserving the posterior column or both columns in order to facilitate reconstruction and improve function. However, these still require complex reconstructions and can necessitate intra-pelvic dissection. TECHNIQUE: We describe here an extra-articular hip resection technique for tumours of the proximal femur invading the joint, with maintenance of pelvic continuity by preserving both columns and the quadrilateral plate of the acetabulum, without intra-pelvic dissection, that can be performed on patients in whom the medial wall of the acetabulum is thick enough. Our preliminary assessments have included studies on dry bone and imaging analyses. The technique was first tested on a single cadaver pelvis (encompassing 2 hips) and subsequently performed on a patient with a pathological fracture of the femoral neck due to osteosarcoma secondary to Paget's disease. CONCLUSIONS: Further clinical applications are essential to evaluate the overall effectiveness, safety and impact on patient functionality of this experimental technique.

Recurrent and novel fusions detected by targeted RNA sequencing as part of the diagnostic workflow of soft tissue and bone tumours.

The identification of gene fusions has become an integral part of soft tissue and bone tumour diagnosis. We investigated the added value of targeted RNA-based sequencing (targeted RNA-seq, Archer FusionPlex) to our current molecular diagnostic workflow of these tumours, which is based on fluorescence in situ hybridisation (FISH) for the detection of gene fusions using 25 probes. In a series of 131 diagnostic samples targeted RNA-seq identified a gene fusion, BCOR internal tandem duplication or ALK deletion in 47 cases (35.9%). For 74 cases, encompassing 137 FISH analyses, concordance between FISH and targeted RNA-seq was evaluated. A positive or negative FISH result was confirmed by targeted RNA-seq in 27 out of 49 (55.1%) and 81 out of 88 (92.0%) analyses, respectively. While negative concordance was high, targeted RNA-seq identified a canonical gene fusion in seven cases despite a negative FISH result. The 22 discordant FISH-positive analyses showed a lower percentage of rearrangement-positive nuclei (range 15-41%) compared to the concordant FISH-positive analyses (>41% of nuclei in 88.9% of cases). Six FISH analyses (in four cases) were finally considered false positive based on histological and targeted RNA-seq findings. For the EWSR1 FISH probe, we observed a gene-dependent disparity (p = 0.0020), with 8 out of 35 cases showing a discordance between FISH and targeted RNA-seq (22.9%). This study demonstrates an added value of targeted RNA-seq to our current diagnostic workflow of soft tissue and bone tumours in 19 out of 131 cases (14.5%), which we categorised as altered diagnosis (3 cases), added precision (6 cases), or augmented spectrum (10 cases). In the latter subgroup, four novel fusion transcripts were found for which the clinical relevance remains unclear: NAB2::NCOA2, YAP1::NUTM2B, HSPA8::BRAF, and PDE2A::PLAG1. Overall, targeted RNA-seq has proven extremely valuable in the diagnostic workflow of soft tissue and bone tumours.

Grade 3 Proximal Humerus Giant Cell Tumour: Is Resection and Reconstruction with Prosthesis a Valuable Option?

Grade 3 proximal humerus giant cell tumours are uncommon and the results of their surgical management with prosthetic replacement are not well established. We retrospectively studied patients with grade 3 giant cell tumours of proximal humerus treated with excision and prosthetic replacement from January 2009 and December 2018. We identified 13 patients who underwent tumour excision and prosthetic replacement. There were 7 (53.8%) men and 6 (46.2%) women. The mean age at diagnosis was 30.4 years (range 16-48 years). The mean follow-up period was 72.2 months (range 24-132). Margins were positive in 4 (30.8%) patients, ≤ 1 mm in 8 (61.5%) patients, and negative in 1 (7.7%) patient. Local recurrence was seen in 2 (15.4%) patients. 1 out of 2 (50%) patients with local recurrence had pathological fracture at presentation and margins were positive in both (100%) patients. There were 3 (23.1%) complications-1 patient with joint subluxation, 1 patient with fracture post trauma, and 1 patient with surgical site infection. The mean overall Musculoskeletal Tumour Society score was 24 out of 30 (range 21-29). Resection of grade 3 proximal humerus giant cell tumour and reconstruction with prosthesis results in low recurrence rate and good functional outcome and is a valuable treatment option for such tumours.

The Sarcoma Assessment Measure (SAM): Preliminary Psychometric Validation of a Novel Patient-Reported Outcome Measure.

The Sarcoma Assessment Measure (SAM) was developed as a sarcoma-specific patient-reported outcome measure to be used in clinical practice. We have reported in detail how SAM has been developed in collaboration with patients and healthcare professionals. The aim of this paper is to report the preliminary validation of SAM. The 22-item SAM was administered alongside a validated quality of life questionnaire and measure of activities of daily living. Linear modelling was used to build a measure, which had predictive validity in comparison to more established outcome measures. Of the 762 patients who participated in the study, 44.1% identified as male, and participant age ranged from 13 to 82 years. Clinically, participants presented with a range of soft tissue (82.2%) and bone (21.8%) sarcomas. Our preliminary analysis indicates that SAM accounts for 35% of the global quality of life scale and 18% of the Toronto Extremity Salvage Scale (TESS); so psychometrically, it overlaps with quality of life and activities of daily living, but also measures distinct concerns. This demonstrates that this measure picks up issues that are important to patients with sarcoma that are not reflected in other measures. We have established the preliminary validity of SAM and believe it has utility as a patient-reported outcome measure both as a research tool and for assessing the impact of symptoms and dysfunction related to sarcoma as part of clinical care. Further validation using a larger and more clinically diverse sample is now needed.

Investigation of cognitive functions in children with bone tumours and lymphoma in treatment process.

BACKGROUND: Neurocognitive sequelae are among the most debilitating effects of cancer observed in children. Yet we know very little about the impact on neurocognitive functioning, especially cancer types that develop outside the central nervous system. This study aimed to assess and compare the cognitive functions (CoF) of children with bone tumours and lymphoma in the treatment process. METHODS: The CoF of children with bone tumours (n = 44), lymphoma (n = 42) and their non-cancer peers (n = 55) were assessed with Dynamic Occupational Therapy Assessment for Children. The CoF of children with cancer were compared with their non-cancer peers. Then, children with bone tumours and lymphoma were compared in binary. RESULTS: One-hundred forty-one children aged 6-12 years with a mean age of 9.4 (SD = 1.5) were included in this study. The orientation and visuomotor construction functions of children with bone tumours and orientation, praxis and visuomotor construction functions of children with lymphoma performed worse than their non-cancer peers (pk < 0.001). While orientation, spatial perception, visuomotor construction and thinking operations functions of children with bone tumours and lymphoma were similar (pk > 0.016), praxis functions of children with lymphoma were found to be worse than children with bone tumours (pk < 0.016). CONCLUSIONS: Our findings show that children with bone tumours and lymphoma in the process of treatment are at risk for impairment of their CoF. The findings highlight the importance of assessing CoF in children with bone tumours and lymphoma and considering specific differences between groups. It is essential to assess CoF and develop early intervention plans in these children.

Benign tumours of foot and ankle.

Musculoskeletal tumours of foot or ankle make up about 4-5% of all musculoskeletal tumours. Fortunately, about 80% of them are benign. However, due to the rarity and low prevalence of each single tumour entity, diagnosis is often difficult and delayed. Ultrasonography is an important diagnostic tool to safely recognize ganglion cysts as a frequently encountered 'bump' in the foot. In suspicious lesions, malignancy must be excluded histologically in a tumour center by biopsy after imaging procedures using x-ray, computed tomography (CT) and magnetic resonance imaging (MRI). Most of the benign tumours do not require any further surgical therapy. Resection should be performed in the case of locally aggressive tumour growth or local symptoms of discomfort. In contrast to malignant tumours, the primary purpose in the resection is the least possible loss of function.

Chondrosarcoma of the Proximal Humerus: Does the Margin Affect Survival?

Chondrosarcoma (CS) is the second most common primary malignant bone tumour and, in the absence of reliable chemotherapy and radiotherapy, is effectively a surgical disease. Overall disease specific survival (DSS) is affected by tumour grade, whilst resection margin contributes to local recurrence free survival (LRFS). The aim of this study was to investigate factors that affect the local and systemic prognoses for conventional central CSs arising from the proximal humerus. A multi-centre, retrospective study from three international collaborative sarcoma centres identified 110 patients between 1995 and 2020 undergoing treatment for a conventional central CS of the proximal humerus; 58 patients (53%) had a grade 1 tumour, 36 (33%) had a grade 2 tumour, and 16 patients (13%) had a grade 3 CS. The mean age of patients was 50 years (range 10-85). The incidence of local recurrence (LR) was 9/110 (8.2%), and the disease specific mortality was 6/110 (5.5%). The grade was a statistically significant factor for LRFS (p < 0.001). None of the grade 1 tumours developed LR. The DSS was affected by the grade (p < 0.001) but not by the LR (p = 0.4). Only one patient with a grade 2 tumour died from the disease. The proximal humeral grade 1 CS behaved as a benign tumour, having no cases of LR nor death due to disease. Grade 2 CSs of the proximal humerus behaved in a more indolent way when compared with comparable grade tumours elsewhere in the appendicular skeleton, being locally aggressive with a higher LR rate than grade 1 CSs but still having very low mortality and a high rate of DSS. The LR in grade 2 CSs did not affect the DSS; therefore, surgical management in proximal humeral grade 2 CSs should have a greater emphasis on preserving function whilst maintaining an adequate margin for resection. The proximal humeral grade 3 CS was, as elsewhere in the skeleton, an aggressive, high-grade tumour. Therefore, surgical management should include en bloc resection with clear margins to avoid LR.

Robot-assisted implantation of additively manufactured patient-specific orthopaedic implants: evaluation in a sheep model.

PURPOSE: Bone tumours must be surgically excised in one piece with a margin of healthy tissue. The unique nature of each bone tumour case is well suited to the use of patient-specific implants, with additive manufacturing allowing production of highly complex geometries. This work represents the first assessment of the combination of surgical robotics and patient-specific additively manufactured implants. METHODS: The development and evaluation of a robotic system for bone tumour excision, capable of milling complex osteotomy paths, is described. The developed system was evaluated as part of an animal trial on 24 adult male sheep, in which robotic bone excision of the distal femur was followed by placement of patient-specific implants with operative time evaluated. Assessment of implant placement accuracy was completed based on post-operative CT scans. RESULTS: A mean overall implant position error of 1.05 ± 0.53 mm was achieved, in combination with a mean orientation error of 2.38 ± 0.98°. A mean procedure time (from access to implantation, excluding opening and closing) of 89.3 ± 25.25 min was observed, with recorded surgical time between 58 and 133 min, with this approximately evenly divided between robotic (43.9 ± 15.32) and implant-based (45.4 ± 18.97) tasks. CONCLUSIONS: This work demonstrates the ability for robotics to achieve repeatable and precise removal of complex bone volumes of the type that would allow en bloc removal of a bone tumour. These robotically created volumes can be precisely filled with additively manufactured patient-specific implants, with minimal gap between cut surface and implant interface.

What are the resection accuracy and guide-fitting errors associated with 3D-printed, patient-specific resection guides for bone tumour resections?

AIMS: This study aimed to analyze the accuracy and errors associated with 3D-printed, patient-specific resection guides (3DP-PSRGs) used for bone tumour resection. METHODS: We retrospectively reviewed 29 bone tumour resections that used 3DP-PSRGs based on 3D CT and 3D MRI. We evaluated the resection amount errors and resection margin errors relative to the preoperative plans. Guide-fitting errors and guide distortion were evaluated intraoperatively and one month postoperatively, respectively. We categorized each of these error types into three grades (grade 1, < 1 mm; grade 2, 1 to 3 mm; and grade 3, > 3 mm) to evaluate the overall accuracy. RESULTS: The maximum resection amount error was 2 mm. Out of 29 resection amount errors, 15 (51.7%) were grade 1 errors and 14 (38.3%) were grade 2 errors. Complex resections were associated with higher-grade resection amount errors (p < 0.001). The actual resection margins correlated significantly with the planned margins; however, there were some discrepancies. The maximum guide-fitting error was 3 mm. There were 22 (75.9%), five (17.2%), and two (6.9%) grade 1, 2, and 3 guide-fitting errors, respectively. There was no significant association between complex resection and fitting error grades. The guide distortion after one month in all patients was rated as grade 1. CONCLUSION: In terms of the accurate resection amount according to the preoperative planning, 3DP-PSRGs can be a viable option for bone tumour resection. However, 3DP-PSRG use may be associated with resection margin length discrepancies relative to the planned margins. Such discrepancies should be considered when determining surgical margins. Therefore, a thorough evaluation of the preoperative imaging and surgical planning is still required, even if 3DP-PSRGs are to be used.Cite this article: Bone Joint J 2023;105-B(2):190-197.

CT-Guided RFA for Management of Surgical Relapses of Giant Cell Tumour of Bone.

OBJECTIVES: This retrospective study describes a pilot experience in CT-guided RadioFrequency Ablation (RFA) treatment of 5 Giant Cell Tumour of the bone (GCT) recurrences after surgery. METHODS: After biopsy to confirm the diagnosis of GCT recurrences, all patients were treated with RFA in a single session. A close follow-up was scheduled with contrast-enhanced MRI starting 1 months after treatment. RESULTS: Five lesions were treated in 5 patients. The length of the observation period was between 4 and 100 months. One lesion relapsed 4 months after the RFA treatment, and the patient underwent a second surgical treatment which included the en-block resection and prosthetic implant. No complications were recorded. CONCLUSIONS: The management of GCT relapses with RFA could be an interesting and innovative field. However, the results of this limited series need to be confirmed by further investigations of larger patient cohorts.

Exostosis of Ulna With Developmental Deformity of the Left Forearm: A Rare Case.

This case report presents a rare occurrence of exostosis of the ulna associated with a developmental deformity of the left forearm in a 15-year-old female. The patient reported a history of trauma resulting in a supracondylar humerus fracture managed conservatively eight years prior. The patient presented with a two-year history of pain and swelling over the left forearm. Clinical examination revealed a firm, non-tender, immobile swelling closely associated with the ulna, accompanied by a 20-degree cubitus varus deformity and forearm shortening. Radiographs and computed tomography scans confirmed the presence of a solitary external bony protuberance over the ulna shaft, communicating with the medullary cavity. A preliminary diagnosis of osteochondroma was established based on clinical and imaging findings. The patient underwent extraperiosteal en bloc resection of the lesion under supraclavicular nerve block anesthesia. A histopathological examination confirmed the diagnosis. Postoperative physiotherapy was initiated, and at the one-month follow-up, the patient reported being pain-free. This case highlights the rarity of exostosis of the ulna with associated developmental deformity, emphasizing the importance of a comprehensive diagnostic approach. Early surgical intervention resulted in a successful outcome, underscoring the significance of timely management in improving patient outcomes and quality of life.

A Retrospective Cohort Analysis of Limb Salvage Surgery Using Mega Prosthesis in Bone Tumours at a Tertiary Care Centre in Eastern India.

Background The method known as "limb salvage surgery" (LSS) aids in the removal of extremity tumours, and reconstruction is completed with satisfactory oncologic, functional, and cosmetic outcomes. Oncologic clearance is given first priority, followed by functional outcomes. Worldwide, the trend has already shifted away from amputations and toward limb salvage surgery for eligible patients due to efficient chemotherapy regimens, improved imaging techniques, precise administration of enhanced radiation, better reconstructive choices, and developments in bio-engineering. The purpose of the present study was to determine the clinicopathological characteristics, surgical techniques, functional outcome, and prognostic factors of limb salvage surgery performed using mega prosthesis in primary malignant or benign resectable tumours. Methods Our retrospective cohort study was carried out over a period of two years and included 28 patients who received care for bone tumours. The data gathered comprised the demographic profile, clinical characteristics, histological characteristics, treatments given, functional results, and survival. LSS was performed on all patients by orthopaedics oncologists trained in the surgical oncology department. Following surgery, during the first two years, patients were examined at every three-month interval, then every six months until the fifth year, and then once a year after that. The Kaplan-Meier method was utilized to determine the median follow-up and recurrence-free survival (RFS). Results In our study, the mean age of study subjects was 30.0±10.9 years. Almost all of the subjects included in the study had lower limb bone tumours (96.4%). The most common site for the tumour was the distal femur (57.1%) followed by the proximal femur (32.2%). The most common type of benign tumour was giant cell tumour (GCT) (53.6%), including recurrences of giant cell tumour (GCT), and among malignant tumours, osteosarcoma was the most common (25.0%). The mean surgical resection of bone in limb salvage surgery was 125.2±24.2 mm. The most common post-operative complication was leg length discrepancy (LLD) among 25.0% of subjects, which was managed by shoe raise. The overall mean musculoskeletal tumour society (MTSS) score after LSS was 25.0±4.3. Using the Kaplan-Meier method analysis, we found that relapse-free survival was 83.7% among enrolled subjects at a median follow-up period of 80 months. Conclusion It can be difficult to surgically treat patients who have malignant bone tumours. In limb-sparing surgery for bone tumours, the modular segmental-replacement system prosthesis that we preferred produced satisfactory results in terms of tumour control and limb function. To get good long-term results, the case selection must be appropriate.

Radiodynamic therapy with acridine orange local administration as a new treatment option for primary and secondary bone tumours.

AIMS: Acridine orange (AO) demonstrates several biological activities. When exposed to low doses of X-ray radiation, AO increases the production of reactive radicals (radiodynamic therapy (AO-RDT)). We elucidated the efficacy of AO-RDT in breast and prostate cancer cell lines, which are likely to develop bone metastases. METHODS: We used the mouse osteosarcoma cell line LM8, the human breast cancer cell line MDA-MB-231, and the human prostate cancer cell line PC-3. Cultured cells were exposed to AO and radiation at various concentrations followed by various doses of irradiation. The cell viability was then measured. In vivo, each cell was inoculated subcutaneously into the backs of mice. In the AO-RDT group, AO (1.0 µg) was locally administered subcutaneously around the tumour followed by 5 Gy of irradiation. In the radiation group, 5 Gy of irradiation alone was administered after macroscopic tumour formation. The mice were killed on the 14th day after treatment. The change in tumour volume by AO-RDT was primarily evaluated. RESULTS: The viability of LM8, MDA-MB-231, and PC-3 cells strongly decreased at AO concentration of 1.0 µg/ml and a radiation dose of 5 Gy. In xenograft mouse model, the AO-RDT also showed a strong cytocidal effect on tumour at the backside in osteosarcoma, breast cancer, and prostate cancer. AO-RDT treatment was more effective for tumour control than radiotherapy in breast cancer. CONCLUSION: AO-RDT was effective in preventing the proliferation of osteosarcoma, breast cancer, and prostate cancer cell lines in vitro. The reduction in tumour volume by AO-RDT was also confirmed in vivo.Cite this article: Bone Joint Res 2022;11(10):685-692.

Effects of Resveratrol, Curcumin and Quercetin Supplementation on Bone Metabolism-A Systematic Review.

Phenolic compounds are natural phytochemicals that have recently reported numerous health benefits. Resveratrol, curcumin, and quercetin have recently received the most attention among these molecules due to their documented antioxidant effects. The review aims to investigate the effects of these molecules on bone metabolism and their role in several diseases such as osteopenia and osteoporosis, bone tumours, and periodontitis. The PubMed/Medline, Web of Science, Google Scholar, Scopus, Cochrane Library, and Embase electronic databases were searched for papers in line with the study topic. According to an English language restriction, the screening period was from January 2012 to 3 July 2022, with the following Boolean keywords: ("resveratrol" AND "bone"); ("curcumin" AND "bone"); ("quercetin" AND "bone"). A total of 36 papers were identified as relevant to the purpose of our investigation. The studies reported the positive effects of the investigated phenolic compounds on bone metabolism and their potential application as adjuvant treatments for osteoporosis, bone tumours, and periodontitis. Furthermore, their use on the titanium surfaces of orthopaedic prostheses could represent a possible application to improve the osteogenic processes and osseointegration. According to the study findings, resveratrol, curcumin, and quercetin are reported to have a wide variety of beneficial effects as supplement therapies. The investigated phenolic compounds seem to positively mediate bone metabolism and osteoclast-related pathologies.

Endoprosthetic replacement of the proximal tibia for oncological conditions.

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

Intercalary reconstruction of long bones by massive allograft: Comparison of construct stability ensured by three different host-graft junctions and two types of fixations in a synthetic femur model.

An intercalary segmental allograft is an option for limb salvage in bone tumours. Stable and congruent intercalary reconstructions are a prerequisite for achieving host-graft union. However, a too rigid fixation could increase the risk of late complications correlated with negative bone remodelling. This study compared the reconstruction stiffness achieved by three different host-graft junctions, namely, end-to-end, modified step-cut, and taper. A low-stiffness bone plate was used as the fixation method, except for the taper junction where a low-stiffness intramedullary nail was also used to investigate the effects of different types of fixation on construct stiffness. Composite femora were tested under four loading conditions to determine coronal and sagittal bending stiffness, as well as torsional stiffness in opposite directions. Stiffness values were expressed as a percentage of intact host bone stiffness (%IBS). While a reduction of coronal bending stiffness was found with taper junctions (76%IBS) compared with the high values ensured by end-to-end (96%IBS) and modified step-cut junctions (92%IBS), taper junctions significantly increased stiffness under sagittal bending and torsion in intra- and extra-direction: end-to-end 29%IBS, 7%IBS, 7%IBS, modified step-cut 38%IBS, 20%IBS, 21%IBS, and taper junction 52%IBS, 55%IBS, 56%IBS, respectively. Construct stiffness with taper junctions was decreased by 11-41%IBS by replacing the bone plate with an intramedullary nail. Taper junctions can be an alternative to achieve intercalary reconstructions with more homogeneous and, in three out of four loading conditions, significantly higher construct stability without increasing bone plate stiffness. The risk of instability under high torsional loads increases when taper junctions are associated with a low-stiffness intramedullary nail.

Paediatric Strategy Forum for medicinal product development of multi-targeted kinase inhibitors in bone sarcomas: ACCELERATE in collaboration with the European Medicines Agency with participation of the Food and Drug Administration.

The eighth Paediatric Strategy Forum focused on multi-targeted kinase inhibitors (mTKIs) in osteosarcoma and Ewing sarcoma. The development of curative, innovative products in these tumours is a high priority and addresses unmet needs in children, adolescents and adults. Despite clinical and investigational use of mTKIs, efficacy in patients with bone tumours has not been definitively demonstrated. Randomised studies, currently being planned or in progress, in front-line and relapse settings will inform the further development of this class of product. It is crucial that these are rapidly initiated to generate robust data to support international collaborative efforts. The experience to date has generally indicated that the safety profile of mTKIs as monotherapy, and in combination with chemotherapy or other targeted therapy, is consistent with that of adults and that toxicity is manageable. Increasing understanding of relevant predictive biomarkers and tumour biology is absolutely critical to further develop this class of products. Biospecimen samples for correlative studies and biomarker development should be shared, and a joint academic-industry consortium created. This would result in an integrated collection of serial tumour tissues and a systematic retrospective and prospective analyses of these samples to ensure robust assessment of biologic effect of mTKIs. To support access for children to benefit from these novel therapies, clinical trials should be designed with sufficient scientific rationale to support regulatory and payer requirements. To achieve this, early dialogue between academia, industry, regulators, and patient advocates is essential. Evaluating feasibility of combination strategies and then undertaking a randomised trial in the same protocol accelerates drug development. Where possible, clinical trials and development should include children, adolescents, and adults less than 40 years. To respond to emerging science, in approximately 12 months, a multi-stakeholder group will meet and review available data to determine future directions and priorities.

Impact of racial disparities and insurance status in patients with bone sarcomas in the USA : a population-based cohort study.

AIMS: Socioeconomic and racial disparities have been recognized as impacting the care of patients with cancer, however there are a lack of data examining the impact of these disparities on patients with bone sarcoma. The purpose of this study was to examine socioeconomic and racial disparities that impact the oncological outcomes of patients with bone sarcoma. METHODS: We reviewed 4,739 patients diagnosed with primary bone sarcomas from the Surveillance, Epidemiology and End Results (SEER) registry between 2007 and 2015. We examined the impact of race and insurance status associated with the presence of metastatic disease at diagnosis, treatment outcome, and overall survival (OS). RESULTS: Patients with Medicaid (odds ratio (OR) 1.41; 95% confidence interval (CI) 1.15 to 1.72) and uninsured patients (OR 1.90; 95% CI 1.26 to 2.86) had higher risks of metastatic disease at diagnosis compared to patients with health insurance. Compared to White patients, Black (OR 0.63, 95% CI 0.47 to 0.85) and Asian/Pacific Islander (OR 0.65, 95% CI 0.46 to 0.91) were less likely to undergo surgery. In addition, Black patients were less likely to receive chemotherapy (OR 0.67, 95% CI 0.49 to 0.91) compared to White patients. In patients with chondrosarcoma, those with Medicaid had worse OS compared to patients with insurance (hazard ratio (HR) 1.65, 95% CI 1.06 to 2.56). CONCLUSION: In patients with a bone sarcoma, the cancer stage at diagnosis varied based on insurance status, and racial disparities were identified in treatment. Further studies are needed to identify modifiable factors which can mitigate socioeconomic and racial disparities found in patients with bone sarcomas. Cite this article: Bone Joint Res 2022;11(5):278-291.

CIRSE Standards of Practice on Thermal Ablation of Bone Tumours.

BACKGROUND: Percutaneous thermal ablation is an effective, minimally invasive means of treating a variety of focal benign and malignant osseous lesions. To determine the role of ablation in individual cases, multidisciplinary team (MDT) discussion is required to assess the suitability and feasibility of a thermal ablative approach, to select the most appropriate technique and to set the goals of treatment i.e. curative or palliative. PURPOSE: This document will presume the indication for treatment is clear and approved by the MDT and will define the standards required for the performance of each modality. CIRSE Standards of Practice documents are not intended to impose a standard of clinical patient care, but recommend a reasonable approach to, and best practices for, the performance of thermal ablation of bone tumours. METHODS: The writing group was established by the CIRSE Standards of Practice Committee and consisted of five clinicians with internationally recognised expertise in thermal ablation of bone tumours. The writing group reviewed the existing literature on thermal ablation of bone tumours, performing a pragmatic evidence search using PubMed to search for publications in English and relating to human subjects from 2009 to 2019. Selected studies published in 2020 and 2021 during the course of writing these standards were subsequently included. The final recommendations were formulated through consensus. RESULTS: Recommendations were produced for the performance of thermal ablation of bone tumours taking into account the biologic behaviour of the tumour and the therapeutic intent of the procedure. Recommendations are provided based on lesion characteristics and thermal modality, for the use of tissue monitoring and protection, and for the appropriately timed application of adjunctive procedures such as osseus consolidation and transarterial embolisation. RESULTS: Percutaneous thermal ablation has an established role in the successful management of bone lesions, with both curative and palliative intent. This Standards of Practice document provides up-to-date recommendations for the safe performance of thermal ablation of bone tumours.