Objectives. In this cross sectional study, the impact and the efficacy of a surveillance programme for sarcomas of the extremities was analysed. Methods. All patients who had treatment with curative intent for a high-grade sarcoma and were diagnosed before 2014 were included and followed for a minimum of two years. Results. Of the 909 patients who had a review appointment in 2014, 131 were under review for a high-grade sarcoma of the extremities following treatment with curative intent. Of these patients, three patients died of disease, two patients died of other causes, 12 are alive, with disease, and 114 have no evidence of disease. The surveillance programme accounts for 14% of all review appointments. Four of five patients (80%) who developed local recurrence identified the recurrence themselves. Chest radiographs are adequate in identifying metastatic disease and 11 (73%) of metastases were diagnosed during a routine follow up visit. However, the chance of cure is small and only two patients were referred for a metastatectomy. Of these only one survived for more than two years. The mean time for developing metastatic disease and local recurrence was 2.0 and 3.9 years respectively. Once identified, the mean time to death was 2.1 years for patients with metastatic disease. Conclusions. Surveillance of sarcoma patients makes up a substantial amount of the workload of a sarcoma unit. The chance of cure following identification of local recurrence or metastatic disease, however, is small. Alternative methods of surveillance that allow better evaluation of the patient’s needs are recommended. Cite this article: P. Cool, G. Cribb. The impact and efficacy of surveillance in patients with sarcoma of the extremities. Bone Joint Res 2017;6:224–230. DOI: 10.1302/2046-3758.64.BJR-2016-0253.R1

The December 2014 Oncology Roundup. 360 . looks at: metaphyseal and diaphyseal osteosarcoma subtly different beasts; sports and endoprosthetic reconstruction of the knee; is curettage without tissue diagnosis sensible in cartilaginous tumours?; autoclaved autograft in bone tumour reconstruction; vascularised graft a step too far in bone defects?; interdigitated neoadjuvant chemoradiotherapy in high-grade sarcoma; predicting life expectancy in patients with painful metastasis; and osteolytic lesions of the hands and feet

Introduction and Objectives: This study analyses the results of our experience in reconstruction of high-grade sarcomas of the proximal humerus. Materials and Methods: A total of 37 patients were treated from March 1983 to December 2001. Average age was 24 years (19–38), with 21 males and 16 females, all presenting with high-grade sarcomas of the proximal humerus. The primary tumour was osteosarcoma in 49% (n=18) of cases, chondrosarcoma in 22% (n=8), Ewing’s sarcoma in 13% (n=5), parosteal sarcoma in 8% (n=3), malignant fibrous histiocytoma in 5% (n=2), and adamantinoma in 3% (n=1). All cases were classified as Type 1 Malawer resections (intraarticular resections of the proximal humerus). Results: During this period, scapulohumeral or scapulothoracic disarticulation was performed in 32% of cases (12 patients). In the remaining 68% (25 cases), limb salvage surgery was performed (relative limb salvage rate: 67.5%). In terms of reconstruction type, 6 cases received megaprotheses, and the remaining 19 cases received osteoarticular allografts of the humerus. Four of the 25 patients had reconstructions with sufficient follow-up time to be considered survivors without recurrence. Results were “acceptable” based on the Enneking-MTS functional scale (overall movement arcs in all directions between 60–120° with tolerable subluxations.) From a subjective point of view, all the patients obtained good results in terms of physical health and mobility of distal joints. No analysis was done of survival due to the varied nature of the neoplasias and adjuvant therapies. The main complication was fracture of the osteoarticular allograft, which occurred in 4 of 19 cases (21%). Subluxation was practically constant throughout our series but was of relatively minor clinical significance. Discussion and Conclusions: The results obtained in this study in terms of local and functional control are similar to those reported in the literature. In spite of the poor functional results for the shoulder joint, the functional results for distal joints and the cosmetic appearance of the extremity are satisfactory. However, the high rate of allograft fractures forces us to reconsider our reconstruction technique, placing priority on a mixed reconstruction using both allograft and mega-prosthesis

EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.) is the first prospective multicenter international study for patients 41–65 year old with high-grade bone sarcoma. Patients with HG Osteosarcoma (OS), HG sarcoma NOS (S), Fibrosarcoma, MFH, Leiomyosarcoma, Dedifferentiated Chondrosarcoma (DCh) were included. Chemotherapy: Combinations of cisplatin/doxorubicin (CDP 100mg/m2/ADM 60mg/m2), ifosfamide/CDP(IFO 6g/m2/CDP 100mg/m2) and IFO/ADM (IFO 6g/m2/ADM 60mg/m2) were repeated three times (9 cycles). Surgery was planned after 3 cycles. Methotrexate (8g/m2) was postoperatively added in poor responders. Immediate surgery was allowed and 9 cycles with CDP, ADM, IFO were postoperatively given. At December 2007, 140 patients were registered (median age 51 years). OS (51%), S (16%), and DCh (11%) were the more frequent histotypes. Synchronous metastases in 30 (21%) patients, central location of tumor in 45(32%).Surgical complete remission (SCR) was achieved in 84% of patients, (localized 91%, meta-static 37%) without difference among the histology groups. One surgical-related and one chemotherapy-related death were reported. Grade4 WBC and PLT incidence was 55% and 17%.Renal toxicity and peripheral neurotoxicity were reported in 16% and 20% of patients. With a median follow-up of 25 months (4–68) 3 year OS was 58% (95%CI 48–68%) [7% (95%CI 0–19%) without SCR]. In patients with SCR, 3-year OS and EFS were 46% (95%CI 9–83%) and 0% in case of synchronous metastases and 69% (95%CI58–80%) and 45% (95%CI33–57%) for localized patients; 50% (95%CI 29–71%) and 40% (95%CI 20–59%) for patients with central tumor, 73% (95%CI61–85%) and 44% (95%CI31–57%) for those with extremity tumor; 68% (95%CI 52–83%) and 46% (95%CI 32–54%) for OS, 64% (95%CI 42–85%) and 48% (95%CI 25–71%) for S, 48% (95%CI 13–82%) and 27% (95%CI 1–54%) for DCh. The protocol is feasible, but the chemotherapy-related toxicity is remarkable. Surgical complete remission is the main factor influencing survival. Central location and synchronous metastases are negative prognostic factors, but 50% 3-year OS can be achieved with aggressive local and systemic treatment. Osteosarcoma and high-grade sarcoma NOS benefit from chemotherapy more than patients with dedifferentiated chondrosarcoma

Introduction: Limb salvage surgery has all but replaced amputation as the treatment of choice for sarcomas of the extremities. This dramatic change came about as the result of two important developments: effective chemotherapy and precision imaging techniques.In high-grade sarcomas the most significant predictors of survival are the location of the primary lesion, local control of the tumor, and the degree of necrosis in the primary tumor after intravenous neoadjuvant chemotherapy (histologic response). Aim : To detect the response to preoperative chemotherapy and correlate with the biological characteristic of osteosarcoma. Materials and method:19 Patients wih primary osteo-sarcoma were studied (follow up 9 months to 7 years). Response to preoperative chemotherapy is made histologically according to the HUVOS staging system..Combination chemotherapy was used based on the Rosen T-10 protocol (high dose methotrexate) or the platine and adriamycine protocol. Conclusions :The best response to preoperative chemotherapy was found in osteoblastic osteosarcomas (12% grade IV, 33% grade III, 33% grade II and 22% grade I tumor necrosis).Chondroplastic osteosarcomas showed less sensitivity to chemotherapy (o% grade IV, 40 % grade III, 20% grade II and 40% grade I tumor necrosis) and paraosteal and periosteal osteosarcomas were resistant to preoperarive chemotherapy

Competence of the extensor mechanism is the major determinant of functional outcome after resection of the proximal tibia and tumor prosthesis implantation. Restoration of a compromised active extension of the knee and an extension lag still remains a difficult challenge. Various techniques have been proposed in the past twenty years including direct attachment of the patellar ligament to the prosthesis, transposition of the medial gastrocnemius muscle possibly associated with other muscle flaps, transposition of the fibula and combination of these techniques. Transposition of the fibula was first reported by Kotz in 1983 but not sufficiently described, so that surgeons who want to plane and manage such a procedure can have some difficulties. We present our technique of fibula transposition and report the functional results about seven patients treated for high-grade sarcomas of the proximal tibia. Fibula transposition is carried out only if the entire fibula and its soft-tissues can be preserved. Resection of the tumor and reconstruction is carried out using the same anteromedial approach. After implantation of the prosthesis, the fibula and its muscles are mobilized anteriorly in a ‘baionnette’ shape obtained by performing a two-level osteotomy. The peroneal nerve and the anterior tibial vessels are previously identified and released to prevent tension on these structures during transposition. Care must be taken to preserve as much as possible the muscular insertions on the fibula so that probability of bone fusion increases. The biceps tendon and the lateral collateral ligament inserted in the fibular head are sutured to the patellar ligament. The knee is immobilized in a knee-ankle orthosis for 6 weeks. We have performed this technique in seven cases. A medial gastrocnemius muscle flap was associated in 3 cases to cover the prosthesis. Fusion was achieved in all cases. Full active extension was obtained in all cases with an extensor strength rated 5/5. All patients were ambulatory without external support at the last follow up

Paediatric bone sarcomas around the knee are often amenable to either endoprosthetic reconstruction or rotationplasty. Cosmesis and durability dramatically distinguish these two options, although patient-reported functional satisfaction has been similar among survivors. However, the impact on oncological and surgical outcomes for these approaches has not been directly compared. We retrospectively reviewed all wide resections for bone sarcoma of the distal femur or proximal tibia that were reconstructed either with an endoprosthesis or by rotationplasty at our institution between June 2004 and December 2014 with a minimum two year follow-up. Pertinent demographic information, surgical and oncological outcomes were reviewed. Survival analysis was performed using the Kaplan-Meier method with statistical significance set at p<0.05. Thirty eight patients with primary sarcomas around the knee underwent wide resection and either endoprosthetic reconstruction (n=19) or rotationplasty (n=19). Groups were comparable in terms of demographic parameters and systemic tumour burden at presentation. We found that selection of endoprosthetic reconstruction versus rotationplasty did not impact overall survival for the entire patient cohort but was significant in subgroup analysis. Two-year overall survival was 86.7% and 85.6% in the endoprosthesis and rotationplasty groups, respectively (p=0.33). When only patients with greater than 90% chemotherapy-induced necrosis were considered, overall survival was significantly better in the rotationplasty versus endoprosthesis groups (100% vs. 72.9% at two years, p=0.013). Similarly, while event-free survival was not affected by reconstruction method (60.2% vs. 73.3% at two years for endoprosthesis vs rotationplasty, p=0.27), there was a trend towards lower local recurrence in rotationplasty patients (p=0.07). When surgical outcomes were considered, a higher complication rate was seen in patients that received an endoprosthesis compared to those who underwent rotationplasty. Including all reasons for re-operation, 78.9% (n=15) of the endoprosthesis patients required a minimum of one additional surgery compared with only 26.3% (n=5) among rotationplasty patients (p=0.003). The most common reasons for re-operation in endoprosthesis patients were wound breakdown/infection (n=6), limb length discrepancy (n=6) and periprosthetic fracture (n=2). Excluding limb length equalisation procedures, the average time to re-operation in this patient population was 5.6 months (range 1 week to 23 months). Similarly, the most common reason for a secondary procedure in rotationplasty patients was wound breakdown/infection, although only two patients experienced this complication. Average time to re-operation in this group was 23.8 months (range 5 to 49 months). Endoprosthetic reconstruction and rotationplasty are both viable limb-salvage options following wide resection of high-grade bony sarcomas located around the knee in the paediatric population. Endoprosthetic reconstruction is associated with a higher complication rate and may negatively impact local recurrence. Study of a larger number of patients is needed to determine whether the reconstructive choice affects survival

Purpose: The purpose of this work was to analyse follow-up and prognostic factors in a series of patients treated for soft tissue tumours as a function of the type of facility providing initial care: a supra-regional referral centre (Royal Orthopaedic Hospital, Birmingham), and 38 regional hospitals in the referral area. Material and methods: This series included 260 patients (111 women and 149 men) treated between 1994 and 1996. Mean age at diagnosis was 61 years. Primary care was given to 96 patients (37%) in the referral centre and 164 (63%) in other centres. Minimum follow-up was five years. The risk of local recurrence and survival prognosis were studied by risk factor: grade, localisation (supra versus infra aponeurotic), tumour size, quality of resection margins. Results: High-grade tumours were found in 73% of patients with a supra-aponeurotic localisation in 59%. Mean tumour size was 8.6 cm. Tumours in patients treated in the referral centre were larger (10.3 cm versus 7.5 cm) (p< 0.05). Frequency of local recurrence was 20% for the referral centre versus 37% for the other centres. Overall five-year survival rate was 58% and was correlated with grade, tumour size, and localisation (p< à.05). Overall survival of patients given primary care in the referral centre was not statistically different from those treated in the other centres, but for high-grade tumours (UICC grade III), five-year survival was 41% for the referral centre and 14% for the other centres (p< 0.05). Discussion: Soft tissue sarcomas are rare tumours. For high-grade sarcomas, the rate of recurrence after treatment and the survival rate were better for patients given primary care in the referral centre. The question of centralising patients with this type of disease in referral centres is raised

Introduction and Objectives: Extraskeletal osseous sarcomas are very rare high-grade tumours. They include osteosarcoma, chondrosarcoma, and Ewing’s soft tissue sarcoma and their respective variants. We present a retrospective study of 25 cases covering the period from December 2002 to January 2003. Materials and Methods: This study of 25 cases shows that this heterogeneous group of soft tissue sarcomas primarily affects adults (mean age 50.68; range 17 to 70 years). The thigh (36.60%) and the elbow-forearm (18.18%) are the most common locations for these tumours. In these cases, 14 chondrosarcomas, 8 osteosarcomas, and 3 Ewing’s sarcomas were diagnosed. Mean follow-up time was 48.9 months with a range of 16 to 197 months. There were 16 surgeries performed with wide or compartmental margins, while in the 5 remaining cases, amputations were performed, and one case of Ewing’s sarcoma of the pelvis was treated by means of chemotherapy and radiation therapy, as it was considered non-resectable. All cases of osteosarcoma and Ewing’s sarcoma were treated by means of pre- and postoperative chemotherapy and postoperative radiation therapy. Results: The postoperative duration of symptoms ranged from 2 weeks to 6 years with a mean of 6 months. Local recurrence following surgery with wide or compartmental margens occurred in 8 cases, representing 35.45% (3 chondrosarcomas, 4 osteosarcomas, and 1 Ewing’s sarcoma). The total number of cases surviving without recurrence is 14 (63.6%), surviving cases with one or more recurrences is 5 (22.72%), one case is alive with metastasis (4.59%), and 3 sarcomas (2 ulcerated upon admission) had disseminated and the patients died (10.05%). Discussion and Conclusions: All these tumours were high-grade sarcomas. The most common metastatic localizations were lungs, regional ganglia, and skeleton. The recommended treatment is surgery with wide or compartmental margins, if possible in the early stages, in conjunction with chemotherapy and radiation therapy. The worst prognosis corresponds to the osteosarcoma

Purpose: Surgery is required for primary tumours invading the sacroiliac joint. The purpose of the present work was to analyse results obtained with surgical treatment in order to better ascertain indications. Material and methods: Forty patients (24 men and 16 women), mean age 24 years (range 12–56) underwent surgery for high-grade sarcoma (n=30, osteosarcoma 12, Ewing 13, chondrosarcoma five) or more differentiated tumours (n=10, low-grade S chondrosarcoma five, fibro-sarcoma two, others three). Resection was achieved in 37 cases via two approaches and via a lateral approach in three. Resection involved vertical sacrectomy either via the homolateral foramen (n=27) or via the midline (n=10). Reconstruction consisted in stabilising the iliosa-cral assembly generally associated with vertebral osteo-synthesis, an autologous graft in 36 cases, cement in one, and an allograft in three. Functional outcome was assessed with the MSTS (Enneking). Results: There were three infections (all three with extensive lateral approach) and five cases of postoperative lumbosacral trunk palsy. Late complications were three cases of spondylolisthesis and eight nonunions. Twenty patients died (eight local recurrences, ten metastases, one chemotherapy toxicity, one undetermined cause). Sixteen patients achieved complete remission at six years (follow-up 2–16 years) and four patients were lost to follow-up. Functional outcome was very good in eight, good in ten, fair in twelve, and poor in ten. Survival was 40% among patients with malignant tumours (38 patients) but only 20% for those with osteosarcomas. Discussion: Technical improvements (combined approach rather than wide lateral approach and omentum flap) have allowed a reduction of cutaneous and infectious complications. Mechanical complications can be prevented by systematic lumbosacral fusion on the side opposite the resection reconstruction. This provides good functional results despite sacrificing a hemi-sacral plexus if the lumbo-sacral trunk is preserved. Reconstruction after extension of the resection to the acetabulum raises an unresolved problem and yields mediocre results. The quality of the surgical resection is determinant since risk of local recurrence is 8/100 after a contaminated resection edge. Conclusion: Surgical resection of sacroiliac tumours is a source of numerous complications despite real technical improvements. This approach can be proposed if carcinological resection can be reasonably achieved. Local control is very poor in case of large osteo-osteogenic sarcomas

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.

Soft tissue sarcomas (STS) have not demonstrated favourable clinical responses to emerging immunotherapies such as checkpoint inhibitors. Studies in carcinomas and melanoma have demonstrated that tumours lacking T-cell infiltrates are associated with poor responses to immunotherapies. It is postulated that STS lack tumour asscoiated lymphocytes which renders these tumours insensitive to checkpoint inhibitors. Our objective was to develop a novel syngeneic mouse model of STS and characterize the immune phenotype of these tumours. Additionally, we sought to evaluate the therapeutic responses of these sarcomas to checkpoint inhibitors and a Type I interferon agonist. K-ras mutagenesis and p53 deletion was induced using a Lenti-Cre-recombinase injection into the hindlimb of 3 week old C57BL/6 mice. Tumours were harvested and characterized using standard histopathology techniques and whole trascriptome sequencing (RNAseq). Full body necrospy and histopathology was performed to identify metastases. Flow cytometry and immunohistochemistry was used to evaluate tumour immune phenotypes. Tumours were implanted into syngeneic C57BL/6 mice and the therapeutic responses to anti-CTLA4, anti-PD1 and DMXAA (Type I interferon agonist) were performed. Tumour responses were evaluated using bioluminescent imaging and caliper measurements. Soft tissue sarcomas developed in mice within 2–3 months of Lenti-Cre injection with 90% penetrance. Histologic analyses of tumours was consistent with a high-grade myogenic sarcoma characterized by smooth muscle actin, Desmin and Myogenin D positive immunostaining. Using crossplatform normalization protocols, geneexpression signatures of the mouse tumours most closely correlated with human undifferentiated pleomorphic sarcoma (UPS). Collectively, gene expression signatures of this murine sarcoma correlated with all muscle-derived human sarcomas (ERMS, ARMS, Synovial sarcoma, UPS). No lung or other visceral metastases were observed in all mice who developed spontaneous tumours. Immune phenotyping demonstrated a paucity of tumour-infiltrating lymphocytes (TILs, (TAMs). 50% of identified TILs in these murine sarcomas expressed PD-1, yet tumours were not responsive to anti-PD1 therapy or anti-CTLA4 therapy. A single intra tumoural (i.t.) injection of the Type I interferon agonist, DMXAA resulted in 80–90% tumour necrosis 72 hrs post-injection, decreased tumour viability up to 2 weeks post-injection and a marked infiltration of CD8+ T-cells and anitgen presenting dendritic cells and macrophages. Additional longitudinal experiments demonstrate a sustained and progressive anti-tumour effect in 83% (5/6) mice up to 6weeks following a single i.t. injection of DMXAA. All control treated mice (6/6) reached humane endpoint within 14 days. At 3 months post-DMXAA treatment, 4/6 mice were free of disease. We re-injected UPS tumours into these mice and tumours did not grow, suggesting abscopal effects after DMXAA treatment of primary tumours. We have characterized a new orthotopic and syngeneic mouse model of a myogenic soft tissue sarcoma. Like most human STS sub-types, these tumours have an immune inert tumour microenvironment and are not sensitive to checkpoint inhibitors. This model, syngeneic to C56BL/6 mice will enable future opportunities to investigate how various branches of the immune system can be targetted or manipulated to unearth new immunotherapeutic strategies for sarcoma. Using this model we have demonstrated that a single, intra-tumoural injection of a Type I interferon agonist can result in anti-tumour effects, recruit cytotoxic lymphocytes and antigen presenting cells with into the the tumour microenvironment. Abscopal tumour rejection after DMXAA treatement suggest adaptive T-cell responses against UPS are active in this model. Future work is needed to determine if upregulation of Type I inferferon pathways can be used as a therapeutic strategy for sarcoma or as a sensitization strategy for checkpoint inhibitors

Purpose: Use of massive allografts for reconstruction of major bone stock defects remains a controversial issue. We reviewed our experience to compare results with other methods, particularly free vascularised bone transfer reconstructions. Material and method: Between 1983 and 1995, 36 patients (15 men and 21 women) underwent cancerological resection of a long bone shaft for primary malignant tumour. Mean age was 19 years (8–84). The tumour was a high-grade sarcoma in 26 cases, low grade sarcoma in eight and adamantinoma in two. Mean length of resection was 19 cm (14–34). Cryopreserved allografts were used in 24 cases, gamma irradiated allografts in 12. Various osteosynthesis procedures were used, generally combining an axial assembly with a single or dual epiphyseal construct. Localisations were: femur 24, tibia ten and humerus four. All patients were reviewed at a minimum follow-up of five years (range 5–16 years, mean 8 years). Functional outcome was assessed with the Enneking criteria. Bone healing at junctions was considered to be achieved when there was no clinical expression and radiographic images remained unchanged for two years. Results: All immediate complications were infections (one femur four tibias) and required partial or total ablation of the allograft in four cases and amputation in one (tibia). The predominant late complications were late consolidation (n=13) and stress fracture of the allograft (n=6). Six patients died before bone healing and were not retained for analysis. Among the 28 patients retained for analysis (eight excluded: six deaths, one amputation, one total ablation of the allograft), only ten achieved bone healing after one procedure. The other eighteen required on the average four reoperations to achieve consolidation (3–11 procedures for osteosynthesis and new allograft material). All patients had achieved bone healing at last follow-up. Functional outcome was excellent for femurs, good for tibias, and fair for humeri due to the impact on shoulder function. There was no significant difference in consolidation with cryopreserved and irradiated bone material but two irradiated grafts could not be used correctly because they were to friable. Discussion: These more or less satisfactory results must be examined in light of the context. Cancerologicl resection (periosteum + soft tissue), generally combined with adjuvant treatment (chemotherapy for 24 patients and radiotherapy for three), places the patient in conditions highly unfavourable for bone healing. Use of allografts alone, combined with approximate fixation procedures early in our experience, demonstrated the limitations of the technique (only two primary consolidations among 18 patients). However, when the allograft was combined with axial fixation and immediate allograft or allograft after adjuvant treatment, primary consolidation was achieved in 80% of the cases (eight out of ten). All patients who achieved long-term remission conserved a functional limb with relatively preserved bone stock. Conclusion: Despite controversial results, massive allograft reconstructions can provide a useful alternative to fill major bone stock defects of the femur or humerus. For the tibia the risk of infections may require further discussion before determining the best approach. These results should be compared with those in a homogeneous series of patients treated with a vascularised free bone transfer, but to our knowledge no such series is available in the literature

Chondrosarcoma is the second most common surgically treated primary bone sarcoma. Despite a large number of scientific papers in the literature, there is still significant controversy about diagnostics, treatment of the primary tumour, subtypes, and complications. Therefore, consensus on its day-to-day treatment decisions is needed. In January 2024, the Birmingham Orthopaedic Oncology Meeting (BOOM) attempted to gain global consensus from 300 delegates from over 50 countries. The meeting focused on these critical areas and aimed to generate consensus statements based on evidence amalgamation and expert opinion from diverse geographical regions. In parallel, periprosthetic joint infection (PJI) in oncological reconstructions poses unique challenges due to factors such as adjuvant treatments, large exposures, and the complexity of surgery. The meeting debated two-stage revisions, antibiotic prophylaxis, managing acute PJI in patients undergoing chemotherapy, and defining the best strategies for wound management and allograft reconstruction. The objectives of the meeting extended beyond resolving immediate controversies. It sought to foster global collaboration among specialists attending the meeting, and to encourage future research projects to address unsolved dilemmas. By highlighting areas of disagreement and promoting collaborative research endeavours, this initiative aims to enhance treatment standards and potentially improve outcomes for patients globally. This paper sets out some of the controversies and questions that were debated in the meeting.

Cite this article: Bone Joint J 2024;106-B(5):425–429.

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.

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.

Besides conventional chondrosarcoma, several rare chondrosarcoma subtypes are described, comprising about 15% of all chondrosarcomas. Clear cell chondrosarcoma (CCS) is a low-grade malignant tumour, often recurring after curettage, and showing overall survival of about 85%. Mesenchymal chondrosarcoma (MCS) is a highly malignant tumour occurring in bone and soft tissue of relatively young patients. The tumour shows differentiated cartilage mixed with undifferentiated small round cells. It often metastasises and shows a 5-year overall survival of 55%. Dedifferentiated chondrosarcoma (DDCS) is a tumour containing a high-grade non-cartilaginous sarcoma (DD), and a usually low-grade malignant cartilage-forming tumour (WD). The prognosis is poor. The lack of efficacious treatment of these rare tumours emphasises the need to learn more about their characteristics and to unravel potential targets for therapy. We constructed tissue microarrays (TMAs) with 2mm cores of 45 DDCS (WD and DD), 24 CCS, and 25 MCS, in triplicate. Using immunohistochemistry, we investigated protein expression of estrogen-signaling molecules, growth plate-signaling molecules, and other molecules which might be potential targets for therapy. In addition, we gathered genomic information using Agilent 44K oligo arrays. 30% of the WD components were positive for Cox-2. Almost all others were negative. For Bcl2, 88% of the small cells and 32% of the cartilage in MCS were positive. In CCS, WD, and DD 48%, 4%, and 12% were positive, respectively. We demonstrated the presence of ESR1 and aromatase protein in the majority of tumours in all subtypes. Using array CGH, we observed similar aberrations in the two components of DDCS, with additional aberrations in the DD. Celecoxib treatment is not recommended, as most of the tumours are negative for Cox-2. However, the presence of ESR1 and aromatase support a possible effect of anti-estrogen treatment in all subtypes, and application of Bcl2 inhibitors might chemosensitise MCS

Introduction: Telomerase is a ribonucleoprotein that adds TTA GGG nucleotide repeated into the ends of eukaryotic chromosomes to maintain their integrity. Most of the normal somatic cells do not express telomerase while telomerase is expressed in the vast majority of malignant tumor cells. Contradictory and limited data have been reported concerning the telomerase expression in soft-tissue sarcomas. The current study evaluates telomerase expression in a single histologic type of a high-grade soft-tissue sarcoma. Materials and Methods: A non-radioactive in situ hybridization (ISH) method was used to study the expression of the RNA component of human telomerase in 55 paraffin embedded archives tissue samples of patients who were diagnosed with synovial sarcoma, the diagnosis of which was based on morphologic, immunohistochemical, and cytogenetic characteristics. The intensity and distribution of telomerase RNA was scored by two different investigators. Intensity was graded as weak, moderate, or intensive. These parameters were further correlated to the oncologic status of the patient. Results: The majority of the investigated specimens demonstrated moderate to intensive telomerase RNA intensity with a diffuse distribution throughout the specimen. A positive correlation was found between telomerase intensity and progression of the underlying disease. Conclusions: Results of the current series suggest that upregulating of telomerase expression may play a role in the pathogenesis and biological activity of synovial sarcoma. This upregulation as detected by ISH assay may be a useful prognostic tool in the evaluation of these patients

Purpose: Prosthetic arthroplasty is the most common method of reconstruction of segmental bone defects following resection of bone sarcomas about the knee. The purpose of this study was to determine the survivorship of the reconstructions in short- and long-term follow-up. Methods: A retrospective study was performed on all patients diagnosed with a bone sarcoma between 1984 and 1995 who were treated with a limb-sparing osteoarticular resection and rotating hinge prosthetic knee arthroplasty. Prosthetic survival was calculated with endpoints of analysis based on any event, any prosthesis-related event and aseptic loosening of the prosthesis, which led to prosthetic revision, removal or limb amputation. Results: A total of 154 reconstructions were performed involving the distal femur (n=111) and proximal tibia (n=43). The median resection length was 155 mm (105–250mm) for the distal femur and 117 mm (85–150 mm) for the proximal tibia. All implants were fixed with polymethylmethacrylate cement. Early complications (within one year postoperatively) developed in fewer than 2 % of patients. Aseptic loosening accounted for the majority of events resulting in prosthetic failures (distal femur = 17 [median failure 34 mos]; proximal tibia = 10 [median failure 100 mos]). Polyethylene bushing wear was observed in seven patients (median time to replacement = 156 months). Conclusion: The early outcome of prosthetic arthroplasty was extremely favorable supporting this method of reconstruction following excision of high-grade bone sarcomas about the knee. Long-term survival of these prostheses is suboptimal and can be anticipated to be poor for the proximal tibia. Aseptic loosening continues to be the primary cause of prosthetic failure about the knee

In order to investigate the efficacy of free vascularised fibular graft (VFG) after bone intercalary tumour resection in tibia, we present our results with a minimum follow-up of 2 years. From 1988 to 2001, 47 patients affected by high-grade tibial sarcoma in 31 cases (66%), and low-grade diesease in 16 cases (34%) were treated in our department. Average age was 19 years (range 5–60 years), with a male/female ratio of 1.35. The average length of tibial resection was 15 cm, while the average length of the fibular graft was 19 cm. In 11 cases (21%) VFG was assembled alone, while in 36 cases (79%) a massive bone allograft was associated to the fibula. Three patients developed a deep infection, treated by amputation in two cases and by graft removal and an Ilizarov device in one case. Minor complications occurred in 28 cases (55%) (stress fractures, wound slough, osteosynthesis breakage), all healed by minor surgery or conservative treatment. At an average follow-up of 108 months (range 24 to 185 months), four patients had died of disease and three were lost to follow-up. Regarding the overall results, the combined group of fibula plus massive allograft showed to be more effective than the group of fibula alone in terms of early weight bearing (6 versus 12 months), while VFG showed intrinsic efficacy in achieving early bony fusion at the osteotomy lines and hypertrophy of the graft in both groups. Furthermore, using the combined assembly the articular surface could be spared in all the trans-epiphyseal resections, while VFG alone appeared to be electively indicated for infected or irradiated fields. In conclusion, despite the demanding surgical technique, VFG appears to be a long-lasting and definitive biological reconstruction procedure after intercalary tibial resection