The management of primary malignant bone tumors with metastatic disease at presentation remains a challenge. While surgical resection has been shown to improve overall survival among patients with non-metastatic malignant bone tumors, current evidence regarding the utility of surgery in improving overall survival in metastatic patients remains limited. The 2004–2016 National Cancer Database (NCDB) was queried using International Classification of Diseases 3rd Edition (ICD-O-3) topographical codes to identify patients with primary malignant bone tumors of the extremities (C40.0-C40.3, C40.8 and C40.9) and/or pelvis (C41.4). Patients with malignant bone tumors of the axial skeleton (head/skull, trunk and spinal column) were excluded, as these cases are not routinely encountered and/or managed by orthopaedic oncologists. Histological codes were used to categorize the tumors into the following groups - osteosarcomas, chondrosarcomas, and Ewing sarcomas. Patients who were classified as stage I, II or III, based on American Joint Commission of Cancer (AJCC) guidelines, were excluded. Only patients with metastatic disease at presentation were included in the final study sample. The study sample was divided into two distinct groups – those who underwent surgical resection of the primary tumors vs. those who did not receive any surgery of the primary tumor. Kaplan-Meier survival analysis was used to report unadjusted 5-year overall survival rates between patients who underwent surgical resection of the primary tumor, compared to those who did not. Multi-variate Cox regression analyses were used to assess whether undergoing surgical resection of the primary tumor was associated with improved overall survival, after controlling for differences in baseline demographics, tumor characteristics (grade, location, histological type and tumor size), and treatment patterns (underwent metastatectomy of distal and/or regional sites, positive vs. negative surgical margins, use of radiation therapy and/or chemotherapy). Additional sensitivity analyses, stratified by histologic type for osteosarcomas, chondrosarcomas and Ewing sarcomas, were used to assess prognostic factors for overall survival. A total of 2,288 primary malignant bone tumors (1,121 osteosarcomas, 345 chondrosarcomas, and 822 Ewing sarcomas) with metastatic disease at presentation were included – out of which 1,066 (46.0%) underwent a surgical resection of the primary site. Overall 5-year survival rates, on unadjusted Kaplan-Meier log-rank analysis, were significantly better for individuals who underwent surgical resection vs. those who did not receive any surgery (31.7% vs. 17.3%; p<0.001). After controlling for differences in baseline demographics, tumor characteristics and treatment patterns, undergoing surgical resection of primary site was associated with a reduced overall mortality (HR 0.42 [95% CI 0.36–0.49]; p<0.001). Undergoing metastectomy (HR 0.92 [95% CI 0.81–1.05]; p=0.235) was not associated with a significant improvement in overall survival. On stratified analysis, radiation therapy was associated with improved overall survival for Ewing Sarcoma (HR 0.71 [95% CI 0.57–0.88]; p=0.002), but not for osteosarcoma (HR 1.14 [95% CI 0.91–1.43]; p=0.643) or chondrosarcoma (HR 1.08 [95 % CI 0.78–1.50]; p=0.643). Chemotherapy was associated with improved overall survival for osteosarcoma (HR 0.50 [95% CI 0.39–0.64]; p<0.001) and chondrosarcoma (HR 0.62 [95% CI 0.45–0.85]; p=0.003), but not Ewing sarcoma (HR 0.79 [95% CI 0.46–1.35]; p=0.385). Surgical resection of the primary site significantly improves overall survival for primary malignant bone tumors with metastatic disease at presentation. Physicians should strongly consider surgical resection of the primary tumor, with adjunct systemic and/or radiation therapy (dependent on tumor histology), in patients presenting with metastatic disease at presentation

Introduction and Purposes. Custom made acetabular prosthesis are a valid option for the reconstruction after the resection of pelvic tumors. They should guarantee a stable and reliable reconstruction for the expected survival of the patient. Nevertheless in many cases periacetabular metastatic lesions have been compared to high grade (IIIA-B) Paprosky defects, but treated with low or intermediate longevity implants. Some complex post-traumatic scenarios or total hip arthroplasty (THA) multiple failures need a reconstruction according to oncologic criteria to fill in the huge defect and to obtain an acceptable function. The aim of the study is to compare 3D custom-made implants for tumors and for THA failures. Materials and Methods. Three custom-made implants after tumor resection (group A: 1 chondroblastic osteosarcoma, 1 bifasic synovialsarcoma, 1 high grade chondrosarcoma) were evaluated and compared to 3 acetabular complex reconstructions after non-oncologic bone defect (group B: 3 cases of aseptic loosening after at least 2 revisions). All the implants were case-based designed, 3D printed, and realized with porous or trabecular surfaces on a Titanium base prosthesis. Age range 16–70 ys in oncologic patients and 60–75 ys in non-oncologic patients. The bone defect to be reconstructed after tumor resection was classified according to Enneking zones (1 type 1-2-3 resection, 1 type 2 resection, 1 partial type 2 resection). Non-oncologic cases were comparable in term of remaining bone stock and classified according to Paprosky classification for acetabular defects as 1 type IIIA an 2 type IIIB. Complications, MSTS functional score, necessity of walking-aids were evaluated at minimum follow up of 1 year. Results. In both groups, good functional results were obtained (MSTS score 25/30 in both groups). No cases of aseptic loosening and no infection occured. After 3 months of partial or no weight-bearing on the operated limb, 3 patients were able to walk unaided and 3 walked with one cane or crutch. No limb length discrepancy (major than 2 cm) were observed. Limping was present in oncologic cases due to muscle resection. Overall better results in term of satisfaction and quality of life were obtained in younger (oncologic) patients. Conclusions. Complex THA revision cases can sometimes be considered for a reconstruction with oncologic criteria. Even if indications are limited an acceptable functional results can be obtained. In the past for these cases a Girldestone resection was the only option. A collaboration among orthopaedic oncologist and hip arthroplasty surgeons is advisable in major orthopaedic centers to improve the prosthetic design and the functional results

The orthopaedic surgeon is often consulted to manage pathologic fractures due to metastatic disease, even though he or she may not be an orthopaedic oncologist. A good understanding of the principles of management of metastatic disease is therefore important. The skeleton remains a common site for metastasis, and certain cancers have a predilection for bone, namely, tumors of the breast, prostate, lung, thyroid, and kidney. Myeloma and lymphoma also often involve bone. The proximal femur and pelvis are most commonly affected, so we will focus on those anatomic sites. The patient may present with pain and impending fracture, or with actual fracture. Careful preoperative medical optimization is recommended. If the lesion is solitary, or the primary is unknown, the diagnosis must be made by a full workup and biopsy before definitive treatment is planned. For patients with known metastasis (the most common situation), the options for treatment of pathologic lesions of the proximal femur generally center on internal fixation versus prosthetic replacement. Patients with breast or prostate metastasis can live for several years after pathologic fracture, so constructs must be relatively durable. If fixation is chosen, it must be stable enough to allow full weight bearing, since the overwhelming majority of pathologic fractures will never heal. In general, long constructs are chosen to protect the entire length of the bone. Nails should protect the femoral neck as well, so cephalomedullary devices are typically chosen. Megaprostheses can be useful in situations where bony destruction precludes stable internal fixation. Postoperative radiation is recommended after wound healing. Acetabular involvement typically requires reinforcement rings or cement augmentation with the Harrington technique. Careful multi-disciplinary medical management is recommended to minimise complications

Metastatic bone disease is increasing in association with ever improving medical management of osteophylic malignant conditions. The precise timing of surgical intervention for secondary lesions in long bones can be difficult to determine. This paper aims to validate a classic scoring system. All radiographs were examined twice by 3 orthopaedic oncologists and scored according to the Mirels’ scoring system. The Kappa statistic was used for the purpose of statistical analysis. The results show agreement between observers (κ=0.35–0.61) for overall scores at the 2 time intervals. Inter-observer agreement was also seen with subset analysis of size (κ=0.27–0.60), site (κ=0.77–1.0) and nature of the lesion (κ=0.55–0.81). Similarly, low levels of intra-observer variability were noted for each of the 3 surgeons (κ=0.34, 0.39, 0.78 respectively). These results validate the Mirels’ scoring system across a wide spectrum of malignant pathology. We continue to advocate its use in the management of patients with long bone metastases

Aims

Advances in treatment have extended the life expectancy of patients with metastatic bone disease (MBD). Patients could experience more skeletal-related events (SREs) as a result of this progress. Those who have already experienced a SRE could encounter another local management for a subsequent SRE, which is not part of the treatment for the initial SRE. However, there is a noted gap in research on the rate and characteristics of subsequent SREs requiring further localized treatment, obligating clinicians to extrapolate from experiences with initial SREs when confronting subsequent ones. This study aimed to investigate the proportion of MBD patients developing subsequent SREs requiring local treatment, examine if there are prognostic differences at the initial treatment between those with single versus subsequent SREs, and determine if clinical, oncological, and prognostic features differ between initial and subsequent SRE treatments.

Based on paleopathological findings there is evidence that primary malignant bone und probably soft tissue tumours accompanied mankind from the very beginning. Impressive findings of osteosarcomas have been reported from ancient Peru and medieval Hungary. Astonishingly a report exists on a 3rd century AD amputation of a leg affected by “cancer” and, even more amazing, on the successful reconstruction using a homologous limb transplant. This “miracle” has been attributed to Saints Cosmas and Damian. According to the legenda aurea of Jacobo da Varragine the miraculous treatment of took place in 3rd century Rome. The saints amputated the leg of the Deacon Justinian and successfully transplanted the leg of a black African, who had died some hours ago. According to the legend the deacon was able to walk again and glorify his doctors. This legend inspired artists throughout the centuries as can be seen in a famous 16th century oil painting in Stuttgart’s Landesmuseum Württemberg. The twin saints Cosmas and Damian have been praised before for the first homoplastic limb transplant. The cause for amputation, however, was reported to be a “gangrenous leg” or a “diseased leg”. Looking at the original text of the legenda aurea, a different picture emerges, the cause for surgery being “cancer” of the leg – “…cui cancer unum crus totum còsumpserat”. Also astonishing, at their time and in ours, the saints treated patients without taking any payment. It is not surprising that they were the most renowned of all medically inclined saints and were soon regarded as patron saints of medicine. From today’s medical view, neither resection margins according to Enneking nor a follow-up period were provided by the legenda aurea. It therefore remains elusive whether a local or systemic recurrence occurred. Nevertheless, Saints Cosmas and Damian may well be regarded as Europe’s first orthopaedic oncologists

Introduction and Aims: Limb salvage surgery for the pediatric patient poses unique surgical challenges for the orthopaedic oncologist. In the past, surgeons have advocated amputation or rotationplasty as treatment for the skeletally immature child with a malignant bone tumor that involves a major growth plate and has an expected limb length discrepancy. Method: The Repiphysis is a non-invasively expandable prosthesis. Applications for the knee joint are approved and other joints are pending. The expansion principle involves energy stored in a spring compressed by a locking system. Lengthenings are achieved via exposure to an electromagnetic field that allows controlled release of the spring and can be done without general anesthesia. Numerous small expansions (5–10mm) keep pace as the child grows. Between 1998–2002, 25 prostheses [knee (19), shoulder (five), hip (one)] were implanted in 21 patients. Most were implanted at the time of tumor resection and nine were revisions from previous reconstruction or fusion. Results: Ninety lengthenings were performed with all but two on an outpatient basis. An average of 5mm was obtained per lengthening (range 1–35mm). The average follow-up was 25 months (range 12–48 months) and the latest Musculoskeletal Tumor Society functional scores averaged 87%. Eight revisions were required for mechanical problems or stem loosening. There was one amputation caused by post-operative arterial thrombosis. Conclusion: This unique prosthesis offers many benefits in maintaining limb-length equality in growing patients. We are optimistic that this technology will prove beneficial to patients with malignant bone tumors as well as patients requiring serial limb lengthening or those with spinal deformities

Radiation induced pathologic fractures present a difficult problem for musculoskeletal oncologists. The purpose of this study was to determine the outcomes of management of radiation-induced pathologic fractures in a group of patients who had previously undergone combined management of extremity soft tissue sarcoma. A review of our retrospective database was undertaken. From 1986 to present, thirty-two patients with soft tissue sarcomas were found to have radiation induced pathologic fractures. The records of these patients were reviewed for patient demographics, tumour size and anatomic site, presence of periosteal stripping at time of surgery, radiation dose, time to fracture, fracture treatment and fracture outcome. There were twenty-three females and nine males with a mean age of sixty-three (range thirty-six to eighty-nine) years. Fractures occurred at a mean of forty-five months after resection of the sarcoma (range three to one hundred and fifty months). Anatomic distribution of fractures were : proximal femur(twelve), femoral diaphysis (eight), distal femur (two) tibia (five), acetabulum (two), metatarsal (two) and patella (one). Periosteal stripping was performed in half of the patients. Twenty-three patients had received high dose radiation (6600Gy). Seven fractures were managed conservatively while twenty-five were treated surgically. Only eleven of the thirty-two fractures united. Six patients underwent amputation, three for local recurrence and three for non-union of their fracture. Eight patients ultimately underwent arthroplasty, while seven patients have persistent non-unions. In the proximal femur, only three out of twelve fractures healed while six patients eventually underwent arthroplasty and three continue to have non-unions. Of eight femoral diaphyseal fractures, only one united. Patients who eventually underwent prosthetic replacement had good function and pain relief. Radiation induced pathologic fractures are a difficult clinical problem. In particular patients with fractures in the proximal femur often undergo multiple attempts at fixation before definitive management with resection and endoprosthetic replacement. Fractures of the femoral diaphysis rarely heal despite aggressive surgical management. Primary arthroplasty may be considered in some patients as an alternative to fixation in radiation-induced pathologic fractures of the femur in order to avoid long term morbidity and repeated operations

Introduction and Aims: Orthopaedic oncologists are often consulted regarding problems involving salvage of the distal femur due to bone loss, non-unions, infections. In young patients, extensive bony reconstruction is often necessary; in elderly, low demand patients, replacement with an endoprosthetic device results in decreased surgical time and more rapid mobilisation. Method: Since 1991, 27 patients underwent reconstruction with a custom modular distal femoral replacement and rotating hinged knee joint (DFR). Twenty-two (81%) were revised to a DFR from an existing knee arthroplasty. Diagnoses included fracture, non-union, osteomyelitis, osteolysis or deformity. Average age was 66 (25–85); 83% were female. Most patients had undergone multiple prior surgeries. Patients with a history of infection had undergone aggressive resection and insertion of spacers with prolonged antibiotic administration, however they had no infection at the time of DFR reconstruction. All endoprostheses were cemented. Patients were allowed immediate weight-bearing and rehabilitation similar to patients undergoing TKA. Results: One elderly patient died in the immediate peri-operative period of respiratory failure and one was lost to follow-up after placement in a nursing home. Average follow-up on 25 evaluable patients was 47 months (7–122). Reoperations were for recurrent infection (six) and tibial component loosening (three). Five of the six with infection were treated with synovectomy, antibiotic beads and suppressive oral antibiotics, and all five devices are still in place at an average of 54 months (range, 25–100). One severely diabetic patient had had multiple episodes of sepsis unrelated to the prosthesis which eventually seeded the distal femur and required a hip disarticulation. MSTS functional scores at last follow-up averaged 49% (13–80%) and HSS knee scores averaged 71% (37–90%). Conclusion: DFR is a useful salvage procedure in low demand patients. Initially, six patients were scheduled for transfemoral amputation and three were confined to wheelchairs. Patients other than the hip disarticulation were at minimum household ambulators at last follow-up. In spite of problems with infection, most patients improved in overall function

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

Ilium is the most common site of pelvic Ewing’s sarcoma (ES). Resection of the ilium and iliosacral joint causes pelvic disruption. However, the outcomes of resection and reconstruction are not well described. In this study, we report patients’ outcomes after resection of the ilium and iliosacral ES and reconstruction with a tibial strut allograft.

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

The primary objective of this study was to compare the postoperative infection rate between negative pressure wound therapy (NPWT) and conventional dressings for closed incisions following soft-tissue sarcoma (STS) surgery. Secondary objectives were to compare rates of adverse wound events and functional scores.

Aims

The aim of this study was to assess orthopaedic oncologic patient morbidity resulting from COVID-19 related institutional delays and surgical shutdowns during the first wave of the pandemic in New York, USA.

Objectives

As tumours of bone and soft tissue are rare, multicentre prospective collaboration is essential for meaningful research and evidence-based advances in patient care. The aim of this study was to identify barriers and facilitators encountered in large-scale collaborative research by orthopaedic oncological surgeons involved or interested in prospective multicentre collaboration.

Objectives

We evaluated the accuracy of augmented reality (AR)-based navigation assistance through simulation of bone tumours in a pig femur model.