Search Results (85)

Background: While changes in clinical practice during the COVID-19 pandemic in Japan have been widely studied, data specific to bone and soft tissue tumor care remain limited. Methods: A nationwide web-based survey was conducted among hospitals specializing in musculoskeletal tumors. It assessed the occurrence of COVID-19-related events (patient infections, outbreak clusters, and staff infections), delays in referral and diagnosis, postponement or cancellation of specific treatments, and changes in institutional management strategies. Results: Seventy-eight hospitals (91.7% of all specialized centers) responded. Patient infections, outbreak clusters, and staff infections were reported by 28.2%, 48.7%, and 53.8% of hospitals, respectively. While radiological exams and biopsies were largely maintained, patient referrals decreased significantly. Surgical treatment was more affected than chemotherapy or radiotherapy. Strategy changes included surgery delays or cancellations (48.7%) and prolonged follow-up intervals (20.5%). Among COVID-19-related factors, only direct patient infections were significantly associated with institutional changes in treatment policy. Conclusions: The pandemic substantially disrupted outpatient services and surgical care in musculoskeletal oncology. Patient infection was the main driver of treatment strategy modifications. Full article

Sarcomas are tumors of mesenchymal origin that are generally resistant to systemic therapies and prone to local recurrence despite current multimodal treatment approaches. Focused ultrasound (FUS) is a noninvasive therapeutic technology that may enhance standard treatment strategies for primary solid malignancies. FUS exerts its effects through diverse mechanisms, including high-intensity focused ultrasound (HIFU) thermal ablation, histotripsy, sonodynamic therapy, immunomodulation, and hyperthermia-enhanced drug delivery. In this narrative review, we summarize the mechanisms of focused ultrasound that have been investigated for the treatment of sarcomas and highlight the results of preclinical, veterinary, and clinical studies related to this area. Full article

Cryotherapy could stimulate immune responses and induce abscopal effects. A novel technique was developed for treating spinal bone tumors involving the use of frozen tumor-containing autologous bone grafts for anterior spinal reconstruction following total en-bloc spondylectomy, with the aim of activating cryoimmunity. This study focused on analyzing changes in the T-cell receptor (TCR) repertoire after surgery to evaluate T-cell diversity. Blood samples were collected pre- and post-operatively, with subsequent RNA extraction and immunosequencing. Compared to pre-surgery samples, the diversity and abundance of the Complementarity-Determining Region 3, regions of the TCR α and β chains decreased, suggesting that more selective clones may have emerged and influenced immune responses. Through TCR repertoire analysis, this study demonstrated that transplantation of frozen tumor-containing autologous bone impacted the immune system. This study is expected to provide a foundation for developing treatments that may enhance immune activation. Full article

Osteosarcoma is one of the most common primary bone malignancies, typically occurring around the knee. However, the forearm is a rare site, with tumors in the proximal ulna being extremely uncommon. Primary sarcoma in this location presents a surgical challenge due to the complex anatomy and limited reconstructive options. We report a rare case of a 19-year-old female with non-metastatic, high-grade giant cell-rich osteosarcoma involving the right proximal ulna. To our knowledge, this is only the second reported adult case of this histological subtype in this location. The patient was treated at a specialized oncology center with neoadjuvant and adjuvant chemotherapy, along with wide intra-articular resection for local tumor control. Reconstruction was achieved using a novel, customized 3D-printed articulating cement spacer mold with plate osteosynthesis. Artificial elbow ligamentous reconstruction was performed using FiberTape and FiberWire sutures passed through drill holes, and the triceps tendon was reattached to the cement mold using an endobutton. This cost-effective and personalized surgical approach allowed successful joint reconstruction while maintaining elbow stability and function. Our case highlights a feasible reconstructive option for rare and anatomically challenging osteosarcoma presentations, contributing to the limited literature on proximal ulna giant cell-rich osteosarcoma. Full article

Desmoid tumors are rare, deep-seated myofibroblastic tumors with an unpredictable course, ranging from spontaneous regression to infiltrative growth and locally aggressive behavior, but without metastatic potential. Over the past few decades, advances in understanding their natural history, underlying molecular pathways, and patient care priorities have shifted the treatment paradigm from upfront surgical resection to initial active surveillance, with further treatment dictated by continuous disease progression or associated symptoms. However, there are still specific scenarios where surgery continues to play an important role in locoregional treatment and symptom control. This article will focus on current treatment strategies and surgical indications in adult patients with desmoid tumors, emphasizing patient selection, anatomic site-specific considerations, and surgical technique. Understanding the nuanced role of surgery within the growing treatment landscape is key for individualized patient care in a multidisciplinary setting to optimize quality of life and long-term outcomes. Full article

Diffuse type tenosynovial giant cell tumor (D-TGCT) of the knee presents with stiffness, pain and swelling with surgery being the mainstay treatment. However, the literature lacks data on clinical course of range of motion, pain (ROM), and swelling after open synovectomy for D-TGCT. Therefore, this study aims to evaluate clinical course after open anterior synovectomy. A retrospective analysis was conducted on 214 patients treated for TGCT at our Institutions between 2010 and 2023. 51 patients with anterior knee D-TGCT who underwent open anterior synovectomy were included. Pre- and postoperative assessments included ROM, pain (VAS scale), and reported swelling. The mean knee flexion increased from 100° (SD 14.28) preoperatively to 131.8° (12.64) at 12 months post-surgery. Knee extension remained stable, ranging from 178.4° preoperatively to 179.2° at the final follow-up. Pain decreased from a mean of 5.0 (SD 2.8) preoperatively to 0.5 (SD 0.7) at 12 months. Swelling was initially reported in 90.4% of patients, with 95.74% of them showing improvement at six months, and 100% at 12 months. Open anterior synovectomy effectively improves knee function, pain, swelling in patients with anterior knee D-TGCT, although functional recovery may take up to 6–12 months. Full article

Background: Desmoid fibromatosis (DF) is a rare, locally aggressive soft tissue tumour with unpredictable clinical behaviour. Historically, treatment has involved surgery; however, contemporary guidelines, such as those from the Desmoid Tumour Working Group, advocate active surveillance. This article reviews current perspectives on DF, focusing on epidemiology, pathogenesis, treatment strategies, emerging research directions and cost effectiveness based on our experience at the West of Scotland Musculoskeletal Oncology Service, Glasgow Royal Infirmary (GRI). Methodology: We reviewed 101 patients diagnosed with desmoid fibromatosis between 2010 and 2024. A review of patient records was conducted to gather information on demographics, date of diagnosis, prior treatment, treatment initiation, intervention types, imaging intervals, follow-up duration, recurrence rate for surgery and other intervention, and discharge timelines. All data was systematically organized and analyzed to assess our outcomes. Results: Out of 101 patients with DF in the study, 66% were females. The most common site of primary tumour was lower extremity (39.6%) followed by near equal distribution in upper extremity and trunk. Out of the total cases, 72 (71.2%) were successfully managed with active surveillance involving serial imaging and clinical reviews in accordance with European guidelines. A total of 22 patients (21%) received treatment: 10 underwent surgery alone, 2 had surgery combined with radiotherapy, 8 received only radiotherapy, 1 was treated with hormonal therapy and 1 participated in a trial with Nirogacestat. Of the seven remaining patients, six had unplanned surgery outside followed by active surveillance at GRI. One patient was on alternative treatment modality, homeopathy. The average number of MRI scans per patient was 3.11, with many patients requiring significantly more imaging. MRI surveillance varies significantly in desmoid tumours due to their heterogeneous behaviour. Active or symptomatic tumours often require more frequent scans (every 3–6 months), while stable cases may need only imaging annually or just clinical monitoring. Recurrence was noted in eight patients, all of which were related to prior surgery. The total combined cost of imaging and appointments exceeds £6500 per patient in active surveillance. Conclusions: We conclude that most patients with desmoid fibromatosis in our cohort were effectively treated with active surveillance, consistent with current European guidelines. Surgical management of desmoid fibromatosis in our cohort is historic and has shown a significant recurrence risk. Our study proposes a revised follow-up protocol that significantly reduces costs without compromising on patient care. We suggest a two-year surveillance period for stable disease with patient-initiated return to reduce unnecessary clinic visits, imaging and healthcare costs. Full article

The proximal femur represents the most frequent site in the appendicular skeleton for metastatic bone disease (MBD) to occur, with a high risk for pathologic fracture. While surgical stabilization is typically used to manage fractures, reconstruction approaches are gaining popularity due to improved survival. Previous studies have focused on clinical outcomes, but patient-centered outcomes remain underexplored. This study aims to develop a patient-centered primary outcome for the Proximal FEmur Reconstruction or Internal Fixation fOR Metastases (PERFORM) Randomized Controlled Trial, employing a mixed-methods approach. First, a focus group with advanced cancer patients and caregivers identified relevant outcomes. Next, a discrete choice experiment (DCE) assessed the importance of these outcomes among stakeholders, including surgeons, patients and caregivers. The most important components for the primary outcome were identified: mortality within twelve months, physical function assessed at four months using the PROMIS^® Global Physical Function score, and the number of days at home within twelve months. The DCE further confirmed that survival and physical function were most prioritized. The PERFORM trial’s primary outcome, developed through extensive stakeholder engagement, will guide the evaluation of surgical approaches for MBD of the proximal femur and has the potential to influence patient-centered practice. Full article

Approximately 50–70% of patients with advanced cancer will experience bone metastases. The link between metastatic lesions and pathological bones is especially troubling since more metastases mean a higher chance of painful fractures, which can reduce mobility and often require surgery. Therefore, fracture risk predictions are essential for managing patients with bone metastases. However, the current methods for evaluating the risk of fractures are subjective, with low predictive value. This paper highlights how there being no effective comprehensive models for grouping patients by fracture risk due to skeletal metastases makes it harder to create personalized treatment plans; presents the methods currently used for objective evaluations of the pathological fracture risk in bone metastases; and discusses their pros and cons. Full article

Objective: To systematically evaluate the effectiveness and safety of radiofrequency ablation (RFA) for managing pain caused by spinal metastases. This review aimed to consolidate evidence on RFA’s analgesic efficacy and associated risks to inform clinical practice in palliative cancer care. Methods: A systematic review adhering to PRISMA guidelines was conducted. Databases were searched for studies evaluating RFA for spinal metastases pain. Inclusion criteria specified: randomized or non-randomized studies (prospective/retrospective); ≥3 adult patients; RFA used alone or combined with other treatments; reported pre- and post-RFA pain assessments; English language publication. Data extracted included patient demographics, primary tumor type, lesion location, pain scores (e.g., NRS/VAS), and complications. Pain response was assessed using definitions including the International Consensus Pain Response Endpoints (ICPRE) and definitions for moderate (≥2-point reduction) and high (≥4-point reduction) effectiveness. Results: This review included 33 studies, totaling 1336 patients (53.7% female) and 1787 treated lesions. The majority (85%) of studies reported highly effective pain management (≥4-point pain score reduction). The remaining 15% showed moderate effectiveness (≥2-point reduction). All studies reported achieving at least a partial pain response per ICPRE criteria. Mean pain scores decreased significantly from baseline (7.56/10) within 24–72 h (3.65) and remained low at 4 weeks (2.99), 12 weeks, and 24 weeks (both 2.70). Common primary cancers were lung (27.6%), breast (26.2%), and genitourinary (11.3%). Lesions were primarily in the thoracic (47.9%) and lumbar spine (47.3%). Crucially, no life-threatening (grade IV–V) complications occurred. The overall rate of grade I–III complications was low at 2.11%. Limitations: This systematic review is limited by its study-level nature, preventing detailed subgroup analyses regarding specific metastasis characteristics or the impact of complementary therapies. Conclusions: This systematic review suggests that RFA is a safe and effective treatment for pain control in patients with spinal metastases. It provides both rapid (within 24 h) and durable mid-term (up to 24 weeks) analgesia. The favorable safety profile, with a low complication rate, supports RFA as a valuable complimentary option within the multidisciplinary palliative management of painful spinal secondary tumors. Future randomized-controlled studies may help to further define its role when associated with other treatments. Full article

Desmoid tumors are rare, locally invasive soft-tissue tumors with unpredictable clinical behavior. Imaging plays a crucial role in their diagnosis, measurement of disease burden, and assessment of treatment response. However, desmoid tumors’ unique imaging features present challenges to conventional imaging metrics. The heterogeneous nature of these tumors, with a variable composition (fibrous, myxoid, or cellular), complicates accurate delineation of tumor boundaries and volumetric assessment. Furthermore, desmoid tumors can demonstrate prolonged stability or spontaneous regression, and biologic quiescence is often manifested by collagenization rather than bulk size reduction, making traditional size-based response criteria, such as Response Evaluation Criteria in Solid Tumors (RECIST), suboptimal. To overcome these limitations, advanced imaging techniques offer promising opportunities. Functional and parametric imaging methods, such as diffusion-weighted MRI, dynamic contrast-enhanced MRI, and T2 relaxometry, can provide insights into tumor cellularity and maturation. Radiomics and artificial intelligence approaches may enhance quantitative analysis by extracting and correlating complex imaging features with biological behavior. Moreover, imaging biomarkers could facilitate earlier detection of treatment efficacy or resistance, enabling tailored therapy. By integrating advanced imaging into clinical practice, it may be possible to refine the evaluation of disease burden and treatment response, ultimately improving the management and outcomes of patients with desmoid tumors. Full article

Precision medicine approaches using ex-vivo drug sensitivity testing (DST) have received attention in the cancer research community as a means to improve treatment stratification in populations where multiple treatment attempts are not feasible, or no standard-of-care treatment exists, such as ultra-rare cancers with a significant clinical need for effective treatment options, like osteosarcoma. DST has the potential to supplement existing patient stratification approaches by providing tumor-specific response data to aid in treatment selection at the time of treatment decision. We present the case of a pediatric osteosarcoma patient who was evaluated using DST at the time of standard-of-care treatment to evaluate treatment sensitivity. The DST screen indicated significant treatment sensitivity to anthracyclines and methotrexate, consistent with the first-line standard-of-care therapy (MAP). Clinical follow-up showed treatment sensitivity to standard-of-care MAP treatment and pathology results of 90% necrosis. The present case shows that DST screening is feasible from a technical standpoint, can be performed in a clinically relevant time frame that does not delay treatment start, and provides personalized drug sensitivity information on clinically available agents, and the DST results align with the clinical treatment response. Full article

Bone metastases are a major concern in cancer management since they significantly contribute to morbidity and mortality. Metastatic lesions, commonly arising from breast, prostate, lung, and kidney cancers, affect approximately 25% of cancer patients, leading to severe complications such as pain, fractures, and neurological deficits. This narrative review explores contemporary approaches to bone metastases, emphasizing a multidisciplinary strategy and the evolving concept of oligometastatic disease. Oligometastases, defined by limited metastatic spread (1–5 lesions), offer a potential window for curative treatment through aggressive interventions, including stereotactic ablative radiotherapy and resection surgery. Tumor boards, integrating systemic therapies with local interventions, are crucial to optimize treatment. Despite promising results, gaps remain in defining optimal treatment sequences and refining patient selection criteria. Future research should focus on personalized approaches, leveraging biomarkers and advanced imaging to enhance outcomes and the quality of life in patients with bone metastases. Full article

Pseudomyogenic haemangioendotheliomas (PMH) are exceedingly rare, mostly occurring in soft tissue, with malignant cases even more uncommon. In this report, we present a case of a 28-year-old male initially suspected of having a fibroblastic osteosarcoma of the right femur, which was then correctly diagnosed as a primary pseudomyogenic hemangioendothelioma of the bone with synchronous metastases to other skeletal segments. Molecular analysis through targeted RNA sequencing confirmed the correct diagnosis, revealing a fusion transcript ACTB::FOSB. To our knowledge, this is one of the few reported cases of suffering from multiple pathological fractures. The rapid skeletal progression and the onset of distant metastases in this case is highly unusual considering the typically indolent clinical course commonly reported in the literature for this tumor. Full article

Osteosarcoma is the most common primary malignancy of bone. Previous studies have demonstrated rural-urban disparities in metastatic disease incidence and overall survival in high-grade osteosarcoma patients. However, there is a paucity of literature investigating disease-specific survival (DSS) disparities between rural and urban patients, which is explored herein using the SEER database. Patients with biopsy-proven cases of osteosarcoma were identified from 2000–2021. Statistical analysis was completed using SPSS version 29.0.2 and included chi-squared, Kaplan–Meier and log-rank, and stepwise Cox regressions. Statistical significance was considered at p < 0.05. Kaplan–Meier analysis revealed no significant differences in 5- and 10-year DSS between rural (55.0% and 47.0%) and urban patients (56.0% and 51.0%) (p = 0.107). Multivariable analysis further revealed no significant DSS difference between rural and urban patients (aHR: 1.03; 95% CI: 0.86–1.24; p = 0.757). This study expands upon prior research by investigating DSS between rural and urban osteosarcoma patients and finding no significant differences. While rural living is often associated with worse outcomes, important prognostic factors for osteosarcoma, including metastatic disease at presentation and tumor grade, were not significantly different between rural and urban patients in our study, possibly explaining our DSS-related findings. Factors other than geographical location likely impact outcomes, and future research should examine other ways that rural living may influence cancer care. Full article