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Sarcomas are rare cancers with many subtypes in soft tissues, bone and cartilage. International survival trends in these cancers are not well known. We present 50-year survival trends for soft tissue sarcoma (STS) and bone sarcoma (BS) in Denmark (DK), Finland (FI), Norway (NO) and Sweden (SE). Relative 1-, 5/1 conditional- and 5-year survival data were obtained from the NORDCAN database for years 1971–20. We additionally estimated annual changes in survival rates and determined significant break points. In the last period, 2016–20, 5-year survival in STS was best for NO men (74.6%) and FI women (71.1%). For the rarer BS, survival rates for SE men (72.0%) and DK women (71.1%) were best. Survival in BS was lower than that in STS in 1971–75 and the difference remained in 2016–20 for men, but for women the rates were almost equal. Sex- and country-specific differences in survival in STS were small. The 50-year improvement in 5-year survival in STS was highest in NO men, 34.0 % units and FI women, 30.0 % units. The highest improvements in BS were in SE men 26.2 % units and in FI women 29.2 % units. The steady development in survival over the half century suggests contribution by stepwise improvements in diagnostics, treatment and care. The 10–15% mortality in the first year probably indicates diagnostic delays which could be improved by organizing patient pathways for aggressive rare diseases. Early diagnosis would also reduce metastatic disease and breakthroughs in treatment are a current challenge. •Survival in bone (BS) and soft tissue sarcoma (STS) has increased, as documented.•5-year survival reached 70% in these cancers but male BS remained at over 60%.•Steady survival development suggests contributions by stepwise improvements in care.•Over 10% mortality in the first year is a challenge for survival improvements.•Achieving this would require vigilance and ready care pathways to experts.

ObjectivesThe COVID-19 pandemic is unprecedented as a global crisis over the last century. How do specialist surgeons make decisions about patient care in these unprecedent times?DesignBetween April and May 2020, we conducted an international qualitative study. Sarcoma surgeons from diverse global settings participated in 60 min interviews exploring surgical decision making during COVID-19. Interview data were analysed using an inductive thematic analysis approach.SettingParticipants represented public and private hospitals in 14 countries, in different phases of the first wave of the pandemic: Australia, Argentina, Canada, India, Italy, Japan, Nigeria, Singapore, Spain, South Africa, Switzerland, Turkey, UK and USA.ParticipantsFrom 22 invited sarcoma surgeons, 18 surgeons participated. Participants had an average of 19 years experience as a sarcoma surgeon.Results17/18 participants described a decision they had made about patient care since the start of the pandemic that was unique to them, that is, without precedence. Common to ‘unique’ decisions about patient care was uncertainty about what was going on and what would happen in the future (theme 1: the context of uncertainty), the impact of the pandemic on resources or threat of the pandemic to overwhelm resources (theme 2: limited resources), perceived increased risk to self (theme 3: duty of care) and least-worst decision making, in which none of the options were perceived as ideal and participants settled on the least-worst option at that point in time (theme 4: least-worst decision making).ConclusionsIn the context of rapidly changing standards of justice and beneficence in patient care, traditional decision-making frameworks may no longer apply. Based on the experiences of surgeons in this study, we describe a framework of least-worst decision making. This framework gives rise to actionable strategies that can support decision making in sarcoma and other specialised fields of surgery, both during the current crisis and beyond.

Background Sarcomas are rare, heterogeneous tumours affecting patients of any age. Previous surveys describe that sarcoma patients report a significantly worse experience than those with common cancers. Consequently, Sarcoma UK conducted a national survey and these data were examined for age- and tumour-related differences in patients’ experiences. Methods Patients were randomly selected from respondents to National Cancer Patient Experience Surveys ( n  = 900). Differences between patient groups according to age (Adolescents and Young Adults [AYA] 18–39 years, middle-aged 40–64 years, elderly 65 + years) and tumour type (soft-tissue [STS] vs. bone]) were analysed with t-tests or chi-square tests. Results Survey response rate was 62% ( n  = 558; STS 75%, bone sarcoma 25%). Delay in diagnosis was reported; 27% patients ( n  = 150) waited > 3 months and initial symptoms were incorrectly interpreted; AYA STS patients were significantly more likely to be treated for another condition, or advised that their symptoms were not serious, than older STS patients. Clinical trial participation was low (6%, n  = 35). Symptom burden was high, most commonly daytime fatigue (48%, n  = 277) and pain (44%, n  = 248). AYAs were significantly more likely to report most side-effects and post-treatment concerns than older patients. Elderly patients were more satisfied with the information and emotional support provided than younger patients, however were significantly less likely to be referred to rehabilitation services. Conclusions This study identifies significant age-related differences in the sarcoma patient journey, which are not only related to variation in tumour-types. These results provide rationale for adopting an age-specific approach to the management of sarcoma patients in order to improve overall patient experience.

Background Though relatively rare, bone tumors significantly impact patient health and treatment outcomes. Objective This systematic review analyzes the incidence, types, survival rates, and risk factors associated with bone tumors, including both benign and malignant forms. Methods This systematic review was conducted using the keywords “bone tumors,” “epidemiology,” “benign bone tumors,” “malignant bone tumors,” “osteosarcoma,” “Ewing sarcoma,” “chondrosarcoma,” “risk factors,” and “survival” in electronic databases including PubMed, Scopus, Web of Science, and Google Scholar from 2000 to 2024. The search strategy was based on the PRISMA statement. Finally, 9 articles were selected for inclusion in the study. Results The systematic review highlights that primary bone tumors can be classified into benign and malignant types, with osteosarcoma being the most prevalent malignant form, particularly among adolescents and young adults. The epidemiology of bone tumors is influenced by factors such as age, gender, geographic location, and genetic predispositions. Recent advancements in imaging techniques have improved the detection of these tumors, contributing to an increasing recognition of their prevalence. Data shows that the limited-duration prevalence of malignant bone tumors has increased significantly. This increase is from 0.00069% in 2000 to 0.00749% in 2018, indicating an increasing recognition and diagnosis of these rare tumors over time. Survival rates vary significantly by tumor type, with approximately 50–60% for osteosarcoma and around 70% for Ewing’s sarcoma, though these rates decrease with metastasis. Key risk factors identified include genetic predispositions such as Li-Fraumeni syndrome and TP53 mutations, environmental exposures like radiation, and growth patterns related to height. Conclusion The review highlights the importance of early diagnosis and treatment intervention, as survival rates are significantly better for patients with localized disease compared to those with metastatic conditions. The observed variations in survival rates across different tumor types underscore the need for tailored treatment strategies. Key risk factors include genetic predispositions and environmental exposures, highlighting the need for targeted screening and ongoing research to enhance diagnostic accuracy and treatment strategies.

Introduction: Primary bone and soft tissue sarcomas are rare, but diagnostically and therapeutically challenging group of tumors, requiring multidisciplinary management. There are limited documented studies from multidisciplinary teams , in the form of comprehensive analysis of these tumors, from our country. This study is an analysis of cases of osteosarcomas, Ewing sarcomas (ESs), chondrosarcomas (CSs), and soft-tissue sarcomas (STSs), registered at our institution during 2012. Methods: Clinical details, including outcomes of cases of bone and STSs, during the year 2012, were retrieved from the medical records of our institution and were further analyzed. Results: Ninety-five high-grade, extremity-based, treatment-naïve cases of osteosarcomas were treated with a novel, dose-dense, nonhigh-dose methotrexate-based OGS-12 protocol. Good histopathologic response (necrosis ≥90%) was achieved in 59% nonmetastatic and 56% metastatic patients. At a median follow-up of 48 months, the estimated 5-year event-free survival and overall survival (OS) were 67% and 78%, respectively. In the metastatic cohort at a median follow-up of 51 months, the 5-year estimated progression-free survival was 24% and OS was 26%. Among 87 (73.2%) nonmetastatic and 32 (26.8%) metastatic, analyzable cases of ES, at a median follow-up of 40 months, the disease-free survival (DFS) and OS in the nonmetastatic group were 62% and 83%; in the metastatic group, they were 37.5% and 65.6%, respectively. Among 40 cases of CSs (33 nonmetastatic and 7 metastatic), 21 had limb salvage surgery while 5 had amputation. Microscopically, 90.4% were Grade II CSs. Five-year OS and DFS were 84.6% and 71%, respectively. Among 189 high-grade, extremity-based STSs (89% nonmetastatic), synovial sarcoma was the most common subtype (31%). Eighty-five percent had limb preservation surgery; a majority were offered adjuvant radiation with or without chemotherapy. At a median follow-up of 51 (1-63) months, 3-year local control, DFS, and OS were 81%, 48%, and 64%, respectively. Conclusions: The novel OGS 12 and Ewing Family of Tumors 2001 protocols have shown comparable outcomes to international standards in cases of osteosarcoma and ES, respectively, and merit wider applications, especially in low- and middle-income countries (LMICs). Outcomes in STS and CSs were also comparable and underscore the importance of a multidisciplinary approach for the management of sarcomas in LMICS.

ObjectiveThe aim was to study the incidence and survival of patients with uterine sarcoma diagnosed in the period from 2000 to 2012 based on Surveillance, Epidemiology, and End Results (SEER) database.MethodsAll 18 registries of the SEER database were used to select cases. We included women aged 30 years or older diagnosed with uterine sarcoma. Histological subtypes were defined as leiomyosarcoma, carcinosarcoma, stromal sarcoma, adenosarcoma, and sarcoma not otherwise specified according to the 2003 World Health Organization classification. Using SEER*Stat software version 8.1.2. We calculated the age-adjusted incidence rates, extent of disease at time of diagnosis, and survival rates with different treatment modalities for white, black, and other races. Univariate and multivariate Cox proportional hazards analysis were done to examine factors affecting survival.ResultsWe identified 13,089 patients diagnosed with uterine sarcoma in the period from 2000 to 2012. The age-adjusted incidence rate for patients aged 50 years or older was more than that of younger patients (6.4/105 vs 1.5/105, P < 0.0001). Also, the age-adjusted incidence rate for black women was twice that of white women (7.3/105 vs 3.5/105, P < 0.0001). Carcinosarcoma was the most commonly diagnosed subtype followed by leiomyosarcoma. Women aged 50 years or older had worse survival than those younger than 50 years (hazard ratio, 1.78; 95% confidence interval, 1.64–1.92; P < 0.001). The overall survival of patients who had surgery with radiation was better than those who had surgery alone (hazard ratio, 0.89; 95% confidence interval, 0.83–0.95; P < 0.001). In women with localized disease, surgery was associated with better survival than surgery with radiation (66.4% vs 74.4%, P < 0.00001).ConclusionsUterine sarcoma is an aggressive tumor that occurs more in old age and among women of black race. Poor survival was associated with old age, black race, and advanced disease stage. Radiotherapy in patients with localized stage does not improve the survival.

Purpose Osteosarcoma (OSC) and Ewing sarcoma (EWS) of the skull are rare malignancies in children and adolescents. This study aimed to analyze the demographic, clinical, treatment, and survival characteristics of these tumors using a population-based approach. Methods Data from the Surveillance, Epidemiology, and End Results (SEER) database were used to identify pediatric and adolescent patients (aged 0–19 years) diagnosed with OSC or EWS of the skull from 2000 to 2019. Demographic, clinical, and treatment variables were analyzed. Kaplan–Meier survival analysis and Cox proportional hazards regression models were used to evaluate prognostic factors for overall survival (OS). Results A total of 124 patients were included, with 46% diagnosed with OSC and 54% with EWS. At diagnosis, 43.6% had localized disease, 42.6% had regional disease, and 13.8% presented with distant metastases. Surgical resection was the primary treatment (84.6%), with 84.6% of patients receiving chemotherapy and 43.6% undergoing radiotherapy. The OS rates for the entire cohort were 90.3% at 1 year, 77.9% at 3 years, and 75.6% at 5 years. Younger patients (0–14 years) demonstrated significantly better survival outcomes compared to older adolescents ( P  = 0.003). EWS was associated with higher survival rates than OSC ( P  = 0.004). Patients with localized disease had significantly better survival outcomes compared to those with distant metastases ( P  = 0.003). Multivariable Cox regression analysis identified age, histological subtype, and SEER stage as independent prognostic factors. Older patients had higher mortality risk (HR 2.58; 95% CI, 1.28–5.19; P  = 0.008), as did those with distant-stage disease (HR 4.11; 95% CI, 1.67–10.11; P  = 0.002). EWS was associated with better survival compared to OSC (HR 0.36; 95% CI, 0.13–0.99; P  = 0.048). Conclusions This study highlights significant differences in survival outcomes for OSC and EWS of the skull in children and adolescents. Younger age, localized disease, and EWS subtype were associated with better prognosis. These findings underscore the need for early diagnosis and tailored treatment strategies to improve outcomes in this population.

Background: Primary osseous sarcomas of the mobile spine are rare bony tumors. Ewing sarcoma, chondrosarcoma, chordoma and osteosarcoma constitute the majority of primary bone sarcomas of the spine; however, other rare sarcoma tumors may also affect the spine. In order to perform an epidemiological study of theses tumors, national registries may help to evaluate data for populations with similar characteristics. Methods: A population-based study was designed based on data from the Iran National Cancer Registry (INCR). All morphology codes (M-Code) of primary osseous sarcomas of the mobile spine (C-code 41.2) were derived and analyzed. Results: Among 186 patients with primary osseous sarcomas of the mobile spine, 67.2% were men and 32.8% were women. The median (IQR) age was 37.0 (20.0–59.0) years and the age-standardized incidence rate (ASIR) was 0.37 per million. The majority of cases of Ewing sarcoma (29.5%) were observed in the age group 20–25 years. Among male patients with chondrosarcoma, the median age was 39.0 (30.0–50.0), while females showed a median age of 56.0 (50.0–59.0). The median age of patients with chordoma was 54.0 (47.0–63.0) years. The crude incidence rate of mobile spine osteosarcoma was 0.04 per million. Conclusion: Ewing sarcoma was the most frequent primary osseous sarcoma of the mobile spine. A male predilection was observed among all major sarcomas of the mobile spine. Ewing sarcoma in Iran affects the mobile spine in slightly older ages compared to other studies. Myxoid chondrosarcoma is the most frequent subtype of the mobile spine chondrosarcoma. Chordoma affects male in older ages compared to females. In contrast with other studies which showed a bimodal distribution of osteosarcoma of the spine including young adult and older age groups, 86% of cases in Iran were in the age group of 10–40 years.

•Comprehensive incidence estimates for all the main primary bone sarcomas.•Centralisation of bone sarcoma care improves the clinical outcome in osteosarcoma.•Survival rates did not significantly improve for high-grade bone sarcomas. Chondrosarcoma, osteosarcoma and Ewing sarcoma form the majority of malignant primary tumours of bone. High-grade bone sarcomas require intensive treatment due to their rapid and invasive growth pattern and metastasising capabilities. This nationwide study covers overall incidence, treatment and survival patterns of bone sarcomas in a 15-year period (2000–2014) in the total population of the Netherlands. Data for this study were derived from the Netherlands Cancer Registry, which receives primary notification from the national pathology database. Classification and categorisation was based on the ICD-O-3 classification and the WHO classification 2013 applied according to our clinicopathological expertise. Overall incidence over the 15-year-period was calculated as a rate per 100,000 person-years (using the European Standardised Rate, ESR). Survival was analysed with Kaplan-Meier curves and Cox proportional hazards regression. Incidence for high-grade chondrosarcoma (n = 429) was estimated at 0.15 per 100,000 ESR, and 5-year overall survival at 65.9% (95% confidence interval (CI): 61.0%–70.4%). Incidence for high-grade central osteosarcoma (n = 605) was estimated at 0.25 per 100,000 ESR and 5-year survival at 53.9% (95%CI: 49.7%–58.0%). Ewing sarcoma incidence (n = 334) was estimated at 0.15 per 100,000 ESR and 5-year survival at 59.3% (95%CI: 53.5%–64.6%). For high-grade central osteosarcoma, treatment at a bone tumour centre was associated with better survival (HR 0.593). This study provides comprehensive incidence estimates for all the main primary bone sarcomas over a 15-year time period in a Northern European country with little migration. Centralisation of bone sarcoma care improves the clinical outcome in osteosarcoma.

Objectives Most epidemiologic studies on soft tissue sarcomas (STS) and bone sarcomas (BS) are performed in western countries, with few in the Middle East and North Africa region. We describe the epidemiology of sarcomas in Lebanon using the medical records database at the American University of Beirut Medical Center (AUBMC). Methods This single-center retrospective cohort study included patients with sarcomas registered in the database between 2015 and 2019. Their charts were reviewed for baseline characteristics, tumor biology and location, treatment modalities, recurrence, metastasis, and death. Results The cohort included 234 patients with STS and 99 patients with BS. Most tumors were <10 cm in size. The most common subtypes were liposarcoma for STS and osteosarcoma for BS. The most common location of STS was the thigh. The most frequent sites of STS metastasis were the lungs. Histological subtype, smoking status, and tumor size and grade were significant for progression-free survival (PFS) in patients with STS. By multivariable analysis, smoking was significantly associated with poorer PFS in STS. For BS, only tumor grade was significant for PFS. Conclusion The epidemiology of sarcomas at AUBMC is similar to that previously reported. Smoking history was associated with poorer survival in patients with STS.