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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.

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.

The present study aimed to compare cancer incidence and trends in survival for children diagnosed in Japan and England, using population‐based cancer registry data. The analysis was based on 5192 children with cancer (age 0‐14 years) from 6 prefectural cancer registries in Japan and 21 295 children diagnosed in England during 1993‐2010. Differences in incidence rates between the 2 countries were measured with Poisson regression models. Overall survival was estimated using the Kaplan–Meier method. Incidence rates for Hodgkin lymphoma, renal tumors and Ewing sarcomas in England were more than twice as high as those in Japan. Incidence of germ cell tumors, hepatic tumors, neuroblastoma and acute myeloid leukemia (AML) was higher in Japan than in England. Incidence of all cancers combined decreased in Japan throughout the period 1993 to 2010, which was mainly explained by a decrease in registration of neuroblastoma in infants. For many cancers, 5‐year survival improved in both countries. The improvement in survival in chronic myeloid leukemia (CML) was particularly dramatic in both countries. However, 5‐year survival remained less than 80% in 2005‐2008 in both countries for AML, brain tumors, soft tissue sarcomas, malignant bone tumors and neuroblastoma (age 1‐14 years). There were significant differences in incidence of several cancers between countries, suggesting variation in genetic susceptibility and possibly environmental factors. The decrease in incidence for all cancers combined in Japan was related to the cessation of the national screening program for neuroblastoma. The large improvement in survival in CML coincided with the introduction of effective therapy (imatinib). For many of childhood cancers, 5‐year survival improved in Japan and England. The improvement in survival in chronic myeloid leukaemia (CML) was particularly dramatic in both countries.

Purpose Little is known regarding long-term neurocognitive outcomes in osteosarcoma and Ewing sarcoma (EWS) survivors despite potential risk factors. We evaluated associations among treatment exposures, chronic health conditions, and patient-reported neurocognitive outcomes in adult survivors of childhood osteosarcoma and EWS. Methods Five-year survivors of osteosarcoma ( N  = 604; median age 37.0 years) and EWS ( N  = 356; median age 35.0 years) diagnosed at < 21 years from 1970 to 1999, and 697 siblings completed the Childhood Cancer Survivor Study Neurocognitive Questionnaire and reported chronic health conditions, education, and employment. Prevalence of reported neurocognitive difficulties were compared between diagnostic groups and siblings. Modified Poisson regression identified factors associated with neurocognitive difficulties. Results Osteosarcoma and EWS survivors, vs. siblings, reported higher prevalences of difficulties with task efficiency (15.4% [ P  = 0.03] and 14.0% [ P  = 0.04] vs. 9.6%, respectively) and emotional regulation (18.0% [ P  < 0.0001] and 15.2% [ P  = 0.03] vs. 11.3%, respectively), adjusted for age, sex, and ethnicity/race. Osteosarcoma survivors reported greater memory difficulties vs. siblings (23.5% vs. 16.4% [ P  = 0.01]). Comorbid impairment (i.e., ≥ 2 neurocognitive domains) was more prevalent in osteosarcoma (20.0% [ P  < 0.001]) and EWS survivors (16.3% [ P  = 0.02]) vs. siblings (10.9%). Neurological conditions were associated with worse task efficiency (RR = 2.17; 95% CI = 1.21–3.88) and emotional regulation (RR = 1.88; 95% CI = 1.01–3.52), and respiratory conditions were associated with worse organization (RR = 2.60; 95% CI = 1.05–6.39) for EWS. Hearing impairment was associated with emotional regulation difficulties for osteosarcoma (RR = 1.98; 95% CI = 1.22–3.20). Patient report of cognitive difficulties was associated with employment but not educational attainment. Conclusions Survivors of childhood osteosarcoma and EWS are at increased risk for reporting neurocognitive difficulties, which are associated with employment status and appear related to chronic health conditions that develop over time. Implications for Cancer Survivors Early screening, prevention, and treatment of chronic health conditions may improve/prevent long-term neurocognitive outcomes.

•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.

Background Ewing sarcoma, the second most frequent bone tumor in children and adolescents, is often presented with localized disease or metastatic-related symptoms. In this study, we aim to construct and validate a nomogram for patients with Ewing sarcoma to predict the 3- and 5-year overall survival (OS) based on the Surveillance, Epidemiology, and End Results (SEER) database. Methods Demographic and clinic pathological characteristics of patients with Ewing sarcoma diagnosed between 2010 and 2015 were extracted from SEER database. Univariate and multivariate Cox analyses were carried out to identify the independent characteristics. The independent factors were further included into the construction of a nomogram. Finally, c-index and calibration curves were used to validate the nomogram. Results A total of 578 patients were enrolled into our analysis. The results of univariate Cox analysis showed that age, 7th AJCC stage, 7th AJCC T stage, 7th AJCC N stage, 7th AJCC M stage, metastatic status to lung, liver and bone were significant factors. Multivariate Cox analysis was performed and it confirmed age, N stage and bone metastasis as independent variables. Next, a nomogram was constructed using these independent variables in prediction to the 3- and 5-year OS. Furthermore, favorable results with c-indexes (0.757 in training set and 0.697 in validation set) and calibration curves closer to ideal curves indicated the accurate predictive ability of this nomogram. Conclusions The individualized nomogram demonstrated a good ability in prognostic prediction for patients with Ewing sarcoma.

Abstract Background: The impact of surgical compliance on survival outcomes in patients with Ewing sarcoma (ES) is unclear, so this study was performed to explore the association between them. Methods: We used the SEER*Stat software (version 8.3.6.1) to extract information on ES patients from the SEER database. Patients were divided into two groups based on their adherence to surgical recommendations: the surgical compliance group and the surgical noncompliance group. Categorical variables were expressed as percentages. Multivariate logistic regression and Chi-square test were used to explore variables related to surgical compliance. Univariate Cox regression analysis was used to initially select potential prognostic factors, and then the factors selected in the univariate Cox regression analysis were further analyzed in a multivariate Cox proportional risk model to ultimately determine the risk prognostic factors significantly related to the survival of patients with ES. Results: Multiple logistic regression analysis suggested that adults (OR = 0.373, 95% confidence interval (CI): 0.164-0.849), Grade IV (OR = 0.373, 95% CI: 0.164-0.849), and unmarried patients (OR = 0.568, 95% CI: 0.339-0.954) were more inclined to accept surgery recommendations, while patients from 2001 to 2010 were less compliant with surgery. Multifactorial Cox regression analysis suggested that surgical compliance was an independent prognostic factor for patients with ES. Through the Kaplan-Meier survival curves, we could clearly observe that the overall survival was higher in the surgical compliance group than in the surgical noncompliance group. Furthermore, subgroup analysis also reached similar conclusions. Conclusion: In this study, we found that surgical compliance was an independent predictor of patient prognosis. Furthermore, we found that age, tumor grade, year of diagnosis, and marital status may be related to surgical compliance.

Background This study is to determine the risk factors for metastasis of Ewing sarcoma (ES) patients in SEER database. Then explore clinicopathological factors associated with poor prognosis. Furthermore, develop the nomogram to predict the probability of overall survival and cancer-specific survival Methods Thus, we collected clinicopathological data of ES patients in SEER database, and then used chi-square test and logistic regression to determine risk factors associated to metastasis. We also did survival analysis including Kaplan-Meier curve and Cox proportional hazard model to explore the risk factors associated to overall survival and cancer-specific survival, and then developed the nomogram to visualize and quantify the probability of survival. Results After statistics, we find that patients with older ages (11–20 years old: OR = 1.517, 95% confidence interval [CI] 1.033–2.228, p = 0.034; 21–30 years old: OR = 1.659. 95%CI 1.054–2.610, p = 0.029), larger tumor size (> 8 cm: OR = 1.914, 95%CI 1.251–2.928, p = 0.003), and pelvic lesions (OR = 2.492, 95%CI 1.829–3.395, p < 0.001) had a higher risk of metastasis. ROC curves showed higher AUC (0.65) of combined model which incorporate these three factors to predict the presence of metastasis at diagnosis. In survival analysis, patients with older ages (11–20 years: HR = 1.549, 95%CI 1.144–2.099, p = 0.005; 21–30 years: HR = 1.808, 95%CI 1.278–2.556, p = 0.001; 31–49 years: HR = 3.481, 95%CI 2.379–5.094, p < 0.001; ≥ 50 years: HR = 4.307, 95%CI 2.648–7.006, p < 0.001) , larger tumor size (5–8 cm: HR = 1.386, 95%CI 1.005–1.991, p = 0.046; > 8 cm: HR = 1.877, 95%CI 1.376–2.561, p < 0.001), black race (HR = 2.104, 95%CI 1.296–3.416, p = 0.003), and wider extension (regional: HR = 1.373, 95%CI 1.033–1.823, p = 0.029; metastatic: HR = 3.259, 95%CI 2.425–4.379, p < 0.001) were associated with worse prognosis. Chemotherapy was associated with better prognosis (HR = 0.466, 95%CI 0.290–0.685, p < 0.001). The nomogram which developed by training set and aimed to predict OS and CSS showed good consistency with actual observed outcomes internally and externally. Conclusion In conclusion, tumor size and primary site were associated with distant metastasis at diagnosis. Age, tumor size, primary site, tumor extent, and chemotherapy were associated with overall survival and cancer-specific survival. Nomogram could predict the probability of OS and CSS and showed good consistency with actual observed outcomes internally and externally.

BackgroundCommon primary bone tumors include osteosarcomas (OSC) and Ewing sarcomas (EWS). The skull is a rare site, and literature about their treatment and survival is scarce. Using the Surveillance, Epidemiology, and End Results (SEER) database, this study aims to assess the treatment and survival of skull OSC and skull EWS, as well as predictors for survival.MethodsSkull OSC and EWS cases were obtained from the SEER database. Patient and tumor characteristics, treatment modalities, and survival were extracted. Overall survival (OS) was assessed using multivariable Cox proportional hazard regression stratified by tumor histology. Kaplan-Meier curves were constructed for OS comparing OSC and EWS, as well as histological subtypes in OSC.ResultsA total of 321 skull OSC and 80 skull EWS patients were registered from 1973 to 2013. EWS was more common in younger patients (p < 0.001). Resection was the predominant treatment strategy (80.1%), frequently in combination with adjuvant radiotherapy (30.4%). The 5-year survival rate varied significantly between OSC and EWS (51.0% versus 68.5%, p = 0.02). Kaplan-Meier curves show that EWS had a significantly better survival compared to OSC. Comparing histological subtypes of skull OSC, chondroblastic OSC had the best OS, Paget OSC the worst. Older age, tumor advancement, no surgical treatment, and the use of radiotherapy were identified as independent predictors of decreased OS in skull OSC.ConclusionOverall prognosis is better for EWS compared to OSC. Chondroblastic OSC have the best overall survival, while OSC associated with Paget’s disease of the bone has the poorest overall survival.

Background Ewing sarcoma (ES) is a malignant bone tumor most commonly affecting non‐Hispanic White (NHW) adolescent males, though recognition among Hispanic individuals is rising. Prior population‐based studies in the United States (US), utilizing Surveillance, Epidemiology, and End Results (SEER) have shown higher all‐cause mortality among White Hispanics, Blacks, and those of low socioeconomic status (SES). Florida is not part of SEER but is home to unique Hispanic populations including Cubans, Puerto Ricans, South Americans that contrasts with the Mexican Hispanic majority in other US states. This study aimed to assess racial/ethnic disparities on incidence and survival outcomes among this diverse Florida patient population. Methodology Our study examined all patients diagnosed with osseous ES (2005–2018) in Florida (n = 411) based on the state's population‐based cancer registry dataset. Florida Age‐adjusted Incidence Rates (AAIRs) were computed by sex and race‐ethnicity and compared to the equivalent populations in SEER. Cause‐specific survival disparities among Florida patients were examined using Kaplan–Meier analysis. Univariable and multivariable analyses using Cox regression were performed for race/ethnicity, with adjustment for age, sex, year of diagnosis, site of disease, staging, SES, and insurance type. Results There was a significantly higher incidence of osseous ES in Florida Hispanic males (AAIR 2.6/1,000,000); (95% CI: 2.0–3.2 per 1,000,000; n = 84) compared to the SEER Hispanic males (AAIR 1.2/1,000,000;1.1–1.4 per 1,000,000; n = 382). Older age, distant metastasis, lack of chemotherapy or surgical resection were statistically significant determinants of poor survival while SES, insurance status and race‐ethnicity were not. However, among nonmetastatic ES, Florida Hispanics had an increased risk of death compared to Florida NHW (adjusted Hazard Ratio 2.32; 95%CI: 1.20–4.46; p = 0.012). Conclusions Florida Hispanic males have a higher‐than‐expected incidence of osseous ES compared to the US. Hispanics of both sexes show remarkably worse survival for nonmetastatic disease compared to NHW. This disparity is likely multifactorial and requires further in‐depth studies.