Patterns and treatment outcomes of primary bone tumors in children treated at tertiary referral hospital, Ethiopia.

BACKGROUND: Bone tumors account for approximately 6% of all cancers in children. Malignant bone tumors, commonly occurring in children and adolescents, are associated with high mortality and morbidity. The overall survival of children with primary malignant bone tumors is affected by the stage of disease, time of diagnosis, and treatment response. Despite advanced treatment modalities with chemotherapy, surgery, and radiotherapy, bone tumor is the third leading cause of death in children with malignancy. Patients with metastatic disease at diagnosis have poor outcomes compared to localized disease at presentation. The 5-year Overall Survival and event-free survival in children with primary malignant bone tumors were 85.2% and 69.2%. The study aimed to assess the clinicopathological profile and treatment outcomes of children with primary malignant bone tumors in our setup. MATERIALS AND METHODS: A hospital-based cross-sectional study was conducted on 95 children who met the inclusion criteria through structured questionnaire. The collected data were analyzed using a statistical package for social sciences (SPSS) version 25. P-value < 0.05 was considered to be statistically significant. Kaplan Meier survival estimate was used for overall and event-free survival analysis. RESULTS: A total of ninety-five patients met the study inclusion criteria and the median age at diagnosis with primary malignant bone tumors was 10 years, with an interquartile range of 8-12 years. The duration of the illness from the onset of symptoms to the oncologic treatment center ranges from three weeks to 2 years with a mean duration of five months. Swelling was the commonest presenting symptom accounting for 95.8% (n = 91). Lower extremity was the commonest primary site of involvement accounting for 55.8% (n = 53) of children with primary malignant bone tumors. Osteosarcoma was the commonest malignant bone tumor constituted 66.3% (n = 63), followed by Ewing sarcoma at 33.7% (n = 32). About 41.2% (n = 39) of children had metastatic disease at presentation and the lung was the commonest site of distant metastasis. The Kaplan Meier survival estimate analysis showed the 1-year and 5-year overall survival probabilities for all pediatric primary malignant bone tumor patients were 65% (95% CI: 0.3-0.56) and 38% (95% CI:0.19-0.47) respectively. The 1-year and 5-year event-free survival probabilities were 55% (95% CI: 0.32-0.73) and 33% (95% CI: 0.10-0.59). The stage of the disease at presentation had a significant association with the outcome (p = 0.023). CONCLUSION: Our study showed the mean duration of the illness from the onset of symptoms to the oncologic treatment center was 5 months ranging from 3 weeks to 2 years. More than one-third of the presented with metastatic disease at presentation. The 1-year and 5-year overall survival (OS) probabilities of children with primary malignant bone tumors were low in our setup compared to other studies.

Li-Fraumeni-associated osteosarcomas: The French experience.

PURPOSE: Describe clinical characteristics and outcome of Li-Fraumeni syndrome (LFS)-associated osteosarcomas. METHODS: TP53 germline pathogenic/likely pathogenic variant carriers diagnosed with osteosarcoma in France between 1980 and 2019 were identified via the French Li-Fraumeni database at Rouen University Hospital. Sixty-five osteosarcomas in 52 patients with available clinical and histological data were included. The main clinical characteristics were compared with data from National Cancer Institute's SEER (Surveillance, Epidemiology, and End Results) for patients of the same age group. RESULTS: Median age at first osteosarcoma diagnosis was 13.7 years (range: 5.9-36.7). Compared to unselected osteosarcomas, LFS-associated osteosarcomas occurred more frequently in patients less than 10 years of age (23% vs. 9%), and when compared with osteosarcomas in patients less than 25 years were characterized by an excess of axial (16% vs. 10%) and jaw sites (15% vs. 3%) and histology with predominant chondroblastic component and periosteal subtypes (17% vs. 1%). Metastases incidence (25%) was as expected in osteosarcomas. After the first osteosarcoma treatment, the rate of good histologic response (62%) and the 5-year progression-free survival (55%, 95% confidence interval [CI]: 42.6-71.1) were as expected in unselected series of osteosarcomas, whereas the 5-year event-free survival was 36.5% [95% CI: 25.3-52.7] due to the high incidence of second malignancies reaching a 10-year cumulative risk of 43.4% [95% CI: 28.5-57.5]. CONCLUSION: In osteosarcoma, young age at diagnosis, axial and jaw sites, histology with periosteal or chondroblastic subtype, and synchronous multifocal tumors should prompt suspicion of a germline TP53 mutation. Standard treatments are effective, but multiple malignancies impair prognosis. Early recognition of these patients is crucial for tailored therapy and follow-up.

Treatments and prognostic factors for bone and soft tissue sarcoma in non-urban areas in Japan.

BACKGROUND: Although bone and soft tissue sarcoma is recognized as a rare cancer that originates throughout the body, few comprehensive reports regarding it have been published in Japan. PATIENTS AND METHODS: Bone and soft tissue sarcomas were tabulated from the Cancer Registries at eight university hospitals in the Chugoku-Shikoku region. Prognostic factors in cases were extracted in a single facility and have been analyzed. RESULTS: From 2016 to 2019, 3.4 patients with bone and soft tissue sarcomas per a general population of 100,000 were treated at eight university hospitals. The number of patients who underwent multidisciplinary treatment involving collaboration among multiple clinical departments has been increasing recently. In the analysis carried out at a single institute (Ehime University Hospital), a total of 127 patients (male/female: 54/73) with an average age of 67.0 y (median 69.5) were treated for four years, with a 5-year survival rate of 55.0%. In the analysis of prognostic factors by multivariate, disease stage and its relative treatment, renal function (creatinine), and a patient's ability of self-judgment, and a patient's mobility and physical capability were associated with patient prognosis regarding bone and soft tissue sarcomas. Interestingly, age did not affect the patient's prognosis (> 70 vs ≦ 70). CONCLUSIONS: Physical and social factors may affect the prognosis of patients with bone and soft tissue sarcomas, especially those living in non-urban areas.

Statistics of bone sarcoma in Japan: report from the population-based cancer registry in Japan.

BACKGROUND: No previous reports have characterized national bone sarcoma profiles overall. We examined the nationwide statistics for bone sarcoma in Japan using data from the National Cancer Registry (NCR), a population-based cancer registry. METHODS: We identified 3,755 patients with bone sarcomas entered in the NCR during 2016-2019 using International Classification of Diseases-Oncology, Third Edition codes for cancer topography and morphology. We extracted data on patient demographics, tumor details (reason for diagnosis, tumor location, histology, extent of disease), hospital volume/type, treatment, and prognosis for each patient. RESULTS: Bone sarcoma showed a slight male preponderance. The age distribution peaked at ages 10-20 and 60-80; approximately 44% of patients were aged over 60 years. Chordoma, chondrosarcoma, and malignant fibrous histiocytoma of bone peaked in the elderly, and Ewing's sarcoma peaked in children. Osteosarcoma had two peaks in Japan as well as in Western countries. The most frequent tumor locations were the limb (45%) and the pelvis (21%). Extent of disease was categorized as: "localized" (39%), "regional" (27%), and "distant" (11%). We found significant associations between overall survival and age, tumor location, facility type, hospital volume, histologic subtype, reason for diagnosis, and extent of disease. The latter had the poorest survival. CONCLUSIONS: This is the first study to outline the epidemiology, clinical features, treatment, prognosis, and significant factors affecting prognosis of bone sarcoma in Japan using the NCR. Documenting our data regarding elderly patients' outcomes is essential so other countries showing similar population-aging trends can learn from our experiences. LEVEL OF EVIDENCE: Prognostic studies, Level III.

Bone metastases among newly diagnosed cancer patients: a population-based study.

PURPOSE: (i) To analyze age-adjusted incidence rates of synchronous bone metastases diagnosed alongside primary malignancy from 2010 to 2018 in the US population, (ii) determine the incidence proportions (IPs) and characteristics of synchronous bone metastases among newly diagnosed cancer patients in the USA especially pediatric cases, and (iii) assess the implications of synchronous bone metastases on cancer patient's survival, and identify the survival risk factors for these cancer patients. METHODS: Utilizing data from the Surveillance, Epidemiology, and End Results (SEER) program (2010-2018), we calculated age-adjusted IPs and annual percentage change (APC), and employed logistic regression and Cox regression models for our analysis. RESULTS: 3 300 736 cancer patients were identified. The age-adjusted incidence rates of synchronous bone metastases increased from 2010 (18.04/100 000) to 2018 (20.89/100 000; APC: 2.3, 95% confidence interval [CI], 1.4-3.1), but decreased in lung cancer (average APC: -1.0, 95% CI, -1.8 to -0.3). The highest IPs were observed in pediatric neuroblastoma (43.2%; 95% CI, 39.8%-46.7%) and adult small cell carcinoma (23.1%; 95% CI, 22.7%-23.4%). Multivariate logistic analyses revealed that primary tumor characteristics were correlated with higher bone metastases risk. Survival analyses also showed varied prognostic outcomes based on metastasis sites and demographics among cancer patients. Landmark analyses further indicated among long-term cancer survivors (≥3 and ≥5 years), patients with de novo bone metastases had the poorest survival rates compared with those with other synchronous metastases (P < 0.001). CONCLUSION: This study provides a population-based estimation of the incidence and prognosis for synchronous bone metastases. Our findings highlight the need for early identification of high-risk groups and multidisciplinary approaches to improve prognosis of cancer patients with de novo bone metastases.

Incidence and management of secondary deformities after megaendoprosthetic proximal femur replacement in skeletally immature bone sarcoma patients.

INTRODUCTION: Megaendoprosthetic reconstruction of bone defects in skeletally immature patients has led to the development of unique complications and secondary deformities not observed in adult patient cohorts. With an increasing number of megaendoprosthetic replacements performed, orthopedic oncologists still gain experience in the incidence and type of secondary deformities caused. In this study, we report the incidence, probable cause and management outcome of two secondary deformities after megaendoprosthetic reconstruction of the proximal femur: hip dysplasia and genu valgum. MATERIALS AND METHODS: Retrospective analysis of 14 patients who underwent primary and/or repeat reconstruction/surgery with a megaendoprosthetic proximal femur replacement between 2018 and 2022. RESULTS: Mean patient age was 9.1 years (range 4-17 years). Stress shielding was observed in 71.4%. Hip dislocation was the most frequent complication (50%). While four dislocations occurred without an underlying deformity, secondary hip dysplasia was identified in 58.3% (n = 7/12) of intraarticular resections and reconstructions, leading to dislocation in 71.4% (n = 5/7). A genu valgum deformity was observed in 41.6% (n = 5/12). The incidence of secondary hip dysplasia and concomitant genu valgum was 42.9% (n = 3/7). Triple pelvic osteotomy led to rebound hip dysplasia in two cases (patients aged < 10 years), whereas acetabular socket replacement led to stable hip joints over the course of follow-up. Temporary hemiepiphyseodesis was applied to address secondary genu valgum. CONCLUSIONS: Patients aged < 10 years were prone to develop secondary hip dysplasia and genu valgum following proximal femur replacement in this study. Management of secondary deformities should depend on remaining skeletal growth. Stress shielding was observed in almost all skeletally immature patients.

Epidemiology of bone tumors in children and adolescents: a retrospective study of 266 patients in the south of Tunisia.

Although bone tumors (BT) are relatively uncommon among the human neoplasm, they constitute the most frequent tumors in children and adolescents (CAA). Little information is available about the epidemiologic features of BT in CAA. We aimed to present and discuss epidemiological characteristics of BT in CAA in southern Tunisia, regarding the different histological types. This is a retrospective study including cases of BT in CAA collected in the pathology department at the Habib Bourguiba university hospital over a period of 15 years (2006- 2020). A total of 266 BT was diagnosed in our institution (42,7% among all BT in Southern Tunisia) divided into 200 benign bone tumors (BBT) (75,2%) and 66 malignant bone tumors (MBT) (24,8%). The mean age for all BT was 14,2 years (3-20 years) with male predominance (sex ratio: 1,48). The most common tumor was osteochondroma (42.2%) followed by osteosarcoma (14.6%) and Ewing sarcoma (6.4%). For BBT, the most affected age group was the 16 to 20 year - old - group (50,7%) with a male predominance (59.8%) and a predilection for lower limb (66.8%) then the upper limb (16,8%). Osteochondroma was the most common histological type (56.5%) followed by aneuvrysmal cyst (8,5%) and osteoid osteoma (6,5%). For MBT, the mean age was 12,5 years (5-20 years) and the most affected age group was the 11 to 15 year -old -group (59%). Boys were more affected (60.6%), with a preference for the lower limb (57%) followed by the pelvis (15,6%). Osteosarcoma was the most common MBT (60%) followed by Ewing sarcoma (24%). Given their rarity and heterogeneity, the diagnosis of BT is particular in CAA and requires a multidisciplinary approach. The reporting of epidemiological studies remains essential in order to expand our knowledge regarding these uncommon tumors.

Clinical and pathological characterization of 158 consecutive and unselected oligometastatic breast cancers in a single institution.

PURPOSE: Data about incidence, biological, and clinical characteristics of oligometastatic breast cancer (OMBC) are scarce. However, these data are essential in determining optimal treatment strategy. Gaining knowledge of these elements means observing and describing large, recent, and consecutive series of OMBC in their natural history. METHODS: We collected data retrospectively at our institution from 998 consecutive patients diagnosed and treated with synchronous or metachronous metastatic breast cancer (MBC) between January 2014 and December 2018. The only criterion used to define OMBC was the presence of one to five metastases at diagnosis. RESULTS: Of 998 MBC, 15.8% were classified OMBC. Among these, 88% had one to three metastases, and 86.7% had only one organ involved. Bone metastases were present in 52.5% of cases, 20.9% had progression to lymph nodes, 14.6% to the liver, 13.3% to the brain, 8.2% to the lungs, and 3.8% had other metastases. 55.7% had HR+/HER2- OMBC, 25.3% had HER2+OMBC, and 19% had HR-/HER2- OMBC. The HR+/HER2- subtype statistically correlated with bone metastases (p = 0.001), the HER2+subtype with brain lesions (p = 0.001), and the HR-/HER2- subtype with lymph node metastases (p = 0.008). Visceral metastases were not statistically associated with any OMBC subtypes (p = 0.186). OMBC-SBR grade III was proportionally higher than in the ESME series of 22,109 MBC (49.4% vs. 35.1%, p < 0.001). CONCLUSION: OMBC is a heterogeneous entity whose incidence is higher than has commonly been published. Not an indolent disease, each subgroup, with its biological and anatomical characteristics, merits specific management.

Bone Metastases in Patients with Pancreatic NETs: Prevalence and Prognosis.

The clinical relevance of bone metastases (BM) in advanced pancreatic neuroendocrine tumors (PanNETs) is poorly described. We analyzed 314 consecutive PanNET patients treated at the European Neuroendocrine Tumour Society (ENETS) Center Essen between 2009 and 2021 in terms of the occurrence and clinical and prognostic impact of BM using hybrid imaging with 68Ga-DOTATOC PET/CT. According to UICC staging, 171/314 (54.5%) patients had stage IV PanNETs. BM was diagnosed in 62/171 (36.3%) patients. Initially, 35% of BMs were visible by pathological tracer uptake only. Skeletal-related events (SREs) were detected in 11 of the 62 patients (17.7%). Patients with antiresorptive therapy had a significantly lower rate of SRE (2/36, 5.6%) than individuals without bone-specific therapy (9/26, 34.6%) (odds ratio 9.0, p=0.0054, Fisher's exact test). The median overall survival (OS) was 82 months (53.6-110.4, 95% CI) in the stage IV PanNET cohort. The median OS was significantly lower for patients with BM (63 months; 49.9-76.0, 95% CI) than for patients with distant metastases other than BM (116 months; 87.6-144.3, 95% CI) (p=0.016, log-rank test). BM occurs in more than one-third of advanced PanNETs and is associated with an unfavorable prognosis. One in five patients experiences a persistent quality-of-life-lowering SRE. Antiresorptive therapy is associated with a more favorable risk of SREs and should be offered to all patients with BM in PanNETs.

The national incidence of chondrosarcoma of bone; a review.

BACKGROUND: Chondrosarcoma (CS) epidemiology has been studied by a number of authors using national cancer registry cohorts. Many reports share the common findings of a slight increase in incidence, but not all. The patterns and causes for these changes are divergent while reflection concerning methodological challenges are often missing. METHOD: We have performed a structured literature review to find national analyses of CS incidence published from 2010 to 2020. We included eight studies of national incidence of CS, summarise their findings and patterns of change. We further discuss explanations given for these changes to better understand the real patterns and raise awareness in their interpretation. RESULTS: Reported crude incidence ranges from 0.27 per million per year overall in Saudi Arabia to 5.4 in the Netherlands. Four studies from the USA, England, Switzerland and France report age standardised rates of 2.0-4.1 per million per year overall. While some countries report stable patterns, most report a slight increase. The Netherlands is the only country reporting a large increase, driven by a 10-fold increase in the incidence of ACT/grade 1 CS during the study period. We challenge the explanations given for this and suggest that this most likely is a result of variable interpretation and definition of CS at the lower levels of disease aggressiveness. This should raise awareness to possible over-treatment of CS in the Netherlands. CONCLUSION: The most likely national incidence of CS of bone is between 2-4 per million per year. Three modern reports present an incidence of 3.4-4.1 per million per year.

Sociodemographic Disparities in Presentation and Survival of Pediatric Bone Cancers.

Osteosarcoma (OST) and Ewing sarcoma (ES) are the most common pediatric bone cancers. Patients with metastatic disease at diagnosis have poorer outcomes compared with localized disease. Using the Surveillance, Epidemiology, and End Results registries, we identified children and adolescents diagnosed with OST or ES between 2004 and 2015. We examined whether demographic and socioeconomic disparities were associated with a higher likelihood of metastatic disease at diagnosis and poor survival outcomes. In OST, Hispanic patients and those living in areas of high language isolation were more likely to have metastatic disease at diagnosis. Regardless of metastatic status, OST patients with public insurance had increased odds of death compared to those with private insurance. Living in counties with lower education levels increased odds of death for adolescents with metastatic disease. In ES, non-White adolescents had higher odds of death compared with white patients. Adolescents with metastatic ES living in higher poverty areas had increased odds of death compared with those living in less impoverished areas. Disparities in both diagnostic and survival outcomes based on race, ethnicity, and socioeconomic factors exist in pediatric bone cancers, potentially due to barriers to care and treatment inequities.

Impact of Prior Cancer on the Prognosis of Patients with Chondrosarcoma: A Population-Based Study of the SEER Database.

Background: Patients with prior cancer are generally exempt from cancer experiments. This research aims to describe the prevalence, clinical features, and effects of past malignancy among patients with chondrosarcoma. Methods: Chondrosarcoma patients diagnosed between 2010 to 2015 were collected from the SEER database. The propensity score matching method was used to reconcile the disparity in baseline attributes. Kaplan-Meier analysis was employed to explore the outcomes of prior cancer on overall survival. The proportional hazards assumption was used to certain whether the covariate matched the Cox regression model. The potential outliers were estimated by deviance residuals type. Results: A total of 1,721 unique individuals were collected, of those 284 (16.50%) patients had a history of cancer, with prostate cancer being commonly documented (n = 49, 17.25%). Approximately half of the previous tumors are diagnosed within 5 years before the diagnosis of chondrosarcoma. Chondrosarcoma patients with prior cancers have a lower survival rate than those without prior malignancy (P < .001). A multivariable Cox analysis reveals that past cancer is a distinct risk factor for lifespan (hazard ratio = 2.489, P < .001). Conclusion: This study initially discovered that chondrosarcoma patients with past cancer have a bad prognosis. Different types of past cancer have varying effects on survival. We urgently propose that cancer trial exclusion criteria be set specifically by cancer classification, rather than accepting the unchangeable criterion for default.

Anatomical distribution, the incidence of malignancy and diagnostic workup in the pathological lesions of the clavicle: a review of 410 cases.

BACKGROUND: The clavicle poses a diagnostic dilemma of the pathological lesions due to the wide range of pathologies seen at this site. This study aimed to identify and stratify various pathologies seen in the clavicle and to guide ways of investigation for diagnosis based on age, site and investigation findings. MATERIALS AND METHODS: Four hundred and ten cases with clavicle lesions were identified in our database. Data were collected about the patient's medical history, previous investigation, inflammatory markers radiological investigations and biopsy. All patients were worked up and managed after discussion in a multidisciplinary team meeting (MDT). RESULTS: Non-malignant lesions accounted for 79% of cases. Infection was the most common diagnosis (39%) and the commonest diagnosis in those less than 20 years of age. 73% of the lesions were found at the medial end of the clavicle. Malignant tumours were 21%, while primary benign bone tumours accounted for only 14%. 50% of the malignant lesions were due to metastatic disease. The risk of malignancy increases with advancing age. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were not sensitive as a diagnostic tool in cases of osteomyelitis confirmed by histology. Magnetic resonance imaging (MRI) was noted to have high sensitivity and specificity for identifying the nature of a lesion and diagnosis. CONCLUSION: We have identified age as a positive predictor of a malignant cause in pathological lesions of the clavicle. MRI should be considered in all these cases. CRP and ESR have poor predictive values in diagnosing infection in the clavicle. Patients presenting with clavicle lesions should be discussed in a specialist MDT and undergo a systemic diagnostic workup, still in some cases, diagnosis can be speculated based on the patient's age, location of the lesion within the clavicle and the features seen on the MRI scan. LEVEL OF EVIDENCE: IV.

Risk factors for appendicular osteosarcoma occurrence in large and giant breed dogs in western Canada.

Objective: Risk factors for the development of canine appendicular osteosarcoma (OSA) have been investigated in numerous studies, but with contradictory results. The aim of this study was to analyze weight, age, breed, sex, neuter status, body condition score, and previous lameness in a population of large and giant breed dogs in western Canada with and without appendicular OSA. Animals and procedure: Medical records of 227 large or giant breed dogs diagnosed with appendicular OSA were compared to records from a control population of 454 large and giant breed dogs from the years 2000 to 2020. Results: Gonadectomized dogs, body condition score (BCS), and a history of lameness condition(s) (other than OSA) were associated with increased odds for presentation with OSA. Breeds shown to have increased odds for appendicular OSA occurrence included Rottweilers and Great Danes relative to Labrador retrievers. Conclusion and clinical relevance: Obesity and lameness appear to be independently associated with appendicular osteosarcoma. This study demonstrated that spayed females had the greatest risk compared to other sex and neuter status combinations; further investigation of these factors would be beneficial.

Facteurs de risque d'apparition d'ostéosarcome appendiculaire chez les chiens de grandes races et de races géantes dans l'Ouest canadien. Objectif: Les facteurs de risque de développement de l'ostéosarcome (OSA) appendiculaire canin ont été étudiés dans de nombreuses études, mais avec des résultats contradictoires. Le but de cette étude était d'analyser le poids, l'âge, la race, le sexe, la stérilisation, le score d'état corporel et les boiteries antérieures dans une population de chiens de grande race et de race géante de l'Ouest canadien avec et sans OSA appendiculaire. Animaux et procédure: Les dossiers médicaux de 227 chiens de grande race ou de race géante diagnostiqués avec l'OSA appendiculaire ont été comparés aux dossiers d'une population témoin de 454 chiens de grande race et de race géante des années 2000 à 2020. Résultats: Les chiens gonadectomisés, le score d'état corporel (BCS) et des antécédents de condition(s) de boiterie (autres que l'OSA) étaient associés à une probabilité accrue de présentation d'OSA. Les races dont le risque d'apparition d'OSA appendiculaire était plus élevé comprenaient les Rottweilers et les Grands Danois par rapport aux Labrador retrievers. Conclusion et pertinence clinique: L'obésité et la boiterie semblent être indépendamment associées à l'ostéosarcome appendiculaire. Cette étude a démontré que les femelles stérilisées présentaient le plus grand risque par rapport aux autres combinaisons de sexe et de statut neutre, une enquête plus approfondie sur ces facteurs serait bénéfique.(Traduit par Dr Serge Messier).

Incidence and progression of osteoarthritis following curettage and cementation of giant cell tumor of bone around the knee: long-term follow-up.

BACKGROUND: Giant cell tumor of bone (GCTB) is a benign locally aggressive tumor frequently treated with intralesional curettage and cementation. The aim of this study was to investigate the long-term incidence of arthritic changes following curettage and cementation of GCTB around the knee. MATERIALS AND METHODS: This study was a retrospective review of patients with GCTB around the knee treated with curettage and cementation with a minimum follow-up of 10 years. The functional results were assessed using the Musculoskeletal Tumor Society (MSTS) score. The arthritic changes were classified using the Kellgren-Lawrence (KL) classification system of osteoarthritis. RESULTS: This study included 119 patients, 54 males and 65 females, with a mean age of 29.4 ± 9.2 years. There were 35 (29.4%) patients with pathological fractures. There were 84 (70.6%) patients with de novo lesions and 35 (29.4%) with recurrent lesions. The mean follow-up period was 13.2 ± 3.16 years. The mean MSTS score was 28.5 ± 1.9. Overall, 25 (21%) patients developed variable degrees of arthritis of KL grade 1 (n = 7), KL grade 2 (n = 11), KL grade 3 (n = 4), and KL grade 4 (n = 3). Ten patients showed progression of arthritis during the follow-up period. Age at presentation, gender, presence of pathological fracture, whether the tumor was de novo or recurrent, and tumor location were not associated with arthritis incidence. CONCLUSIONS: Curettage and cementation can be used safely to treat GCTB around the knee. Arthritis of the knee is a possible complication, but mild grades are expected in most cases. There was no association between arthritis incidence and age, gender, pathological fractures, tumor location, or recurrent tumors. LEVEL OF EVIDENCE: Level IV.

Overall and net survival of patients with sarcoma between 2005 and 2010: Results from the French Network of Cancer Registries (FRANCIM).

BACKGROUND: Sarcomas are rare, heterogeneous, ubiquitously localized malignancies with many histologic subtypes and genomic patterns. The survival of patients with sarcoma has rarely been described based on this heterogeneity; therefore, the authors' objective was to estimate survival outcomes in patients who had sarcomas using the 2020 version of the World Health Organization classification of soft tissue and bone tumors. METHODS: Patients older than 15 years who had incident sarcoma diagnosed between 2005 and 2010 were extracted from 14 French population-based cancer registries covering 18% of the French metropolitan population. Vital status for each patient was actively followed up to June 30, 2013. Net survival (NS) was estimated using the unbiased Pohar-Perme method. RESULTS: Overall, 4202 patients were included. NS declined with increasing age at diagnosis. According to topographic groups, large 5-year NS disparities were observed, ranging from 47% among women with gynecologic sarcomas to 89% among patients with skin sarcomas. Patients with soft tissue, bone, and gastrointestinal sarcomas had 5-year NS rates of 53%, 61%, and 70%, respectively. Similar heterogeneity was observed according to histologic subtypes, with 5-year NS ranging from 19% for patients with angiosarcomas to 96% for patients with dermatofibrosarcomas. Patients with sarcoma who displayed missense mutations had a better 5-year NS (74%); those with MDM2-amplified sarcomas had the worst NS (45%). CONCLUSIONS: NS rates in patients with sarcoma are presented here for the first time based on the 2020 World Health Organization classification applied to population-based registry data. Large prognostic heterogeneity was observed based on age, topographic and histologic groups, and genomic alteration profiles, constituting a benchmark for future studies and clinical trials.

Osteosarcoma: A Surveillance, Epidemiology, and End Results program-based analysis from 1975 to 2017.

BACKGROUND: Osteosarcoma is the most common primary bone malignancy. As a rare cancer, population-based studies remain small with limited information on finer demographic categories. Recent studies have reported important genetic differences based on age and ethnicity, and more detailed studies are needed to better understand potentially important osteosarcoma risk groups. METHODS: Incidence and survival rates for 5016 patients with osteosarcoma from the Surveillance, Epidemiology, and End Results (SEER) program (1975-2017) were analyzed by age (0-9, 10-24, 25-59, and >60 years old), race/ethnicity, histologic subtype, stage, and tumor location using SEER*Stat software. RESULTS: For cases 0 to 9 years old, incidence of primary osteosarcoma was similar between the sexes, increased significantly throughout the study period (P < .05), and the 5-year relative survival has steadily increased over time. Blacks had the highest incidence in all aged cases combined and a significant increase in incidence throughout the study period (P < .05). Overall, survival rates for all cases have remained relatively unchanged over recent decades, with worse survival observed in males, American Indian/Alaska Native cases, older patients, metastatic disease, axial tumors, and subsequent osteosarcoma cases. For cases 0 to 24 years old, the incidence of subsequent osteosarcoma increased 3-fold since the 2000s. CONCLUSION: Important differences in osteosarcoma incidence and survival, particularly for the youngest children, ethnic minorities, and subsequent osteosarcoma, are identified. A genetic risk factor may be associated with observed ancestry-specific incidence differences and illustrates the importance of analyzing osteosarcoma by specific age groups and ethnicities to better understand their unique epidemiology and underlying biology. LAY SUMMARY: Osteosarcoma is the most common bone cancer, but still a relatively rare disease, and previous studies have had limited information on finer demographics. Using a large database, osteosarcoma incidence and survival patterns are thoroughly evaluated and important differences, especially for the youngest children, ethnic minorities, and subsequent osteosarcoma cases, are identified.

Secondary malignant neoplasms after bone and soft tissue sarcomas in children, adolescents, and young adults.

BACKGROUND: Increased survival in young sarcoma patients comes along with a higher incidence of second malignant neoplasms (SMNs). The incidence, latency, histiotype, and outcome of these patients were analyzed because this information is essential to design evidence-based long-term follow-up care programs for young sarcoma survivors. METHODS: Patients entered on clinical trials or registered in registries with a primary sarcoma in 1 of the cooperative sarcoma study groups in the framework of the Society for Pediatric Oncology and Hematology (GPOH) were screened for SMNs. Descriptive analysis, the Kaplan-Meier method, the Gray model, the Fine-Gray model, and the Cox regression model were used for the statistical analyses. RESULTS: A total of 159 out of 7079 (2.2%) patients were registered with a SMN. Among them, 104 solid SMNs (65%) and 56 hematologic SMNs (35%) occurred. Median latency from first diagnosis of sarcoma to the diagnosis of SMN was 6.8 years (range, 0-26.7 years). Cumulative incidence of SMN was 8.8% after 30 years. Five-year-survival was 67.1% (95% confidence interval [CI], 66.0-68.2) for the 7079 patients and it was 45.1% (95% CI, 36.2-53.6) after the diagnosis of a SMN (subcohort of n = 159 patients). CONCLUSIONS: There is a remarkable high cumulative incidence of SMNs after bone and soft tissue sarcomas in children, adolescents, and young adults. Therefore, effective transition as well as risk adapted long-term follow-up care programs should be developed and offered to young sarcoma survivors. LAY SUMMARY: Bone sarcomas and soft tissue tumors are rare tumors in children, adolescents, and young adults. The treatment varies, but may comprise chemotherapy, surgery, and/or radiotherapy. Developing a subsequent malignant tumor is a long-term risk for the patients. To better characterize this risk, we analyzed the data of 7079 patients (up to 21 years old) with bone sarcomas or soft tissue tumors. Our findings provide a basis to counsel young sarcoma survivors on their individual risk of subsequent malignant tumors. Moreover, these data can help to establish recommendations for aftercare in young sarcoma survivors.

Sex, racial/ethnic and socioeconomic disparities in patients with metastatic bone disease.

BACKGROUND: We have analyzed sex, race/ethnicity or socioeconomic disparities in the incidence of metastatic bone disease (MBD). METHODS: Patients with the diagnosis of MBD at presentation for five most common primary anatomical sites was extracted from Surveillance, Epidemiology, and End Results Census tract-level dataset. Mean incidence of MBD for different sex, racial/ethnic and socioeconomic groups were compared. RESULTS: The five most common anatomical sites with MBD at presentation include "lung: (n = 59 739), "prostate" (n = 19 732), "breast" (n = 16 244), "renal" (n = 7718) and "colon" (n = 3068). There was an increase in incidence of MBD among cancers originating from prostate (annual percentage change [APC] 4.94), renal (APC 2.55), and colon (APC 3.21) (p < 0.05 for all). Non-Hispanic Blacks had higher incidence of MBD for prostate and breast primary sites (p < 0.001). Non-Hispanic American Indian Alaskan Native had higher incidence of MBD for cancers originating from renal (p < 0.001) and colon (p = 0.049). A higher incidence of MBD was seen in lower socioeconomic status (SES) groups for the selected sites (p < 0.001). CONCLUSIONS: These findings suggest that there are multiple sex-related, racial/ethnic and SES disparities in the incidence of MBD from the 5 most common primary sites. Higher incidence seen among lower SES suggests delay in diagnosis and limited access to screening modalities.

Association between TBXT rs2305089 polymorphism and chordoma in Iranian patients identified by a developed T-ARMS-PCR assay.

BACKGROUND: Chordoma is a locally aggressive bone tumor with a high capability of recurrence. Because chordoma often occurs at critical locations next to neurovascular structures, there is an urgent need to introduce validated biomarkers. T-box transcription factor T (TBXT; OMIM: 601397) plays an important role in the pathogenesis and survival of chordoma cells. METHODS: Herein, we aimed to show whether rs2305089 polymorphism is correlated with chordoma in the Iranian population. In order to detect rs2305089, tetra-primer amplification refractory mutation system-polymerase chain reaction (T-ARMS-PCR) was used. In total, 19 chordoma patients and 108 normal healthy individuals were recruited and screened using T-ARMS-PCR. The results were subsequently validated by Sanger sequencing. RESULTS: The genotype distributions and allele frequencies were significantly different among the patient and healthy groups (p-value <0.05). The A allele of rs2305089 showed a significant positive association with chordoma risk (p-value <0.05). DNA sequencing verified the T-ARMS-PCR results as well. This study demonstrated the association between TBXT rs2305089 and chordoma in an Iranian population using a simple, accurate, and cost-effective T-ARMS-PCR assay. CONCLUSIONS: Our results were in line with those of previous studies showing that TBXT rs2305089 is associated with chordoma development. We also developed an efficient T-ARMS-PCR assay to determine the genotype of rs2305089.