Ewing and chondrosaroma are two forms of primary bone cancer. The few clinical studies that are published in recent decades show disappointing results. With a 5 year survival of 10% for inoperable... Show moreEwing and chondrosaroma are two forms of primary bone cancer. The few clinical studies that are published in recent decades show disappointing results. With a 5 year survival of 10% for inoperable Ewing sarcoma and 2% for inoperable chondrosarcoma patients the outcome is poor. If a patient has not responded to standard treatment, they are often treated with combinations based on expert opinion of the treating physician. These treatment combinations have not been investigated in studies because of the small patient numbers which makes starting a study very difficult and time-consuming. One combination is of the chemotherapeutic agents etoposide with carboplatin or cisplatin. Analysis of the survival data of patients treated with these combinations shows that they are comparable to other standard treatment options. New non-chemotherapy treatment options are currently being investigated. Examples are the IGF-1R and PARP route. The current knowledge of both routes shows that disregulation causes malignant transformation and / or disease progression. Inhibition of these routes is therefore a possible treatment. In clinical studies, the results are not positive for all patients. This is due to incorrect patient selection and not being tested in combination with already used treatments. Show less
Aims: 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... Show moreAims: 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.Patients and methods: 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.Results: 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).Conclusions: 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. Show less
