Incidence and survival of primary bone sarcoma diagnosed in England between 1996 and 2020; An analysis from the National Disease Registration Service.

BACKGROUND: Population-based studies detailing the incidence and survival of patients with primary bone sarcomas (PBS) are limited and often group morphologies and primary sites together. We examined incidence and outcomes for PBS in England and evaluated changes over time, within and across groups. METHODS: All patients diagnosed with PBS in England between 1996 and 2020 were identified from the National Cancer Registration Dataset. Age-specific incidence rates and five-year average age-standardised incidence rates (ASRs) were calculated using the 2013 European Standard Population. Five-year relative survival (RS) was estimated by diagnosis period, histological subtype and patient subgroup. Excess mortality models were used to assess survival trends over time. RESULTS: Between 1996 and 2020, 12,282 patients were diagnosed with PBS (mean 491 annually). Chondrosarcoma (31%) and osteosarcoma (28%) were the most common subtypes. Overall PBS incidence changed little over the study period; however, incidence increased for chondrosarcoma and chordoma and decreased for osteosarcoma. Five-year RS for all PBS improved from 52% in 1998-2002-65% in 2013-2017. Significant survival improvements were observed for chondrosarcoma, chordoma and osteosarcoma. In Ewing sarcoma, survival improved particularly among patients with pelvic tumours. Improvements remained evident after adjustment for measured case mix. Younger age, female sex and extremity primary site were associated with better survival in several subtypes. CONCLUSIONS: This national population-based analysis provides contemporary estimates of PBS incidence and survival in England. Despite the rarity and heterogeneity of these tumours, survival improved for PBS overall and for several clinically important patient subgroups. These findings provide an important benchmark for international comparisons and ongoing evaluation of sarcoma services and outcomes.