Synovial sarcoma (SS) is a rare and aggressive soft tissue sarcoma which primarily affects the extremities of arms and legs. It also occurs in head and neck, trunk, lungs and pleura. Brennan et al. (2016) reported a SS incidence rate of 1.4 per million in 2009 in England. There are no other population-based studies providing the burden of SS in Europe (EU). In addition, the age- and gender- specific distribution of SS patient population have not been published. The objective of this study was to estimate the incidence, prevalence and mortality of SS in 2019 for the EU28 countries.
Data from the United States (US) Surveillance, Epidemiology, and End Results (SEER) 18 Registries, Nov. 2018 (2000-2016) was analyzed using SEER*Stat software (v8.3.5) to calculate the incidence, prevalence and mortality of SS (overall, and by age groups and gender) for 2019. The estimated age-standardized incidence rate of SS, based on this SEER analysis in the US, was similar to that reported by Brennan et al. (2016). These rates were applied to the overall, and age- and gender-specific 2019 EU28 population to estimate the burden of SS in EU28.
In the EU28 countries in 2019, there will be an estimated 993 incident cases of SS, 3,550 prevalent patients and 520 deaths. Most (74%) of the prevalent patients with SS will be 20-64 years old, and only 17% will be 65 years and older. Approximately 40% of patients newly diagnosed with SS will be of ages 20-44 years, and 32% of ages 45-64 years. There will be a low occurrence (<10%) of SS in the pediatric population ages 0-19 years, but more frequent in males for this age group. The incidence and prevalence of SS will not vary between males and females. However, the estimated mortality among adults will be higher in males than females. Accounting for the differences in incidence, among the pediatric SS patients, the mortality in females will be higher than males. 73% of the SS mortality in 2019 will be observed in 20-64 year old patients.
SS is a rare disease. Life expectancy of patients with SS is lower than the general population in EU28 due to high mortality in the 20-64 year age group. SS also occurs among children. Population-based studies are needed to better characterize SS patient population for improving patient care and survival in EU28.
Clinical trial identification
Legal entity responsible for the study
N. Joseph: Shareholder / Stockholder / Stock options, Full / Part-time employment: GlaxoSmithKline. S. St. Laurent: Shareholder / Stockholder / Stock options, Full / Part-time employment: GlaxoSmithKline. S. Zheng: Full / Part-time employment: GlaxoSmithKline. H. Stirnadel-Farrant: Shareholder / Stockholder / Stock options, Full / Part-time employment: GlaxoSmithKline. C. Dharmani: Full / Part-time employment: GlaxoSmithKline.