OBJECTIVES: Soft tissue sarcomas (STS) are rare malignant tumours, comprising over 50 histological variants. The epidemiological evidence is available for individual sub-types of STS. However, limited evidence is available for overall STS (including all the sub-types). The objective of this research was to understand the epidemiology of overall STS globally.

METHODS: A literature review was performed in English language (January 2008 - December 2018) using biomedical databases (MEDLINE^®, EMBASE^®, Google Scholar) and other sources (conference proceedings, registries). Studies reporting epidemiology for two or more STS sub-types were included, while those reporting individual STS sub-types were excluded. The quality assessment of relevant studies was conducted, and low-quality studies were excluded from the analysis. Incidence, prevalence, mortality and survival data was extracted from observational studies and registries.

RESULTS: 49 studies reporting the epidemiological data for overall STS were identified. The studies included patients covering age groups from 0 to ≥80 years, and were conducted across the globe (US, Canada, Europe, Asia-Pacific and Middle East). Incidence and prevalence of STS varied substantially worldwide. The incidence rate (IR) ranged from 0.002 - 3.33 per 100,000, Canada having the highest and India the lowest IR. The global IR in children (aged 0 - 14 years) varied from 0.06 - 1.84 per 100,000. Prevalence ranged from 0.002 - 3.74 per 100,000 globally, with highest in Ireland and lowest in Russia. The mortality rate varied from 4.2% - 13.4% in different regions. Survival rate was higher in females. STS is more common in infants and children as compared to adults. Varying classification for STS subtypes were observed across geographies.

CONCLUSIONS: It is evident from the current review that epidemiological data is rare which establishes STS as an orphan disease. The incidence, prevalence and mortality data variation is attributable to case ascertainment criteria and demographic factors.