OBJECTIVES: GIST is the most common sarcoma of the GI tract, yet estimates of incidence vary greatly. The objective of this study was to review existing literature to identify published incidence rates for GIST in Europe, to enable the assessment of the financial burden to society.

METHODS: To understand the incidence of GIST across Europe, a systematic literature search was conducted utilising these search platforms: Embase, Medline, International Pharmaceutical Abstracts, PubMed Central, and Directory of Open Access Journals databases. The search terms used included gastrointestinal stromal tumour; ‘GIST’; gastrointestinal stromal neoplasm; gastrointestinal stromal sarcoma AND incidence; occurrence; epidemiology; frequency. The search was completed by a manual review of the articles identified. Study selection and data extraction were performed independently by 2 individuals. Data extracted included the region where the study was conducted, population examined, years included, reported number of GIST cases, estimated incidence per million persons, median age of diagnosis, and the estimated incidence. Inclusion criteria: Publication with original analysis including explanation of how GIST patients were identified.

RESULTS: The literature search identified 120 publications of potential interest of which 23 met inclusion criteria. In those studies, European crude GIST incidence rates ranged from 1.0–21 per-million-per-year (pmy). Post-2010, age-standardised rates ranged from 10–19.4 pmy. Some studies further stratified incidence by gender. Higher quality studies included post 2010 data, use of ICD-10 codes and large populations. The values for these studies centered around a crude rate of 12–15 pmy.

CONCLUSIONS: Incidence rates vary from country to country and even intranational. The highest quality studies are national studies from France, Germany, and the Netherlands. After correction for age progression in 2020, the true value today is likely to be 13–16 pmy. Rates appear to be increasing with time. There is no clear pattern of difference due to gender.