Global epidemiology of gastrointestinal stromal tumours (GIST): A systematic review of population-based cohort studies

•Gastrointestinal stromal tumours (GISTs) incidence shows variation globally.•High and low incidence outliers are found across continents.•Stomach and small bowel are consistently the most frequent locations of GIST.•One in five GISTs are discovered incidentally.•The low number of small, low-risk ca...

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Published inCancer epidemiology Vol. 40; pp. 39 - 46
Main Authors Søreide, Kjetil, Sandvik, Oddvar M., Søreide, Jon Arne, Giljaca, Vanja, Jureckova, Andrea, Bulusu, V. Ramesh
Format Journal Article
LanguageEnglish
Published Netherlands Elsevier Ltd 01.02.2016
Elsevier Limited
Subjects
Online AccessGet full text
ISSN1877-7821
1877-783X
DOI10.1016/j.canep.2015.10.031

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Abstract •Gastrointestinal stromal tumours (GISTs) incidence shows variation globally.•High and low incidence outliers are found across continents.•Stomach and small bowel are consistently the most frequent locations of GIST.•One in five GISTs are discovered incidentally.•The low number of small, low-risk category tumours may suggest an underreporting. Gastrointestinal stromal tumours (GISTs) are rare, yet the most common mesenchymal tumour within the digestive tract. Lack of diagnostic criteria and no specific code in the ICD system has prevented epidemiological evaluation except from overt malignant cases in the past. A global estimate of incidence and disease patterns has thus not been available. A systematic literature search of all available population-based studies on GIST published between January 2000 and December 2014 were reviewed. Descriptive epidemiological data are presented. The search found 29 studies of more than 13,550 patients from 19 countries that reported sufficient data for regional or national population-based statistics. Age at diagnosis ranged from 10 to 100 years, with median age being mid 60s across most studies. Gender distribution was equal across studies. On average, 18% of patients had an incidental diagnosis (range from 5% to 40%). Anatomical location of primary tumour in 9747 GISTs demonstrated gastric location as the most frequent (55.6%) followed by small bowel (31.8%), colorectal (6.0%), other/various location (5.5%) and oesophagus (0.7%). Most studies reported incidence at 10–15 per million per year. Notably, lowest incidence was in China (Shanxi province) with 4.3 per million per year. Highest incidence rates were reported also from China (Hong Kong and Shanghai areas), and in Taiwan and Norway (Northern part), with up to 19–22 per million per year. Epidemiology of GIST demonstrates some consistent features across geographical regions. Whether the reported extreme differences in incidence reflect real variation in population risk warrants further investigation.
AbstractList Highlights • Gastrointestinal stromal tumours (GISTs) incidence shows variation globally. • High and low incidence outliers are found across continents. • Stomach and small bowel are consistently the most frequent locations of GIST. • One in five GISTs are discovered incidentally. • The low number of small, low-risk category tumours may suggest an underreporting.
•Gastrointestinal stromal tumours (GISTs) incidence shows variation globally.•High and low incidence outliers are found across continents.•Stomach and small bowel are consistently the most frequent locations of GIST.•One in five GISTs are discovered incidentally.•The low number of small, low-risk category tumours may suggest an underreporting. Gastrointestinal stromal tumours (GISTs) are rare, yet the most common mesenchymal tumour within the digestive tract. Lack of diagnostic criteria and no specific code in the ICD system has prevented epidemiological evaluation except from overt malignant cases in the past. A global estimate of incidence and disease patterns has thus not been available. A systematic literature search of all available population-based studies on GIST published between January 2000 and December 2014 were reviewed. Descriptive epidemiological data are presented. The search found 29 studies of more than 13,550 patients from 19 countries that reported sufficient data for regional or national population-based statistics. Age at diagnosis ranged from 10 to 100 years, with median age being mid 60s across most studies. Gender distribution was equal across studies. On average, 18% of patients had an incidental diagnosis (range from 5% to 40%). Anatomical location of primary tumour in 9747 GISTs demonstrated gastric location as the most frequent (55.6%) followed by small bowel (31.8%), colorectal (6.0%), other/various location (5.5%) and oesophagus (0.7%). Most studies reported incidence at 10–15 per million per year. Notably, lowest incidence was in China (Shanxi province) with 4.3 per million per year. Highest incidence rates were reported also from China (Hong Kong and Shanghai areas), and in Taiwan and Norway (Northern part), with up to 19–22 per million per year. Epidemiology of GIST demonstrates some consistent features across geographical regions. Whether the reported extreme differences in incidence reflect real variation in population risk warrants further investigation.
Gastrointestinal stromal tumours (GISTs) are rare, yet the most common mesenchymal tumour within the digestive tract. Lack of diagnostic criteria and no specific code in the ICD system has prevented epidemiological evaluation except from overt malignant cases in the past. A global estimate of incidence and disease patterns has thus not been available. A systematic literature search of all available population-based studies on GIST published between January 2000 and December 2014 were reviewed. Descriptive epidemiological data are presented. The search found 29 studies of more than 13,550 patients from 19 countries that reported sufficient data for regional or national population-based statistics. Age at diagnosis ranged from 10 to 100 years, with median age being mid 60s across most studies. Gender distribution was equal across studies. On average, 18% of patients had an incidental diagnosis (range from 5% to 40%). Anatomical location of primary tumour in 9747 GISTs demonstrated gastric location as the most frequent (55.6%) followed by small bowel (31.8%), colorectal (6.0%), other/various location (5.5%) and oesophagus (0.7%). Most studies reported incidence at 10-15 per million per year. Notably, lowest incidence was in China (Shanxi province) with 4.3 per million per year. Highest incidence rates were reported also from China (Hong Kong and Shanghai areas), and in Taiwan and Norway (Northern part), with up to 19-22 per million per year. Epidemiology of GIST demonstrates some consistent features across geographical regions. Whether the reported extreme differences in incidence reflect real variation in population risk warrants further investigation.
Background Gastrointestinal stromal tumours (GISTs) are rare, yet the most common mesenchymal tumour within the digestive tract. Lack of diagnostic criteria and no specific code in the ICD system has prevented epidemiological evaluation except from overt malignant cases in the past. A global estimate of incidence and disease patterns has thus not been available. Methods A systematic literature search of all available population-based studies on GIST published between January 2000 and December 2014 were reviewed. Descriptive epidemiological data are presented. Results The search found 29 studies of more than 13,550 patients from 19 countries that reported sufficient data for regional or national population-based statistics. Age at diagnosis ranged from 10 to 100 years, with median age being mid 60s across most studies. Gender distribution was equal across studies. On average, 18% of patients had an incidental diagnosis (range from 5% to 40%). Anatomical location of primary tumour in 9747 GISTs demonstrated gastric location as the most frequent (55.6%) followed by small bowel (31.8%), colorectal (6.0%), other/various location (5.5%) and oesophagus (0.7%). Most studies reported incidence at 10-15 per million per year. Notably, lowest incidence was in China (Shanxi province) with 4.3 per million per year. Highest incidence rates were reported also from China (Hong Kong and Shanghai areas), and in Taiwan and Norway (Northern part), with up to 19-22 per million per year. Conclusions Epidemiology of GIST demonstrates some consistent features across geographical regions. Whether the reported extreme differences in incidence reflect real variation in population risk warrants further investigation.
BACKGROUNDGastrointestinal stromal tumours (GISTs) are rare, yet the most common mesenchymal tumour within the digestive tract. Lack of diagnostic criteria and no specific code in the ICD system has prevented epidemiological evaluation except from overt malignant cases in the past. A global estimate of incidence and disease patterns has thus not been available.METHODSA systematic literature search of all available population-based studies on GIST published between January 2000 and December 2014 were reviewed. Descriptive epidemiological data are presented.RESULTSThe search found 29 studies of more than 13,550 patients from 19 countries that reported sufficient data for regional or national population-based statistics. Age at diagnosis ranged from 10 to 100 years, with median age being mid 60s across most studies. Gender distribution was equal across studies. On average, 18% of patients had an incidental diagnosis (range from 5% to 40%). Anatomical location of primary tumour in 9747 GISTs demonstrated gastric location as the most frequent (55.6%) followed by small bowel (31.8%), colorectal (6.0%), other/various location (5.5%) and oesophagus (0.7%). Most studies reported incidence at 10-15 per million per year. Notably, lowest incidence was in China (Shanxi province) with 4.3 per million per year. Highest incidence rates were reported also from China (Hong Kong and Shanghai areas), and in Taiwan and Norway (Northern part), with up to 19-22 per million per year.CONCLUSIONSEpidemiology of GIST demonstrates some consistent features across geographical regions. Whether the reported extreme differences in incidence reflect real variation in population risk warrants further investigation.
Author Søreide, Kjetil
Sandvik, Oddvar M.
Jureckova, Andrea
Bulusu, V. Ramesh
Søreide, Jon Arne
Giljaca, Vanja
Author_xml – sequence: 1
  givenname: Kjetil
  surname: Søreide
  fullname: Søreide, Kjetil
  email: ksoreide@mac.com
  organization: Department of Gastrointestinal Surgery, Stavanger University Hospital, Stavanger, Norway
– sequence: 2
  givenname: Oddvar M.
  surname: Sandvik
  fullname: Sandvik, Oddvar M.
  organization: Department of Gastrointestinal Surgery, Stavanger University Hospital, Stavanger, Norway
– sequence: 3
  givenname: Jon Arne
  surname: Søreide
  fullname: Søreide, Jon Arne
  organization: Department of Gastrointestinal Surgery, Stavanger University Hospital, Stavanger, Norway
– sequence: 4
  givenname: Vanja
  orcidid: 0000-0002-1719-0424
  surname: Giljaca
  fullname: Giljaca, Vanja
  organization: Department of Internal medicine, Division of Gastroenterology, University Hospital Center Rijeka, Croatia
– sequence: 5
  givenname: Andrea
  surname: Jureckova
  fullname: Jureckova, Andrea
  organization: Masaryk Memorial Cancer Institute Brno, Czech Republic
– sequence: 6
  givenname: V. Ramesh
  surname: Bulusu
  fullname: Bulusu, V. Ramesh
  organization: Oncology Centre, Cambridge University Hospitals, Cambridge, UK
BackLink https://www.ncbi.nlm.nih.gov/pubmed/26618334$$D View this record in MEDLINE/PubMed
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IngestDate Fri Jul 11 16:16:34 EDT 2025
Sat Jul 26 03:12:21 EDT 2025
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Keywords GIST
Risk category
Registry
Epidemiology
Distribution
Incidence
Language English
License http://creativecommons.org/licenses/by-nc-nd/4.0
Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.
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Snippet •Gastrointestinal stromal tumours (GISTs) incidence shows variation globally.•High and low incidence outliers are found across continents.•Stomach and small...
Highlights • Gastrointestinal stromal tumours (GISTs) incidence shows variation globally. • High and low incidence outliers are found across continents. •...
Gastrointestinal stromal tumours (GISTs) are rare, yet the most common mesenchymal tumour within the digestive tract. Lack of diagnostic criteria and no...
Background Gastrointestinal stromal tumours (GISTs) are rare, yet the most common mesenchymal tumour within the digestive tract. Lack of diagnostic criteria...
BACKGROUNDGastrointestinal stromal tumours (GISTs) are rare, yet the most common mesenchymal tumour within the digestive tract. Lack of diagnostic criteria and...
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SubjectTerms Adolescent
Adult
Aged
Cancer
Distribution
Epidemiology
Female
Gastrointestinal Stromal Tumors - epidemiology
GIST
Hematology, Oncology and Palliative Medicine
Humans
Incidence
Internal Medicine
Male
Metastasis
Middle Aged
Population
Registry
Risk Assessment
Risk category
Studies
Tumors
Young Adult
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Title Global epidemiology of gastrointestinal stromal tumours (GIST): A systematic review of population-based cohort studies
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