Journal of Gastrointestinal Surgery

, Volume 20, Issue 6, pp 1132–1140 | Cite as

Population-Based Epidemiology and Mortality of Small Malignant Gastrointestinal Stromal Tumors in the USA

  • Taylor M. Coe
  • Katherine E. Fero
  • Paul T. Fanta
  • Robert J. Mallory
  • Chih-Min Tang
  • James D. Murphy
  • Jason K. Sicklick
Original Article


Background and Aims

Gastrointestinal stromal tumors (GISTs) have significant variability in size and malignant behavior. Our current understanding is limited to pathological analyses, autopsy studies, and small case series. The aim of the current study is to define the risk factors, incidence, and mortality rates of GIST <2 cm in the National Cancer Institute’s Surveillance, Epidemiology, and End Results database.


Patients with histologically confirmed malignant GIST <2 cm were studied from 2001 to 2011. GIST was defined by GI tumor site codes and GIST-specific histology codes.


We identified 378 patients with GIST <2 cm. The average age at diagnosis was 64.0 years with equal sex distribution. The most common tumor location was the stomach (62.2 %), followed by the small intestine (23.3 %), colon (5.6 %), and rectum (3.4 %). Most patients had localized disease (79.4 %), but 11.4 % had regional/distant metastatic disease. The annual incidence rate was 4.2 per 10,000,000 (10M). This was the highest among Blacks (7.6 per 10M). Among patients with GIST and no additional cancers, the 5-year GIST-specific mortality was 12.9 %. Moreover, there was a significantly increased 5-year GIST-specific mortality in those patients who had regionally advanced (34.0 %) or metastatic GIST (34.3 %), as compared to those patients with localized GIST (5.6 %).


This study represents the first population-based analysis of malignant GIST <2 cm. While quite rare, these tumors have an underappreciated disease-specific mortality. Further studies are needed to define the underlying reasons for the identified racial differences, to develop novel risk assessment schema for patients with these small tumors, and to determine appropriate indications for resection and/or medical therapy.


ICC hyperplasia Minute GIST GIST tumorlet Small GIST 





European Society of Medical Oncology


Gastrointestinal stromal tumor


Interstitial cells of Cajal


National Comprehensive Cancer Network


Surveillance, Epidemiology and End Results



The authors acknowledge the support of NIH TL1 TR001443 (K.E. Fero), NIH KL2 RR031978 (J.D. Murphy), and NIH K08 CA168999 (J.K. Sicklick).

Authors’ Contributions

There were no contributions to this manuscript by persons other than the authors. TMC and JKS were responsible for the study concept and design; TMC, KEF, and JDM for the acquisition of data; TMC, KEF, JDM, and JKS for the analysis and interpretation of data; TMC, KEF, CMT, JDM, and JKS for the drafting of the manuscript; TMC, KEF, PTF, RJM, CMT, JDM, and JKS for the critical revision of the manuscript for important intellectual content; KEF, JDM, and JKS for the statistical analysis; TMC, KEF, and JDM for the obtained funding; and JKS for the study supervision.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no competing interests.

Supplementary material

11605_2016_3134_MOESM1_ESM.docx (70 kb)
ESM 1 (DOCX 70 kb)


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Copyright information

© The Society for Surgery of the Alimentary Tract 2016

Authors and Affiliations

  • Taylor M. Coe
    • 1
  • Katherine E. Fero
    • 1
  • Paul T. Fanta
    • 1
    • 2
  • Robert J. Mallory
    • 3
  • Chih-Min Tang
    • 3
  • James D. Murphy
    • 1
    • 4
  • Jason K. Sicklick
    • 1
    • 3
  1. 1.School of MedicineUniversity of CaliforniaLa JollaUSA
  2. 2.Division of Hematology and Oncology, Moores Cancer CenterUniversity of California, San DiegoLa JollaUSA
  3. 3.Division of Surgical Oncology and Department of Surgery, Moores Cancer CenterUniversity of California, San DiegoLa JollaUSA
  4. 4.Department of Radiation Medicine and Applied Sciences, Moores Cancer CenterUniversity of California, San DiegoLa JollaUSA

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