Population-Based Epidemiology and Mortality of Small Malignant Gastrointestinal Stromal Tumors in the USA
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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.
KeywordsICC 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).
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.
- 4.Ma GL, Murphy JD, Martinez ME, Sicklick JK. Epidemiology of gastrointestinal stromal tumors in the era of histology codes: results of a population-based study. Cancer Epidemiology, Biomarkers & Prevention: a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology. 2015;24(1):298–302.CrossRefGoogle Scholar
- 17.Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER*Stat Database: released April 2014 (updated 5/7/2014), based on the November 2013 submission.
- 18.Day JC. Population projections of the United States by age, sex, race, and Hispanic origin: 1995 to 2050. U.S. Bureau of the Census, Current Population Reports, P25-1130, U.S. Government Printing Office Washington, DC, 1996.Google Scholar
- 20.Murphy JD, Ma GL, Baumgartner JM, et al. Increased risk of additional cancers among patients with gastrointestinal stromal tumors: a population-based study. Cancer. 2015.Google Scholar
- 23.Choi AH, Hamner JB, Merchant SJ, et al. Underreporting of gastrointestinal stromal tumors: is the true incidence being captured? Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract. 2015.Google Scholar
- 24.Miettinen M, Lasota J. Gastrointestinal stromal tumors: review on morphology, molecular pathology, prognosis, and differential diagnosis. Archives of Pathology & Laboratory Medicine. 2006;130(10):1466–1478.Google Scholar