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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

Abstract

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.

Methods

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.

Results

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 %).

Conclusions

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.

Keywords

ICC hyperplasia Minute GIST GIST tumorlet Small GIST 

Abbreviations

10M

10,000,000

ESMO

European Society of Medical Oncology

GIST

Gastrointestinal stromal tumor

ICC

Interstitial cells of Cajal

NCCN

National Comprehensive Cancer Network

SEER

Surveillance, Epidemiology and End Results

Notes

Acknowledgments

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)

References

  1. 1.
    Hirota S, Isozaki K, Moriyama Y, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science. 1998;279(5350):577–580.PubMedCrossRefGoogle Scholar
  2. 2.
    DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival. Ann Surg. 2000;231(1):51–58.PubMedPubMedCentralCrossRefGoogle Scholar
  3. 3.
    Roggin KK, Posner MC. Modern treatment of gastric gastrointestinal stromal tumors. World Journal of Gastroenterology: WJG. 2012;18(46):6720–6728.PubMedPubMedCentralCrossRefGoogle Scholar
  4. 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
  5. 5.
    Chetty R. Small and microscopically detected gastrointestinal stromal tumours: an overview. Pathology. 2008;40(1):9–12.PubMedCrossRefGoogle Scholar
  6. 6.
    Dirnhofer S, Leyvraz S. Current standards and progress in understanding and treatment of GIST. Swiss Medical Weekly. 2009;139(7–8):90–102.PubMedGoogle Scholar
  7. 7.
    Rammohan A, Sathyanesan J, Rajendran K, et al. A gist of gastrointestinal stromal tumors: a review. World Journal of Gastrointestinal Oncology. 2013;5(6):102–112.PubMedPubMedCentralCrossRefGoogle Scholar
  8. 8.
    Demetri GD, von Mehren M, Antonescu CR, et al. NCCN Task Force report: update on the management of patients with gastrointestinal stromal tumors. Journal of the National Comprehensive Cancer Network: JNCCN. 2010;8 Suppl 2:S1-41; quiz S42-44.PubMedPubMedCentralGoogle Scholar
  9. 9.
    Miettinen M, Lasota J. Gastrointestinal stromal tumors. Gastroenterology Clinics of North America. 2013;42(2):399–415.PubMedPubMedCentralCrossRefGoogle Scholar
  10. 10.
    Scherubl H, Faiss S, Knoefel WT, Wardelmann E. Management of early asymptomatic gastrointestinal stromal tumors of the stomach. World Journal of Gastrointestinal Endoscopy. 2014;6(7):266–271.PubMedPubMedCentralCrossRefGoogle Scholar
  11. 11.
    Abraham SC, Krasinskas AM, Hofstetter WL, Swisher SG, Wu TT. “Seedling” mesenchymal tumors (gastrointestinal stromal tumors and leiomyomas) are common incidental tumors of the esophagogastric junction. The American Journal of Surgical Pathology. 2007;31(11):1629–1635.PubMedCrossRefGoogle Scholar
  12. 12.
    Agaimy A, Wunsch PH, Hofstaedter F, et al. Minute gastric sclerosing stromal tumors (GIST tumorlets) are common in adults and frequently show c-KIT mutations. The American Journal of Surgical Pathology. 2007;31(1):113–120.PubMedCrossRefGoogle Scholar
  13. 13.
    Kawanowa K, Sakuma Y, Sakurai S, et al. High incidence of microscopic gastrointestinal stromal tumors in the stomach. Hum Pathol. 2006;37(12):1527–1535.PubMedCrossRefGoogle Scholar
  14. 14.
    Chan CHF, Cools-Lartigue J, Marcus VA, Feldman LS, Ferri LE. The impact of incidental gastrointestinal stromal tumours on patients undergoing resection of upper gastrointestinal neoplasms. Canadian Journal of Surgery. 2012;55(6):366–370.PubMedCentralCrossRefGoogle Scholar
  15. 15.
    Rossi S, Gasparotto D, Toffolatti L, et al. Molecular and clinicopathologic characterization of gastrointestinal stromal tumors (GISTs) of small size. The American Journal of Surgical Pathology. 2010;34(10):1480–1491.PubMedCrossRefGoogle Scholar
  16. 16.
    Tran T, Davila JA, El-Serag HB. The epidemiology of malignant gastrointestinal stromal tumors: an analysis of 1,458 cases from 1992 to 2000. Am J Gastroenterol. 2005;100(1):162–168.PubMedCrossRefGoogle Scholar
  17. 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. 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
  19. 19.
    Tiwari RC, Clegg LX, Zou Z. Efficient interval estimation for age-adjusted cancer rates. Statistical Methods in Medical Research. 2006;15(6):547–569.PubMedCrossRefGoogle Scholar
  20. 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
  21. 21.
    Miettinen M, Sobin LH, Lasota J. Gastrointestinal stromal tumors of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 1765 cases with long-term follow-up. The American Journal of Surgical Pathology. 2005;29(1):52–68.PubMedCrossRefGoogle Scholar
  22. 22.
    Call J, Walentas CD, Eickhoff JC, Scherzer N. Survival of gastrointestinal stromal tumor patients in the imatinib era: life raft group observational registry. BMC Cancer. 2012;12:90.PubMedPubMedCentralCrossRefGoogle Scholar
  23. 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. 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
  25. 25.
    Patil DT, Rubin BP. Genetics of gastrointestinal stromal tumors: a heterogeneous family of tumors? Surgical Pathology Clinics. 2015;8(3):515–524.PubMedCrossRefGoogle Scholar
  26. 26.
    Rossi S, Gasparotto D, Miceli R, et al. KIT, PDGFRA, and BRAF mutational spectrum impacts on the natural history of imatinib-naive localized GIST: a population-based study. The American Journal of Surgical Pathology. 2015;39(7):922–930.PubMedCrossRefGoogle Scholar
  27. 27.
    Kramer K, Knippschild U, Mayer B, et al. Impact of age and gender on tumor related prognosis in gastrointestinal stromal tumors (GIST). BMC Cancer. 2015;15:57.PubMedPubMedCentralCrossRefGoogle Scholar

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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