Gastrointestinal stromal tumors: The incidence, prevalence, clinical course, and prognostication in the preimatinib mesylate era

A population-based study in Western Sweden

Authors

  • Bengt Nilsson M.D., Ph.D.,

    1. Department of Surgery, The Lundberg Laboratory for Cancer Research, Sahlgrenska Academy at the University of Göteborg, Göteborg, Sweden
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    • Drs. Lars-Gunnar Kindblom and Bengt Nilsson have acted as members of a Novartis Glivec® Advisory Board.

  • Per Bümming M.D.,

    1. Department of Surgery, The Lundberg Laboratory for Cancer Research, Sahlgrenska Academy at the University of Göteborg, Göteborg, Sweden
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  • Jeanne M. Meis-Kindblom M.D.,

    1. Department of Pathology, The Lundberg Laboratory for Cancer Research, Sahlgrenska Academy at the University of Göteborg, Göteborg, Sweden
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  • Anders Odén Ph.D.,

    1. Department of Pathology, The Lundberg Laboratory for Cancer Research, Sahlgrenska Academy at the University of Göteborg, Göteborg, Sweden
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  • Aydin Dortok M.D.,

    1. Novartis Oncology, Florham Park, New Jersey
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    • Drs. Aydin Dortok, Bengt Gustavsson, and Katarzyna Sablinska are employed by Novartis Pharma.

  • Bengt Gustavsson Ph.D.,

    1. Novartis Oncology, Stockholm, Sweden
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    • Drs. Aydin Dortok, Bengt Gustavsson, and Katarzyna Sablinska are employed by Novartis Pharma.

  • Katarzyna Sablinska M.D., M.P.H.,

    1. Novartis Oncology, Basel, Switzerland
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    • Drs. Aydin Dortok, Bengt Gustavsson, and Katarzyna Sablinska are employed by Novartis Pharma.

  • Lars-Gunnar Kindblom M.D., Ph.D.

    Corresponding author
    1. Department of Pathology, The Lundberg Laboratory for Cancer Research, Sahlgrenska Academy at the University of Göteborg, Göteborg, Sweden
    • Department of Pathology, Sahlgren University Hospital, S41345 Göteborg, Sweden
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    • Drs. Lars-Gunnar Kindblom and Bengt Nilsson have acted as members of a Novartis Glivec® Advisory Board.

    • Fax: (011) 46 31827194


  • Presented in part as invited lectures at the European Society of Medical Oncology meeting in Nice, France, October 20, 2002, and at the American Society of Clinical Oncologists meeting in Chicago, Illinois, June 3, 2003.

Abstract

BACKGROUND

Recent breakthroughs regarding gastrointestinal stromal tumors (GIST) and their pathogenesis have redefined diagnostic criteria and have led to the development of molecularly targeted drug therapy. New treatment options mandate more accurate information regarding the incidence, prevalence, clinical behavior, and prognostic factors of GIST.

METHODS

All patients (n = 1460) who potentially had GIST diagnosed from 1983 to 2000 in western Sweden (population, 1.3–1.6 million) were reviewed, and 288 patients with primary GIST were identified. The incidence and prevalence of GIST were determined, and predictive prognostic factors, including current risk-group stratifications, were analyzed statistically.

RESULTS

Ninety percent of GISTs were detected clinically due to symptoms (69%) or were incidental findings at surgery (21%); the remaining 10% of GISTs were found at autopsy. Forty-four percent of symptomatic, clinically detected GISTs were categorized as high risk (29%) or overtly malignant (15%), with tumor-related deaths occurring in 63% of patients and 83% of patients, respectively (estimated median survival, of 40 months and 16 months, respectively). Tumor-related deaths occurred in only 2 of 170 of patients (1.2%) with very-low-risk, low-risk, or intermediate-risk tumors. The annual incidence of GIST was 14.5 per million. The prevalence of all GIST risk groups was 129 per million (31 per million for the high-risk group and the overtly malignant group).

CONCLUSIONS

GIST has been under recognized: Its incidence, prevalence, and clinical aggressiveness also have been underestimated. Currently existing risk-group stratification systems based on tumor size and mitotic rate delineate GIST patients who have a poor prognosis. Prognostication in patients with GIST can be refined using a proposed risk score based solely on tumor size and proliferative index. Cancer 2005. © 2005 American Cancer Society.

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