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Diagnosis of Gastrointestinal Stromal Tumors:A Consensus Approach

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston MA

Jules J. Berman, MD, PhD

Cancer Diagnosis Program, National Cancer Institute, National Institutes of Health, Bethesda, MD

Christopher Corless, MD, PhD

Department of Pathology, Oregon Health Sciences University, Portland, OR

Fred Gorstein, MD

Department of Pathology, Thomas Jefferson University, Philadelphia, PA

Jerzy Lasota, MD, PhD

Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC

B. Jack Longley, MD

Department of Pathology and Dermatology, College of Physicians and Surgeons, Columbia University, New York, NY

Markku Miettinen, MD

Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC

Timothy J. O'Leary, MD, PhD

Department of Cellular Pathology and Genetics, Armed Forces Institute of Pathology, Washington, DC

Helen Remotti, MD

Department of Gastrointestinal Pathology, Armed Forces Institute of Pathology, Washington, DC

Brian P. Rubin, MD, PhD

Department of Pathology, University of Washington Medical Center, Seattle, WA

Barry Shmookler, MD

Department of Pathology, Suburban Hospital, Bethesda, MD

Leslie H. Sobin, MD

Department of Gastrointestinal Pathology, Armed Forces Institute of Pathology, Washington, DC

Sharon W. Weiss, MD

Department of Pathology & Laboratory Medicine, Emory UniversityHospital, Atlanta, GA

As a result of major recent advances in understanding the biology of gastrointestinal stromal tumors (GIST), specifically recognition of the central role of activating KIT mutations and associated KIT protein expression in these lesions, and thedevelop-ment of novel and effective therapy for GISTs using thereceptor tyrosine kinase in hibitor STI-571, these tumors have become the focus of considerable attention among pathologists, clinicians, and patients. Stromal/mesenchymal tumors of the gastrointestinal tract have long been a sourceof confusion and controversy with regard to classification, line(s) of differentiation, and prognostication. Characterization of the KIT pathway and its phenotypic implications has helped to resolve some but not all of these issues. Given the now critical role of accurate and reproducible pathologic diagnosis in ensuring appropriate treatment for patients with GIST, the National Institutes of Health (NIH) convened a GIST workshop in April 2001 with the goal of developing a consensus approach to diagnosis and morphologic prognostication. Key elements of the consensus, as described herein, are the defining role of KIT immunopositivity indiagnosis and a proposed scheme for estimating metastatic riskin these lesions, based on tumor size and mitotic count, recognizing that it is probably unwise to use the definitive term benign for any GIST, at least at the present time.

Key Words: gastrointestinal stromal tumor • GIST • sarcoma • diagnosis • guidelines • KIT • CD117

International Journal of Surgical Pathology, Vol. 10, No. 2, 81-89 (2002)
DOI: 10.1177/106689690201000201


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