Diagnosis of gastrointestinal stromal tumors: a consensus approach.
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 the development of novel and effective therapy for GISTs using the receptor tyrosine kinase inhibitor 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 source of 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 in diagnosis and a proposed scheme for estimating metastatic risk in 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.
Fletcher
Christopher D M
CD
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston MA 02115, USA.
Berman
Jules J
JJ
Corless
Christopher
C
Gorstein
Fred
F
Lasota
Jerzy
J
Longley
B Jack
BJ
Miettinen
Markku
M
O'Leary
Timothy J
TJ
Remotti
Helen
H
Rubin
Brian P
BP
Shmookler
Barry
B
Sobin
Leslie H
LH
Weiss
Sharon W
SW
D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides; D014408:Biomarkers, Tumor / Q000378:metabolism; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism; D006801:Humans; D000068877:Imatinib Mesylate; D009154:Mutation; D015513:Oncogene Proteins; D010879:Piperazines / Q000627:therapeutic use; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics / Q000378:metabolism; D011743:Pyrimidines / Q000627:therapeutic use; D017154:Stromal Cells / Q000473:pathology
D000970:Antineoplastic Agents; D001549:Benzamides; D014408:Biomarkers, Tumor; D015513:Oncogene Proteins; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D011505:Protein-Tyrosine Kinases; D019009:Proto-Oncogene Proteins c-kit