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Diagnosis of gastrointestinal stromal tumors: a consensus approach
Christopher D M Fletcher1, Jules J Berman, Christopher Corless
1Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston MA 02115, USA.
Abstract:
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.
Insights
Recent advances in gastrointestinal stromal tumors (GIST) biology, particularly KIT mutations, have led to new therapies. A consensus approach for GIST diagnosis and prognostication, focusing on KIT immunopositivity and risk factors, is now established.
Area of Science:
- Oncology
- Gastroenterology
- Pathology
Background:
- Gastrointestinal stromal tumors (GIST) classification and prognostication were historically challenging.
- Recent discoveries highlight the role of KIT mutations and KIT protein expression in GIST pathogenesis.
- The development of targeted therapy, such as STI-571, has increased focus on GIST.
Framework:
- A National Institutes of Health (NIH) workshop convened in April 2001 to establish diagnostic and prognostic consensus for GIST.
- The consensus emphasizes the critical role of accurate and reproducible pathologic diagnosis for effective patient treatment.
- Key elements include defining KIT immunopositivity for diagnosis and a risk stratification scheme.
Implementation:
- Diagnosis of GIST is now critically dependent on demonstrating KIT immunopositivity.
- A proposed scheme for estimating metastatic risk in GIST is based on tumor size and mitotic count.
- The consensus advises caution in using the term 'benign' for any GIST.
Implications:
- This consensus provides a standardized approach to GIST diagnosis and prognostication.
- Accurate diagnosis and risk assessment are crucial for guiding appropriate GIST treatment strategies.
- Further research may refine prognostication and therapeutic approaches for GIST.