The reappraisal of gastrointestinal stromal tumors: from Stout to the KIT revolution

摘要: For five decades gastrointestinal stromal tumors (GISTs) truly have represented one of the most confusing as well neglected areas both surgical pathology and clinical oncology. The recognition central role played by KIT expression in development interstitial cell Cajal activating mutations pathogenesis GIST been keys for a more precise categorization this long elusive clinicopathological entity. A Consensus Conference held at National Institutes Health 2001 provided an evidence-based definition practical scheme assessment risk aggressive behavior. This is based on evaluation size mitotic rate tumors, its use strongly advocated. On basis current data GISTs can be defined distinctive group KIT-expressing mesenchymal neoplasms tract, showing differentiation towards Cajal, also known pacemaker cells. Metastatic virtually incurable disease until elucidation mutations. STI-571 (imatinib mesylate) molecule that inhibits function various receptors with tyrosine kinase activity, such abl, bcr-abl chimeric product, platelet-derived growth factor receptor, KIT. Following successful treatment chronic myeloid leukemia, has proved extremely effective targeting metastatic GIST. Data regarding duration response to therapy are not yet available, therefore any overenthusiasm should avoided. Nonetheless, story remains paradigmatic totally innovative approach cancer which now elegant translation biology experimental knowledge into practice.