Surgical Pathology of Gastrointestinal Stromal Tumors: Practical Implications of Morphologic and Molecular Heterogeneity for Precision Medicine.

摘要: Gastrointestinal stromal tumor (GIST), the most common mesenchymal neoplasm of gastrointestinal tract, exhibits diverse histologic and clinical manifestations. With its putative origin in pacemaker cell Cajal, GIST can arise association with any portion tubular tract. Morphologically, GISTs are classified as spindled or epithelioid, though each these subtypes encompasses a broad spectrum microscopic appearances, many which mimic other entities. Despite this morphologic ambiguity, diagnosis is aided cases by immunohistochemical detection KIT (CD117) DOG1 expression. The natural history ranges from that cured surgical resection to locally advanced even widely metastatic, ultimately fatal, disease. This clinicopathologic heterogeneity paralleled an underlying molecular diversity: majority associated spontaneous activating mutations KIT, PDGFRA, BRAF, while additional subsets driven genetic lesions-often inherited-of NF1 components succinate dehydrogenase enzymatic complex. Specific gene correlate particular anatomic characteristics and, turn, distinct behaviors. Therefore, prognostication treatment increasingly dictated not only clues, but also accompanying features. In review, we provide comprehensive description heterogenous underpinnings GIST, including implications for practicing pathologist regard identification, diagnosis, management.