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GISTs — Gastrointestinal Stromal Tumors pp 3–12Cite as

Anatomic and Clinical Pathology

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Abstract

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors in the GI tract. These tumors derive from Cajal cells or their precursors. The oncogenic drivers are tyrosine kinase enzymes (KIT) and, to a lesser degree, platelet- derived growth factor receptor alpha (PDGFRA), both of which become constitutively activated following certain primary mutations. These mutations are mostly in the c- KIT gene and rarely in PDGFRA.

The diagnosis relies on morphological and immunohistochemical features; most of the tumors show KIT (CD117) or DOG1 positive cells. In negative cases, a mutational analysis is recommended. The introduction of imatinib mesylate — a potent and selective tyrosine kinase inhibitor (TKI) — and the understanding of GIST biology have made the tumor a paradigm for molecularly targeted therapy. The discovery of new mechanisms of resistance to TKIs has resulted in the development of new strategies for treatment. These different options demand exact morphological classification and risk assessment.

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Authors and Affiliations

  1. Department of Anatomic Pathology, National Cancer Institute of Naples, Naples, Italy

    Annarosaria De Chiara

  2. Department of Oncology, ULSS 9, Treviso, Italy

    Angelo Paolo Dei Tos

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  1. Annarosaria De Chiara
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Editors and Affiliations

  1. Radiology Unit, National Cancer Institute G. Pascale, Naples, Italy

    Elisabetta de Lutio di Castelguidone

  2. Department of Radiology and Radiotherapy, National Cancer Institute, Milan, Italy

    Antonella Messina

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De Chiara, A., Tos, A.P.D. (2011). Anatomic and Clinical Pathology. In: de Lutio di Castelguidone, E., Messina, A. (eds) GISTs — Gastrointestinal Stromal Tumors. Springer, Milano. https://doi.org/10.1007/978-88-470-1869-3_1

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