Abstract
GISTs have become a model for the use of novel target therapies in solid tumors. Thanks to the constitutive activation of KIT or PDGFRA receptor, due to oncogene mutations, they can be targeted effectively by anti-tyrosine kinase agents. Currently, In the first-line therapy of advanced GISTs, imatinib has brought median survival from around one to 5 years. Tumor response rate Is high and predictable through the mutational status of the disease. Indeed, different drugs may exert a different antitumor effect against different muations. Patterns of tumor response are representative of the antltumor effect of target therapy in solid tumors and In GISTs reflect a myxoid tissue degeneration. Imatinib Is administered Indefinitely, until progression (or toxicity, which however Is usually limited). Secondary resistance is the limiting factor of target therapy. In GISTs, It emerges after a median of 2 years from starting Imatinib, even though a proportion of advanced patients become long-term progression-free survivors. Sunitinib Is the standard second-line therapy, and new agents are under study. It Is unknown whether adjuvant complete surgery of responding residual metastases may impact the risk of secondary resistance. On the contrary, there is evidence that Imatinib following surgery of localized disease delays recurrences in significant-risk GIST patients, although it is still unknown if the relapse rate will decrease. The optimal duration of adjuvant therapy is unknown as well.
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Fumagalli, E., Apice, G., Casali, P.G. (2011). Medical Treatment. 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_9
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DOI: https://doi.org/10.1007/978-88-470-1869-3_9
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