Gastrointestnal stromal tumors (GIST are the most common tumors of the gastrointestinal tract. They are characterized by gain of function mutations in KIT or PDGFRA genes. Imatinib is the gold standard therapy for the managment of these tumors and it led a terrific improvment in GIST patients' prognosis. GIST patients respond very well to the tyrosine kinase inhibitor (TKI) imatinib for about 18-36 months; however, the occurrence o resistance ater this time is extremely common. Clearly, TKIs have been developed as target therapy or GIST with specific mutation in KIT and PDGFRA. Unfortunately, 10-15% of all GIST are classified as wild-type (WT) GIST, not harbouring any mutation in KIT/PDGFRA. This sugroup o patients, althoug characterized by a unique phenotype, are however treated with TKI. Despite all these observation, imatinib remains the onl irst choice and best treatment in GIST patients. in view of these consideration, the identiication o new molecules, beyond TKIs is highly needed. the aim o this chapter is to highlight the state of the art of GIST management, with particular ocus on TKIs resistant patients and wild-type GIST.
Management of Gastrointestinal Stromal Tumors: present and future directions
RAVEGNINI, GLORIA;ANGELINI, SABRINA
2015
Abstract
Gastrointestnal stromal tumors (GIST are the most common tumors of the gastrointestinal tract. They are characterized by gain of function mutations in KIT or PDGFRA genes. Imatinib is the gold standard therapy for the managment of these tumors and it led a terrific improvment in GIST patients' prognosis. GIST patients respond very well to the tyrosine kinase inhibitor (TKI) imatinib for about 18-36 months; however, the occurrence o resistance ater this time is extremely common. Clearly, TKIs have been developed as target therapy or GIST with specific mutation in KIT and PDGFRA. Unfortunately, 10-15% of all GIST are classified as wild-type (WT) GIST, not harbouring any mutation in KIT/PDGFRA. This sugroup o patients, althoug characterized by a unique phenotype, are however treated with TKI. Despite all these observation, imatinib remains the onl irst choice and best treatment in GIST patients. in view of these consideration, the identiication o new molecules, beyond TKIs is highly needed. the aim o this chapter is to highlight the state of the art of GIST management, with particular ocus on TKIs resistant patients and wild-type GIST.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
