Gastrointestinal Stromal Tumor….Systemic Therapy Agents and Regimens for GIST...Preferred regimens: Imatinib for KIT or PDGFRA mutations (excluding PDGFRA exon 18 mutations that are insensitive to imatinib, including D842V).

...- Positive immunohistochemical staining for c-KIT (CD117); or negative staining for KIT, but with either positive staining for DOG1 or an identified mutation of KIT or PDGFRA gene...

...To determinate the objective response rate (ORR, complete response + partial response) To determinate the time to tumor progression (TTP) To evaluate the safety and toxicity profiles of AUY922 To evaluate the pharmacokinetics profile of AUY922 in Taiwan GIST population To access the pharmacodynamic effect of AUY922 on HSP client proteins in blood and tumor if feasible , i.e. HSP70, in Taiwan GIST population To access the tissue biomarkers pre-treatment and 4wks post treatment if feasible, i.e. HSP70, c-KIT, PDGFRA mutation, ...etc in Taiwan GIST population...

...Histologically confirmed GIST of any anatomical location and confirmed by the RRePS Network ; positive immunohistochemical staining for c-KIT (CD117); or negative staining for KIT, but with either positive staining for DOG1 or an identified mutation of KIT or PDGFRA gene 3. ...

...Exception to this rule is GIST patients with documented KIT or PDGFRA mutations.4. ...

...Patients with demonstrated mutation in KIT or PDGFRA may be entered to the study despite negative immunostaining for KIT and DOG-1 provided that tumour histology is compatible with GIST. ...

...- Histologically or cytologically confirmed GIST with KIT mutation or PDGFRA mutation (non-D842V)...

...Patients with demonstrated mutation in KIT or PDGFRA may be entered to the study despite negative immunostaining for KIT and DOG-1 provided that tumour histology is compatible with GIST....

...- Gene mutation test report including c-kit exons 9,11,13 and 17 and platelet-derived growth factor receptor alpha (PDGFRA) exons 12 and 18...

...Histologically confirmed GIST of any anatomical location and confirmed by the RRePS Network ; positive immunohistochemical staining for c-KIT (CD117); or negative staining for KIT, but with either positive staining for DOG1 or an identified mutation of KIT or PDGFRA gene 3....

...Number of patients with a molecular analysis positive for KIT mutation`KIT Gene mutation`Number of patients with a molecular analysis positive for PDGFRA mutation`PDGFRA Gene mutation`Number of patients with immunohistochemical expression of CD117`Expression of CD117`Number of patients with immunohistochemical expression of DOG1`Expression of DOG1`Number of patients with recurrence after treatment with tyrosinkin inhibitors.`...

...- Histologically confirmed gastrointestinal stromal tumor expressing CD117+ or with documented mutation of the KIT or PDGFRA gene...

The majority of GIST cases harboured exon 11 KIT mutations (88 cases, 82%), 11 cases had exon 9 KIT mutations (10%), 8 had other KIT/PDGFRA mutations potentially sensitive to imatinib. The disease relapse was detected in 19 patients, of them in 5 cases in exon 9 KIT mutants (45%), and 14 cases in patients with exon 11 KIT mutations (11%) [p < 0.01].

A 38-year-old male presented with a 11.5 cm epigastric GIST status... PDGFRA gene mutation detected by genetic analysis….The patient was managed with continuous imatinib treatment and life-long follow up.