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Most GISTs are driven by a mutation in exon 11 of the KIT gene, which keeps the KIT growth receptor switched on. Imatinib blocks it: given for three years after surgery in higher-risk tumours it prevents relapse and extends life, and in metastatic disease it controls the tumour for years before resistance develops.
Gastrointestinal stromal tumours arise from the interstitial cells of Cajal in the gut wall, and in 1998 Hirota showed that most carry activating mutations of KIT. Exon 11 mutations, which affect the juxtamembrane domain that normally holds the receptor inactive, account for around two thirds of all GISTs, occur at every site, and are the most sensitive to imatinib at 400 mg daily. Deletions involving codons 557 and 558 carry a worse prognosis than substitutions. Mutation testing is required before treatment because exon 9, PDGFRA D842V and wild-type tumours behave differently, and risk of relapse after surgery is estimated from size, mitotic count and site using the Miettinen or modified NIH criteria.
Surgery removes localised tumours with clear margins and without lymph node dissection, since GIST rarely spreads to nodes. Adjuvant imatinib for one year improved recurrence-free survival in ACOSOG Z9001 (2009), and the Scandinavian SSG XVIII trial (JAMA 2012) showed that three years beat one in high-risk tumours, with five-year overall survival of 92 percent against 82 percent; three years is standard for high-risk disease, and trials of five years are ongoing. Neoadjuvant imatinib shrinks large or awkwardly placed tumours, at the rectum or gastro-oesophageal junction, to allow organ-sparing surgery.
In metastatic disease imatinib controls the tumour for a median of around two years before secondary mutations, in the ATP-binding pocket (exons 13 and 14) or activation loop (exons 17 and 18), cause resistance; the drug is continued indefinitely because stopping it leads to rapid progression. Response is judged on CT with Choi criteria, since tumours may become cystic without shrinking. On progression, dose escalation to 800 mg, then sunitinib, regorafenib and ripretinib follow, and circulating tumour DNA is increasingly used to identify the secondary mutation and choose between them.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
About two thirds of gastrointestinal stromal tumours carry a mutation in exon 11 of KIT, the juxtamembrane domain; this is the imatinib-sensitive majority in whom the drug turned a lethal sarcoma into a chronic disease.
Squamous cancers sit in the upper and middle oesophagus, adenocarcinomas at the junction and in the stomach; the stomach wall also gives rise to GIST from its pacemaker cells.
Same organ: Gastric & gastro-oesophageal junction cancer, HER2-positive gastric cancer, Claudin 18.2-positive gastric cancer, PD-L1-high gastric cancer, Microsatellite-unstable (MSI-high) gastric cancer, Early gastric cancer, Oesophageal squamous cell carcinoma, Oesophageal and junctional adenocarcinoma, Oesophageal cancer, Gastrointestinal stromal tumour (GIST), PDGFRA D842V-mutant GIST, Imatinib-resistant GIST
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Also on OnCo: Symptoms and red flags · Early detection roadmap.
Complete surgical resection without lymphadenectomy; laparoscopic for smaller gastric tumours; neoadjuvant imatinib for large or poorly placed tumours.
Three years of adjuvant imatinib 400 mg (SSG XVIII); trials of longer courses.
Imatinib 400 mg continued until progression, with CT response assessment by Choi criteria.
Dose escalation to 800 mg, then sunitinib, regorafenib and ripretinib, ideally guided by the secondary mutation on circulating tumour DNA.
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Three years of adjuvant imatinib is standard for high-risk resected GIST with imatinib-sensitive mutations; longer courses are being tested.
Mutation testing is standard before imatinib in GIST: exon 11 tumours receive 400 mg, exon 9 tumours are dosed at 800 mg, and PDGFRA D842V tumours are given avapritinib instead.
Imatinib is the standard first-line treatment for advanced GIST and the model for kinase-targeted therapy in solid tumours.
KIT immunohistochemistry and mutation testing define GIST, and the exon 11 mutations described here are the ones most sensitive to imatinib.
Query for this cancer: (TITLE:"KIT exon 11-mutant GIST" OR ABSTRACT:"KIT exon 11-mutant GIST" OR TITLE:"Imatinib-sensitive GIST" OR ABSTRACT:"Imatinib-sensitive GIST" OR TITLE:"KIT-mutant gastrointestinal stromal tumour" OR ABSTRACT:"KIT-mutant gastrointestinal stromal tumour" OR TITLE:"Classic GIST" OR ABSTRACT:"Classic GIST") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about KIT exon 11-mutant GIST, not a curated reading list.
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Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Take with a meal and a large glass of water.
Take with a low-fat breakfast (under 30% fat).
Avoid grapefruit.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
See all on the product pages:ImatinibRegorafenibRipretinibSunitinib·Printable cards in the navigator
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