The first 60 days: Gastrointestinal stromal tumour (GIST)
GIST is a sarcoma of the gut wall driven almost always by a KIT or PDGFRA mutation. It was the proof that a pill can control a solid tumour: imatinib turned a median survival of about a year into one of eight years or more, and the mutation now dictates which drug to use. Below, week by week, is what OnCo's record of Gastrointestinal stromal tumour (GIST) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Advanced, second line.
- SurgeonNamed in the standard of care for: Localised, resectable.
- Medical oncologistNamed in the standard of care for: Localised, resectable, Advanced, first line, Advanced, second line, Advanced, third/fourth line.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
- 1.Localised, resectableNCCN category Category 1 (adjuvant imatinib, high risk), ESMO-MCBS A, NCCN Guidelines: GIST
Complete resection without lymphadenectomy; adjuvant imatinib 3 years for high-risk (SSGXVIII); neoadjuvant imatinib to downsize when organ-sparing matters.
Imatinib 400 mg (800 mg for KIT exon 9); avapritinib for PDGFRA D842V; continue until progression.
Sunitinib (or ripretinib for KIT exon 11 + 17/18 secondary mutations per ctDNA, INSIGHT).
- 4.Advanced, third/fourth lineNCCN category Category 1 (regorafenib, ripretinib), NCCN Guidelines: GIST
Regorafenib, then ripretinib (INVICTUS); rechallenge or continue TKI beyond progression; clinical trials (bezuclastinib-sunitinib).
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example KIT and PDGFRA mutation status, Secondary KIT mutations by ctDNA at progression, SDHB immunohistochemistry, Mitotic rate, size, site, CD117and DOG1 IHC), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include KIT exon 11-mutant, KIT exon 9-mutant, PDGFRA-mutant.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised, resectable
- For my situation (localised, resectable), which of the standard options do you recommend and why?Guideline options include: Complete resection without lymphadenectomy; adjuvant imatinib 3 years for high-risk (SSGXVIII); neoadjuvant imatinib to downsize when organ-sparing matters.
- Am I a candidate for Imatinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Guideline options include: Imatinib 400 mg (800 mg for KIT exon 9); avapritinib for PDGFRA D842V; continue until progression.
- Am I a candidate for Imatinib, Avapritinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, second line
- For my situation (advanced, second line), which of the standard options do you recommend and why?Guideline options include: Sunitinib (or ripretinib for KIT exon 11 + 17/18 secondary mutations per ctDNA, INSIGHT).
- Am I a candidate for Sunitinib, Ripretinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Advanced, third/fourth line
- For my situation (advanced, third/fourth line), which of the standard options do you recommend and why?Guideline options include: Regorafenib, then ripretinib (INVICTUS); rechallenge or continue TKI beyond progression; clinical trials (bezuclastinib-sunitinib).
- Am I a candidate for Regorafenib, Ripretinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Ripretinib, Avapritinib, Liquid biopsy (ctDNA), Velzatinib?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “Polyclonal resistance: different lesions carry different secondary KIT mutations”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “SDH-deficient and other wild-type GIST have no effective TKI”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- (Peak) A Phase 3 Randomized Trial of CGT9486+Sunitinib vs. Sunitinib in Subjects With Gastrointestinal Stromal TumorsPhase 3 · active · NCT05208047A Phase 3 Randomized, Open-Label, Multicenter Clinical Study of CGT9486+Sunitinib vs. Sunitinib in Subjects With Locally Advanced, Unresectable, or Metastatic Gastrointestinal Stromal Tumors
- A Study of IDRX-42 (GSK6042981) Versus (vs) Sunitinib in Participants With Gastrointestinal Stromal Tumors After Imatinib TherapyPhase 3 · recruiting · NCT07218926A Phase 3, Randomized, Multicenter, Open-Label Study of IDRX-42 (GSK6042981) Versus Sunitinib in Participants With Metastatic and/or Unresectable Gastrointestinal Stromal Tumors (GIST) After Imatinib Therapy (StrateGIST 3)
- A Study of Olverembatinib in SDH-deficient GIST.Phase 3 · recruiting · NCT06640361A Single-Arm Registrational Phase III Study of Olverembatinib in the Treatment of Patients With SDH-Deficient Gastrointestinal Stromal Tumor (POLARIS-3)
- A Study of Ripretinib vs Sunitinib in Advanced GIST Patients After Treatment With ImatinibPhase 3 · active · NCT03673501A Phase 3, Interventional, Randomized, Multicenter, Open-Label Study of Ripretinib vs Sunitinib in Patients With Advanced Gastrointestinal Stromal Tumor (GIST) After Treatment With Imatinib
- A Study to Investigate Velzatinib Compared With Imatinib in Adult Participants With Previously Untreated Metastatic and/or Unresectable Gastrointestinal Stromal Tumors (StrateGIST Frontline)Phase 3 · recruiting · NCT07585266A Phase 3, Randomized, Multicenter, Open-Label Study of Velzatinib (GSK6042981) Versus Imatinib in Participants With Previously Untreated Metastatic and/or Unresectable Gastrointestinal Stromal Tumors (GIST)
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Gastrointestinal stromal tumour (GIST): the full pageGIST is a sarcoma of the gut wall driven almost always by a KIT or PDGFRA mutation. It was the proof that a pill can control a solid tumour: imatinib turned a median survival of about a year into one of eight years or more, and the mutation now dictates which drug to use.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Drug resistance (primary and acquired): Why cancer drugs stop working: the tumour either never depended on the target or evolves around the block.
- Oncogene addiction: When a cancer depends so completely on one mutated gene that blocking it collapses the tumour.
- Circulating tumour DNA (ctDNA): Circulating tumour DNA (ctDNA) consists of fragments of DNA shed by tumour cells into the blood, detectable with sensitive sequencing.
- Rare cancers: Rare cancers are those with fewer than about 6 new cases per 100,000 people per year.
Every term links to the glossary.