The first 60 days: PDGFRA D842V-mutant GIST
PDGFRA D842V GIST is driven by a mutation in the PDGFRA receptor rather than KIT, and it does not respond to imatinib at all. Avapritinib, designed to fit the mutant activation loop, shrinks nearly nine in ten of these tumours and is the standard treatment for advanced disease; localised tumours are cured by surgery alone. Below, week by week, is what OnCo's record of PDGFRA D842V-mutant 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: Progression on avapritinib.
- SurgeonNamed in the standard of care for: Localised, resectable, Progression on avapritinib.
- Medical oncologistNamed in the standard of care for: Advanced, first line, Progression on avapritinib.
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.
Surgical resection; no adjuvant imatinib because the mutation is resistant to it.
Avapritinib 300 mg daily (NAVIGATOR), with monitoring for cognitive effects and bleeding.
No established therapy; clinical trials, surgery or embolisation for isolated progression; regorafenib and other kinase inhibitors have low activity.
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 PDGFRA exon 18 D842V mutation, Weak or absent KITstaining with positive DOG1, Epithelioid histology, Mitotic count and size, Secondary PDGFRA mutations at progression on avapritinib), 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 PDGFRA D842V-mutant gastric GIST, epithelioid, Other PDGFRA exon 18 mutations, PDGFRA exon 12 and 14-mutant GIST.
- 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: Surgical resection; no adjuvant imatinib because the mutation is resistant to it.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Guideline options include: Avapritinib 300 mg daily (NAVIGATOR), with monitoring for cognitive effects and bleeding.
- Am I a candidate for Avapritinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Progression on avapritinib
- For my situation (progression on avapritinib), which of the standard options do you recommend and why?Guideline options include: No established therapy; clinical trials, surgery or embolisation for isolated progression; regorafenib and other kinase inhibitors have low activity.
- Am I a candidate for Regorafenib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of VOYAGER apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Any stage
- Are there clinical trials I could join, for example of Avapritinib, Liquid biopsy (ctDNA), IDRX-42?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 “No proven therapy after progression on avapritinib”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Cognitive side effects limit dose and quality of life for some patients”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- PDGFRA D842V-mutant GIST: the full pagePDGFRA D842V GIST is driven by a mutation in the PDGFRA receptor rather than KIT, and it does not respond to imatinib at all. Avapritinib, designed to fit the mutant activation loop, shrinks nearly nine in ten of these tumours and is the standard treatment for advanced disease; localised tumours are cured by surgery alone.
- 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.