The first 60 days: Inflammatory myofibroblastic tumour (IMT)
IMT is a rare tumour of spindle cells mixed with inflammatory cells, most often in the lung or abdomen of children and young adults. Surgery cures most, and about half carry an ALK gene fusion, so the ALK-blocking pill crizotinib is approved for those that cannot be removed, one of the first targeted approvals for a childhood solid tumour. Below, week by week, is what OnCo's record of Inflammatory myofibroblastic tumour (IMT) 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: Unresectable, ALK-negative with other fusion.
- RadiologistNamed in the standard of care for: Resectable.
- SurgeonNamed in the standard of care for: Resectable.
- Medical oncologistNamed in the standard of care for: Resectable, Unresectable, recurrent or metastatic, ALK-positive, Unresectable, ALK-negative with other fusion.
- Palliative and supportive care teamNamed in the standard of care for: Unresectable, ALK-negative with other fusion.
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.
Complete surgical excision; no adjuvant therapy in most cases, surveillance imaging for recurrence.
- 2.Unresectable, recurrent or metastatic, ALK-positiveNCCN category Category 2A, NCCN Soft Tissue Sarcoma
Crizotinib (FDA approval July 2022, children 1 year and older and adults); alectinib, ceritinib or lorlatinib at progression.
Match to fusion: entrectinib or crizotinib for ROS1, larotrectinib or entrectinib for NTRK, imatinib for PDGFRB; steroids or NSAIDs for symptom control in indolent disease.
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 ALK immunohistochemistry and FISH or RNA fusion panel, ROS1, NTRK, PDGFRB, RET fusions in ALK-negative tumours, Inflammatory markersas systemic markers, Site and resectability), 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 Classic IMT, ALK-negative IMT, Epithelioid inflammatory myofibroblastic sarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Resectable
- For my situation (resectable), which of the standard options do you recommend and why?Guideline options include: Complete surgical excision; no adjuvant therapy in most cases, surveillance imaging for recurrence.
Unresectable, recurrent or metastatic, ALK-positive
- For my situation (unresectable, recurrent or metastatic, alk-positive), which of the standard options do you recommend and why?Guideline options include: Crizotinib (FDA approval July 2022, children 1 year and older and adults); alectinib, ceritinib or lorlatinib at progression.
- Am I a candidate for Crizotinib, Alectinib, Lorlatinib or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Unresectable, ALK-negative with other fusion
- For my situation (unresectable, alk-negative with other fusion), which of the standard options do you recommend and why?Guideline options include: Match to fusion: entrectinib or crizotinib for ROS1, larotrectinib or entrectinib for NTRK, imatinib for PDGFRB; steroids or NSAIDs for symptom control in indolent disease.
- Am I a candidate for Entrectinib, Larotrectinib, Imatinib or related drugs, 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 Crizotinib, Lorlatinib, Repotrectinib?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 “How long to continue ALK inhibition in children with complete response, and whether surgery after response can allow stopping”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “The fusion-negative minority: RNA sequencing to find drivers”. 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.
- Inflammatory myofibroblastic tumour (IMT): the full pageIMT is a rare tumour of spindle cells mixed with inflammatory cells, most often in the lung or abdomen of children and young adults. Surgery cures most, and about half carry an ALK gene fusion, so the ALK-blocking pill crizotinib is approved for those that cannot be removed, one of the first targeted approvals for a childhood solid tumour.
- 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.
- RACE for Children Act: A US law that makes drug companies test new targeted cancer drugs in children whenever the drug's target matters in a childhood cancer, instead of letting them skip children because their cancers are rare.
- Gene fusion: A gene fusion is two genes broken and joined together, creating a hybrid protein that can drive cancer.
- 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.