The first 60 days: Wilms tumour (nephroblastoma)
Wilms tumour is a kidney cancer of young children and one of paediatric oncology's success stories: surgery plus a few months of chemotherapy cures about nine in ten. Today's trials aim to give the lowest-risk children almost no chemotherapy while finding the few with aggressive biology. Below, week by week, is what OnCo's record of Wilms tumour (nephroblastoma) 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: Very low risk (stage I FH, <2 years, <550 g).
- SurgeonNamed in the standard of care for: Very low risk (stage I FH, <2 years, <550 g), Stage I-II favourable histology, Stage III-IV favourable histology, Diffuse anaplastic or relapsed.
- Medical oncologistNamed in the standard of care for: Stage I-II favourable histology, Stage III-IV favourable histology, Diffuse anaplastic or relapsed.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Stage III-IV favourable histology, Diffuse anaplastic or relapsed.
- Transplant and cell therapy teamNamed in the standard of care for: Diffuse anaplastic or relapsed.
- Palliative and supportive care teamNamed in the standard of care for: Stage III-IV favourable histology, Diffuse anaplastic or relapsed.
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.
Nephrectomy alone with close surveillance (AREN0532).
Nephrectomy then vincristine + actinomycin D for 18 weeks (EE-4A), or SIOP pre-op VA ×4 weeks then stage-adapted post-op therapy.
Vincristine, actinomycin D, doxorubicin (DD-4A) for 24 weeks; flank/abdominal radiotherapy for stage III; whole-lung radiotherapy for lung metastases not in rapid complete response (AREN0533).
Intensive UH-1/UH-2 (vincristine, doxorubicin, cyclophosphamide, carboplatin, etoposide) with radiotherapy; relapse: ICE-type regimens, surgery, RT, high-dose therapy or trials.
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 Stage, Histology: anaplasia; SIOP risk group after pre-op chemotherapy, 1p and 16q loss of heterozygosity, 1q gain, TP53 mutation), 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 Favourable histology, Diffuse anaplastic, Focal anaplastic.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Very low risk (stage I FH, <2 years, <550 g)
- For my situation (very low risk (stage i fh, <2 years, <550 g)), which of the standard options do you recommend and why?Guideline options include: Nephrectomy alone with close surveillance (AREN0532).
Stage I-II favourable histology
- For my situation (stage i-ii favourable histology), which of the standard options do you recommend and why?Guideline options include: Nephrectomy then vincristine + actinomycin D for 18 weeks (EE-4A), or SIOP pre-op VA ×4 weeks then stage-adapted post-op therapy.
- Am I a candidate for Vincristine, Dactinomycin (actinomycin D), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Stage III-IV favourable histology
- For my situation (stage iii-iv favourable histology), which of the standard options do you recommend and why?Guideline options include: Vincristine, actinomycin D, doxorubicin (DD-4A) for 24 weeks; flank/abdominal radiotherapy for stage III; whole-lung radiotherapy for lung metastases not in rapid complete response (AREN0533).
- Am I a candidate for Vincristine, Dactinomycin (actinomycin D), Doxorubicin, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Diffuse anaplastic or relapsed
- For my situation (diffuse anaplastic or relapsed), which of the standard options do you recommend and why?Guideline options include: Intensive UH-1/UH-2 (vincristine, doxorubicin, cyclophosphamide, carboplatin, etoposide) with radiotherapy; relapse: ICE-type regimens, surgery, RT, high-dose therapy or trials.
- Am I a candidate for Cyclophosphamide, Carboplatin, Etoposide 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 Vincristine, Dactinomycin (actinomycin D)?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 “Diffuse anaplastic and relapsed disease: survival ~50% or lower”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Global inequity: Wilms is curable, yet most children with it worldwide lack access to the treatment that cures it; adapted regimens (SIOP PODC) are the response”. 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.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Wilms tumour (nephroblastoma): the full pageWilms tumour is a kidney cancer of young children and one of paediatric oncology's success stories: surgery plus a few months of chemotherapy cures about nine in ten. Today's trials aim to give the lowest-risk children almost no chemotherapy while finding the few with aggressive biology.
- 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.
- Nephrectomy: Removing a kidney (radical) or just the tumour-bearing part of it (partial).
- Germline vs somatic mutations: Germline mutations are inherited and in every cell; somatic mutations arise in the tumour only.
Every term links to the glossary.