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Teaching pack: Wilms tumour (nephroblastoma)

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8 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.

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  1. Teaching pack · Cancer · paediatric

    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.

    Teaching pack: Wilms tumour (nephroblastoma) · OnCo, CC BY 4.0 · not medical advice1 / 8
  2. What it is

    In two paragraphs

    Wilms tumour is an embryonal kidney cancer arising from nephrogenic rests, associated with WT1, CTNNB1, WTX, TP53 (anaplastic), and microRNA-processing gene (DROSHA, DGCR8) mutations, and with predisposition syndromes (WAGR, Denys-Drash, Beckwith-Wiedemann). Two cooperative-group philosophies coexist: COG (upfront nephrectomy, then risk-stratified chemotherapy using stage, histology, 1p/16q loss of heterozygosity and 1q gain) and SIOP (pre-operative vincristine-actinomycin then nephrectomy, with post-operative therapy by histologic response and stage; UMBRELLA protocol).

    Favourable-histology stage I-II disease is treated with vincristine and actinomycin D (EE-4A) or, for very low-risk stage I tumours in children under 2 with tumours <550 g, surgery alone; stage III-IV adds doxorubicin and flank/whole-lung radiotherapy, with lung irradiation omitted in rapid complete responders without 1p/16q LOH (AREN0533). Diffuse anaplastic tumours need intensive regimen UH-1/UH-2 with carboplatin, cyclophosphamide and etoposide; bilateral tumours receive neoadjuvant chemotherapy and nephron-sparing surgery. Relapse is treated by risk group (ICE regimens, high-dose chemotherapy in some). Survivorship issues include cardiotoxicity, renal function, second cancers and fertility (radiation).

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  3. Standard of care

    What is given today, by setting

    SettingApproachGuideline
    Very low risk (stage I FH, <2 years, <550 g)Nephrectomy alone with close surveillance (AREN0532).not mapped
    Stage I-II favourable histologyNephrectomy then vincristine + actinomycin D for 18 weeks (EE-4A), or SIOP pre-op VA ×4 weeks then stage-adapted post-op therapy.not mapped
    Stage III-IV favourable histologyVincristine, 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).not mapped
    Diffuse anaplastic or relapsedIntensive UH-1/UH-2 (vincristine, doxorubicin, cyclophosphamide, carboplatin, etoposide) with radiotherapy; relapse: ICE-type regimens, surgery, RT, high-dose therapy or trials.not mapped
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  4. State of the art

    Where the field stands

    • Survival ~90% with steadily less therapy: surgery-only for the youngest lowest-risk children, radiation omitted for lung responders.
    • Molecular markers (1p/16q LOH, 1q gain) already stratify therapy, a rare achievement in paediatric solid tumours.
    • Two cooperative strategies (COG vs SIOP) reach similar outcomes, giving the field a natural experiment.
    • The largest gap is geographic: mortality in sub-Saharan Africa is several-fold higher, driven by late presentation, abandonment and supportive-care shortfalls (SIOP PODC adapted regimens).
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  5. History

    How we got here

    1. 1899Max Wilms's monograph on mixed tumours of the kidney
    2. 1956Actinomycin D active in Wilms tumour (Farber)
    3. 1969National Wilms Tumor Study (NWTS-1) begins
    4. 1990WT1 cloned (Call, Gessler)
    5. 20051p/16q LOH predicts relapse (NWTS-5, Grundy)
    6. 2018AREN0533: lung radiotherapy omitted for rapid complete responders
    7. 2019AREN0532: surgery alone confirmed for very-low-risk stage I
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  6. Pipeline

    What is coming

    • Vincristine (product)
    • Dactinomycin (actinomycin D) (product)
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  7. Open problems

    What nobody has solved

    • Diffuse anaplastic and relapsed disease: survival ~50% or lower.
    • Global inequity: Wilms is curable, yet most children with it worldwide die.
    • Late effects of doxorubicin and radiation in 90% survivors.
    • Bilateral disease: preserving kidney function.
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  8. Sources

    Read the primary sources

    • NCI PDQ: Wilms tumour: https://www.cancer.gov/types/kidney/patient/wilms-treatment-pdq
    • SIOP-RTSG UMBRELLA protocol (Nat Rev Urol 2017): https://doi.org/10.1038/nrurol.2017.163
    • Children's Oncology Group: https://childrensoncologygroup.org/
    • Wikipedia: https://en.wikipedia.org/wiki/Wilms%27_tumor
    • Guideline: https://doi.org/10.1200/JCO.18.02102
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