Wilms tumour and renal malignancies

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Target exams

Red flags

• A painless abdominal mass in a young child, found by the parent at bath time, is a malignant renal tumour until imaging and specialist review prove otherwise
• A renal mass that is hard, irregular, fixed or associated with haematuria or hypertension is a Wilms tumour or another paediatric renal malignancy until staged and treated
• A needle or open biopsy of a radiologically typical Wilms tumour is avoided in the Children's Oncology Group approach because tumour spill upstages the disease to stage three and raises the recurrence risk
• A child with a renal tumour and an intravascular tumour thrombus extending into the inferior vena cava or the right atrium needs the thrombus defined before the surgery because it changes the operative plan
• An infant with a rapidly enlarging abdominal mass and the clear cell sarcoma or the malignant rhabdoid tumour needs the bone and the brain imaging because both tumours metastasise there

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognises the school-age child with the painless abdominal mass as the classic Wilms tumour presentation, and names it the commonest renal malignancy of childhood
• Identifies the red flag of a hard, fixed or rapidly enlarging abdominal mass with the haematuria and hypertension that declare a renal tumour
• Explains why an abdominal mass in a child is a malignant renal or adrenal tumour until the imaging and the specialist review settle the question
• Explains the biology of the Wilms tumour from the nephrogenic rest and the WT1, 11p15 and WTX genetic hits, and the syndromic predisposition of WAGR, Beckwith-Wiedemann and Denys-Drash
• Contrasts the Children's Oncology Group strategy of the upfront radical nephrectomy without the biopsy against the SIOP strategy of the preoperative chemotherapy, and defends each
• Coordinates the pathway from the ultrasound and the computed tomography or magnetic resonance imaging through the surgical staging and the risk-adapted chemotherapy and radiotherapy to the late-effects surveillance
• A firm painless abdominal mass in a young child that does not cross the midline is a Wilms tumour until imaging proves otherwise, and the biopsy is avoided in the Children's Oncology Group approach because of the tumour spill and the upstaging
• The classic triad of the abdominal mass, the haematuria and the hypertension points to the renal tumour, and the first test is the ultrasound with the Doppler of the renal vein and the cava
• The favourable-histology Wilms is cured in around ninety percent, while the anaplastic Wilms, the clear cell sarcoma and the malignant rhabdoid tumour carry the worse prognosis
• Counsels the family on the high cure rate of the favourable-histology Wilms tumour, the surgery-first pathway, and the late effects of the doxorubicin cardiotoxicity and the renal irradiation
• Builds the multidisciplinary plan that runs the paediatric oncology, the surgery, the radiation oncology, the nephrology and the cardiology together, with the renal function and the cardiac surveillance built in
• Synthesises the long-case child treated for a Wilms tumour into a survivorship plan covering the renal function, the cardiotoxicity, the secondary malignancy and the pregnancy risk
• Level 1: Recognition of the painless abdominal mass as a possible renal malignancy and the urgent referral for the imaging and the specialist review
• Level 2: Application of the diagnostic pathway with the ultrasound and the cross-sectional imaging, the avoidance of the biopsy in the typical case, and the staging
• Level 3: Coordination of the risk-adapted surgery, chemotherapy and radiotherapy, and the long-term late-effects surveillance across the oncology, the nephrology and the cardiology
• Wilms tumour as the commonest renal malignancy of childhood, the peak age of three to four years, and the genetics of the WT1 and the Beckwith-Wiedemann 11p15 locus
• The clinical presentation of the painless abdominal mass with the haematuria and the hypertension, and the first-line ultrasound and the computed tomography or magnetic resonance imaging
• The favourable versus the anaplastic histology and the differential of the clear cell sarcoma and the malignant rhabdoid tumour of the kidney
• Structured abdominal examination of the child with the suspected renal mass for the size, the mobility, the crossing of the midline and the signs of the hypertension and the haematuria
• Communication of a new Wilms tumour diagnosis to the child and the family, including the high cure rate, the surgery-first pathway, and the chemotherapy and its late effects
• Recognition of the oncologic emergencies of the tumour rupture and the intravascular tumour thrombus extending into the cava
• Wilms tumour as the commonest renal malignancy of childhood and a leading paediatric solid tumour
• The clinical presentation by age, the abdominal mass, the haematuria and the hypertension, and the syndromic associations
• The principles of the multimodal therapy with the upfront nephrectomy, the vincristine and dactinomycin chemotherapy, and the selective radiotherapy, and the age-related and the histology-related prognosis
• Recognition of the red flag clinical features of the paediatric renal mass that mandate the urgent imaging and the referral to the specialist centre
• Application of the initial stabilisation of the child with the renal tumour including the blood pressure control and the avoidance of the biopsy in the typical case
• Coordination of the diagnostic and the multidisciplinary treatment pathway for the child with the Wilms or another renal tumour
• Canadian approach to the urgent evaluation and referral of the child with the suspected renal mass
• The staging and the risk-adapted therapy of the Wilms tumour under the Children's Oncology Group and the SIOP protocols
• The long-term late effects of the doxorubicin and the abdominal radiotherapy and the surveillance of the paediatric Wilms tumour survivor