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Paeds Caseshaematology-oncology-and-transfusion

Paeds Cases · haematology-oncology-and-transfusion

Wilms tumour and renal malignancies: Case

Clinical long case of a three-year-old girl presenting with a painless abdominal mass found at the bath time, with the hypertension, from a localised favourable-histology Wilms tumour, covering the recognition of the renal tumour, the first-line ultrasound with the Doppler of the cava, the cross-sectional imaging and the staging, the Children's Oncology Group do-not-biopsy strategy of the upfront radical nephrectomy, the risk-adapted vincristine and dactinomycin chemotherapy, and the long-term survivorship plan.

paediatric oncology long case4 min readVerification in progress

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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A three-year-old girl is brought to the general practitioner after her mother felt a lump in her abdomen at the bath time. She is otherwise well, with no pain, no weight loss and no haematuria. On examination she is alert and comfortable, with a smooth, firm, non-tender mass in the right flank that does not cross the midline, and a blood pressure at the ninety-fifth percentile for her age. The examiner asks how you frame the problem, how you confirm and stage the diagnosis, how you treat the tumour, and how you build the survivorship plan.

Framing the case

This three-year-old girl has the classic presentation of a Wilms tumour. The painless abdominal mass found by the parent at the bath time, the smooth firm flank mass that does not cross the midline, and the hypertension, together make the renal tumour the working diagnosis, and the first decision is to move the child from the routine workup to the urgent imaging and the specialist referral. The framework that organises the case is the age, the preschool child, and the biology, the favourable-histology Wilms that is cured in around ninety percent. [1][4]

Immediate assessment and stabilisation

The assessment begins with the airway, the breathing and the circulation, and the blood pressure, because the hypertension is common and the tumour rupture is the emergency. The child is comfortable and stable, so the focus turns to the imaging. The hypertension, at the ninety-fifth percentile, is noted and the careful antihypertensive is begun if it worsens, but it is not the emergency here. The family is told that the mass is a tumour until the imaging settles it, and the child is kept calm and at rest to avoid the rupture. [9]

The diagnostic pathway

The abdominal ultrasound with the Doppler of the renal vein and the inferior vena cava is the first test, and it confirms the solid renal mass and shows no tumour thrombus in the cava. The computed tomography or the magnetic resonance imaging of the abdomen defines the size and the local extension, and the chest computed tomography shows no pulmonary metastases. In the Children's Oncology Group approach, the radiologically typical tumour proceeds to the radical nephrectomy without the biopsy, because the biopsy breaches the capsule and spills the tumour, which upstages the disease to the stage three and commits the child to the radiotherapy. [4][9]

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References6ShowHide
  1. [1]Spreafico F, Fernandez CV, Brok J Wilms tumour Nat Rev Dis Primers, 2021.PMID 34650095
  2. [4]Dome JS, Mullen EA, Dix DB Impact of the First Generation of Children's Oncology Group Clinical Trials on Clinical Practice for Wilms Tumor J Natl Compr Canc Netw, 2021.PMID 34416705
  3. [5]Graf N, Tournade MF, de Kraker J The role of preoperative chemotherapy in the management of Wilms' tumor. The SIOP studies Urol Clin North Am, 2000.PMID 10985144
  4. [6]Kalish JM, Becktell KD, Bougeard G Update on Surveillance for Wilms Tumor and Hepatoblastoma in Beckwith-Wiedemann Syndrome and Other Predisposition Syndromes Clin Cancer Res, 2024.PMID 39320341
  5. [8]Gadd S, Huff V, Walz AL A Children's Oncology Group and TARGET initiative exploring the genetic landscape of Wilms tumor Nat Genet, 2017.PMID 28825729
  6. [9]Benedetti DJ, Varela CR, Renfro LA Treatment of children with favorable histology Wilms tumor with extrapulmonary metastases: a report from the COG studies AREN0533 and AREN03B2 and NWTSG study NWTS-5 Cancer, 2024.PMID 37933882
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