Pediatric Wilms tumor

Pediatric Wilms tumor

Updated: 03/10/2021

© Jun Wang, MD, PhD

 

General features

  • Most common pediatric renal tumor
  • Majority diagnosed prior to age 6 years
  • Usually sporadic
  • May be associated with WAGR syndrome, Denys-Drash syndrome, Beckwith-Wiedemann syndrome, nephroblastomatosis
  • Metastasis: commonly lymph nodes, lung, liver, etc
  • Most patients survive with current multimodality therapy

WAGR syndrome

  • Complex of congenital developmental abnormalities including aniridia, genitourinary malformations, and mental retardation
  • Deletion of the short arm of chromosome 11: WT1, PAX6
  • High risk for Wilms tumor

Denys-Drash syndrome

  • Triad of congenital nephropathy, Wilms tumor and intersex disorders
  • WT1 mutation

Beckwith-Wiedemann syndrome

  • Most common overgrowth syndrome in infancy
  • Triad of congenital exomphalos, macroglossia, and gigantism
  • Mental retardation common
  • Abnormalities 11p15.5 (WT-2)
  • Complex pathogenesis
  • Probably associated with IGF-2 action
  • Wilms tumor: most common cancer in affected children

Clinical features

  • Abdominal mass
  • Abdominal pain
  • Hematuria
  • May spread to perirenal tissue/vessel, lymph nodes

Pathological features

Markers

  • Positive: WT-1 in all three components
  • Negative: S-100, unless neuronal differentiation in stroma
  • Negative: Vimentin is negative in epithelial elements

Genetic abnormality

  • WT1
  • PAX6 if with WT1
  • WT-2
  • Beta-catenin 

Diagnosis

  • Radiologic studies
  • Biopsy

Treatment

  • Nephrectomy followed by chemotherapy

Poor prognostic indicator

  • Anaplasia
  • High stage
  • Age > 2 years
  • Large size

 

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