Updated: 12/27/2021

Wilms Tumor

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  • Snapshot
    • A 15-month-old male is brought in to his pediatrician for routine exam. On exam, his pediatrician notices that he has no irises, consistent with aniridia. He is also found with a palpable, non-tender mass on the left side of his abdomen. On further questioning, his mother reveals that her cousin had a similar abdominal mass at a young age. The patient and his mom are referred to pediatric surgeons for further management. An abdominal CT scan is obtained.
  • Introduction
    • Embryonal malignancy of the kidney
      • embryonic glomerular structure
      • aka nephroblastoma
      • derived from the metanephros
  • Epidemiology
    • Most common renal tumor of childhood
    • Seen in otherwise healthy children < 4 years old
  • ETIOLOGY
    • Pathogenesis
      • loss of function mutation of tumor suppressor genes WT1 or WT2
    • Risk factors
      • family history
      • horseshoe kidney
    • Associated conditions
      • WAGR syndrome
        • Wilms tumor
        • Aniridia
        • Genitourinary anomalies
        • Retardation
      • Beckwith-Wiedemann syndrome
        • overgrowth
          • macrosomia
          • macroglossia
          • hemihyperplasia
          • organomegaly
        • abdominal wall defects
        • embryonal tumors
        • neonatal hypoglycemia
      • neurofibromatosis
  • Presentation
    • Abdominal mass in previously healthy, asymptomatic child
    • Symptoms (25-30% of children)
      • fever
      • hematuria
      • dysuria
      • constipation
      • abdominal pain
    • Unusual presentation of clinical emergency
      • massive bleeding from ruptured tumor
      • acute abdomen
    • Physical exam
      • palpable, painless abdominal mass
        • does not cross midline
      • hypertension
      • some present with aniridia
  • IMAGING
    • Best initial test
      • abdominal ultrasound
    • Most accurate test
      • CT with contrast
    • Radiography or CT to detect lung metastases
  • STUDIES
    • Tissue diagnosis after surgical resection
      • blastemal, epithelial, and stromal cells
      • biopsy not indicated
      • diagnostic confirmation with tissue from surgery
  • Differential Diagnosis
    • Neuroblastoma
    • Polycystic kidney disease
    • Other primary renal malignancies
  • Treatment
    • Prevention
      • genetic counseling based on family history
    • Standard therapy
      • nephrectomy
      • chemotherapy
    • If with metastases
      • radiation added
    • Post-surgery
      • screening for recurrence with abdominal ultrasound every 3 months
  • Complications
    • Nephropathy
    • Hypertension
    • Renal failure
  • Prognosis
    • Better prognosis with younger age
    • Overall 5-year survival rate > 90%
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