Updated: 2/13/2018

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
  • 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
Evaluation
  • Best initial test
    • abdominal ultrasound
  • Most accurate test
    • CT with contrast
  • Radiography or CT to detect lung metastases
  • 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
  • Standard therapy
    • nephrectomy
    • chemotherapy
  • If with metastases
    • radiation added
  • Post-surgery
    • screening for recurrence with abdominal ultrasound every 3 months
Prognosis, Prevention, and Complications
  • Prognosis
    • better prognosis with younger age
    • overall 5-year survival rate > 90%
  • Prevention
    • genetic counseling based on family history
  • Complications
    • nephropathy
    • hypertension
    • renal failure

 

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