Nephroblastoma

Aka: Nephroblastoma, Wilms Tumor, Wilm Tumor, Kidney Adenomyosarcoma

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II. Epidemiology

  1. Age Peak Incidence: 2 to 5 years old
    1. Earlier onset in familial type Nephroblastoma
  2. Accounts for 5-7% of all Pediatric Cancers
    1. U.S.: 600 new cases per year
    2. Most common renal Cancer in Children

III. Pathophysiology

  1. Embryonal pediatric tumor of the Kidney
  2. Mixture of cell types (blastemal, stromal, and epithelial)

IV. Symptoms

  1. Constitutional
    1. Fever
    2. Appetite loss
    3. Weight loss
    4. Malaise
  2. Gastrointestinal
    1. Abdominal Pain
    2. Nausea or Vomiting
    3. Constipation
  3. Genitourinary
    1. Hematuria
    2. Scrotal Swelling
  4. Miscellaneous
    1. Anemia
    2. Hypertension

V. Findings: Presentations

  1. Incidental Abdominal Mass
    1. May occur as sporadic, non-syndromic case (or as part of WAGR syndrome)
    2. Firm unilateral mass below rib margin
    3. May present with complications (e.g. Small Bowel Obstruction)
  2. WAGR Syndrome
    1. Wilms Tumor
    2. Aniridia
    3. Genital anomalies
    4. Mental Retardation
  3. Metastatic Disease
    1. Present in 10-15% of Nephroblastoma cases at presentation
    2. Metastases are most common to regional Lymph Nodes, lung and liver

VI. Imaging

  1. Abdominal XRay
    1. May demonstrate complications such as Small Bowel Obstruction
  2. Abdominal Ultrasound
    1. May define Renal Mass, and demonstrate complications
  3. CT Abdomen and Pelvis with contrast (or MRI Abdomen and Pelvis)
    1. Preferred study to characterize tumor, extent of involvement and complications (e.g. Hemorrhage)
    2. Often obtained with CT chest with contrast in suspected Wilm Tumor (Consult oncology)

VII. Management

  1. Consult pediatric oncology
  2. Typical transfer to tertiary center for multispecialty care
  3. Small Bowel Obstruction
    1. Consult pediatric surgery
    2. Decompression of Small Bowel Obstruction (Nasogastric Tube suction, NPO)

VIII. References

  1. Collyer, Huang and Seo (2026) Crit Dec Emerg Med 40(1): 26-33
  2. Leslie (2023) Wilms Tumor, StatPearls
    1. https://www.ncbi.nlm.nih.gov/books/NBK442004/

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