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Updated: Dec 26 2021


  • Snapshot
    • A 4-year-old girl is brought to the emergency room after her parents felt an abdominal mass during her bath. She complains of headaches. On physical exam, her eyes jerk around erratically. Her feet also twitch occasionally. The abdominal mass is irregular, firm, and clearly crosses the midline. An MRI of her head shows potential metastasis to the skull.
  • Introduction
    • Malignant tumor from cells of sympathetic nervous system
      • most commonly in adrenal medulla and paraspinal region
      • most common adrenal tumor in kids (vs pheochromocytoma, most common in adults)
  • Epidemiology
    • < 4 years old
    • Most common cancer in infancy
    • Most common extracranial solid malignancy
    • Pathogenesis
      • neural crest cell origin
      • anywhere along sympathetic chain
    • Genetics
      • associated with amplification of N-Myc oncogene
    • Associated conditions
      • Beckwith-Wiedemann syndrome
      • sometimes (2-3%) seen with opsoclonus myoclonus ataxia syndrome
        • with or without ataxia
  • Presentation
    • Symptoms
      • abdominal distention
      • abdominal pain
      • hallmark of disease
        • hypsarrythmia (dancing feet)
        • opsoclonus (dancing eyes)
    • Physical exam
      • palpable, firm, and irregular mass
        • crosses midline (vs Wilms' tumor, unilateral, smooth)
      • may have diastolic hypertension
  • Evaluation
    • Biopsy
      • histology
        • Homer-Wright rosettes
        • small round blue/purple cells
        • Bombesin +
          • stimulates neuroblastoma growth and expression of angiogenic markers
      • electron microscopy
        • neurosecretory granules
    • Urine (breakdown products of dopamine)
      • ↑ homovanillic acid
      • ↑ vanillyl mandelic acid
    • Imaging
      • 131I-MIBG body scan to detect metastasis
      • MRI
  • Differential Diagnosis
    • Wilms' tumor
    • Rhabdomyosarcoma
    • Germ cell tumors
    • Hepatoblastoma
  • Treatment
    • Surgical resection
    • With or without chemotherapy
  • Complications
    • Metastases to bone and skin
    • Permanent neurological sequelae from opsoclonus myoclonus syndrome
  • Prognosis
    • Younger patients with better prognosis
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