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Neuroblastoma

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Neuroblastoma, Schwannian Stroma-Poor, Malignant Neural Crest Tumor

  • See Also
  1. Childhood Cancer
  2. Nephroblastoma
  • Epidemiology
  1. Most common extracranial solid tumor in children
  2. Accounts for 6 to 10% of all Childhood Cancers
  3. Incidence: 550 new cases in U.S. per year
  4. Age at diagnosis
    1. Age <1 year in 40% of cases
    2. Median age: 17 months
    3. Age <10 years in 97% of cases
  • Pathophysiology
  1. Cancer derived from postganglionic sympathetic neuroblasts (from neural crest)
  2. May arise anywhere along the sympathetic chain (adrenal is most common)
  • Risk Factors
  1. Maternal Opiate use (esp. Codeine)
  2. Maternal Folate Deficiency
  3. Gestational Diabetes Mellitus
  • Associated Conditions
  1. Central Hypoventilation
  2. Hirschprung Disease
  3. Neurofibromatosis Type 1
  4. Turner Syndrome
  • Presentations
  • Based on distribution or primary lesions
  1. Abdominal primary lesions (most common - 40% adrenal, 25% other abdominal)
    1. Abdominal Pain
    2. Abdominal fullness
    3. Retroperitoneal mass, Adrenal Mass or hepatic mass (e.g. Ultrasound)
    4. May be complicated by Small Bowel Obstruction, Intussusception
  2. Chest primary lesions (15%)
    1. Incidental Chest XRay lesions
  3. Cervical or high chest region primary lesions (5%)
    1. Horner Syndrome (Miosis, Ptosis, Anhidrosis)
  4. Skull base and orbital floor
    1. Periorbital Ecchymosis (raccoon eyes)
    2. Opsoclonus-myoclonus syndrome (Paraneoplastic Syndrome)
      1. Myoclonic Jerks
      2. Multidirectional chaotic eye movement
  5. Paraspinal primary lesions
    1. Spinal Cord Compression (radiculopathy, back pain, extremity weakness, Bladder dysfunction)
  6. Metastatic Neuroblastoma
    1. Fever
    2. Bone pain
    3. Limping Child
    4. Anemia
    5. Periorbital Ecchymosis or Raccoon Eyes (from retrobulbar tumor infiltration)
    6. Opsoclonus-myoclonus syndrome
    7. Blue Skin Nodules (infants with Stage 4S tumors)
  • Imaging
  1. MRI of specifically involved regions
  2. Iodine-Metaiodobenzylguanidine (I-MIBG) Body Scan
  • Evaluation
  1. Evaluation (advanced imaging, labs) is typically deferred to tertiary center work-up
  2. Urine Catecholamines (e.g. vanillylmandelic acid)
  • 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)
  • References
  1. Arndt in Burg (2006) Current Pediatric Therapy, Saunders Elsevier, Philadelphia, p. 1185-7
  2. Collyer, Huang and Seo (2026) Crit Dec Emerg Med 40(1): 26-33
  3. Shohet and Nuchtern in Park (2016) UpToDate, accessed on IOS 2/19/2016