HemeOnc

Neuroblastoma

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Neuroblastoma, Schwannian Stroma-Poor, Malignant Neural Crest Tumor

  • Epidemiology
  1. Most common extracranial solid tumor in children
  2. Accounts for 8 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)
  • 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% abdominal)
    1. Abdominal Pain
    2. Abdominal fullness
    3. Retroperitoneal mass, Adrenal Mass or hepatic mass (e.g. Ultrasound)
  2. Chest primary lesions (15%)
    1. Incidental Chest XRay lesions
  3. Cervical or high chest region primary lesions (5%)
    1. Horner Syndrome (Miosis, Ptosis, anhydrosis)
  4. Paraspinal primary lesions
    1. Spinal cord compression (radiculopathy, back pain, extremity weakness, Bladder dysfunction)
  5. 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)
  • Evaluation
  1. Evaluation (advanced imaging, labs) is typically deferred to tertiary center work-up
  2. Urine Catecholamines (e.g. vanillylmandelic acid)
  • References
  1. Arndt in Burg (2006) Current Pediatric Therapy, Saunders Elsevier, Philadelphia, p. 1185-7
  2. Shohet and Nuchtern in Park (2016) UpToDate, accessed on IOS 2/19/2016