II. Epidemiology
- Most common extracranial solid tumor in children
- Accounts for 8 to 10% of all Childhood Cancers
- Incidence: 550 new cases in U.S. per year
- Age at diagnosis
- Age <1 year in 40% of cases
- Median age: 17 months
- Age <10 years in 97% of cases
III. Pathophysiology
- Cancer derived from postganglionic sympathetic neuroblasts (from neural crest)
IV. Risk Factors
- Maternal Opiate use (esp. Codeine)
- Maternal Folate Deficiency
- Gestational Diabetes Mellitus
V. Associated Conditions
- Central Hypoventilation
- Hirschprung Disease
- Neurofibromatosis Type 1
- Turner Syndrome
VI. Presentations: Based on distribution or primary lesions
- Abdominal primary lesions (most common - 40% adrenal, 25% abdominal)
- Abdominal Pain
- Abdominal fullness
- Retroperitoneal mass, Adrenal Mass or hepatic mass (e.g. Ultrasound)
-
Chest primary lesions (15%)
- Incidental Chest XRay lesions
- Cervical or high chest region primary lesions (5%)
- Paraspinal primary lesions
- Spinal Cord Compression (radiculopathy, back pain, extremity weakness, Bladder dysfunction)
- Metastatic Neuroblastoma
- Fever
- Bone pain
- Limping Child
- Anemia
- Periorbital Ecchymosis or Raccoon Eyes (from retrobulbar tumor infiltration)
- Opsoclonus-myoclonus syndrome
- Blue Skin Nodules (infants with Stage 4S tumors)
VII. Evaluation
- Evaluation (advanced imaging, labs) is typically deferred to tertiary center work-up
- Urine Catecholamines (e.g. vanillylmandelic acid)
VIII. References
- Arndt in Burg (2006) Current Pediatric Therapy, Saunders Elsevier, Philadelphia, p. 1185-7
- Shohet and Nuchtern in Park (2016) UpToDate, accessed on IOS 2/19/2016