Neuroblastoma

• • Most common extracranial solid tumor and most common abdominal solid malignancy in children

  • Associated with the following:

  • -Neurofibromatosis type I

    -Beckwith-Wiedemann syndrome

    -Hirschsprung disease

    -Musculoskeletal and cardiovascular malformation

    -Turner syndrome

  • Factors that place patient at high risk include:

  • -Age > 1 year

    -Stage 3 and 4

    -Unfavorable Shimada classification (histology)

    -N-myc amplification

    -No trk expression

    -Diploid

    -1p deletion

    -> 142 ferritin ng/mL

    -> 1500 LDH IU/L

Epidemiology

• • Incidence is approaching 7%

  • Median age of diagnosis is age 2, with 80% being younger than 4 years

Symptoms and Signs

• • Cervical mass

  • Airway compression

  • Abdominal mass

  • Horner syndrome

  • Recurrent urinary tract infections

  • Hydronephrosis

  • Constipation

  • Periorbital ecchymosis

  • Opsoclonus-myoclonus

  • Secretory diarrhea

Laboratory Findings

• • 24-hour urine collection showing elevated metanephrine, dopamine, and vanillylmandelic acid (VMA)

Imaging Findings

• • CT or MRI: Demonstrate extent of disease and location of primary tumor, which can be found anywhere along sympathetic chain

  • Bone scan: Occasionally shows cortical involvement or marrow involvement

  • MIBG scan: Shows nodal or metastatic disease

• • Stage, high-risk factors, and major vessel involvement

• • History and physical exam

  • CT or MRI

  • Bone scan

  • Bone marrow biopsy

  • MIBG scan

Surgery

• • Resection of all gross tumor and regional nodes as well as vascular dissection

  • Second debulking resection for high-risk tumors following chemotherapy and radiation therapy is controversial but may be indicated in some cases

Medications

• • Chemotherapy and radiation for high-risk tumors

Treatment Monitoring

• • Urinary catecholamines

  • CT scanning

Complications

• • Damage to neurovascular structures

Prognosis

• • 10-30% overall survival for high-risk tumors

  • 3-year survival ranging from 40% to 97% (dependent on stage)

References