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  • • 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

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Epidemiology

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  • • Incidence is approaching 7%

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

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Symptoms and Signs

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  • • Cervical mass

    • Airway compression

    • Abdominal mass

    • Horner syndrome

    • Recurrent urinary tract infections

    • Hydronephrosis

    • Constipation

    • Periorbital ecchymosis

    • Opsoclonus-myoclonus

    • Secretory diarrhea

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Laboratory Findings

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

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Imaging Findings

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  • 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

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

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  • • History and physical exam

    • CT or MRI

    • Bone scan

    • Bone marrow biopsy

    • MIBG scan

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Surgery

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  • • 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

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Medications

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  • • Chemotherapy and radiation for high-risk tumors

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Treatment Monitoring

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  • • Urinary catecholamines

    • CT scanning

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Complications

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  • • Damage to neurovascular structures

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Prognosis

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

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

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References

Maris JM et al: Neuroblastoma. Lancet 2007;369:2106.  [PubMed: 17586306]
Shimada H et al. International neuroblastoma pathology classification for prognostic evaluation of patients with peripheral neuroblastic tumors: a report from the Children's Cancer Group. Cancer. 2001;92:2451.  [PubMed: 11745303]

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