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Neuroblastoma

Neuroblastoma is a topic covered in the 5-Minute Clinical Consult.

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Basics

Most common tumor of infants and one of the most common tumors of children

Description

A neoplasm developed from immature sympathetic ganglion cells of neural crest origin that may arise anywhere along the sympathetic chain or in the adrenal medulla

  • Sites of disease
    • Adrenal medulla, 40%
    • Sympathetic ganglia
      • Abdominal, 25%
      • Thoracic, 15%
      • Cervical, 5%
      • Pelvic, 5%
    • Other, 10%
  • Metastasis: lymphatic and hematogenous to bone, bone marrow, liver, skin, dura, orbits

Epidemiology

  • Third most common pediatric malignancy
  • Most common tumor in infants <1 year of age; accounts for 8–10% of all childhood cancers and 15% of all childhood oncologic deaths
  • Predominant age at diagnosis
    • Within first 5 years of life: 90%
    • Children in the 1st year of life: 30%
    • Between ages 1 and 4 years: 50%
    • Median age 17.3 months
  • Predominant sex: males > females (1.2:1)
  • Predominant race: white > black (1.7:1 for males, 1.9:1 for females)
  • Screening: no impact on mortality (60% regress spontaneously)

Incidence
  • From birth to age 1 year in the United States: 52.1 cases per million children per year
  • From age 1 to 4 years in the United States: 19.1 cases per million children per year
  • First 15 years of life in the United States: 9.8 cases per million children per year

Etiology and Pathophysiology

Genetics
  • 80% of tumors have genetic abnormalities.
  • Genetic alterations associated with aggressive tumor behavior and poor outcomes (1)
    • Deletions in short arm of chromosome 1p36
    • Unbalanced gain of long arm of chromosome 17 (trisomy 17q)
    • Polymorphism at chromosome 6p22
    • N-myc amplification on chromosome 2 (20–25%)
    • Anaplastic lymphoma kinase (ALK) gene associated with hereditary neuroblastoma
  • Genetic alterations associated with better outcomes: 11q23, 14q
  • DNA ploidy: Hyperdiploidy in infants <1 year of age is associated with excellent long-term survival; diploidy is associated with treatment failure.
  • Familial cases (1–2%)
    • Median age at diagnosis: 9 months
    • Autosomal dominant inheritance with incomplete penetrance

Risk Factors

  • Maternal phenytoin or opiates
  • Folate deficiency
  • Opsoclonus-myoclonus syndrome
    • 50% have neuroblastoma.
  • Beckwith-Wiedemann syndrome
  • Pancreatic islet cell dysplasia
  • Fetal alcohol syndrome
  • Hirschsprung disease
  • Family history of neuroblastoma
  • Neurofibromatosis type 1
  • Congenital central hypoventilation syndrome
  • Gestational diabetes mellitus

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Citation

Stephens, Mark B., et al., editors. "Neuroblastoma." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816193/all/Neuroblastoma.
Neuroblastoma. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816193/all/Neuroblastoma. Accessed March 19, 2019.
Neuroblastoma. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816193/all/Neuroblastoma
Neuroblastoma [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 March 19]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816193/all/Neuroblastoma.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Neuroblastoma ID - 816193 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816193/all/Neuroblastoma PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -