Practice Clinical updates

Neuroblastoma

BMJ 2017; 357 doi: https://doi.org/10.1136/bmj.j1863 (Published 03 May 2017) Cite this as: BMJ 2017;357:j1863
  1. Jason Shohet, associate professor and co-chairman of the Neuroblastoma Research Program1 2,
  2. Jennifer Foster, assistant professor, clinical director of the Neuroblastoma Program1 3
  1. 1Texas Children’s Cancer Center, 6701 Fannin Street, Houston, Texas 77030, USA
  2. 2Center for Cell and Gene Therapy, Baylor College of Medicine, Houston, Texas
  3. 3Department of Pediatrics, Section of Hematology-Oncology, Baylor College of Medicine, Houston, Texas
  1. Correspondence to: J Foster jhfoster{at}txch.org

What you need to know

  • Neuroblastoma is the most common extracranial solid tumour in children; most patients are diagnosed by 5 years of age

  • Diagnosis can usually be confirmed by urine catecholamines and imaging; however, biopsy of the primary site (or bone marrow for staging purposes) is required to establish biology and risk stratification

  • Treatment varies from observation alone for certain low risk patients to intense multimodal therapy for high risk patients

  • Prognosis is excellent for patients with low risk disease and poor for those with high risk disease

  • Relapsed or refractory (high risk) disease is difficult to cure and is associated with extremely low survival

Neuroblastoma most commonly arises from the adrenal gland(s), but can form anywhere that sympathetic nervous tissue is present, including paraspinal sympathetic ganglia in the chest and abdomen.123

The term neuroblastoma is commonly used to describe a spectrum of neuroblastic tumours including neuroblastomas (the most common type), ganglioneuroblastomas, and ganglioneuromas.

Who gets it?

The vast majority of neuroblastomas are diagnosed in children younger than 5 years old, and nearly all patients are diagnosed by the time they are 10 years old. The median age at diagnosis is around 18 months.1

Neuroblastoma accounts for nearly 8% of all childhood malignancies and is the most common solid tumour in children not arising from the brain. This incidence is similar across industrialised nations.45

What causes it?

Given the rarity of the disease, strict associations are hard to prove, and no specific environmental exposure has been implicated in the development of neuroblastoma.

Neuroblastoma is classically an embryological malignancy derived from neural crest cells. The neural crest is a group of neuronal cells that migrate from the spinal cord to form many structures, including the sympathetic nervous system, during fetal development. Therefore, the tumour is found to originate from sympathetic ganglia near the spinal cord …

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