תאריך: 01/02/2015
KEY POINTS
- Neuroblastoma (NB) is the most common extracranial pediatric tumor, most frequently diagnosed cancer in infancy, and has a heterogeneous presentation and prognosis.
- Clinical and biological prognostic factors are used to risk stratify patients into groups with low, intermediate, and high risk for recurrence; most protocols now use the International Neuroblastoma Risk Group classification system.
- Age, stage, histology, and amplification of the MYCN oncogene are currently the most robust prognostic factors.
- Outcomes for low- and intermediate-risk NB are excellent, but survival for high-risk NB is less than 50%.
- High-risk NB tumors contain many segmental chromosome aberrations (eg, loss of heterozygosity 1p, 11q); but recurrent somatic mutations are rare, with anaplastic lymphoma kinase (ALK) being the most commonly altered gene in approximately 10% of NB.
- Survival after relapse of metastatic NB is uncommon; current and upcoming trials will rely on incorporation of novel immunotherapies, inhibitors of aberrant pathways (eg MYC, ALK), and radioisotope-containing regimens, such as high-dose iodine-131-metaiodobenzylguanidine.
Meredith S. Irwin, MD, Julie R. Park, MD
Pediatr Clin N Am 62 (2015) 225–256
https://dx.doi.org/10.1016/j.pcl.2014.09.015
