Neuroblastoma

Rare, malignant tumor arising from the embryological neural crest element of the peripheral sympathetic nervous system.

The most common extracranial solid tumor in children; the majority of 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 (i.e., cytogenetics, pathology) and risk stratification.

Treatment depends primarily on risk classification and 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.

A malignant tumor arising from the embryological neural crest element of the peripheral sympathetic nervous system. It 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.[1]Brodeur G, Hogarty M, Bagatell R, et al. Neuroblastoma. In: Pizzo P, Poplack D, eds. Principles and practice of pediatric oncology. Philidelphia, PA: Lippincott Williams & Wilkins; 2016:772.[2]Brisse HJ, McCarville MB, Granata C, et al. Guidelines for imaging and staging of neuroblastic tumors: consensus report from the International Neuroblastoma Risk Group Project. Radiology. 2011 Oct;261(1):243-57. http://pubs.rsna.org/doi/full/10.1148/radiol.11101352 http://www.ncbi.nlm.nih.gov/pubmed/21586679?tool=bestpractice.com [3]Angstman KB, Miser JS, Franz WB 3rd. Neuroblastoma. Am Fam Physician. 1990 Jan;41(1):238-44. http://www.ncbi.nlm.nih.gov/pubmed/2403727?tool=bestpractice.com

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