More than 600 cases are diagnosed in the United States each year, and neuroblastoma accounts for approximately 15% of all pediatric cancer fatalities.¹

The median age at diagnosis is 17.3 months, and 40% of patients are diagnosed before 1 year of age.¹,²

Neuroblastomas are the most common cancer among infants younger than 12 months, in whom the incidence rate is almost twice that of leukemia.³

The incidence of neuroblastoma is greater among white than black infants (ratio of 1.7:1 and 1.9:1 for males and females, respectively), but less if any racial difference is apparent among older children.¹

Neuroblastoma is slightly more common among boys compared with girls.¹

Neuroblastoma is commonly used to refer to a spectrum of neuroblastic tumors (including neuroblastomas, ganglioneuroblastomas, and ganglioneuromas) that arise from primitive sympathetic ganglion cells.

The neuroectodermal cells that comprise neuroblastic tumors originate from the neural crest during fetal development and migrate to the adrenal medulla and sympathetic nervous system.

The International Neuroblastoma Pathology Classification classifies tumors of neuroblastic origin according to the balance between neural-type cells and Schwann-type cells into neuroblastoma, ganglioneuroblastoma, or ganglioneuroma.

Neuroblastomas are the most undifferentiated and aggressive of this family of tumors.⁴

The degree of differentiation and stromal component of neuroblastoma tumors can be predictive of outcome and are used for treatment considerations.

Neuroblastomas are composed almost entirely of neuroblasts, with very few Schwannian (or stromal) cells (Figure 48.1).

These are called “stroma-poor” tumors.⁵

Ganglioneuroblastomas are “intermixed stroma-rich” tumors because of the increased proportion of Schwannian cells.

These tumors generally have intermediate malignant potential, between that of neuroblastomas and ganglioneuromas.

Ganglioneuromas are predominantly composed of Schwannian cells studded with maturing or fully mature ganglion cells.⁴,⁶,⁷

These tumors tend to occur in older children aged 5 to 7 years rather than the more aggressive neuroblastomas.

They are considered to be benign but can metastasize.⁸,⁹,¹⁰

The prognosis is excellent, even when complete tumor removal is not possible.¹¹

Risk factors can include maternal, fetal, and genetic factors.

Folate deficiency, other congenital abnormalities, maternal opiate consumption, small- or large-for-gestational-age babies, and maternal gestational diabetes mellitus have all shown a correlation with and may play a role in development of neuroblastoma.

A higher incidence of neuroblastoma has been suggested in girls with Turner syndrome.¹²

Hirschsprung disease, central hypoventilation, and neurofibromatosis type 1 have also been described in association with neuroblastoma.¹³,¹⁴,¹⁵,¹⁶