Introduction

The pathological findings of neuroblastoma, a childhood malignancy of neural crest origin, were initially described by James Homer Wright in 1910. Extracranial neuroblastoma may be found anywhere along the sympathetic axis, including the neck, posterior mediastinum, retroperitoneum, adrenals, and pelvis. The seemingly unpredictable character of neuroblastoma has frustrated physicians for nearly a century. However, data collected over the past 40 years through the large cooperative groups, both in the USA and in Europe, has allowed the stratification of patients into risk groups. The risk of neuroblastoma progression may be predicted, based on the age at presentation, stage of disease, histology, and biological markers. By stratifying patients into risk categories, children with good prognosis receive less intensive therapy, minimizing their morbidity, while children with poor prognosis are treated aggressively, in order to achieve long-term survival.

Presentation

The presentation of neuroblastoma varies with age and stage of disease. Newborns may present with an asymptomatic mass or with paraplegia. Infants may present with skin metastases or abdominal distention secondary to liver metastases and resultant hepatomegaly (stage 4S). Children with early stage (1 or 2) tumors are usually well and present with a mass found by a family member, an examining physician, or noted incidentally on a radiological examination. In contrast, children with advanced disease usually present with a lingering illness lasting 1–3 weeks and a mass found either by family or physician. Occasionally, children may present with lower extremity weakness resulting from impingement of the spinal cord by tumor.