Neuroblastoma

Abstract

Neuroblastoma is the most frequent extracranial solid malignancy in children less then 5 years of age and accounts for 6–10% of all pediatric malignancies and accounts for about 15% of cancer death in children. It arises from the sympathetic nervous system, most likely from the adrenal gland but also from sympathetic side chain. 40% of neuroblastomas are incidental findings. Most patients with clinical signs present with abdominal distention. The most common localization of neuroblastoma is the adrenal glands. Metastatic spread to the lymph nodes and bone marrow is common. MYCN is an oncogene and the most important molecular marker for poor prognosis. Image defined Risk Factors (IDRF) describe the extent and invasive/extrusive growth of neuroblastoma in different anatomical areas, such as neck, thorax or abdomen. Primary complete resection should be attempted if no IDRF are present. Although complete resection (R0) is the goal in surgical oncology, recent studies indicate that <10% of residual tumor after resection (gross total resection) can be tolerated does not affect outcome.