**Neuroblastoma, Clinical**

**Chapter 1**

**Clinical Presentation of Neuroblastoma**

Neuroblastoma is a cancer of the peripheral sympathetic nervous system, derived from embryonic neural crest cells (a neuroendocrine tumor) and is one of the few cancers known to

Neuroblastoma is the most common extracranial solid tumor in children, accounting for 7% to 8% of all childhood cancers. The prevalence is about 1 case per 7,000 live births. This incidence is fairly uniform throughout the world, at least for industrialized nations. Neuro‐ blastoma is slightly more common in boys than in girls, with a male-to-female sex ratio of 1.1 to 1 in most large studies [1]. While it accounts for 7% of all childhood malignancies, neuro‐ blastoma accounts for 10% of childhood cancer mortality. Neuroblastoma is a pediatric neoplasm that is the most common cancer diagnosed during infancy. POG and CCG institu‐ tions from 1986 to 2001 showed a median age at diagnosis of about 19 months. In this cohort, 36% were infants, 89% were younger than 5 years, and 98% were diagnosed by 10 years of age. The distribution of cases by age shows that this is a disease of infancy and early childhood,

Patients with low- and intermediate-risk NBL have an overall survival rate exceeding 90% with a trend toward minimization of therapy [3,4,5]. Standard therapy for patients with highrisk NBL involves multi-agent chemotherapy induction, surgery and external beam radio‐ therapy, myeloablative consolidation with autologous hematopoietic stem cell recue and biologic agents, including the recent demonstration that GD2- directed immunotherapy combined with cytokines significantly improves survival. Despite these achievements, 50% of

> © 2013 Malis; licensee InTech. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use,

© 2013 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution,

distribution, and reproduction in any medium, provided the original work is properly cited.

and reproduction in any medium, provided the original work is properly cited.

undergo spontaneous regression from an undifferentiated state to a benign tumor.

with the highest number of cases diagnosed in the first month of life [2].

Additional information is available at the end of the chapter

Josef Malis

**1. Introduction**

**2. Current outcome**

http://dx.doi.org/10.5772/55921
