Introduction

Peripheral neuroblastic tumors (pNTs), which include neuroblastoma, ganglioneuroblastoma, and ganglioneuroma, are common pediatric tumors (Ross et al. 1996). These tumors are derived from immature sympathetic neuroblasts during embryonic, fetal, or early postnatal development, and their morphological features appear to recapitulate developmental stages of sympathetic ganglia. Their primary sites are anatomically related to the embryological distribution of neural crest cells, and include adrenal gland and structures of the sympathetic nervous system.

For many years pNTs were characterized as "enigmatic" because of their unexpected clinical behaviors, such as involution/spontaneous regression, maturation, or aggressive progression. Because of recent advances in clinical and basic research, pNTs now are considered to be biologically heterogeneous, and their individual molecular properties like account for their unique clinical behaviors (Brodeur and Maris 2002). Based primarily on their clinical biology, the International Neuroblastoma Pathology Classification (Shimada et al. 1999a,b) was established by adopting the concept of the original Shimada system (age-linked evaluation of the morphological features in this disease). In this chapter, histopathology of the pNTs is illustrated according to the Classification along with its biological relevance.

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