Neural Crest Cell-Derived Tumors: An Overview

Abstract

Neural crest cells are able to give rise to various differentiated cell types, tissues, and organs. Regarding the multipotent differentiation potential of these cells, it is not surprising that tumors that have originated from neural crest cells represent a heterogeneous group of neoplasms. These tumors can arise in various locations throughout the body where neural crest derived cells are normally present. However, they can be found in some unusual location as well, probably arising from neural crest stem cells, which shown to be present in a wide range of tissues in adults including those that are not specifically derived from neural crest. Some types of neural crest cell-derived tumors can arise both in peripheral locations, and in the CNS, while others are specific only to the CNS or can occur only outside the CNS. To some extent, the classification of neural crest cell-derived tumors has changed over time. Some tumors that were previously proposed to be of neural crest origin such as those arising from neuroendocrine cells of the gastrointestinal tract were shown to have a different embryologic origin. In other neoplasms, mostly from the heterogeneous group of primitive neuroectodermal tumors that are generally regarded as neural crest-cell derived tumors, the precise origin remains unknown. In this chapter, we will review the current concept of neural crest-cells derived tumors, focusing on tumors of the CNS.