**1. Introduction**

In the embryonic period, the nervous system originates from a layer of ectodermal cells which is called neuroectoderm. The neuroectoderm extends along the axis of the body to form the neural plate. The latter, turns inward and surrounds itself of neural folds, which then merge together giving life to the neural tube that will develop all the components of the central nervous system and the spinal cord. Finally, from the posterior portion of the neural tube, specialized cells will separate to form the neural crest. Various differentiated cell types, tissues and organs develop from neural crest cells, the mechanisms for this are not well known. However, these cells are multipotent and their subsequent specialization it could be conditioned by the activity of particular genes and by the microenvironment into which they migrate [1]. Hence, neural crest cells have a multipotent differentiation potential. From these cells originate neurons and glial cells, melanocytes, Schwann cells, parafollicular cells of the thyroid, cells of the adrenal medulla, endothelial cells of large vessels and some components of the connective and skeletal tissue of the head [2] (**Figure 1**). Based on these concepts, the tumors that develop from the cells of the neural crest represent a very varied and heterogeneous group of neoplasms, these can affect different body locations where the neural crest cellsderived are normally present. However, neural crest stem cells are also present in some adult tissues that normally do not originate from the neural crest, such as skin and bone marrow [3]. Heterogeneity of tumors originating from neural crest cells is confirmed by the fact that some of them can arise in both peripheral sites or in the central nervous system, while others are specific to the central nervous system or affect only other peripheral locations. Neural crest cell-derived tumors can be grouped into: tumors of peripheral and cranial nerves, melanocytic tumors, peripheral neuroblastic tumors, embryonal tumors of the central nervous system, paraganglioma group, and other tumors of neural crest origin. Most of these are sporadic, some can be hereditary (hereditary paraganglioma-pheochromocytoma syndrome, von Hippel–Lindau disease, neurofibromatosis, schwannomatosis, and multiple neuroendocrine neoplasia (MEN I). Pheochromocytoma/Paraganglyoma

**Figure 1.** *Segments of neural crest with relative adult tissues and pathologies derivate [2].*

and neuroblastoma are the most common neural crest-derived tumors in adults and children, respectively. These neoplasm are both associated with significant morbidity and mortality.
