From the Department of Radiology, Stead Family Children's Hospital, University of Iowa, University of Iowa Hospitals and Clinics, 200 Hawkins Dr, 3889 JPP, Iowa City, IA 52242.
Radiographics. 2019 Nov-Dec;39(7):2085-2102. doi: 10.1148/rg.2019190086.
The neural crest is an important transient structure that develops during embryogenesis in vertebrates. Neural crest cells are multipotent progenitor cells that migrate and develop into a diverse range of cells and tissues throughout the body. Although neural crest cells originate from the ectoderm, they can differentiate into mesodermal-type or endodermal-type cells and tissues. Some of these tissues include the peripheral, autonomic, and enteric nervous systems; chromaffin cells of the adrenal medulla; smooth muscles of the intracranial blood vessels; melanocytes of the skin; cartilage and bones of the face; and parafollicular cells of the thyroid gland. Neurocristopathies are a group of diseases caused by the abnormal generation, migration, or differentiation of neural crest cells. They often involve multiple organ systems in a single person, are often familial, and can be associated with the development of neoplasms. As understanding of the neural crest has advanced, many seemingly disparate diseases, such Treacher Collins syndrome, 22q11.2 deletion syndrome, Hirschsprung disease, neuroblastoma, neurocutaneous melanocytosis, and neurofibromatosis, have come to be recognized as neurocristopathies. Neurocristopathies can be divided into three main categories: dysgenetic malformations, neoplasms, and combined dysgenetic and neoplastic syndromes. In this article, neural crest development, as well as several associated dysgenetic, neoplastic, and combined neurocristopathies, are reviewed. Neurocristopathies often have clinical manifestations in multiple organ systems, and radiologists are positioned to have significant roles in the initial diagnosis of these disorders, evaluation of subclinical associated lesions, creation of treatment plans, and patient follow-up. ©RSNA, 2019.
神经嵴是脊椎动物胚胎发生过程中形成的一种重要的短暂结构。神经嵴细胞是多能祖细胞，它们迁移并发育成全身各种细胞和组织。尽管神经嵴细胞起源于外胚层，但它们可以分化为中胚层型或内胚层型细胞和组织。其中一些组织包括周围、自主和肠神经系统；肾上腺髓质的嗜铬细胞；颅内血管的平滑肌；皮肤的黑素细胞；面部的软骨和骨骼；以及甲状腺的滤泡旁细胞。神经嵴病变是一组由神经嵴细胞异常产生、迁移或分化引起的疾病。它们通常涉及一个人多个器官系统，通常是家族性的，并且可能与肿瘤的发展有关。随着对神经嵴的认识不断提高，许多看似不同的疾病，如特雷彻·柯林斯综合征、22q11.2 缺失综合征、先天性巨结肠、神经母细胞瘤、神经皮肤黑色素细胞增多症和神经纤维瘤病，已被认为是神经嵴病变。神经嵴病变可分为三大类：发育不良性畸形、肿瘤和发育不良性与肿瘤性联合综合征。本文综述了神经嵴的发育以及几种相关的发育不良性、肿瘤性和发育不良性与肿瘤性联合神经嵴病变。神经嵴病变通常在多个器官系统中有临床表现，放射科医生在这些疾病的初步诊断、亚临床相关病变的评估、治疗计划的制定和患者随访中具有重要作用。 ©RSNA，2019 年。
