Journal article
Neurocristopathies: Enigmatic Appearances of Neural Crest Cell-derived Abnormalities
Radiographics, Vol.39(7), pp.2085-2102
11/01/2019
DOI: 10.1148/rg.2019190086
PMID: 31697622
Abstract
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. (C) RSNA, 2019
Details
- Title: Subtitle
- Neurocristopathies: Enigmatic Appearances of Neural Crest Cell-derived Abnormalities
- Creators
- T. Shawn Sato - University of Iowa Hospitals and ClinicsAtsuhiko Handa - University of Iowa Hospitals and ClinicsSarv Priya - University of Iowa Hospitals and ClinicsPankaj Watal - University of Iowa Hospitals and ClinicsRobert M. Becker - University of IowaYutaka Sato - University of Iowa Hospitals and Clinics
- Resource Type
- Journal article
- Publication Details
- Radiographics, Vol.39(7), pp.2085-2102
- Publisher
- Radiological Soc North America
- DOI
- 10.1148/rg.2019190086
- PMID
- 31697622
- ISSN
- 0271-5333
- eISSN
- 1527-1323
- Number of pages
- 18
- Language
- English
- Date published
- 11/01/2019
- Academic Unit
- Radiology; Stead Family Department of Pediatrics
- Record Identifier
- 9984318685702771
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