| Neuroendocrine tumors and tumor syndromes in childhood. | |
| | |
MedLine Citation:
|
PMID: 19708762 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
|
Abstract Endocrine and neuroendocrine cells form a large and diverse array of cell types. They are present in the form of specialized organs, such as the pituitary, parathyroid, thyroid, and adrenal gland, or in the form of the diffuse neuroendocrine system in the respiratory and digestive tracts. Neuroendocrine tumors are a heterogeneous group of neoplasms, yet they present certain unifying features. These include frequent hormonal overproduction that leads to specific symptoms and a typical immunohistochemical staining profile with chromogranin A and synaptophysin reactivity. Over the past decades, many neuroendocrine tumors have been described in the context of heritable tumor syndromes, and there exist several syndromes that are almost entirely composed of neuroendocrine tumors. Tumors occurring as part of these hereditary syndromes are characterized by specific genetic abnormalities that have helped our understanding of tumorigenesis, and they frequently appear at a young age. It is therefore important for the pediatric pathologist to be aware of specific histologic characteristics of neuroendocrine tumors in childhood and of their association with specific tumor syndromes. This may alert other clinicians to the possibility of multiple tumors in the patient or his family members. This review focuses on hereditary syndromes with neuroendocrine tumors, including multiple endocrine neoplasia types 1 and 2, Von Hippel-Lindau disease, neurofibromatosis type 1, Carney complex, pheochromocytoma-paraganglioma syndrome, and familial nonmedullary thyroid carcinoma. In addition, several individual neuroendocrine tumors are described, such as medullary thyroid carcinoma, gastroenteropancreatic tumors, pheochromocytoma, and paraganglioma, emphasizing specific histopathologic characteristics. |
| | |
Authors:
|
José Gaal; Ronald R de Krijger |
Publication Detail:
|
Type: Journal Article Date: 2009-08-26 |
Journal Detail:
|
Title: Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society Volume: 13 ISSN: 1093-5266 ISO Abbreviation: Pediatr. Dev. Pathol. Publication Date: 2010 Nov-Dec |
Date Detail:
|
Created Date: 2011-02-24 Completed Date: - Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 9809673 Medline TA: Pediatr Dev Pathol Country: United States |
Other Details:
|
Languages: eng Pagination: 427-41 Citation Subset: IM |
Affiliation:
|
Department of Pathology, Josephine Nefkens Institute, Erasmus MC-University Medical Center, Rotterdam, The Netherlands. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Polymer crystallization in the presence of "sticky" additives.
Next Document: Controllable expansion of primary cardiomyocytes by reversible immortalization.