An Update on the Histology of Pheochromocytomas: How Does it Relate to Genetics?
Journal
Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme
ISSN: 1439-4286
Titre abrégé: Horm Metab Res
Pays: Germany
ID NLM: 0177722
Informations de publication
Date de publication:
Jul 2019
Jul 2019
Historique:
pubmed:
25
8
2018
medline:
20
12
2019
entrez:
25
8
2018
Statut:
ppublish
Résumé
Pheochromocytomas are rare neuroendocrine tumors of the adrenal gland, whereas any extra-adrenal tumor with similar histology is designated as paraganglioma. These tumors have a very high rate of germline mutations in a large number of genes, up to 35% to 40%, frequently predisposing for other tumors as well. Therefore, they represent a phenomenal challenge for treating physicians. This review focuses on pheochromocytomas only, with special attention to gross and microscopic clues to the diagnosis of genetic syndromes, including the role of succinate dehydrogenase subunit A and subunit B immunohistochemistry as surrogate markers for genetic analysis in the field of succinate dehydrogenase subunit gene mutations.
Substances chimiques
Neoplasm Proteins
0
Electron Transport Complex II
EC 1.3.5.1
SDHA protein, human
EC 1.3.5.1
SDHB protein, human
EC 1.3.5.1
Succinate Dehydrogenase
EC 1.3.99.1
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
403-413Informations de copyright
© Georg Thieme Verlag KG Stuttgart · New York.
Déclaration de conflit d'intérêts
The authors declare that they have no conflict of interest.