Pheochromocytomas and Paragangliomas: New Developments with Regard to Classification, Genetics, and Cell of Origin.
GAPP
PASS
SDHB
paraganglioma
pheochromocytoma
scoring system
tumor classification
Journal
Cancers
ISSN: 2072-6694
Titre abrégé: Cancers (Basel)
Pays: Switzerland
ID NLM: 101526829
Informations de publication
Date de publication:
29 Jul 2019
29 Jul 2019
Historique:
received:
19
06
2019
revised:
22
07
2019
accepted:
23
07
2019
entrez:
1
8
2019
pubmed:
1
8
2019
medline:
1
8
2019
Statut:
epublish
Résumé
Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors that arise in the adrenal medulla and in extra-adrenal locations, such as the head, neck, thorax, abdomen, and pelvis. Classification of these tumors into those with or without metastatic potential on the basis of gross or microscopic features is challenging. Recent insights and scoring systems have attempted to develop solutions for this, as described in the latest World Health Organization (WHO) edition on endocrine tumor pathology. PCC and PGL are amongst the tumors most frequently accompanied by germline mutations. More than 20 genes are responsible for a hereditary background in up to 40% of these tumors; somatic mutations in the same and several additional genes form the basis for another 30%. However, this does not allow for a complete understanding of the pathogenesis or targeted treatment of PCC and PGL, for which surgery is the primary treatment and for which metastasis is associated with poor outcome. This review describes recent insights into the cell of origin of these tumors, the latest developments with regard to the genetic background, and the current status of tumor classification including proposed scoring systems.
Identifiants
pubmed: 31362359
pii: cancers11081070
doi: 10.3390/cancers11081070
pmc: PMC6721302
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
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