15 YEARS OF PARAGANGLIOMA: The association of pituitary adenomas and phaeochromocytomas or paragangliomas.

Abstract:

:The combination of pituitary adenomas (PA) and phaeochromocytomas (phaeo) or paragangliomas (PGL) is a rare event. Although these endocrine tumours may occur together by coincidence, there is mounting evidence that, in at least some cases, classical phaeo/PGL-predisposing genes may also play a role in pituitary tumorigenesis. A new condition that we termed '3Pas' for the association of PA with phaeo and/or PGL was recently described in patients with succinate dehydrogenase mutations and PAs. It should also be noted that the classical tumour suppressor gene, MEN1 that is the archetype of the PA-predisposing genes, is also rarely associated with phaeos in both mice and humans with MEN1 defects. In this report, we review the data leading to the discovery of 3PAs, other associations linking PAs with phaeos and/or PGLs, and the corresponding clinical and molecular genetics.

journal_name

Endocr Relat Cancer

journal_title

Endocrine-related cancer

authors

O'Toole SM,Dénes J,Robledo M,Stratakis CA,Korbonits M

doi

10.1530/ERC-15-0241

subject

Has Abstract

pub_date

2015-08-01 00:00:00

pages

T105-22

issue

4

eissn

1351-0088

issn

1479-6821

pii

ERC-15-0241

journal_volume

22

pub_type

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