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

    1. Márta Korbonits
    1. Department of Endocrinology, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK
      1Hereditary Endocrine Cancer Group, Spanish National Cancer Center, Madrid and ISCIII Center for Biomedical Research on Rare Diseases (CIBERER), Madrid, Spain
      2Section on Endocrinology and Genetics , Eunice Kennedy Shriver Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
    1. Correspondence should be addressed to M Korbonits; Email: m.korbonits{at}qmul.ac.uk


    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.

    • Revision received 9 June 2015
    • Accepted 15 June 2015
    • Made available online as an Accepted Preprint 25 June 2015
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