The causes and consequences of pituitary gigantism

[en] In the general population, height is determined by a complex interplay between genetic and environmental factors. Pituitary gigantism is a rare but very important subgroup of patients with excessive height, as it has an identifiable and clinically treatable cause. The disease is caused by chronic growth hormone and insulin-like growth factor 1 secretion from a pituitary somatotrope adenoma that forms before the closure of the epiphyses. If not controlled effectively, this hormonal hypersecretion could lead to extremely elevated final adult height. The past 10 years have seen marked advances in the understanding of pituitary gigantism, including the identification of genetic causes in ~50% of cases, such as mutations in the AIP gene or chromosome Xq26.3 duplications in X-linked acrogigantism syndrome. Pituitary gigantism has a male preponderance, and patients usually have large pituitary adenomas. The large tumour size, together with the young age of patients and frequent resistance to medical therapy, makes the management of pituitary gigantism complex. Early diagnosis and rapid referral for effective therapy appear to improve outcomes in patients with pituitary gigantism; therefore, a high level of clinical suspicion and efficient use of diagnostic resources is key to controlling overgrowth and preventing patients from reaching very elevated final adult heights.

Disciplines :

Endocrinology, metabolism & nutrition

Author, co-author :

Beckers, Albert ; Université de Liège - ULiège > Département des sciences cliniques > Endocrinologie

PETROSSIANS, Patrick ; Centre Hospitalier Universitaire de Liège - CHU > Département de médecine interne > Service d'endocrinologie clinique

Hanson, Julien ; Université de Liège - ULiège > Département de pharmacie > Chimie pharmaceutique

Daly, Adrian ; Université de Liège - ULiège > Département des sciences cliniques > Endocrinologie

Language :

English

Title :

The causes and consequences of pituitary gigantism

Publication date :

25 October 2018

Journal title :

Nature Reviews. Endocrinology

ISSN :

1759-5029

eISSN :

1759-5037

Publisher :

Nature Publishing Group, United Kingdom

Volume :

Pages :

705-720

Peer reviewed :

Peer Reviewed verified by ORBi

Additional URL :

https://doi.org/10.1038/s41574-018-0114-1

Available on ORBi :

since 26 October 2018

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