Diagnosis and management of pituitary adenomas in children and adolescents.

Eur J Endocrinol

INSERM-U1185, Paris Sud Paris-Saclay University and AP-HP Division of Endocrinology and Diabetes for Children, Bicêtre Paris Sud Hospital, 64 Gabriel Péri Street, 94270 Le Kremlin Bicêtre, France.

Published: September 2024

AI Article Synopsis

  • Pituitary adenomas (PA), or Pit-NETS, are rare in children and especially unusual in those under 10, with the majority of research conducted on adult cases.
  • This review addresses the unique clinical considerations, diagnosis, and treatment of PA in young patients, highlighting the importance of involving specialized teams that include both pediatric and adult medical experts.
  • Pediatric PAs tend to be larger, more aggressive, and often lead to hormonal imbalances that can affect growth and puberty, with the most common type being prolactinomas; genetic testing is recommended for young patients due to potential hereditary mutations linked to these tumors.

Article Abstract

Background: Pituitary adenomas (PAs)-also now called pituitary neuroendocrine tumours or Pit-NETS-are rare in children and adolescents and exceptional below the age of 10. Most evidence-based high-quality data are derived from larger studies in adult patients.

Aims: We will review recent knowledge on the epidemiology, clinical features, diagnosis, and treatment modalities of the different types of pituitary adenomas diagnosed in children and adolescents, emphasizing the many reasons why these cases should be discussed within pituitary-specific multidisciplinary teams with experts from both paediatric and adult practice.

Conclusions: Paediatric PA presents multiple peculiarities that may challenge their adequate management. They are overall proportionally larger and more aggressive than in adults, with potential mass effects including hypopituitarism. Hormonal hypersecretion is frequent, resulting in clinical syndromes affecting normal growth and pubertal development. Prolactinomas represent the most frequent subtype of PA found during childhood, followed by adrenocorticotropin (ACTH) and growth hormone (GH)-secreting adenomas, while clinically non-functioning adenomas are exceptionally diagnosed before the age of 16. The occurrence of a pituitary tumour in a young individual should also prompt genetic testing in each case, searching for either germline mutations in one of the known genes that may drive inherited/familial PA (such as the multiple endocrine neoplasia type 1 or MEN1 gene, or the aryl hydrocarbon receptor interacting protein or AIP gene), or for a mosaic activating mutation of GNAS as found in the McCune-Albright syndrome.

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http://dx.doi.org/10.1093/ejendo/lvae120DOI Listing

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