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- Review Article Pituitary Neuroendocrine Tumors in Multiple Endocrine Neoplasia
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Sang Ouk Chin1
, Constance Chik2, Toru Tateno2
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DOI: https://doi.org/10.3803/EnM.2024.2074 [Epub ahead of print]
Published online: August 30, 2024
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2Division of Endocrinology and Metabolism, Department of Medicine, University of Alberta, Edmonton, AB, Canada
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Corresponding author:
Toru Tateno, Tel: +1-780-492-3626, Fax: +1-780-492-6444,
Email: tateno@ualberta.ca
Abstract
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal-dominant disorder characterized by tumors of the pituitary, parathyroid, and endocrine-gastrointestinal tract. Pituitary neuroendocrine tumors (PitNETs) occur in about 40% of MEN1 cases, with 10% being the first manifestation. Recent studies show a slight female predominance, with microPitNETs (<1 cm) being more common than macroPitNETs (>1 cm). Functional PitNETs (FPitNETs) are more frequent than non-functional ones (36% to 48%), with prolactinomas being the most common FPitNETs. MEN1-associated PitNETs are often plurihormonal, larger, and more invasive compared to sporadic types, though patient age and FPitNET proportions are similar. MEN1 mutation-negative patients tend to have larger, symptomatic PitNETs at diagnosis. Six patients with MEN1 have been reported to have pituitary carcinomas, including a mutation- negative patient. Treatment approach between PitNETs in MEN1 and sporadic types appears to be similar. PitNETs also occur in MEN4, but their epidemiology is less understood. In patients with a MEN1-like phenotype and negative genetic testing, MEN4 should be considered.
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