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Multicenter Study
. 2024 Oct 8:15:1427821.
doi: 10.3389/fendo.2024.1427821. eCollection 2024.

Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1: a nationwide study

Nuria Valdés  1   2 , Ana Romero  3 , Estrella Diego  4 , María Calatayud  5 , Cristina Lamas  6 , Marta Araujo-Castro  7 , Cristina Álvarez-Escolá  8 , José A Díaz  9 , Victoria Alcázar  10 , Julia Sastre  11 , Rosa Martínez  12 , Josep Oriola  13 , Miguel Paja  14 , Paula Sánchez-Sobrino  15 , Isabel Salinas  16 , José María Recio-Córdova  17 , Elena Navarro  3 , María Dolores Chiara  18   19 , Luis Castaño  2 , Anna Casterás  20
Affiliations
Multicenter Study

Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1: a nationwide study

Nuria Valdés et al. Front Endocrinol (Lausanne). .

Abstract

Introduction: Historically, Multiple Endocrine Neoplasia type 1 (MEN1)-related pituitary adenomas (PAs) were considered more aggressive and treatment-resistant than sporadic PAs. However, recent studies suggest similarities in their behavior. This study aimed to evaluate the long-term outcomes of MEN1 PAs and identify predictive factors.

Methods: Nationwide multicenter retrospective cohort study of MEN1-related PAs with a minimum 1-year follow-up, collecting patient demographics, germline MEN1 pathogenic variants (PV), PA size, secretory profile, radiological characteristics, treatments, and outcomes.

Results: We analyzed 84 PAs, 69%in females and 31% in males (P<0.001), diagnosed at a mean age of 35.2±14.9 years, mostly through screening (60.7%). Median follow-up was 9 years (IQR:4-16). Prolactin-secreting PAs (PRLomas) (53.5%) and microadenomas (65.5%) were most common. Dopamine agonist treatment was first line for 16 macroPRLomas and 25 microPRLomas, 60.9% of them achieved PRL normalization. There was no significant association observed with tumor size, sex, treatment duration or MEN1 PV. The risk of progression from micro-PA to invasive macro-PA was 7.2% (4/55), after 8 years (IQR:4-13), all of them were microPRLomas. Kaplan-Meier estimation curve showed significantly higher progression probability in microPRLomas than in other microadenomas subtypes (P=0.017) or microNFPAs (P=0.032). No differences were found between sex, age, or germline MEN1 PV.

Conclusion: MEN1-related micro-PAs have a low risk of progressing to invasive macro-PAs, regardless of sex, age at diagnosis, or MEN1 germline PV. The risk is higher for microPRLomas over the long term. Therefore, long-term surveillance with reduced frequency, rather than intensive short-term monitoring, may be appropriate for patients with MEN1-related PAs.

Keywords: Multiple Endocrine Neoplasia type 1; macroadenomas; microadenomas; non-functioning pituitary adenomas; outcomes; pituitary adenomas; progression; prolactinomas.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Flowchart of patients selection. MEN1, Multiple Endocrine Neoplasia Type 1.
Figure 2
Figure 2
Flowchart of treatment and outcomes for prolactin secreting pituitary adenomas. DA, dopamine agonist; PRL, prolactin; HC, Hardy´s classification.
Figure 3
Figure 3
Flowchart of treatment and outcomes for non-functioning pituitary adenomas.
See this image and copyright information in PMC

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