Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1

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 ME...

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Detalles Bibliográficos
Autores: Valdés, Nuria, Romero, Ana, Diego, Estrella, Calatayud, María|||0000-0002-6737-7289, Lamas, Cristina|||0000-0003-4622-9508, Araujo-Castro, Marta|||0000-0002-0519-0072, Álvarez-Escolá, Cristina|||0000-0002-4684-1652, Díaz, José A., Alcázar, Victoria, Sastre, Julia|||0000-0002-1445-2601, Martínez, Rosa, Oriola, Josep, Paja, Miguel|||0009-0002-1079-4943, Sánchez-Sobrino, Paula|||0000-0002-1142-180X, Salinas, Isabel, Recio-Córdova, José María, Navarro Vives, Elena, Chiara, María Dolores|||0000-0002-1112-1583, Castaño, Luis|||0000-0003-0437-9401, Casterás, A.
Tipo de recurso: artículo
Fecha de publicación:2024
País:España
Institución:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:311598
Acceso en línea:https://ddd.uab.cat/record/311598
https://dx.doi.org/urn:doi:10.3389/fendo.2024.1427821
Access Level:acceso abierto
Palabra clave:Pituitary adenomas
Multiple Endocrine Neoplasia type 1
Non-functioning pituitary adenomas
Prolactinomas
Outcomes
Microadenomas
Macroadenomas
Progression
Descripción
Sumario: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.

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