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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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
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spelling Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1a nationwide studyValdés, NuriaRomero, AnaDiego, EstrellaCalatayud, María|||0000-0002-6737-7289Lamas, Cristina|||0000-0003-4622-9508Araujo-Castro, Marta|||0000-0002-0519-0072Álvarez-Escolá, Cristina|||0000-0002-4684-1652Díaz, José A.Alcázar, VictoriaSastre, Julia|||0000-0002-1445-2601Martínez, RosaOriola, JosepPaja, Miguel|||0009-0002-1079-4943Sánchez-Sobrino, Paula|||0000-0002-1142-180XSalinas, IsabelRecio-Córdova, José MaríaNavarro Vives, ElenaChiara, María Dolores|||0000-0002-1112-1583Castaño, Luis|||0000-0003-0437-9401Casterás, A.Pituitary adenomasMultiple Endocrine Neoplasia type 1Non-functioning pituitary adenomasProlactinomasOutcomesMicroadenomasMacroadenomasProgressionIntroduction: 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. 22024-01-0120242024-01-01Articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://ddd.uab.cat/record/311598https://dx.doi.org/urn:doi:10.3389/fendo.2024.1427821reponame:Dipòsit Digital de Documents de la UABinstname:Universitat Autònoma de BarcelonaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.https://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:ddd.uab.cat:3115982026-06-06T12:50:31Z
dc.title.none.fl_str_mv Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1
a nationwide study
title Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1
spellingShingle Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1
Valdés, Nuria
Pituitary adenomas
Multiple Endocrine Neoplasia type 1
Non-functioning pituitary adenomas
Prolactinomas
Outcomes
Microadenomas
Macroadenomas
Progression
title_short Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1
title_full Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1
title_fullStr Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1
title_full_unstemmed Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1
title_sort Long term outcomes of pituitary adenomas in Multiple Endocrine Neoplasia type 1
dc.creator.none.fl_str_mv 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.
author Valdés, Nuria
author_facet 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.
author_role author
author2 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.
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Pituitary adenomas
Multiple Endocrine Neoplasia type 1
Non-functioning pituitary adenomas
Prolactinomas
Outcomes
Microadenomas
Macroadenomas
Progression
topic Pituitary adenomas
Multiple Endocrine Neoplasia type 1
Non-functioning pituitary adenomas
Prolactinomas
Outcomes
Microadenomas
Macroadenomas
Progression
description 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.
publishDate 2024
dc.date.none.fl_str_mv 2
2024-01-01
2024
2024-01-01
dc.type.none.fl_str_mv Article
http://purl.org/coar/resource_type/c_6501
VoR
http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://ddd.uab.cat/record/311598
https://dx.doi.org/urn:doi:10.3389/fendo.2024.1427821
url https://ddd.uab.cat/record/311598
https://dx.doi.org/urn:doi:10.3389/fendo.2024.1427821
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
https://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:Dipòsit Digital de Documents de la UAB
instname:Universitat Autònoma de Barcelona
instname_str Universitat Autònoma de Barcelona
reponame_str Dipòsit Digital de Documents de la UAB
collection Dipòsit Digital de Documents de la UAB
repository.name.fl_str_mv
repository.mail.fl_str_mv
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