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Bone phenotypes in multiple endocrine neoplasia type 1: survey on the MEN1 Florentine database

Multiple endocrine neoplasia type 1 (MEN1) is a rare, inherited cancer syndrome characterized by the development of multiple endocrine and non-endocrine tumors. MEN1 patients show a reduction of bone mass and a higher prevalence of early onset osteoporosis, compared to healthy population of the same...

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Autores principales: Marini, Francesca, Giusti, Francesca, Iantomasi, Teresa, Cioppi, Federica, Brandi, Maria Luisa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bioscientifica Ltd 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9175581/
https://www.ncbi.nlm.nih.gov/pubmed/35324455
http://dx.doi.org/10.1530/EC-21-0456
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author Marini, Francesca
Giusti, Francesca
Iantomasi, Teresa
Cioppi, Federica
Brandi, Maria Luisa
author_facet Marini, Francesca
Giusti, Francesca
Iantomasi, Teresa
Cioppi, Federica
Brandi, Maria Luisa
author_sort Marini, Francesca
collection PubMed
description Multiple endocrine neoplasia type 1 (MEN1) is a rare, inherited cancer syndrome characterized by the development of multiple endocrine and non-endocrine tumors. MEN1 patients show a reduction of bone mass and a higher prevalence of early onset osteoporosis, compared to healthy population of the same age, gender, and ethnicity. During the monitoring and follow-up of MEN1 patients, the attention of clinicians is primarily focused on the diagnosis and therapy of tumors, while the assessment of bone health and mineral metabolism is, in many cases, marginally considered. In this study, we retrospectively analyzed bone and mineral metabolism features in a series of MEN1 patients from the MEN1 Florentine database. Biochemical markers of bone and mineral metabolism and densitometric parameters of bone mass were retrieved from the database and were analyzed based on age ranges and genders of patients and presence/absence of the three main MEN1-related endocrine tumor types. Our evaluation confirmed that patients with a MEN1 diagnosis have a high prevalence of earlyonset osteopenia and osteoporosis, in association with levels of serum and urinary markers of bone turnover higher than the normal reference values, regardless of their different MEN1 tumors. Fifty percent of patients younger than 26 years manifested osteopenia and 8.3% had osteoporosis, in at least one of the measured bone sites. These data suggest the importance of including biochemical and instrumental monitoring of bone metabolism and bone mass in the routine medical evaluation and follow-up of MEN1 patients and MEN1 carriers as important clinical aspects in the management of the syndrome.
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spelling pubmed-91755812022-06-14 Bone phenotypes in multiple endocrine neoplasia type 1: survey on the MEN1 Florentine database Marini, Francesca Giusti, Francesca Iantomasi, Teresa Cioppi, Federica Brandi, Maria Luisa Endocr Connect Research Multiple endocrine neoplasia type 1 (MEN1) is a rare, inherited cancer syndrome characterized by the development of multiple endocrine and non-endocrine tumors. MEN1 patients show a reduction of bone mass and a higher prevalence of early onset osteoporosis, compared to healthy population of the same age, gender, and ethnicity. During the monitoring and follow-up of MEN1 patients, the attention of clinicians is primarily focused on the diagnosis and therapy of tumors, while the assessment of bone health and mineral metabolism is, in many cases, marginally considered. In this study, we retrospectively analyzed bone and mineral metabolism features in a series of MEN1 patients from the MEN1 Florentine database. Biochemical markers of bone and mineral metabolism and densitometric parameters of bone mass were retrieved from the database and were analyzed based on age ranges and genders of patients and presence/absence of the three main MEN1-related endocrine tumor types. Our evaluation confirmed that patients with a MEN1 diagnosis have a high prevalence of earlyonset osteopenia and osteoporosis, in association with levels of serum and urinary markers of bone turnover higher than the normal reference values, regardless of their different MEN1 tumors. Fifty percent of patients younger than 26 years manifested osteopenia and 8.3% had osteoporosis, in at least one of the measured bone sites. These data suggest the importance of including biochemical and instrumental monitoring of bone metabolism and bone mass in the routine medical evaluation and follow-up of MEN1 patients and MEN1 carriers as important clinical aspects in the management of the syndrome. Bioscientifica Ltd 2022-03-23 /pmc/articles/PMC9175581/ /pubmed/35324455 http://dx.doi.org/10.1530/EC-21-0456 Text en © The authors https://creativecommons.org/licenses/by-nc/4.0/This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. (https://creativecommons.org/licenses/by-nc/4.0/)
spellingShingle Research
Marini, Francesca
Giusti, Francesca
Iantomasi, Teresa
Cioppi, Federica
Brandi, Maria Luisa
Bone phenotypes in multiple endocrine neoplasia type 1: survey on the MEN1 Florentine database
title Bone phenotypes in multiple endocrine neoplasia type 1: survey on the MEN1 Florentine database
title_full Bone phenotypes in multiple endocrine neoplasia type 1: survey on the MEN1 Florentine database
title_fullStr Bone phenotypes in multiple endocrine neoplasia type 1: survey on the MEN1 Florentine database
title_full_unstemmed Bone phenotypes in multiple endocrine neoplasia type 1: survey on the MEN1 Florentine database
title_short Bone phenotypes in multiple endocrine neoplasia type 1: survey on the MEN1 Florentine database
title_sort bone phenotypes in multiple endocrine neoplasia type 1: survey on the men1 florentine database
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9175581/
https://www.ncbi.nlm.nih.gov/pubmed/35324455
http://dx.doi.org/10.1530/EC-21-0456
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