Cargando…
Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Single Center Experience
Background: Primary hyperparathyroidism (PHPT) in the most common and earliest manifestation of multiple endocrine neoplasia type-1 (MEN1). Epidemiological data have been reported in MEN1 patients but data on long-term follow-up focusing on PHPT are scarce. Methods: In this retrospective cohort stud...
Autores principales: | , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8999236/ https://www.ncbi.nlm.nih.gov/pubmed/35407574 http://dx.doi.org/10.3390/jcm11071967 |
_version_ | 1784685140739358720 |
---|---|
author | Yavropoulou, Maria P. Vlachou, Sofia Tsoli, Marina Fostira, Florentia Kaltsas, Gregory Kassi, Eva |
author_facet | Yavropoulou, Maria P. Vlachou, Sofia Tsoli, Marina Fostira, Florentia Kaltsas, Gregory Kassi, Eva |
author_sort | Yavropoulou, Maria P. |
collection | PubMed |
description | Background: Primary hyperparathyroidism (PHPT) in the most common and earliest manifestation of multiple endocrine neoplasia type-1 (MEN1). Epidemiological data have been reported in MEN1 patients but data on long-term follow-up focusing on PHPT are scarce. Methods: In this retrospective cohort study, we included patients diagnosed with MEN1-related PHPT that were under regular follow-up in our institution. Results: Data on 68 patients (39 males), with a mean age at MEN1-diagnosis of 39 ± 13.06 years, were analyzed. Pancreatic neuroendocrine tumors were encountered in 82% (71% nonsecreting) followed by pituitary adenomas in 66% (49% nonsecreting). Mean age at PHPT diagnosis was 35.2 ± 4.0 years. Parathyroidectomy was performed in 57 patients (82.3%), of whom 56% achieved long-term remission, while 12.2% and 31.5% had persistent and recurrent disease, respectively (median follow-up of 4 years; range 1–21 years). Cinacalcet restored serum calcium levels in 33.8%, both as first and as a second line treatment. Permanent hypoparathyroidism occurred in 19.2%. MEN1 pathogenic variants were identified in 77.2% of the tested individuals, but no genotype-phenotype associations were reported. Conclusions: MEN1-related PHPT involves a multiglandular disease and its management remains a therapeutic challenge, as recurrent disease can develop even after 20 years of follow-up. Prolonged follow-up of these patients at referral centers is critical for their optimal management. |
format | Online Article Text |
id | pubmed-8999236 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-89992362022-04-12 Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Single Center Experience Yavropoulou, Maria P. Vlachou, Sofia Tsoli, Marina Fostira, Florentia Kaltsas, Gregory Kassi, Eva J Clin Med Article Background: Primary hyperparathyroidism (PHPT) in the most common and earliest manifestation of multiple endocrine neoplasia type-1 (MEN1). Epidemiological data have been reported in MEN1 patients but data on long-term follow-up focusing on PHPT are scarce. Methods: In this retrospective cohort study, we included patients diagnosed with MEN1-related PHPT that were under regular follow-up in our institution. Results: Data on 68 patients (39 males), with a mean age at MEN1-diagnosis of 39 ± 13.06 years, were analyzed. Pancreatic neuroendocrine tumors were encountered in 82% (71% nonsecreting) followed by pituitary adenomas in 66% (49% nonsecreting). Mean age at PHPT diagnosis was 35.2 ± 4.0 years. Parathyroidectomy was performed in 57 patients (82.3%), of whom 56% achieved long-term remission, while 12.2% and 31.5% had persistent and recurrent disease, respectively (median follow-up of 4 years; range 1–21 years). Cinacalcet restored serum calcium levels in 33.8%, both as first and as a second line treatment. Permanent hypoparathyroidism occurred in 19.2%. MEN1 pathogenic variants were identified in 77.2% of the tested individuals, but no genotype-phenotype associations were reported. Conclusions: MEN1-related PHPT involves a multiglandular disease and its management remains a therapeutic challenge, as recurrent disease can develop even after 20 years of follow-up. Prolonged follow-up of these patients at referral centers is critical for their optimal management. MDPI 2022-04-01 /pmc/articles/PMC8999236/ /pubmed/35407574 http://dx.doi.org/10.3390/jcm11071967 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Yavropoulou, Maria P. Vlachou, Sofia Tsoli, Marina Fostira, Florentia Kaltsas, Gregory Kassi, Eva Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Single Center Experience |
title | Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Single Center Experience |
title_full | Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Single Center Experience |
title_fullStr | Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Single Center Experience |
title_full_unstemmed | Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Single Center Experience |
title_short | Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Single Center Experience |
title_sort | management and long-term follow-up of hyperparathyroidism in multiple endocrine neoplasia type 1: single center experience |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8999236/ https://www.ncbi.nlm.nih.gov/pubmed/35407574 http://dx.doi.org/10.3390/jcm11071967 |
work_keys_str_mv | AT yavropouloumariap managementandlongtermfollowupofhyperparathyroidisminmultipleendocrineneoplasiatype1singlecenterexperience AT vlachousofia managementandlongtermfollowupofhyperparathyroidisminmultipleendocrineneoplasiatype1singlecenterexperience AT tsolimarina managementandlongtermfollowupofhyperparathyroidisminmultipleendocrineneoplasiatype1singlecenterexperience AT fostiraflorentia managementandlongtermfollowupofhyperparathyroidisminmultipleendocrineneoplasiatype1singlecenterexperience AT kaltsasgregory managementandlongtermfollowupofhyperparathyroidisminmultipleendocrineneoplasiatype1singlecenterexperience AT kassieva managementandlongtermfollowupofhyperparathyroidisminmultipleendocrineneoplasiatype1singlecenterexperience |