MEN1 in children and adolescents: Data from patients of a regional referral center for hereditary endocrine tumors

Endocrine
Letizia VannucciMaria Luisa Brandi

Abstract

To retrospectively evaluate the age of onset of MEN1-associated lesions in a group of affected children and adolescents and to compare the clinical features of our series with the evidence derived from the literature. The study population consisted of 22 Italian children and adolescents (age 6-31 years at the time of the inclusion in this study) all with a clinical and/or a genetic diagnosis of MEN1 performed before the age of 16 who have been followed-up regularly from 1998 to 2016 at the Regional Referral Center for Hereditary Endocrine Tumors. Clinical, biochemical, imaging and genetic data have been collected for each patient. Ten subjects (45.5%) have not yet presented any clinical/biochemical/radiological manifestation of MEN1 disease, whereas 12 patients (54.5%) developed at least one MEN1-associated endocrine manifestation. The second group of patients was significantly older than the first one. The most frequent manifestation was primary hyperparathyroidism (50%), followed by pituitary tumors (prolactinomas) (31.8%) and nonfunctioning pancreatic neuroendocrine tumors (9%). The earliest cases of primary hyperparathyroidism and prolactinoma were a 12-year-old girl and a 13-year-old boy, respectively. MEN1 disease seems t...Continue Reading

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Citations

Aug 5, 2017·Endocrine-related Cancer·Rachel S van LeeuwaardeGerlof D Valk
Jan 9, 2020·Reviews in Endocrine & Metabolic Disorders·Christina Tatsi, Constantine A Stratakis
Nov 16, 2018·Orphanet Journal of Rare Diseases·Francesca MariniMaria Luisa Brandi
Mar 23, 2019·Frontiers in Endocrinology·Fabio Luiz de Menezes MontenegroSergio Pereira de Almeida Toledo
May 1, 2020·Frontiers in Endocrinology·Delmar M Lourenço, Wouter W de Herder
Nov 30, 2020·Endocrine Reviews·Maria Luisa BrandiRajesh V Thakker
Mar 16, 2021·Frontiers in Endocrinology·Jenny E Blau, William F Simonds
Apr 12, 2021·Annales d'endocrinologie·Aurore GeslotDelphine Vezzosi
May 4, 2021·Case Reports in Endocrinology·Gerdi TuliLuisa De Sanctis

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