Outcome and Long-term Follow-up of Adrenal Lesions in Multiple Endocrine Neoplasia Type 1

Overview

Journal Arch Endocrinol Metab

Specialty Endocrinology

Date 2019 Sep 5

PMID 31482957

Citations 6

Authors

Mara Ventura

Miguel Melo

Francisco Carrilho

Affiliations

Soon will be listed here.

Abstract

Objective: To describe the prevalence, clinical characteristics and outcome of adrenal lesions in long-term follow-up of Multiple endocrine neoplasia type 1 (MEN1) patients.

Subjects And Methods: We retrospectively studied sixteen patients from six families of individuals with MEN1. Adrenal involvement was evaluated using clinical, biochemical and imaging data.

Results: Adrenal lesions were identified in nine of sixteen (56.3%) patients: seven women and two men (mean age: 52.2 years). Adrenal involvement was detected at MEN1 diagnosis in more than half of the patients. Eighteen adrenal nodules were founded (median of two nodules per patient) with mean adrenal lesion diameter of 17.4 mm. Three patients had unilateral adrenal involvement. Hormonal hypersecretion (autonomous cortisol secretion) was found in two patients. None of the patients was submitted to adrenalectomy, presented an aldosterone-secreting lesion, a pheochromocytoma, an adrenal carcinoma or metastatic disease during the follow-up. A predominance of stable adrenal disease, in terms of size and hormonal secretion, was observed. Adrenal lesions were evenly distributed between the germline mutations.

Conclusion: Adrenal tumours are a common feature of MEN1 that can affect more than half of the patients. Most of the tumours are bilateral non-functional lesions, but hormonal secretion may occur and should be promptly identified to reduce the morbidity/mortality of the syndrome. Periodic surveillance of these patients should be performed.

Citing Articles

Beyond the three P's: adrenal involvement in MEN1.

Clemente-Gutierrez U, Pieterman C, Lui M, Szabo Yamashita T, Tame-Elorduy A, Huang B Endocr Relat Cancer. 2023; 31(2).

PMID: 38108666 PMC: 10854230. DOI: 10.1530/ERC-23-0162.

Clinical and molecular features of four Brazilian families with multiple endocrine neoplasia type 1.

Miranda I, Valadares L, Barra G, Mesquita P, Bandeira de Santana L, de Castro L Front Endocrinol (Lausanne). 2023; 14:1117873.

PMID: 36967793 PMC: 10036827. DOI: 10.3389/fendo.2023.1117873.

A MEN1 Patient Presenting With Multiple Parathyroid Adenomas and Transient Hypercortisolism: A Case Report and Literature Review.

Chen F, Xu Q, Yue W, Yu X, Shao S Front Endocrinol (Lausanne). 2022; 13:802453.

PMID: 35370956 PMC: 8965320. DOI: 10.3389/fendo.2022.802453.

Overview of the 2022 WHO Classification of Adrenal Cortical Tumors.

Mete O, Erickson L, Juhlin C, de Krijger R, Sasano H, Volante M Endocr Pathol. 2022; 33(1):155-196.

PMID: 35288842 PMC: 8920443. DOI: 10.1007/s12022-022-09710-8.

Coexistence of Renin-independent Aldosterone Secretion and Multiple Endocrine Neoplasia Type 1 Within a Family.

Obata Y, Takayama K, Maruo Y, Yamaguchi H, Fujii K, Hata S J Endocr Soc. 2022; 6(3):bvac009.

PMID: 35187382 PMC: 8852679. DOI: 10.1210/jendso/bvac009.

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