Adrenal involvement in MEN1. Analysis of 715 cases from the Groupe d'etude des Tumeurs Endocrines database
Open accessAbstract
OBJECTIVE: Limited data regarding adrenal involvement in multiple endocrine neoplasia type 1 (MEN1) is available. We describe the characteristics of MEN1-associated adrenal lesions in a large cohort to provide a rationale for their management. METHODS: Analysis of records from 715 MEN1 patients from a multicentre database between 1956 and 2008. Adrenal lesions were compared with those from a multicentre cohort of 144 patients with adrenal sporadic incidentalomas. RESULTS: Adrenal enlargement was reported in 20.4% (146/715) of patients. Adrenal tumours (>10 mm in size) accounted for 58.1% of these cases (10.1% of the whole patient cohort). Tumours were bilateral and >40 mm in size in 12.5 and 19.4% of cases respectively. Hormonal hypersecretion was restricted to patients with tumours and occurred in 15.3% of them. Compared with incidentalomas, MEN1-related tumours exhibited more cases of primary hyperaldosteronism, fewer pheochromocytomas and more adrenocortical carcinomas (ACCs; 13.8 vs 1.3%). Ten ACCs occurred in eight patients. Interestingly, ACCs occurred after several years of follow-up of small adrenal tumours in two of the eight affected patients. Nine of the ten ACCs were classified as stage I or II according to the European Network for the Study of Adrenal Tumors. No evident genotype/phenotype correlation was found for the occurrence of adrenal lesions, endocrine hypersecretion or ACC. CONCLUSIONS: Adrenal pathology in MEN1 differs from that observed in sporadic incidentalomas. In the absence of relevant symptoms, endocrine biology can be restricted to patients with adrenal tumours and should focus on steroid secretion including the aldosterone-renin system. MEN1 is a high-risk condition for the occurrence of ACCs. It should be considered regardless of the size of the tumour.
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Associated adrenal lesions, mainly ACAs and uni- or bilateral hyperplasia, occur in 20% to 55% of MEN1 cases.
“Associated adrenal lesions, mainly ACAs and uni- or bilateral hyperplasia, occur in 20% to 55% of MEN1 cases (45–48).”
From Adrenocortical carcinoma (2014) · cites this paper as [48]
Recent analysis of a French multicenter registry determined that ∼10% of MEN1 patients have distinct adrenal tumors, and of these, up to 14% are malignant.
“Recent analysis of a French multicenter registry determined that ∼10% of MEN1 patients have distinct adrenal tumors, and of these, up to 14% are malignant (48).”
From Adrenocortical carcinoma (2014) · cites this paper as [48]
A small fraction of patients with MEN1 will develop ACC.
“A small fraction of patients with MEN1 will develop ACC (46–51).”
From Adrenocortical carcinoma (2014) · cites this paper as [48]
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- Authors
- Gatta-Cherifi, B., Chabre, O., Murat, A., Niccoli, P., Cardot-Bauters, C., Rohmer, V, Young, J., Delemer, B., Du Boullay, H., Verger, M. F., Kuhn, J. M., Sadoul, J. L., Ruszniewski, P., Beckers, A., Monsaingeon, M., Baudin, E., Goudet, P., & Tabarin, A.
- Affiliations
- Centre Hospitalier Universitaire de Grenoble (FR)
- Centre Hospitalier Universitaire de Nantes (FR)
- Hôpital de la Timone (FR)
- Centre Hospitalier Universitaire d'Angers (FR)
- Université Paris-Sud (FR)
- Centre Hospitalier Universitaire de Reims (FR)
- Centre Hospitalier Universitaire de Rouen (FR)
- Centre Hospitalier Universitaire de Nice (FR)
- Hôpital Beaujon (FR)
- Centre Hospitalier Universitaire de Liège (BE)
- Centre Hospitalier Universitaire de Bordeaux (FR)
- Université de Bourgogne (FR)
- Corresponding author
- Tabarin, A
- Venue
- European Journal of Endocrinology
- Publisher
- Oxford University Press (OUP)
- Published
- 2012-02
- Volume
- 166
- Issue
- 2
- Pages
- 269-279
- ISSN
- 0804-4643 (ISSN-L)
- 1479-683X
- OpenAlex
- W2135367483
- Cited by
- 230
- References
- 45
- Citations by year
- 2026: 14 · 2025: 13 · 2024: 17 · 2023: 14 · 2022: 23 · 2021: 22 · 2020: 19 · 2019: 12 · 2018: 17 · 2017: 18 · 2016: 20 · 2015: 18 · 2014: 9 · 2013: 7 · 2012: 4
- FWCI
- 6.54
- Type
- journal article
- Language
- en
- Open access
- green
- orbi.uliege.be
- Licence
- other-oa
- Locations
- European Journal of Endocrinology (publishedVersion)
- PubMed (publishedVersion)
- ORBi (University of Liège) (submittedVersion, other-oa)
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
- Health Sciences › Medicine › Oncology › Ear and Head Tumors
- Keywords
- multiple endocrine neoplasia type 1, adrenal involvement, adrenal incidentaloma, adrenocortical carcinoma, primary aldosteronism, pheochromocytoma, adrenal tumors, adrenal enlargement, hormone hypersecretion, genotype-phenotype correlation
- MeSH terms
- Adolescent, Adrenal Gland Neoplasms, Adult, Aged, Belgium, Child, Child, Preschool, DNA Mutational Analysis, Female, France, Humans, Male, Middle Aged, Pheochromocytoma, Proto-Oncogene Proteins, Cohort Studies, Multicenter Studies as Topic, Case-Control Studies, Multiple Endocrine Neoplasia Type 1, Databases as Topic, Tumor Burden, Young Adult
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