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Cited by 151Neuroendocrine Tumor Research AdvancesAdrenal and Paraganglionic TumorsPituitary Gland Disorders and Treatments

Adrenal involvement in multiple endocrine neoplasia type 1

Langer, Peter, Cupisti, Kenko, Bartsch, Detlef K., Nies, Christoph, Goretzki, Peter E., Rothmund, Matthias, Röher, Hans D.·2002·World Journal of Surgery
DOI 10.1007/s00268-002-6492-4
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Abstract

Adrenal lesions belong to the spectrum of multiple endocrine neoplasia type 1 (MEN-1) syndrome. However, the prevalence of adrenal involvement, the characteristics, and the clinical management of adrenal lesions have not yet been clearly defined. A total of 66 patients with confirmed MEN1 germline mutations and 1 additional patient with typical manifestations in three organ systems were monitored in a regular screening program that included evaluation of the adrenals (median follow-up 96 months; range 12 to 300 months). Age at the diagnosis of MEN-1 and of adrenal tumors and the clinical characteristics, genotype, treatment, and follow-up of adrenal disease were analyzed. Adrenal lesions were identified in 18 of 67 (26.8%) MEN-1 patients and were diagnosed 5 years later than MEN-1. The median tumor diameter at diagnosis was 3.0 cm (range 1.2-15.0 cm), with most tumors being 3 cm or smaller. Eight patients had bilateral tumors. Ten patients had nonfunctional benign tumors, three had benign adrenal Cushing syndrome, and one patient had a pheochromocytoma. Four patients developed adrenocortical carcinomas (ACCs), three of which were functional. Nine adrenalectomies and one subtotal adrenalectomy were performed in six patients. Three patients with ACC died owing to the tumor. Patients with mutations in exons 2 and 10 developed adrenal tumors significantly more often than patients with other mutations (p <0.01). Adrenal tumors are a common feature of MEN-1 but occur later in the course of the disease. The lesions are often small and nonfunctional and can therefore be managed by close surveillance; others have significant malignant potential and should be considered for surgery when they are 3 cm or larger.

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  • 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 [50]

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Authors
Langer, P., Cupisti, K., Bartsch, D. K., Nies, C., Goretzki, P. E., Rothmund, M., & Röher, H. D.
Affiliations
  • Philipps University of Marburg (DE)
  • Heinrich Heine University Düsseldorf (DE)
  • Department of Surgery Philipps‐University Hospital Baldingerstrasse, D‐35043 Marburg Germany<!--<country1>GERMANY</country1>-->
ORCID
Venue
World Journal of Surgery
Publisher
Wiley
Published
2002-08
Volume
26
Issue
8
Pages
891-896
ISSN
  • 0364-2313 (ISSN-L)
  • 1432-2323
DOI
10.1007/s00268-002-6492-4
OpenAlex
W2074878869
Cited by
151
References
22
Citations by year
2026: 1 · 2025: 3 · 2024: 8 · 2023: 8 · 2022: 9 · 2021: 5 · 2020: 6 · 2019: 7 · 2018: 7 · 2017: 6 · 2016: 5 · 2015: 9 · 2014: 9 · 2013: 7 · 2012: 7
FWCI
3.02
Type
journal article
Language
en
Open access
closed
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • World Journal of Surgery (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
Keywords
multiple endocrine neoplasia type 1, adrenal tumors, adrenocortical carcinoma, pheochromocytoma, Cushing's syndrome, adrenalectomy, germline mutations, tumor surveillance
MeSH terms
Adolescent, Adrenal Gland Neoplasms, Adult, Aged, Exons, Female, Genotype, Humans, Introns, Male, Middle Aged, Phenotype, Prevalence, Germ-Line Mutation, Multiple Endocrine Neoplasia Type 1

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