Adrenal lesion in multiple endocrine neoplasia type 1
Abstract
Background Multiple endocrine neoplasia (MEN) type 1 is accompanied by adrenal involvement, but characteristics and clinical handling of this lesion have been insufficiently explored.Methods Patients with MEN 1 (n = 43) were monitored (mean, 6.3 years) with annual biochemical and radiologic adrenal evaluation. Adrenal specimens were examined by in situ RNA-RNA hybridization for expression of the MEN1 candidate gene phospholipase C beta 3 (PLC beta 3) and immunostaining for insulin-like growth factor-1 receptor.Results Altogether 17 patients (40%) displayed adrenal enlargement, which was limited to the adrenal cortex and showed signs of progression, marked atypia, and cancer development in three of them. Only the carcinoma exhibited adrenocortical hormone excess. PLC beta 3 was expressed in the hyperplastic and adenomatous proliferation but not the carcinoma. Pancreatic endocrine tumors with insulin-proinsulin excess were overrepresented in the patients with adrenocortical involvement, but significant insulin-like growth factor-1 receptor immunoreactivity was restricted to the carcinoma.Conclusions The prevalent adrenocortical lesion associated with MEN 1 requires regular attention because of malignant potential. It was unrelated to loss of constitution heterozygosity for the MEN1 locus (11q13) and PLC beta 3 expression, except for the cortical carcinoma exhibiting allelic losses involving also the Wiedemann-Beckwith gene at 11p15. Mechanisms for mitogenic relationships between the pancreatic and adrenal lesions of MEN 1 demand further clarification.
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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 [46]
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 [46]
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- Authors
- Skogseid, B., Rastad, J., Gobl, A., Larsson, C., Backlin, K., Juhlin, C., Åkerström, G., & Öberg, K.
- Affiliations
- Karolinska University Hospital (SE)
- Corresponding author
- Skogseid, Britt
- Venue
- Surgery
- Publisher
- Elsevier BV
- Published
- 1995-12
- Volume
- 118
- Issue
- 6
- Pages
- 1077-1082
- ISSN
- 0039-6060 (ISSN-L)
- OpenAlex
- W1972860148
- Cited by
- 111
- References
- 20
- Citations by year
- 2024: 3 · 2023: 2 · 2022: 1 · 2021: 2 · 2020: 2 · 2019: 6 · 2018: 2 · 2017: 7 · 2015: 4 · 2013: 4 · 2012: 2
- FWCI
- 2.07
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- 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 mass, adrenocortical carcinoma, adrenal hyperplasia, IGF-1 receptor, pancreatic neuroendocrine tumors, Beckwith-Wiedemann syndrome, 11p15, tumor progression
- MeSH terms
- Adrenal Gland Diseases, Adrenal Gland Neoplasms, Adrenal Glands, Adult, Aged, Aged, 80 and over, Female, Humans, Immunoenzyme Techniques, Isoenzymes, Male, Middle Aged, Pancreatic Diseases, Type C Phospholipases, RNA, Messenger, In Situ Hybridization, Receptor, IGF Type 1, Multiple Endocrine Neoplasia Type 1, Phospholipase C beta
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