CoChat
Sign Up
Cited by 104Adrenal and Paraganglionic TumorsNeuroendocrine Tumor Research AdvancesPituitary Gland Disorders and Treatments

Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening

Waldmann, J., Bartsch, D. K., Kann, P. H., Fendrich, V., Rothmund, M., Langer, P.·2007·Langenbeck s Archives of Surgery
DOI 10.1007/s00423-006-0124-7
Open this paper in CoChat — free account

Abstract

Background Adrenal tumors are a common manifestation of the multiple endocrine neoplasia type 1 (MEN-1) syndrome. Prevalence in recent studies varies between 9 and 45%. A genotype-phenotype correlation has been described as well as the development of adrenocortical carcinomas. Long-term prospective data are still lacking.Materials and methods Thirty-eight MEN-1 patients with proven germline mutations have been prospectively observed in a regular screening program in our hospital. Adrenal glands have been screened by biochemical analysis and either by endoscopic ultrasound (EUS) or computed tomography (CT) or both. Median follow-up was 48 months (12-108 months). Age at diagnosis of MEN-1, type of adrenal tumor, genotype, therapy, and clinical characteristics have been analyzed.Results In 21 (55%) patients, adrenal involvement of the disease was detected. Adrenal lesions were detected in average 6.9 years after the initial diagnosis of MEN-1. Median tumor size was 12 mm (5-40 mm). Tumor size smaller than 10 mm was observed in 11 patients. Twelve patients had unilateral while nine had bilateral adrenal lesions. EUS detected all adrenal tumors, whereas CT failed in seven cases. In three patients, functioning tumors (one pheochromocytoma, one bilateral Cushing adenoma, and one adrenocortical carcinoma) and one nonfunctioning adenoma were diagnosed by histology and biochemical assessment. Two laparoscopic adrenalectomies and one laparoscopic subtotal resection were performed. Nonfunctioning adrenal lesions, not characterized by histology yet, were found in 18 patients. There was no statistical difference with regard to adrenal involvement between patients with germline mutations in exons 2 and 10 (12/21) and those with mutations in exons 3-9 (6/11).Conclusion MEN-1-associated adrenal tumors are mostly small, benign, and nonfunctioning and much more common than previously reported. EUS was the most sensitive imaging procedure. The genotype-pheotype correlation previously suggested by our group could not be confirmed.

Integrity signals

Registry

Crossref · 10.1007/s00423-006-0124-7

Cites

51 of 51 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • 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 [47]

  • 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 [47]

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Related

Authors
Waldmann, J., Bartsch, D. K., Kann, P. H., Fendrich, V., Rothmund, M., & Langer, P.
Affiliations
Philipps University of Marburg (DE)
Corresponding author
Langer, P.
ORCID
Venue
Langenbeck s Archives of Surgery
Publisher
Springer Science and Business Media LLC
Published
2007-01-19
Volume
392
Issue
4
Pages
437-43
ISSN
  • 0023-8236 (ISSN-L)
  • 1435-2443
  • 1435-2451
DOI
10.1007/s00423-006-0124-7
OpenAlex
W2169457322
Cited by
104
References
51
Citations by year
2026: 2 · 2025: 1 · 2024: 5 · 2023: 5 · 2022: 7 · 2021: 3 · 2020: 2 · 2019: 9 · 2018: 5 · 2017: 6 · 2016: 6 · 2015: 6 · 2014: 4 · 2013: 7 · 2012: 4
FWCI
6.97
Type
journal article
Language
en
Open access
closed
Licence
Locations
  • Langenbeck s Archives of Surgery (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
Keywords
multiple endocrine neoplasia type 1, adrenal tumors, adrenocortical carcinoma, pheochromocytoma, non-functioning adrenal adenoma, germline mutations, genotype-phenotype correlation, endoscopic ultrasound, computed tomography, adrenalectomy, tumor size
MeSH terms
Adolescent, Adrenal Gland Neoplasms, Adrenalectomy, Adult, Aged, DNA Mutational Analysis, Female, Genotype, Humans, Male, Middle Aged, Phenotype, Prospective Studies, Prevalence, Germ-Line Mutation, Multiple Endocrine Neoplasia Type 1, Endosonography

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review