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Cited by 313Neuroendocrine Tumor Research AdvancesPituitary Gland Disorders and TreatmentsGenetic factors in colorectal cancer

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Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome: A Prospective Study of 107 Cases and Comparison With 1009 Cases From the Literature

Gibril, Fathia, Schumann, Michael, Pace, Andrea, Jensen, Robert T.·2004·Medicine
DOI 10.1097/01.md.0000112297.72510.32
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Abstract

In patients with multiple endocrine neoplasia type 1 (MEN1), the most common functional pancreatic endocrine tumor (PET) syndrome is Zollinger-Ellison syndrome (ZES). ZES has been well studied in its sporadic form (that is, without MEN1); however, there are limited data on patients with MEN1 and ZES (MEN1/ZES), and the long-term natural history is largely unknown. To address this issue we report the results of a prospective long-term National Institutes of Health (NIH) study of 107 MEN1/ZES patients and compare our results with those of 1009 MEN1/ZES patients in 278 case reports and small series in the literature. Patients were clinically, radiologically, and biochemically evaluated yearly for all MEN1 manifestations (mean follow-up, 10 yr; range, 0.1-31 yr). Compared with patients from the literature, the NIH MEN1/ZES patients more frequently had pituitary (60%) and adrenal (45%) disease and carcinoid tumors (30%), but had equal frequency of hyperparathyroidism (94%), thyroid disease (6%), or lipomas (5%). Twenty-five percent of both the NIH and the literature patients lacked a family history of MEN1; ZES was the initial clinical manifestation of MEN1 in 40%. ZES onset preceded the diagnosis of hyperparathyroidism in 45%. However, ZES was rarely (8%) the only initial manifestation of MEN1 if careful testing was done. ZES occurred before age 40 years in 50%-60% of the current patients, in contrast to older studies. The diagnosis of ZES is delayed 3-5 years from its onset and is delayed as long as in sporadic ZES cases. Pituitary disease and carcinoid tumors (gastric > bronchial, thymic) are more frequent than generally reported, whereas a second functional PET is uncommon. In patients with MEN1/ZES without a family history of MEN1, the MEN1 manifestations are not as severe. This study shows that MEN1/ZES patients differ in many aspects from those commonly reported in older studies involving few MEN1/ZES patients. In this study we have identified a number of important clinical and laboratory features of MEN1/ZES that were not previously appreciated, which should contribute to earlier diagnosis and improve both short- and long-term management.

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Citations

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Research works printed but not linked: 25

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Metadata

Authors
Gibril, F., Schumann, M., Pace, A., & Jensen, R. T.
Affiliations
National Institutes of Health (US)
ORCID
Venue
Medicine
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2004-01
Volume
83
Issue
1
Pages
43-83
ISSN
0025-7974 (ISSN-L)
DOI
10.1097/01.md.0000112297.72510.32
OpenAlex
W2064893457
Cited by
313
References
531
Citations by year
2026: 1 · 2025: 10 · 2024: 7 · 2023: 11 · 2022: 14 · 2021: 15 · 2020: 11 · 2019: 11 · 2018: 15 · 2017: 21 · 2016: 15 · 2015: 20 · 2014: 16 · 2013: 11 · 2012: 23
FWCI
11.26
Type
journal article
Language
en
Open access
closed
Locations
  • Medicine (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Oncology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Genetic factors in colorectal cancer
Keywords
multiple endocrine neoplasia type 1, Zollinger-Ellison syndrome, pancreatic neuroendocrine tumors, hyperparathyroidism, pituitary disorders, adrenal disorders, carcinoid tumor, gastric carcinoid, thymic carcinoid, bronchial carcinoid, delayed diagnosis, natural history (disease), early-onset disease
MeSH terms
Adult, Aged, Diagnosis, Differential, Female, Follow-Up Studies, Genetic Testing, Hormones, Humans, Male, Middle Aged, Prospective Studies, Tomography, X-Ray Computed, Zollinger-Ellison Syndrome, Gastrinoma, Survival Analysis, Treatment Outcome, Age of Onset, Age Distribution, Disease Progression, Multiple Endocrine Neoplasia Type 1

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