Gastric endocrine cell behavior in Zollinger-Ellison patients upon long-term potent antisecretory treatment

Lehy, Thérèse; Mignon, M; Cadiot, Guillaume; Elouaer-Blanc, L; Ruszniewski, P.; Lewin, M. J. M.; Bonfils, S

doi:10.1016/0016-5085(89)91620-x

Cited by 113 publications

(59 citation statements)

References 29 publications

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“…This patient is slightly atypical for an MEN1/ZES patient because she had pituitary disease, which is reported to occur in only 16%-31% 276,285,286,371 of patients with MEN1/ZES and was present in only 11.7% of 1009 MEN1/ZES literature cases reviewed in this study (see Table 7). She is also atypical because of the presence of gastric carcinoids, which are reported to occur in 13-37% of patients with MEN1/ZES in various reports where they were specifically sought 198,200,239,240,285 and in only 3.5% of 1009 cases of ZES/MEN1 reviewed from the literature in this study (see Figure 9). This low incidence of gastric carcinoids in the literature likely reflects the fact that their increased frequency in ZES/MEN1 was emphasized only recently 42,46,49,105,334,410,411 .…”

Section: Casementioning

confidence: 81%

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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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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Section: Casementioning

confidence: 81%

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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“…The intensity of ectopic expression of α-HCG independently correlated with the presence of dysplasia [39]. Gastric carcinoids are reported to be as much as 100 times more frequent in patients with ZES/MEN1 than in those with sporadic ZES, occurring in 13% to 43% in ZES/MEN1 patients [19,39,46,49,50]. A proportion of these gastric carcinoids (type 2) is reported to be malignant (30% lymph node and 4%-11% liver metastases), although the exact percentage is unclear [45,51].…”

Section: Diagnosis Of Gastric Carcinoidsmentioning

confidence: 99%

Zollinger-Ellison syndrome revisited: Diagnosis, biologic markers, associated inherited disorders, and acid hypersecretion

Gibril

Jensen

2004

Curr Gastroenterol Rep

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Despite general awareness of Zollinger-Ellison syndrome (ZES) by most physicians and more than 3000 articles written about it since 1955, the diagnosis of ZES is still delayed for a mean of 5 years. Recent studies show it is being delayed even more with the widespread use of proton pump inhibitors. A number of tumor markers, in addition to assessing serum gastrin, such as chromogranin A, neuron-specific enolase, and subunits of chorionic gonadotropin, have been proposed for use in either the diagnosis of pancreatic endocrine tumors, such as gastrinomas, or for assessment of tumor extent and growth. In this article important recent insights into the diagnosis of ZES as well as the clinical usefulness of assessing tumor markers for diagnosis and determination of disease extent and growth are discussed. Approximately 25% of ZES cases are due to multiple endocrine neoplasia type 1 (MEN1). A number of important studies in this group of patients are also reviewed. Finally, almost every patient with ZES has marked gastric acid hypersecretion, and its current treatment as well as the long-term possible side effects are reviewed briefly.

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“…Thyroid functional test and thyroperoxidase antibodies should be assessed at diagnosis to better define chronic atrophic gastritis and possible association with autoimmune thyroiditis [28] . In patients with ZES, laboratory tests are limited to CgA and serum gastrin levels measurement [31,32,83,84] . EUS is necessary before endoscopic resection of big polyps, and in patients affected by type 2 g-NENs diagnostic work-up for MEN-1 syndrome has to be followed (CT/MRI useful).…”

Section: Gastric Neuroendocrine Tumorsmentioning

confidence: 99%