Diagnosis of Zollinger–Ellison Syndrome in the Era of Ppis, Faulty Gastrin Assays, Sensitive Imaging and Limited Access to Acid Secretory Testing

Metz, David C.; Cadiot, Guillaume; Poitras, Pierre; Ito, Tetsuhide; Jensen, Robert T.

doi:10.2217/ije-2017-0018

Cited by 62 publications

(53 citation statements)

References 165 publications

(329 reference statements)

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“…14,15 Gastrinoma diagnosis frequently differs from these criteria, because of the lack of gastric pH measurement, the unavailability of secretin testing and the use of immunohistochemistry and imaging as alternatives. 26 In daily clinical practice of the 14,15 Although 52% had a pNET at the moment of gastrinoma diagnosis, only 19% of all patients in this cohort had pNETs >2.0 cm on cross-sectional imaging. Especially, in the coexistence of hypergastrinemia and pNETs ≥2.0 cm, the optimal surgical strategy is hard to establish.…”

Section: Prognostic Factors For Osmentioning

confidence: 86%

“…Current guidelines recommend the combination of hypergastrinemia and basal gastric acid hypersecretion (pH < 2) . Gastrinoma diagnosis frequently differs from these criteria, because of the lack of gastric pH measurement, the unavailability of secretin testing and the use of immunohistochemistry and imaging as alternatives . In daily clinical practice of the DMSG, gastrinoma diagnosis was complicated by the unavailability of stimulation tests, no routine measurements of gastric pH and widespread use of PPI.…”

Section: Discussionmentioning

confidence: 99%

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Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

Beek

Nell

Pieterman

et al. 2019

Journal of Surgical Oncology

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Background and objectivesGastrinomas are the most prevalent functioning neuroendocrine tumors (NET) in multiple endocrine neoplasia type 1 (MEN1). Guidelines suggest medical therapy in most patients, but surgery may be considered in a subgroup. Currently, factors to guide management are necessary. This population‐based cohort study assessed prognostic factors of survival in patients with MEN1‐related gastrinomas.MethodsPatients with MEN1 having gastrinomas were identified in the Dutch MEN1 database from 1990 to 2014 based on fasting serum gastrin (FSG) levels and/or pathology. Predictors of overall survival were assessed using Cox regression.ResultsSixty‐three patients with gastrinoma (16% of the MEN1 population) were identified. Five‐ and 10‐year overall survival rates were 83% and 65%, respectively. Prognostic factors associated with overall survival were initial FSG levels ≥20x upper limit of normal (ULN) (hazard ratio [HR], 6.2 [95% confidence interval, 1.7‐23.0]), pancreatic NET ≥2 cm (HR 4.5; [1.5‐13.1]), synchronous liver metastases (HR 8.9; [2.1‐36.7]), gastroduodenoscopy suspicious for gastric NETs (HR 12.7; [1.4‐115.6]), and multiple concurrent NETs (HR 5.9; [1.2‐27.7]).ConclusionLife expectancy of patients with MEN1 gastrinoma is reduced. FSG levels and pancreatic NETs ≥2 cm are prognostic factors. FSG levels might guide surveillance intensity, step‐up to additional diagnostics, or provide arguments in selecting patients who might benefit from surgery.

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Section: Prognostic Factors For Osmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

Beek

Nell

Pieterman

et al. 2019

Journal of Surgical Oncology

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“…The most interesting aspect of this case is related to the appearance of a de novo gastrinoma 25 years after the first episode. Although frequent, as more than 50% of all surgically treated patients relapse, it occurs mostly within the first 2 years [7]. The risk factors are not well known, but size, histological grade, and neural invasion seem to be implicated.…”

Section: Discussionmentioning

confidence: 99%

De novo Gastrinoma: A Case Report

Barbosa¹,

Gomes²,

Fonseca³

et al. 2019

GE Port J Gastroenterol

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Gastrinomas are neuroendocrine tumors characterized by gastrin overexpression -80% are sporadic and 20% are associated with multiple endocrine neoplasia type 1. A 75-yearold male patient, surgically treated at the age of 50 years for gastrinoma, followed on an outpatient basis because of chronic non-bloody diarrhea, was admitted to our hospital because of abdominal pain, watery diarrhea, and nonbiliary vomits. He was hypotensive and showed no response to fluids. Blood cultures were positive for Salmonella, and a diagnosis of septic shock due to Salmonella infection was made. The patient's condition improved, but the history of chronic diarrhea was still not explained. To investigate chronic diarrhea, gastrinoma recurrence was considered. Serum gastrin measurement was five times higher than the upper limit of the normal range (536 pg/mL). A positive somatostatin receptor scintigraphy was diagnostic for neuroendocrine tumor. Metastases were excluded. The patient was proposed to curative surgery, and a diagnosis of a well-differentiated neuroendocrine tumor was made. para tumor neuroendócrino. Foram excluídas metástases. O doente foi proposto para cirurgia curativa e foi feito o diagnóstico de tumor neuroendócrino bem diferenciado.

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“…The diagnosis of insulinoma was based on the presence of hypoglycemic symptoms associated with low plasma glucose concentrations and abnormally high serum insulin or C-peptide [ 14 ]. Zollinger–Ellison syndrome criteria were the presence of continuous specific clinical symptoms associated with ZES features found on endoscopy, an inability to discontinue high-dose proton pump inhibitors, and at least 2 out of the 4 National Institute of Health (NIH) criteria and a histological confirmation of gastrinoma [ 15 , 16 ]. A diagnosis of VIPoma was confirmed in patients with the association of watery diarrhea and a high serum vasoactive intestinal peptide level.…”

Section: Methodsmentioning

confidence: 99%

Pancreatoduodenectomy for Neuroendocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1: An AFCE (Association Francophone de Chirurgie Endocrinienne) and GTE (Groupe d’étude des Tumeurs Endocrines) Study

et al. 2021

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Aim To assess postoperative complications and control of hormone secretions following pancreatoduodenectomy (PD) performed on multiple endocrine neoplasia type 1 (MEN1) patients with duodenopancreatic neuroendocrine tumors (DP-NETs). Background The use of PD to treat MEN1 remains controversial, and evaluating the right place of PD in MEN1 disease makes sense. Methods Thirty-one MEN1 patients from the Groupe d’étude des Tumeurs Endocrines MEN1 cohort who underwent PD for DP-NETs between 1971 and 2013 were included. Early and late postoperative complications, secretory control and overall survival were analyzed. Results Indication for surgery was: Zollinger–Ellison syndrome (n = 18; 58%), nonfunctioning tumor (n = 9; 29%), insulinoma (n = 2; 7%), VIPoma (n = 1; 3%) and glucagonoma (n = 1; 3%). Mean follow-up was 141 months (range 0–433). Pancreatic fistulas occurred in 5 patients (16.1%), distant metastases in 6 (mean onset of 43 months; range 13–110 months), postoperative diabetes mellitus in 7 (22%), and pancreatic exocrine insufficiency in 6 (19%). Five-year overall survival was 93.3% [CI 75.8–98.3] and ten-year overall survival was 89.1% [CI 69.6–96.4]. After a mean follow-up of 151 months (range 0–433), the biochemical cure rate for MEN-1 related gastrinomas was 61%. Conclusion In MEN1 patients, pancreatoduodenectomy can be used to control hormone secretions (gastrin, glucagon, VIP) and to remove large NETs. PD was found to control gastrin secretions in about 60% of cases.

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Diagnosis of Zollinger–Ellison Syndrome in the Era of Ppis, Faulty Gastrin Assays, Sensitive Imaging and Limited Access to Acid Secretory Testing

Cited by 62 publications

References 165 publications

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

De novo Gastrinoma: A Case Report

Pancreatoduodenectomy for Neuroendocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1: An AFCE (Association Francophone de Chirurgie Endocrinienne) and GTE (Groupe d’étude des Tumeurs Endocrines) Study

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