Zollinger–Ellison syndrome

Ito, Tetsuhide; Igarashi, Hisato; Jensen, Robert T.

doi:10.1097/mog.0b013e328365efb1

Cited by 74 publications

(71 citation statements)

References 145 publications

(388 reference statements)

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“…It is now known that 70–80% of patinets the gastrinomas are duodenal in location, in contrast to older studies which primarily reported a pancreatic location [4–6]. The duodenal and pancreatic gastrinomas behave similarly clinically[2].…”

Section: Relevant Backgroundmentioning

confidence: 99%

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

Metz

Cadiot

Poitras

et al. 2017

Int. J. Endo. Oncol.

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In recent years the diagnosis of Zollinger-Ellison syndrome (ZES) has become increasingly controversial with several new approaches and criteria proposed, differing from the classical biochemical criterion of inappropriate hypergastrinemia (i.e., hypergastrinemia in the presence of hyperchlorhydria) (Table 1). These changes have come about because of the difficulty and potential dangers of stopping proton pump inhibitors (PPIs) for gastric acid analysis; the recognition than many of the current assays used to assess gastrin concentrations are unreliable; the development of sensitive imaging modalities that detect neuroendocrine tumors (NETs) including an increasing number of the primary gastrinomas; the increased use of percutaneous or endoscopic ultrasound (EUS)-directed biopsies/cytology and the general lack of availability of acid secretory testing. In this article we will discuss the basis for these controversies, review the proposed changes in diagnostic approaches and make recommendations for supporting the diagnosis of ZES in the modern era.

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Section: Relevant Backgroundmentioning

confidence: 99%

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

Metz

Cadiot

Poitras

et al. 2017

Int. J. Endo. Oncol.

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“…These tumours occur either sporadically or in the context of multiple endocrine neoplasia type 1 (MEN 1) in approximately 25-30% of the cases (Kaltsas et al 2004, Jensen et al 2012. The syndrome should also be suspected in patients with peptic ulcer disease resistant to treatment with PPIs and concomitant diarrhoea, and in the presence of hyperplastic gastric folds -'rugal hypertrophy' (Kaltsas et al 2004, Ito et al 2013. As with other functioning NETs, the therapeutic strategy aims at controlling hormonal excess and tumour growth.…”

Section: Gastrinoma and The Zollinger-ellison Syndromementioning

confidence: 99%

“…In the acute setting where oral medications may be contraindicated, intravenous 80 mg of pantoprazole given by 15 min infusion every 8 h controlled gastric acid hypersecretion in all ZES patients studied both acutely and up to 7 days (Lew et al 2000). Patients should maintain their medication with PPIs for some weeks after surgery, since many continue to hypersecrete acid for some time (Ito et al 2013). Recently, oral gastrin receptor antagonists have also been synthesised that have a rapid mode of action and can induce prolonged gastric acid output suppression (Fossmark et al 2012).…”

Section: Gastrinoma and The Zollinger-ellison Syndromementioning

confidence: 99%

“…High initial doses of PPIs (such as 60-80 mg/day of omeprazole) are frequently prescribed, with dose adjustment as necessary based on symptom response. Additionally, the dose can also be titrated to reduce the acid hypersecretion to ≤10 mEq/h, or to lower values in patients with previous acid-reducing surgery or severe 23:9 reflux disease (Ito et al 2013). Long-term treatment with PPIs has been shown to be safe with the only potential side effects being PPI-induced achlorhydria that may lead to B12 deficiency, although there is a suggestion of an increased incidence of bone fractures (Jensen et al 2012).…”

Section: Gastrinoma and The Zollinger-ellison Syndromementioning

confidence: 99%

“…Indeed, patients with a number of secretory syndromes related to GEP-NETs obtained substantial symptomatic and biochemical responses to these agents, although their effect on tumour growth was not as prominent (Modlin et al 2010). In addition, proton pump inhibitors (PPIs) have also proven to be efficacious in controlling the secretory syndrome related to excessive gastrin secretion (Ito et al 2013). The introduction of these medications along with the use of various other drugs has resulted in a significant reduction in morbidity and mortality related to functioning GEP-NETs.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours

Dimitriadis¹,

Weickert²,

Randeva³

et al. 2016

Endocrine-Related Cancer

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Although recent epidemiological evidence indicates that the prevalence of non-functioning gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) is rising, a significant number of GEP-NETs still present with symptoms related to the secretion of biologically active substances leading to the development of distinct clinical syndromes. In the past, these syndromes were associated with substantial morbidity and mortality due to the lack of specific therapies; however, since the introduction of long-acting somatostatin analogues and medications such as proton pump inhibitors, their control has been greatly improved. As a result, nowadays, the main cause of morbidity and mortality in GEP-NETs is mostly directly related to tumour growth and the extent of metastatic disease. However, in some patients with functioning tumours and extensive disease, control of the secretory syndrome still remains problematic, necessitating the employment of several cytoreductive techniques, which may not always be sufficient. Recently, new agents directed against tumour growth, or exerting increased binding activity to receptors expressed in these tumours, or interfering with the synthetic pathway of some of the compounds secreted by these tumours, have been developed. Since there are no specific guidelines addressing the totality of the management of the secretory syndromes related to GEP-NETs, this review aims at critically analysing the medical management of previously recognised secretory syndromes; it also addresses areas of uncertainty, assesses the newer therapeutic developments and also addresses recently described but poorly characterised secretory syndromes related to GEP-NETs.

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