Primary thoracic gastrinoma causing Zollinger-Ellison syndrome

Rafieian, Shahab; Vahedi, Matin; Jahanbin, Behnaz; Ghasemloee, Amir

doi:10.1007/s12055-021-01211-3

Cited by 3 publications

(4 citation statements)

References 7 publications

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“…During the test, secretin normally has an inhibitory effect on gastrin release; however, in gastrinoma patients, it will increase gastrin release. A rise in gastrin levels of more than 120 pg/mL after secretin administration of 2 U/kg is 100% specific for Zollinger-Ellison syndrome [93].…”

Section: Gastrinomamentioning

confidence: 99%

“…Zollinger-Ellison-syndrome-related ulcers can occur in unusual locations, such as in the third part of the duodenum and small intestine, and can lead to strictures, bleeding, penetration and perforation. While chronic gastroesophageal reflux disease with heartburn is a manifestation of Zollinger-Ellison syndrome, such patients more frequently experience esophageal strictures caused by acid reflux [93].…”

Section: Gastrinomamentioning

confidence: 99%

“…Gastrinoma: Heartburn, abdominal pain, diarrhea and weight loss. Strictures, bleeding and perforation [93]. Somatostatinomas: Weight loss, abdominal pain, diarrhea or statorrhea, diabetes mellitus and cholelithiasis [100].…”

Section: Pancreatic Nensmentioning

confidence: 99%

“…Glucagon level of more than 500 to 1000 pg/mL (normal 50 to 150 pg/mL) [62]. Fasting serum gastrin level of 1000 pg/mL [93]. A fasting plasma hormone concentration of more than 3-fold the normal [62].…”

Section: Pancreatic Nensmentioning

confidence: 99%

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A Comprehensive Review on Neuroendocrine Neoplasms: Presentation, Pathophysiology and Management

Sultana

Kar

Verma

et al. 2023

JCM

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This review focuses on neuroendocrine neoplasms (NENs), which represent a heterogeneous group of relatively uncommon tumors that arise from cells of the endocrine and nervous systems. Although diverse, these tumors share common features, such as an alike histological appearance, the presence of secretory granules and, often, the generation and release of hormones or biogenic amines. NENs have been categorized based on their clinical behavior, histology, anatomic location and proliferation rate—factors relevant to their treatment and prognosis. Previously, chiefly diagnosed by their clinical features, their presence incidental to widespread imaging techniques appears to be on the rise in the US and several other countries. In this light, this article summarizes their pathophysiology, staging and recent changes to clinical practice pertinent to their diagnosis and treatment. Neuroendocrine neoplasms (NENs) are a group of heterogeneous tumors with neuroendocrine differentiation that can arise from any organ. They account for 2% of all malignancies in the United States. A significant proportion of NEN patients experience endocrine imbalances consequent to increased amine or peptide hormone secretion, impacting their quality of life and prognosis. Over the last decade, pathologic categorization, diagnostic techniques and therapeutic choices for NENs—both well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs)—have appreciably evolved. Diagnosis of NEN mostly follows a suspicion from clinical features or incidental imaging findings. Hormonal or non-hormonal biomarkers (like serum serotonin, urine 5-HIAA, gastrin and VIP) and histology of a suspected NEN is, therefore, critical for both confirmation of the diagnosis and classification as an NET or NEC. Therapy for NENs has progressed recently based on a better molecular understanding, including the involvement of mTOR, VEGF and peptide receptor radionuclide therapy (PRRT), which add to the growing evidence supporting the possibility of treatment beyond complete resection. As the incidence of NENs is on the rise in the United States and several other countries, physicians are more likely to see these cases, and their better understanding may support earlier diagnosis and tailoring treatment to the patient. We have compiled clinically significant evidence for NENs, including relevant changes to clinical practice that have greatly updated our diagnostic and therapeutic approach for NEN patients.

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Section: Gastrinomamentioning

confidence: 99%

Section: Gastrinomamentioning

confidence: 99%

Section: Pancreatic Nensmentioning

confidence: 99%

Section: Pancreatic Nensmentioning

confidence: 99%

See 2 more Smart Citations

A Comprehensive Review on Neuroendocrine Neoplasms: Presentation, Pathophysiology and Management

Sultana

Kar

Verma

et al. 2023

JCM

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Successful Lifetime/Long-Term Medical Treatment of Acid Hypersecretion in Zollinger-Ellison Syndrome (ZES): Myth or Fact? Insights from an Analysis of Results of NIH Long-Term Prospective Studies of ZES

Ito

Ramos-Álvarez

Jensen

2023

Cancers

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Analysis of the efficacy/pharmacology of long-term/lifetime medical treatment of acid hypersecretion in a large cohort of ZES patients in a prospective study. This study includes the results from all 303 patients with established ZES who were prospectively followed and received acid antisecretory treatment with either H2Rs or PPIs, with antisecretory doses individually titrated by the results of regular gastric acid testing. The study includes patients treated for short-term periods (<5 yrs), patients treated long-term (>5 yrs), and patients with lifetime treatment (30%) followed for up to 48 years (mean 14 yrs). Long-term/lifelong acid antisecretory treatment with H2Rs/PPIs can be successfully carried out in all patients with both uncomplicated and complicated ZES (i.e., with MEN1/ZES, previous Billroth 2, severe GERD). This is only possible if drug doses are individually set by assessing acid secretory control to establish proven criteria, with regular reassessments and readjustments. Frequent dose changes both upward and downward are needed, as well as regulation of the dosing frequency, and there is a primary reliance on the use of PPIs. Prognostic factors predicting patients with PPI dose changes are identified, which need to be studied prospectively to develop a useful predictive algorithm that could be clinically useful for tailored long-term/lifetime therapy in these patients.

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Long-Term Proton Pump Inhibitor–Acid Suppressive Treatment Can Cause Vitamin B12 Deficiency in Zollinger–Ellison Syndrome (ZES) Patients

Ito,

Ramos-Alvarez,

Jensen

2024

IJMS

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Whether the long-term treatment of patients with proton pump inhibitors (PPIs) with different diseases [GERD, Zollinger–Ellison syndrome (ZES), etc.] can result in vitamin B12 (VB12) deficiency is controversial. In this study, in 175 patients undergoing long-term ZES treatment with anti-acid therapies, drug-induced control acid secretory rates were correlated with the presence/absence of VB12 deficiency, determined by assessing serum VB12 levels, measurements of VB12 body stores (blood methylmalonic acid (MMA) and total homocysteine[tHYC]), and other features of ZES. After a mean of 10.2 yrs. of any acid treatment (5.6 yrs. with PPIs), 21% had VB12 deficiency with significantly lower serum and body VB12 levels (p < 0.0001). The presence of VB12 deficiency did not correlate with any feature of ZES but was associated with a 12-fold lower acid control rate, a 2-fold higher acid control pH (6.4 vs. 3.7), and acid control secretory rates below those required for the activation of pepsin (pH > 3.5). Over a 5-yr period, the patients with VB12 deficiency had a higher rate of achlorhydria (73% vs. 24%) and a lower rate of normal acid secretion (0% vs. 49%). In conclusion, in ZES patients, chronic long-term PPI treatment results in marked acid hyposecretion, resulting in decreased serum VB12 levels and decreased VB12-body stores, which can result in VB12 deficiency.

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Primary thoracic gastrinoma causing Zollinger-Ellison syndrome

Cited by 3 publications

References 7 publications

A Comprehensive Review on Neuroendocrine Neoplasms: Presentation, Pathophysiology and Management

A Comprehensive Review on Neuroendocrine Neoplasms: Presentation, Pathophysiology and Management

Successful Lifetime/Long-Term Medical Treatment of Acid Hypersecretion in Zollinger-Ellison Syndrome (ZES): Myth or Fact? Insights from an Analysis of Results of NIH Long-Term Prospective Studies of ZES

Long-Term Proton Pump Inhibitor–Acid Suppressive Treatment Can Cause Vitamin B12 Deficiency in Zollinger–Ellison Syndrome (ZES) Patients

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