Materiały źródłowe

• #1 Zollinger-Ellison Syndrome – Gastrinoma | Choose the Right Test

  https://arupconsult.com/content/zollinger-ellison-syndrome

  Zollinger-Ellison syndrome (ZES) is characterized by refractory peptic ulcer disease, diarrhea, and gastric acid hypersecretion as a result of a functional pancreatic or duodenal neuroendocrine tumor (NET). ZES is also referred to as gastrinoma. […] Diagnosis is delayed an average of 4-6 years after symptom onset. […] Definitive diagnosis requires biopsy and pathologist examination. […] Elevated fasting serum gastrin (FSG) 10x upper limit of normal, and gastric pH 2 are criteria for diagnosis. […] Gastrin testing patient should be off antacids and proton pump inhibitors 10 days to 2 weeks. […] Secretin stimulation test especially recommended for indeterminate FSG findings (200-1000 pg/mL). […] Positive secretin stimulation test (increase in serum gastrin by 120 pg/mL) confirms the need to search for presence of gastrinoma.

• #2 Zollinger-Ellison syndrome | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/zollinger-ellison-syndrome?lang=us

  Zollinger-Ellison syndrome (ZES) is a clinical syndrome that occurs secondary to elevated gastrin levels produced by a gastrinoma. […] Diagnosis is often delayed by 5-7 years after the onset of symptoms. […] Increased gastrin levels in fasting patients (but not specific, and some data suggest that clinical assays may be unreliable). […] Surgery plays a vital role. […] Death from complications of Zollinger-Ellison syndrome (e.g. perforation, hemorrhage) can occur. […] Diagnosis of Zollinger-Ellison syndrome: increasingly difficult.

• #3 Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome – PubMed

  https://pubmed.ncbi.nlm.nih.gov/28776139/

  Zollinger-Ellison syndrome (ZES) results from an ectopic gastrin-secreting tumor leading to peptic ulcer disease, reflux, and chronic diarrhea. […] While early recognition portends an excellent prognosis with 80% survival at 15 years, symptoms are often nonspecific making the diagnosis difficult to establish. Diagnosis involves a series of tests, including fasting gastrin, gastric pH, chromogranin A, and secretin stimulation. Performing these tests in the correct sequence and at the proper time is essential to avoid inaccurate results. […] Tumor localization is equally nuanced. Although providers have classically used 111indium-radiolabeled octreotide with somatostatin receptor scintigraphy to evaluate tumor size and metastases, recent studies have shown superior results with newer imaging modalities. In particular, 68gallium (68Ga)-labeled somatostatin radiotracers (i.e., 68Ga-DOTATOC, 68Ga-DOTANOC and 68Ga-DOTATATE) used with positron emission tomography/computed tomography can provide excellent results. Endoscopic ultrasound is another useful modality, particularly in patients with ZES in the setting of multiple endocrine neoplasia type 1. This review aims to provide clinicians with an overview of ZES with a focus on both clinical presentation and the proper utilization of the various biochemical and imaging tests available.

• #4

  https://link.springer.com/article/10.1007/s10620-017-4695-7

  ZollingerEllison syndrome (ZES) results from an ectopic gastrin-secreting tumor leading to peptic ulcer disease, reflux, and chronic diarrhea. […] While early recognition portends an excellent prognosis with 80% survival at 15 years, symptoms are often nonspecific making the diagnosis difficult to establish. Diagnosis involves a series of tests, including fasting gastrin, gastric pH, chromogranin A, and secretin stimulation. Performing these tests in the correct sequence and at the proper time is essential to avoid inaccurate results. […] Although providers have classically used 111indium-radiolabeled octreotide with somatostatin receptor scintigraphy to evaluate tumor size and metastases, recent studies have shown superior results with newer imaging modalities. In particular, 68gallium (68Ga)-labeled somatostatin radiotracers (i.e., 68Ga-DOTATOC, 68Ga-DOTANOC and 68Ga-DOTATATE) used with positron emission tomography/computed tomography can provide excellent results.

• #5 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  Diagnostic laboratory studies include measurement of gastric pH and levels of fasting gastrin and chromogranin A, as well as secretin stimulation. […] Fasting serum gastrin is the best single screening test for Zollinger-Ellison syndrome (ZES). Preferably, patients should not be taking gastric antisecretory medications at the time of the test, but this is not essential for the initial screen. […] Because fasting gastrin levels can fluctuate from day to day and can appear to be normal, serial measurements on different days should be performed. […] Normal levels of serum gastrin in untreated ZES are extremely rare (1%). […] Basal acid output (BAO) greater than 15 mEq/h or greater than 5 mEq/h in patients with a prior vagotomy and partial gastrectomy is suggestive of ZES. […] Gastric pH less than 2.0 in the presence of a large gastric volume (140 mL over 1 h in patients without prior gastric acid-reducing surgery) is highly suggestive of ZES.

• #6 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  Diagnostic laboratory studies include measurement of gastric pH and levels of fasting gastrin and chromogranin A, as well as secretin stimulation. […] Fasting serum gastrin is the best single screening test for Zollinger-Ellison syndrome (ZES). Preferably, patients should not be taking gastric antisecretory medications at the time of the test, but this is not essential for the initial screen. […] Because fasting gastrin levels can fluctuate from day to day and can appear to be normal, serial measurements on different days should be performed. […] Normal levels of serum gastrin in untreated ZES are extremely rare (1%). […] Basal acid output (BAO) greater than 15 mEq/h or greater than 5 mEq/h in patients with a prior vagotomy and partial gastrectomy is suggestive of ZES. […] Gastric pH less than 2.0 in the presence of a large gastric volume (140 mL over 1 h in patients without prior gastric acid-reducing surgery) is highly suggestive of ZES.

• #7 Gastrinoma Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/184332-workup

  Fasting hypergastrinemia is present (150 pg/mL with levels 100,000 pg/mL in some patients; a serum gastrin level 1,000 pg/mL in the appropriate clinical setting is virtually diagnostic of Zollinger-Ellison syndrome [ZES]). […] This is the most sensitive test for the diagnosis of ZES. […] Levels higher than 1000 pg/mL with acid hypersecretion are highly suggestive of ZES. […] In patients with intermediate gastrin levels (150-1000 pg/mL) and acid secretion, the secretin stimulation test can help diagnose the presence of gastrinoma. […] Imaging studies are helpful in localizing the tumor. […] Somatostatin receptor scintigraphy (SRS) is very useful to identify the primary lesions preoperatively. […] Endoscopic ultrasonography has also been found to be useful in helping to detect the primary tumor, with a reported overall sensitivity and accuracy greater than 90% for intrapancreatic gastrinomas.

• #8 Zollinger Ellison Syndrome – El Atlas Gastrointestinal – gastrointestinalatlas.com

  https://www.gastrointestinalatlas.com/english/zollinger_ellison_syndrome_.html

  Gastrinomas are an integral part of the Zollinger-Ellison syndrome (ZES). In fact, ZES is also known as gastrinoma. This syndrome consists of ulcer disease in the upper gastrointestinal tract, marked increases in the secretion of gastric acid in the stomach, and tumors of the islet cells in the pancreas. The tumors produce large amounts of gastrin that are responsible for the characteristics of Zollinger-Ellison syndrome, namely severe ulcer disease. Although usually located within the pancreas, they may occur in other organs. […] The diagnosis of Zollinger-Ellison syndrome is made on the demonstration of high serum gastrin levels. Fasting gastrin levels in normals and in patients with ordinary duodenal ulcer average approximately 60 pg/ml. Patients with gastrinoma almost always have levels greater than 150 pg/ml and not uncommonly greater than 1,000 pg/ml. The diagnosis is also suspected on the clinical history, marked acid hypersecretion, prominence of mucosal folds in the stomach, duodenum and sometimes jejunum or SR. It may be confirmed by several provocative tests involving measurement of serum gastrin levels in response to calcium infusion, secretin injection or a standard test meal.

• #9 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  Diagnostic laboratory studies include measurement of gastric pH and levels of fasting gastrin and chromogranin A, as well as secretin stimulation. […] Fasting serum gastrin is the best single screening test for Zollinger-Ellison syndrome (ZES). Preferably, patients should not be taking gastric antisecretory medications at the time of the test, but this is not essential for the initial screen. […] Because fasting gastrin levels can fluctuate from day to day and can appear to be normal, serial measurements on different days should be performed. […] Normal levels of serum gastrin in untreated ZES are extremely rare (1%). […] Basal acid output (BAO) greater than 15 mEq/h or greater than 5 mEq/h in patients with a prior vagotomy and partial gastrectomy is suggestive of ZES. […] Gastric pH less than 2.0 in the presence of a large gastric volume (140 mL over 1 h in patients without prior gastric acid-reducing surgery) is highly suggestive of ZES.

• #10 Zollinger–Ellison Syndrome – Diagnosis

  https://www.medindia.net/health/conditions/zollinger-ellison-syndrome-diagnosis.htm

  Tests used to diagnose Zollinger-Ellison syndrome are: […] Blood tests show a rise in gastrin levels. However, other conditions can also result in increased gastrin levels; thus the test used alone is not diagnostic of gastrinoma. […] Gastrinoma patients usually have a gastrin level more than 150-200 pg/mL. Levels of gastrin are obtained with patients not taking H2-receptor antagonists for 24 hours or proton pump inhibitors for 6 days. […] A basal gastric pH greater than or equal to 3 excludes gastrinomas. […] An increase of plasma gastrin levels of over 200 pg/mL within 2-30 minutes is seen in 85% of patients with gastrinoma. […] Imaging studies are required to locate the gastrinomas and evaluate their stage. An important imaging study in this context is the somatostatin receptor scintigraphy (SRS) using 111In-pentetreotide with single photon emission tomography (SPECT) scanning.

• #11 Zollinger–Ellison Syndrome – Diagnosis

  https://www.medindia.net/health/conditions/zollinger-ellison-syndrome-diagnosis.htm

  Tests used to diagnose Zollinger-Ellison syndrome are: […] Blood tests show a rise in gastrin levels. However, other conditions can also result in increased gastrin levels; thus the test used alone is not diagnostic of gastrinoma. […] Gastrinoma patients usually have a gastrin level more than 150-200 pg/mL. Levels of gastrin are obtained with patients not taking H2-receptor antagonists for 24 hours or proton pump inhibitors for 6 days. […] A basal gastric pH greater than or equal to 3 excludes gastrinomas. […] An increase of plasma gastrin levels of over 200 pg/mL within 2-30 minutes is seen in 85% of patients with gastrinoma. […] Imaging studies are required to locate the gastrinomas and evaluate their stage. An important imaging study in this context is the somatostatin receptor scintigraphy (SRS) using 111In-pentetreotide with single photon emission tomography (SPECT) scanning.

• #12 Zollinger-Ellison syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/zollinger-ellison-syndrome/diagnosis-treatment/drc-20379046

  Endoscopy also can find out whether the stomach is making acid. If the stomach is making acid and the gastrin level is high, then the diagnosis of Zollinger-Ellison can be established. […] Imaging techniques may be used to look for tumors, such as CT scans, MRI imaging and Ga-DOTATATE PET-CT scanning. […] Certain acid-reducing medicines, such as proton pump inhibitors or H-2 blockers, can affect the results of some tests used to diagnose Zollinger-Ellison syndrome. […] What tests do I need to confirm the diagnosis? How should I prepare for those tests?

• #13 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  The secretin stimulation test is the provocative test of choice because of its higher sensitivity. In this test, a 2-U/kg bolus of secretin is administered intravenously after an overnight fast, and serum levels of gastrin are determined at 0, 2, 5, 10, and 15 minutes. An increase in serum gastrin of greater than 200 pg/mL is diagnostic. […] A suggested algorithm for the evaluation of a patient with suspected gastrinoma is as follows: Step 1: Check the fasting gastrin level. Measure at least 3 fasting levels of gastrin on different days. […] Step 2: Perform gastric acid secretory studies. A BAO value of greater than 15 mEq/h or a gastric volume of greater than 140 mL and pH of less than 2.0 are highly suggestive of gastrinoma. […] Step 3: Perform a provocative test. The secretin stimulation test is the preferred test.

• #14 Gastrinoma Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/184332-workup

  Fasting hypergastrinemia is present (150 pg/mL with levels 100,000 pg/mL in some patients; a serum gastrin level 1,000 pg/mL in the appropriate clinical setting is virtually diagnostic of Zollinger-Ellison syndrome [ZES]). […] This is the most sensitive test for the diagnosis of ZES. […] Levels higher than 1000 pg/mL with acid hypersecretion are highly suggestive of ZES. […] In patients with intermediate gastrin levels (150-1000 pg/mL) and acid secretion, the secretin stimulation test can help diagnose the presence of gastrinoma. […] Imaging studies are helpful in localizing the tumor. […] Somatostatin receptor scintigraphy (SRS) is very useful to identify the primary lesions preoperatively. […] Endoscopic ultrasonography has also been found to be useful in helping to detect the primary tumor, with a reported overall sensitivity and accuracy greater than 90% for intrapancreatic gastrinomas.

• #15 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  The secretin stimulation test is the provocative test of choice because of its higher sensitivity. In this test, a 2-U/kg bolus of secretin is administered intravenously after an overnight fast, and serum levels of gastrin are determined at 0, 2, 5, 10, and 15 minutes. An increase in serum gastrin of greater than 200 pg/mL is diagnostic. […] A suggested algorithm for the evaluation of a patient with suspected gastrinoma is as follows: Step 1: Check the fasting gastrin level. Measure at least 3 fasting levels of gastrin on different days. […] Step 2: Perform gastric acid secretory studies. A BAO value of greater than 15 mEq/h or a gastric volume of greater than 140 mL and pH of less than 2.0 are highly suggestive of gastrinoma. […] Step 3: Perform a provocative test. The secretin stimulation test is the preferred test.

• #16 Zollinger–Ellison Syndrome – Diagnosis

  https://www.medindia.net/health/conditions/zollinger-ellison-syndrome-diagnosis.htm

  Tests used to diagnose Zollinger-Ellison syndrome are: […] Blood tests show a rise in gastrin levels. However, other conditions can also result in increased gastrin levels; thus the test used alone is not diagnostic of gastrinoma. […] Gastrinoma patients usually have a gastrin level more than 150-200 pg/mL. Levels of gastrin are obtained with patients not taking H2-receptor antagonists for 24 hours or proton pump inhibitors for 6 days. […] A basal gastric pH greater than or equal to 3 excludes gastrinomas. […] An increase of plasma gastrin levels of over 200 pg/mL within 2-30 minutes is seen in 85% of patients with gastrinoma. […] Imaging studies are required to locate the gastrinomas and evaluate their stage. An important imaging study in this context is the somatostatin receptor scintigraphy (SRS) using 111In-pentetreotide with single photon emission tomography (SPECT) scanning.

• #17 Zollinger Ellison Syndrome Mnemonic

  https://www.picmonic.com/pathways/medicine/courses/standard/pathology-196/neuroendocrine-disorders-39305/zollinger-ellison-syndrome-diagnosis-and-treatment_1343

  The secretin stimulation test is the most specific and sensitive test for identifying gastrinomas causative of Zollinger-Ellison syndrome (ZES). The secretin stimulation test is considered a provocative test, as the response to administered secretin is measured, and the patients response aids in diagnosis. In this test, secretin is administered as a bolus after an overnight fast, and gastrin levels are measured in response at different time intervals. An increase in serum gastrin of greater than 200 pg/mL yields a positive test result and is diagnostic of ZES.

• #18

  https://step2.medbullets.com/gastrointestinal/120150/zollinger-ellison-syndrome

  differentiates gastrinomas from other causes of hypergastrinemia (e.g., antral G-cell hyperplasia) […] patients with gastrinomas will have a dramatic rise in serum gastrin following secreting stimulation (normal gastric G cells are inhibited by secretin) […] calcium infusion study […] indicated in patients with strong clinical suspicion despite a negative secretin stimulation test.

• #19

  https://step2.medbullets.com/gastrointestinal/120150/zollinger-ellison-syndrome

  differentiates gastrinomas from other causes of hypergastrinemia (e.g., antral G-cell hyperplasia) […] patients with gastrinomas will have a dramatic rise in serum gastrin following secreting stimulation (normal gastric G cells are inhibited by secretin) […] calcium infusion study […] indicated in patients with strong clinical suspicion despite a negative secretin stimulation test.

• #20 Gastrinoma Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/184332-workup

  Selective arterial secretin injection, involving the gastroduodenal, splenic, and superior mesenteric arteries, with assessment of the hepatic vein serum gastrin concentrations is one among several diagnostic procedures proposed for helping identify gastrinomas. […] The calcium infusion test, calcium gluconate, is infused intravenously over 180 minutes with serum gastrin levels obtained at set intervals.

• #21 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  Diagnostic laboratory studies include measurement of gastric pH and levels of fasting gastrin and chromogranin A, as well as secretin stimulation. […] Fasting serum gastrin is the best single screening test for Zollinger-Ellison syndrome (ZES). Preferably, patients should not be taking gastric antisecretory medications at the time of the test, but this is not essential for the initial screen. […] Because fasting gastrin levels can fluctuate from day to day and can appear to be normal, serial measurements on different days should be performed. […] Normal levels of serum gastrin in untreated ZES are extremely rare (1%). […] Basal acid output (BAO) greater than 15 mEq/h or greater than 5 mEq/h in patients with a prior vagotomy and partial gastrectomy is suggestive of ZES. […] Gastric pH less than 2.0 in the presence of a large gastric volume (140 mL over 1 h in patients without prior gastric acid-reducing surgery) is highly suggestive of ZES.

• #22 Zollinger-Ellison Syndrome: Not Your Average Peptic Ulcer Disease | MDedge

  https://community.the-hospitalist.org/content/zollinger-ellison-syndrome-not-your-average-peptic-ulcer-disease

  A more severe variant of peptic ulcer disease, Zollinger-Ellison syndrome (ZES) is a rare, chronic, and potentially life-threatening ulcerative disorder. Because the syndrome can be easily misdiagnosed based on clinical presentation alone, primary care clinicians need to be aware of its diagnostic features and know when referral to a gastroenterologist is necessary. Clinicians should suspect ZES in patients with peptic ulcer disease that is refractory to traditional medications. […] Patients with symptoms refractory to medical management should be referred to a specialist for further testing. Once a patient is referred, a gastroenterologist will perform lab tests and imaging studies. In order to be diagnosed with ZES, the patient must exhibit an acidic environment with a pH less than 2 and an inappropriate release of gastrin with a basal acid output greater than 15 mEq/h (or 5 mEq/h in a patient with prior acid reduction surgery).

• #23 ZOLLINGER-ELLISON SYNDROME (GASTRINOMA) | Harrison’s Manual of Medicine

  https://harrisons.unboundmedicine.com/harrisons/view/Harrisons-Manual-of-Medicine/623366/all/ZOLLINGER_ELLISON_SYNDROME__GASTRINOMA_

  Consider when ulcer disease is severe, refractory to therapy, associated with ulcers in atypical locations, or associated with diarrhea. […] Basal acid output 15 mmol/h; basal/maximal acid output 60%; large mucosal folds on endoscopy or upper GI radiograph. […] Serum gastrin 1000 ng/L or rise in gastrin of 200 ng/L following IV secretin and, if necessary, rise of 400 ng/L following IV calcium. […] Z-E syndrome, antral G-cell hyperplasia or hyperfunction (? due to H. pylori), postgastrectomy retained antrum, renal failure, massive small bowel resection, chronic gastric outlet obstruction. […] Pernicious anemia, chronic gastritis, gastric cancer, vagotomy, pheochromocytoma.

• #24 Gastroenterology Education and CPD for trainees and specialists » Zollinger Ellison syndrome

  https://www.gastrotraining.com/category/gastro-duodenal/zollinger-ellison-syndrome

  Zollinger Ellison syndrome (ZES) is characterized by peptic ulcers of the upper GIT refractory to medical therapy caused by severe gastric acid hyper secretion associated with non-beta islet cell tumors of the pancreas. […] The diagnosis is usually made between the ages of 30 and 50 years. There is no clear sex prevalence. […] Diagnosis should be suspected in patients with multiple or refractory ulcers, or ulcer location distal to the duodenum, diarrhoea, or a personal or family history of MEN 1. Step 1 – Check fasting serum gastrin. If it is elevated on PPIs, you want to discontinue the PPIs for seven days and then re check. Step 2 – if high need to exclude secondary causes of hypergastrinemia (achlorhydria in pernicious anaemia or atrophic gastritis) pH of the gastric juice can be obtained during endoscopy or through a nasogastric tube. In the presence of gastric acid (i.e., a gastric pH below 5.0), a serum gastrin value greater than 1000 pg/mL (475 pmol/L) is virtually diagnostic of the disorder. Step 3 – If fasting gastrin levels are not diagnostic i.e. if the pH is 5 and the plasma gastrin concentration is between 100 and 1 000 pg/mL, a secretin test must be carried out.

• #25 ZOLLINGER-ELLISON SYNDROME (GASTRINOMA) | Harrison’s Manual of Medicine

  https://harrisons.unboundmedicine.com/harrisons/view/Harrisons-Manual-of-Medicine/623366/all/ZOLLINGER_ELLISON_SYNDROME__GASTRINOMA_

  Consider when ulcer disease is severe, refractory to therapy, associated with ulcers in atypical locations, or associated with diarrhea. […] Basal acid output 15 mmol/h; basal/maximal acid output 60%; large mucosal folds on endoscopy or upper GI radiograph. […] Serum gastrin 1000 ng/L or rise in gastrin of 200 ng/L following IV secretin and, if necessary, rise of 400 ng/L following IV calcium. […] Z-E syndrome, antral G-cell hyperplasia or hyperfunction (? due to H. pylori), postgastrectomy retained antrum, renal failure, massive small bowel resection, chronic gastric outlet obstruction. […] Pernicious anemia, chronic gastritis, gastric cancer, vagotomy, pheochromocytoma.

• #26 Gastrinoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/gastrointestinal-and-pancreatic-neuroendocrine-tumors-nets/gastrinoma

  A gastrinoma is a gastrin-producing tumor usually located in the pancreas or the duodenal wall. […] Diagnosis is by measuring serum gastrin levels. […] Gastrinoma is suspected by history, particularly when symptoms are refractory to standard acid suppressant therapy. […] Serum gastrin level is the most reliable test. All patients have levels 150 pg/mL (72 pmol/L); markedly elevated levels of 1000 pg/mL (480 pmol/L) in a patient with compatible clinical features and gastric acid hypersecretion of 15 mEq/hour establish the diagnosis. […] A secretin provocative test may be useful in patients with gastrin levels 1000 pg/mL (480 pmol/L). […] Once the diagnosis of gastrinoma has been established, the tumor or tumors must be localized. […] Tumors can usually be localized with CT, somatostatin receptor scintigraphy, or positron emission tomography (PET). […] Serum gastrin levels are usually diagnostic, but patients with borderline elevated levels may need a secretin provocative test.

• #27 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  Somatostatin receptor scintigraphy (SRS) is the most sensitive imaging modality for detection of primary or metastatic lesions in Zollinger-Ellison syndrome (ZES); thus, is the imaging modality of choice in ZES. […] Computed tomography (CT) scanning can be performed to localize the tumor and is useful for evaluation for metastatic disease. However, its sensitivity for primary tumor localization is only 50%, and frequently, tumors smaller than 1 cm are missed. […] Endoscopic ultrasonography is one of the relatively newer methods for localizing gastrinomas. Its sensitivity is higher for pancreatic gastrinoma (40-75%) than for duodenal gastrinoma (50%).

• #28 Zollinger-Ellison Syndrome/Gastrinoma – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/other-pancreatic-conditions/zollinger-ellison-syndrome-gastrinoma/

  Zollinger-Ellison syndrome is a rare disorder characterized by the development of a tumor called a Gastrinoma found in the pancreas and/or duodenum. […] A diagnosis of a Gastrinoma should be considered in a patient with peptic ulcers that recur frequently and are resistant to treatment. […] A medical professional may suggest a biochemical study called the Secretin Stimulated blood test to check for an elevated gastrin level after an infusion of secretin. […] A medical professional may suspect Zollinger-Ellison syndrome if blood drawn after the infusion shows an elevated gastrin level. […] Once a diagnosis is made, localizing the tumor is important. […] Some of the tests that are performed to localize the tumor include a CT scan, octreotide scan and MRI and an endoscopic ultrasound. […] An experienced surgeon will detect these tumors at surgery even when they are not visible on preoperative imaging tests.

• #29 Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome – PubMed

  https://pubmed.ncbi.nlm.nih.gov/28776139/

  Zollinger-Ellison syndrome (ZES) results from an ectopic gastrin-secreting tumor leading to peptic ulcer disease, reflux, and chronic diarrhea. […] While early recognition portends an excellent prognosis with 80% survival at 15 years, symptoms are often nonspecific making the diagnosis difficult to establish. Diagnosis involves a series of tests, including fasting gastrin, gastric pH, chromogranin A, and secretin stimulation. Performing these tests in the correct sequence and at the proper time is essential to avoid inaccurate results. […] Tumor localization is equally nuanced. Although providers have classically used 111indium-radiolabeled octreotide with somatostatin receptor scintigraphy to evaluate tumor size and metastases, recent studies have shown superior results with newer imaging modalities. In particular, 68gallium (68Ga)-labeled somatostatin radiotracers (i.e., 68Ga-DOTATOC, 68Ga-DOTANOC and 68Ga-DOTATATE) used with positron emission tomography/computed tomography can provide excellent results. Endoscopic ultrasound is another useful modality, particularly in patients with ZES in the setting of multiple endocrine neoplasia type 1. This review aims to provide clinicians with an overview of ZES with a focus on both clinical presentation and the proper utilization of the various biochemical and imaging tests available.

• #30

  https://link.springer.com/article/10.1007/s10620-017-4695-7

  ZollingerEllison syndrome (ZES) results from an ectopic gastrin-secreting tumor leading to peptic ulcer disease, reflux, and chronic diarrhea. […] While early recognition portends an excellent prognosis with 80% survival at 15 years, symptoms are often nonspecific making the diagnosis difficult to establish. Diagnosis involves a series of tests, including fasting gastrin, gastric pH, chromogranin A, and secretin stimulation. Performing these tests in the correct sequence and at the proper time is essential to avoid inaccurate results. […] Although providers have classically used 111indium-radiolabeled octreotide with somatostatin receptor scintigraphy to evaluate tumor size and metastases, recent studies have shown superior results with newer imaging modalities. In particular, 68gallium (68Ga)-labeled somatostatin radiotracers (i.e., 68Ga-DOTATOC, 68Ga-DOTANOC and 68Ga-DOTATATE) used with positron emission tomography/computed tomography can provide excellent results.

• #31 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  Somatostatin receptor scintigraphy (SRS) is the most sensitive imaging modality for detection of primary or metastatic lesions in Zollinger-Ellison syndrome (ZES); thus, is the imaging modality of choice in ZES. […] Computed tomography (CT) scanning can be performed to localize the tumor and is useful for evaluation for metastatic disease. However, its sensitivity for primary tumor localization is only 50%, and frequently, tumors smaller than 1 cm are missed. […] Endoscopic ultrasonography is one of the relatively newer methods for localizing gastrinomas. Its sensitivity is higher for pancreatic gastrinoma (40-75%) than for duodenal gastrinoma (50%).

• #32 Zollinger–Ellison Syndrome

  https://neuroendocrine.org.au/zollinger-ellison-syndrome/

  ZollingerEllison Syndrome (ZES) is a rare disorder characterised by the formation of gastrin-secreting tumours, known as gastrinomas, which lead to excessive production of gastric acid. […] Understanding and managing ZES is crucial for improving patient outcomes and quality of life. […] The diagnosis of ZES involves several steps to confirm hypergastrinemia and identify gastrinomas. Key diagnostic criteria include: Elevated fasting serum gastrin levels, Gastric pH testing to confirm acid hypersecretion. […] Imaging studies are crucial for locating gastrinomas and assessing their extent. Common imaging modalities include: Computed Tomography (CT) Scan: Useful for detecting primary tumours and metastases, Magnetic Resonance Imaging (MRI): Provides detailed images of soft tissues, Somatostatin Receptor Scintigraphy (SRS): A nuclear medicine test that detects tumours with somatostatin receptors.

• #33 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  Somatostatin receptor scintigraphy (SRS) is the most sensitive imaging modality for detection of primary or metastatic lesions in Zollinger-Ellison syndrome (ZES); thus, is the imaging modality of choice in ZES. […] Computed tomography (CT) scanning can be performed to localize the tumor and is useful for evaluation for metastatic disease. However, its sensitivity for primary tumor localization is only 50%, and frequently, tumors smaller than 1 cm are missed. […] Endoscopic ultrasonography is one of the relatively newer methods for localizing gastrinomas. Its sensitivity is higher for pancreatic gastrinoma (40-75%) than for duodenal gastrinoma (50%).

• #34

  https://link.springer.com/article/10.1007/s10620-017-4695-7

  Endoscopic ultrasound is another useful modality, particularly in patients with ZES in the setting of multiple endocrine neoplasia type 1. […] This review aims to provide clinicians with an overview of ZES with a focus on both clinical presentation and the proper utilization of the various biochemical and imaging tests available.

• #35 Zollinger–Ellison Syndrome

  https://neuroendocrine.org.au/zollinger-ellison-syndrome/

  In addition to imaging, other tests can help confirm the presence of gastrinomas, such as: Secretin Stimulation Test: Measures the increase in gastrin levels after administration of secretin, Endoscopic Ultrasound (EUS): Allows for detailed imaging and biopsy of pancreatic and duodenal lesions. […] ZollingerEllison Syndrome is a complex condition that requires a multidisciplinary approach for effective management. Early diagnosis, appropriate medical and surgical treatments, and ongoing supportive care are essential to improving patient outcomes.

• #36 Zollinger-Ellison Syndrome Causes, Treatments, Symptoms, Prognosis, and More

  https://www.webmd.com/digestive-disorders/zollinger-ellison-syndrome

  How Is Zollinger-Ellison Syndrome Diagnosed? […] If your doctor suspects that you have ZES, they will perform a blood test to look for high levels of gastrin (the hormone secreted by gastrinomas). They may also perform tests to measure how much acid your stomach is producing. […] Your doctor may examine you for gastrinomas by performing an endoscopy. This procedure is done with a flexible, lighted tube (an endoscope) that looks at your esophagus, stomach and duodenum. This is often done with endoscopic ultrasound to see the tumor. […] Other tests your doctor might perform include a CT scan, a special type of X-ray that provides cross-sectional images of the body, a PET scan to locate tumors, and an octreotide scan to look for neuroendocrine tumor cells. […] Despite these tests, gastrinomas can be difficult to find.

• #37 Zollinger Ellison Syndrome – El Atlas Gastrointestinal – gastrointestinalatlas.com

  https://www.gastrointestinalatlas.com/english/zollinger_ellison_syndrome_.html

  Diagnostic imaging techniques help locate the gastrinomas. The most sophisticated is radionuclide octreotide scanning (also known as somatostatin receptor scintigraphy or 111In pentetreotide SPECT. […] Approximately half of all gastrinomas do not show up on imaging studies. Therefore, exploratory surgery is often recommended to try to locate and remove the tumors. […] The radiological detection of endocrine tumors of the pancreas poses a difficult challenge to the radiologist because of the small size of most of these tumors and frequently requires the combined use of different imaging modalities. However, important progress in the detection of these tumors by noninvasive means has been achieved by the introduction of CT and fast MRI and with the combined use of ultrasound, CT and MRI a sensitivity of more than 90% can nowadays be achieved.

• #38 Zollinger Ellison Syndrome – El Atlas Gastrointestinal – gastrointestinalatlas.com

  https://www.gastrointestinalatlas.com/english/zollinger_ellison_syndrome_.html

  Over 90% of gastrinomas are found within an anatomic triangle referred to as the gastrinoma triangle. The 3 points that define this region are: (1) the confluence of the cystic and common bile duct, (2) the junction of the second and third portions of the duodenum, and (3) the junction of the neck and body of the pancreas. […] The mean age at presentation is 45-50 years, and men are affected more often than women. Because of the rarity of the disease, the average interval between onset of symptoms and diagnosis is about 6 years. Most gastrinomas are malignant; therefore, a high index of suspicion remains key to proper and prompt management of the disease. Management is aimed at cure. ZES may present in one of several ways: […] Peptic ulcer disease: This disease is present in 90%-95% of patients with gastrinomas. Patients who are Helicobacter pylori infection-negative and have no history of nonsteroidal anti-inflammatory drug use may have ZES. Peptic ulcers associated with ZES tend to be more persistent and less responsive to therapy than those not associated with ZES. Ulcers occurring in the second, third, or fourth portions of the duodenum or the jejunum should alert one to the possibility of ZES, although a single ulcer in the duodenal bulb is the most common presentation. Gastroesophageal reflux disease complicated by ulcerations and strictures of the esophagus also tends to be more prevalent and more severe in patients with ZES.

• #39 Gastrinoma – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/gastrinoma/

  The presence of multiple ulcers in atypical locations (e.g., the jejunum) should raise suspicion of gastrinoma. […] Obtain cross sectional imaging and either PET/CT or scintigraphy in all patients with confirmed gastrinoma. […] Most gastrinomas are found in an area called the gastrinoma triangle.

• #40 Zollinger-Ellison Syndrome – NIDDK

  https://www.niddk.nih.gov/health-information/digestive-diseases/zollinger-ellison-syndrome

  How do doctors diagnose Zollinger-Ellison syndrome? Doctors will perform a physical exam and ask about your symptoms, medical history, and family medical history. Your doctor may suspect you have Zollinger-Ellison syndrome if you have certain symptoms and signs, such as chronic diarrhea that occurs along with GERD or peptic ulcer disease […] If your doctor suspects you may have Zollinger-Ellison syndrome, he or she will order tests to diagnose this condition. […] What tests do doctors use to diagnose Zollinger-Ellison syndrome? Doctors may order the following tests to diagnose Zollinger-Ellison syndrome and find the tumors that cause this condition. For a blood test, a health care professional will take a blood sample from you and send the sample to a lab. Doctors may order a blood test to check gastrin levels in your blood after you fast […] Doctors may also measure your stomach acid pH during an upper gastrointestinal (GI) endoscopy. Upper GI endoscopy is a procedure during which a doctor uses an endoscope—a flexible tube with a camera—to see the lining of your upper GI tract, including your esophagus, stomach, and duodenum. Upper GI endoscopy may show signs of Zollinger-Ellison syndrome or its complications. Doctors may also order this test to look for the tumors that cause Zollinger-Ellison syndrome.

• #41 Zollinger Ellison Syndrome – El Atlas Gastrointestinal – gastrointestinalatlas.com

  https://www.gastrointestinalatlas.com/english/zollinger_ellison_syndrome_.html

  Over 90% of gastrinomas are found within an anatomic triangle referred to as the gastrinoma triangle. The 3 points that define this region are: (1) the confluence of the cystic and common bile duct, (2) the junction of the second and third portions of the duodenum, and (3) the junction of the neck and body of the pancreas. […] The mean age at presentation is 45-50 years, and men are affected more often than women. Because of the rarity of the disease, the average interval between onset of symptoms and diagnosis is about 6 years. Most gastrinomas are malignant; therefore, a high index of suspicion remains key to proper and prompt management of the disease. Management is aimed at cure. ZES may present in one of several ways: […] Peptic ulcer disease: This disease is present in 90%-95% of patients with gastrinomas. Patients who are Helicobacter pylori infection-negative and have no history of nonsteroidal anti-inflammatory drug use may have ZES. Peptic ulcers associated with ZES tend to be more persistent and less responsive to therapy than those not associated with ZES. Ulcers occurring in the second, third, or fourth portions of the duodenum or the jejunum should alert one to the possibility of ZES, although a single ulcer in the duodenal bulb is the most common presentation. Gastroesophageal reflux disease complicated by ulcerations and strictures of the esophagus also tends to be more prevalent and more severe in patients with ZES.

• #42 Gastrinoma – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/gastrinoma/

  The presence of multiple ulcers in atypical locations (e.g., the jejunum) should raise suspicion of gastrinoma. […] Obtain cross sectional imaging and either PET/CT or scintigraphy in all patients with confirmed gastrinoma. […] Most gastrinomas are found in an area called the gastrinoma triangle.

• #43 Gastrinoma – Hormonal and Metabolic Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/hormonal-and-metabolic-disorders/gastrointestinal-and-pancreatic-neuroendocrine-tumors/gastrinoma

  Gastrinomas arise from cells in the pancreas that produce the hormone gastrin. […] Diagnosis includes blood and imaging tests. […] A doctor suspects a gastrinoma when a person has frequent peptic ulcers or several peptic ulcers that do not respond to the usual ulcer treatments. Blood tests to detect abnormally high levels of gastrin are the most reliable diagnostic tests. […] Once blood tests diagnose gastrinoma, doctors try to locate the tumor using several imaging techniques, such as computed tomography (CT) of the abdomen, scintigraphy (a type of radionuclide scanning), endoscopic ultrasound, positron emission tomography (PET), and arteriography (an x-ray taken after a radiopaque dye is injected into an artery). These tumors may be difficult to find, however, because usually they are small.

• #44 ZOLLINGER-ELLISON SYNDROME (GASTRINOMA) | Harrison’s Manual of Medicine

  https://harrisons.unboundmedicine.com/harrisons/view/Harrisons-Manual-of-Medicine/623366/all/ZOLLINGER_ELLISON_SYNDROME__GASTRINOMA_

  Consider when ulcer disease is severe, refractory to therapy, associated with ulcers in atypical locations, or associated with diarrhea. […] Basal acid output 15 mmol/h; basal/maximal acid output 60%; large mucosal folds on endoscopy or upper GI radiograph. […] Serum gastrin 1000 ng/L or rise in gastrin of 200 ng/L following IV secretin and, if necessary, rise of 400 ng/L following IV calcium. […] Z-E syndrome, antral G-cell hyperplasia or hyperfunction (? due to H. pylori), postgastrectomy retained antrum, renal failure, massive small bowel resection, chronic gastric outlet obstruction. […] Pernicious anemia, chronic gastritis, gastric cancer, vagotomy, pheochromocytoma.

• #45 Zollinger-Ellison Syndrome: Not Your Average Peptic Ulcer Disease | Clinician Reviews

  https://www.mdedge9-ma1.mdedge.com/clinicianreviews/article/140787/gastroenterology/zollinger-ellison-syndrome-not-your-average-peptic

  Once lab tests have been performed, a series of imaging studies are indicated. Endoscopy is used to identify active ulcers and erosions due to long-term acid secretion. […] CT, MRI, and somatostatin receptor scintigraphy (a specialized form of imaging that is the study of choice for localizing gastrinomas) are performed to localize primary tumors and identify any metastatic disease that may be present. […] Finally, after lab tests and imaging studies have been completed, genetic screening for MEN1 is used to determine if the patient has a sporadic or hereditary gastrinoma.

• #46 Zollinger-Ellison syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/zollinger-ellison-syndrome/diagnosis-treatment/drc-20379046

  Endoscopy also can find out whether the stomach is making acid. If the stomach is making acid and the gastrin level is high, then the diagnosis of Zollinger-Ellison can be established. […] Imaging techniques may be used to look for tumors, such as CT scans, MRI imaging and Ga-DOTATATE PET-CT scanning. […] Certain acid-reducing medicines, such as proton pump inhibitors or H-2 blockers, can affect the results of some tests used to diagnose Zollinger-Ellison syndrome. […] What tests do I need to confirm the diagnosis? How should I prepare for those tests?

• #47 Zollinger-Ellison syndrome // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/zollinger-ellison-syndrome

  Upper gastrointestinal endoscopy. This test requires sedation. An endoscopy involves putting a thin, flexible instrument down the throat and into the stomach and duodenum. […] Endoscopy also can find out whether the stomach is making acid. If the stomach is making acid and the gastrin level is high, then the diagnosis of Zollinger-Ellison can be established. […] Imaging tests. Imaging techniques may be used to look for tumors, such as CT scans, MRI imaging and Ga-DOTATATE PET-CT scanning.

• #48 Zollinger-Ellison Syndrome Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/183555-workup

  The secretin stimulation test is the provocative test of choice because of its higher sensitivity. In this test, a 2-U/kg bolus of secretin is administered intravenously after an overnight fast, and serum levels of gastrin are determined at 0, 2, 5, 10, and 15 minutes. An increase in serum gastrin of greater than 200 pg/mL is diagnostic. […] A suggested algorithm for the evaluation of a patient with suspected gastrinoma is as follows: Step 1: Check the fasting gastrin level. Measure at least 3 fasting levels of gastrin on different days. […] Step 2: Perform gastric acid secretory studies. A BAO value of greater than 15 mEq/h or a gastric volume of greater than 140 mL and pH of less than 2.0 are highly suggestive of gastrinoma. […] Step 3: Perform a provocative test. The secretin stimulation test is the preferred test.

• #49 Zollinger-Ellison Syndrome: Symptoms and Treatment

  https://patient.info/doctor/zollinger-ellison-syndrome

  Biochemical serum evaluation for elevated gastrin, followed by radiological or nuclear localisation of the primary lesion, is necessary for establishing the diagnosis. […] Hypergastrinaemia is defined as fasting serum gastrin concentration 100 pg/ml and is seen frequently in clinical conditions other than ZES – eg, proton pump inhibitor (PPI) or H2-receptor antagonist therapy, post-vagotomy, renal failure and chronic atrophic gastritis. […] If the diagnosis is suspected, then more specific tests are required – these should be performed in a specialist setting. The following diagnostic algorithm is taken from consensus guidelines on the management of functional pancreatic neuroendocrine tumours: […] Check fasting serum gastrin (FSG) level – if this is raised, check gastric pH level. […] If ZES is confirmed, it is important to consider MEN1, and calcium, parathormone and prolactin levels should be checked.

• #50 Zollinger-Ellison Syndrome: Symptoms and Treatment

  https://patient.info/doctor/zollinger-ellison-syndrome

  Biochemical serum evaluation for elevated gastrin, followed by radiological or nuclear localisation of the primary lesion, is necessary for establishing the diagnosis. […] Hypergastrinaemia is defined as fasting serum gastrin concentration 100 pg/ml and is seen frequently in clinical conditions other than ZES – eg, proton pump inhibitor (PPI) or H2-receptor antagonist therapy, post-vagotomy, renal failure and chronic atrophic gastritis. […] If the diagnosis is suspected, then more specific tests are required – these should be performed in a specialist setting. The following diagnostic algorithm is taken from consensus guidelines on the management of functional pancreatic neuroendocrine tumours: […] Check fasting serum gastrin (FSG) level – if this is raised, check gastric pH level. […] If ZES is confirmed, it is important to consider MEN1, and calcium, parathormone and prolactin levels should be checked.

• #51 ZOLLINGER-ELLISON SYNDROME (GASTRINOMA) | Harrison’s Manual of Medicine

  https://harrisons.unboundmedicine.com/harrisons/view/Harrisons-Manual-of-Medicine/623366/all/ZOLLINGER_ELLISON_SYNDROME__GASTRINOMA_

  Consider when ulcer disease is severe, refractory to therapy, associated with ulcers in atypical locations, or associated with diarrhea. […] Basal acid output 15 mmol/h; basal/maximal acid output 60%; large mucosal folds on endoscopy or upper GI radiograph. […] Serum gastrin 1000 ng/L or rise in gastrin of 200 ng/L following IV secretin and, if necessary, rise of 400 ng/L following IV calcium. […] Z-E syndrome, antral G-cell hyperplasia or hyperfunction (? due to H. pylori), postgastrectomy retained antrum, renal failure, massive small bowel resection, chronic gastric outlet obstruction. […] Pernicious anemia, chronic gastritis, gastric cancer, vagotomy, pheochromocytoma.

• #52 Zollinger-Ellison Syndrome: Symptoms and Treatment

  https://patient.info/doctor/zollinger-ellison-syndrome

  Biochemical serum evaluation for elevated gastrin, followed by radiological or nuclear localisation of the primary lesion, is necessary for establishing the diagnosis. […] Hypergastrinaemia is defined as fasting serum gastrin concentration 100 pg/ml and is seen frequently in clinical conditions other than ZES – eg, proton pump inhibitor (PPI) or H2-receptor antagonist therapy, post-vagotomy, renal failure and chronic atrophic gastritis. […] If the diagnosis is suspected, then more specific tests are required – these should be performed in a specialist setting. The following diagnostic algorithm is taken from consensus guidelines on the management of functional pancreatic neuroendocrine tumours: […] Check fasting serum gastrin (FSG) level – if this is raised, check gastric pH level. […] If ZES is confirmed, it is important to consider MEN1, and calcium, parathormone and prolactin levels should be checked.

• #53 Zollinger-Ellison Syndrome Causes, Symptoms, Diagnosis and Treatment – Cura4U

  https://cura4u.com/conditions/zollinger-ellison-syndrome

  Zollinger-Ellison syndrome should be differentiated from other conditions that cause abdominal pain and diarrhea. Examples of such conditions include peptic ulcers, gastroesophageal reflux disease, hypergastrinemia, gastric antrum syndrome, Crohns disease, celiac disease, irritable bowel syndrome, etc.

• #54 Zollinger–Ellison syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Zollinger%E2%80%93Ellison_syndrome

  ZollingerEllison syndrome may be suspected when the above symptoms prove resistant to treatment when the symptoms are especially suggestive of the syndrome, or when endoscopy is suggestive. The diagnosis is made through several laboratory tests and imaging studies: […] The diagnosis is also suspected in patients who have severe and recurrent ulceration of the stomach and small bowel, especially if they fail to respond to treatment. […] Diagnosis can sometimes be very difficult, especially considering patients who take a proton pump inhibitor (PPI) medication for gastric reflux, which constitute a large and increasing proportion of people who develop ZollingerEllison syndrome. PPIs inadvertently increase gastrin production, which may cause a false positive for elevated gastrin levels. This can occur even in patients who have been off their medication for weeks, due to the long duration of effects of these medications.

• #55 Zollinger-Ellison Syndrome (ZES) | Loma Linda University Health

  https://lluh.org/conditions/zollinger-ellison-syndrome-zes

  Zollinger-Ellison syndrome (ZES) is sometimes known as gastrinoma, pancreatic ulcerogenic tumor syndrome or Z-E syndrome. […] ZES is a rare condition that is usually diagnosed in patients between the ages of 20 and 60. […] Diagnosis of Zollinger-Ellison syndrome starts with a medical exam and health history. Doctors may order lab work, including a blood test to check for high levels of a certain hormone that is associated with ZES. […] Patients should tell the doctor if they have used over-the-counter, acid-reducing medications. Often, these medications may mask the symptoms of ZES. This could lead to a misdiagnosis or delay the diagnosis.

• #56 Zollinger-Ellison syndrome | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/zollinger-ellison-syndrome?lang=us

  Zollinger-Ellison syndrome (ZES) is a clinical syndrome that occurs secondary to elevated gastrin levels produced by a gastrinoma. […] Diagnosis is often delayed by 5-7 years after the onset of symptoms. […] Increased gastrin levels in fasting patients (but not specific, and some data suggest that clinical assays may be unreliable). […] Surgery plays a vital role. […] Death from complications of Zollinger-Ellison syndrome (e.g. perforation, hemorrhage) can occur. […] Diagnosis of Zollinger-Ellison syndrome: increasingly difficult.

• #57

  https://www.msjonline.org/index.php/ijrms/article/view/13311

  Zollinger-Ellison syndrome (ZES) is characterized by gastrin-secreting neuroendocrine tumors (gastrinomas) in the duodenum or pancreas. […] The diagnosis is confirmed by the secretin stimulation test and imaging studies such as octreotide scintigraphy. […] Diagnosis requires elevated fasting serum gastrin levels and hypersecretion of gastric acid. […] The diagnosis of ZES can be complicated due to the unreliability of the assays and the need for secretin testing. […] Patients should undergo imaging studies for tumor localization and regular monitoring for complications and recurrences.

• #58 Zollinger-Ellison syndrome (gastrinoma): Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/zollinger-ellison-syndrome-gastrinoma-clinical-manifestations-and-diagnosis

  Zollinger-Ellison (ZES) syndrome is characterized by gastric acid hypersecretion resulting in severe acid-related peptic disease and diarrhea. The clinical manifestations and diagnosis of ZES will be reviewed here. […] Zollinger-Ellison syndrome (ZES) is caused by secretion of gastrin by duodenal or pancreatic neuroendocrine tumors (gastrinomas). […] The annual incidence of gastrinomas is 0.5 to 2 per million population. […] The clinical presentation, diagnosis, and management of VIPoma. […] The designation of the tumor as a gastrinoma is based upon the presence of a clinical syndrome that results from tumor production and secretion of gastrin, and not by its morphologic appearance or the presence of gastrin in the secretory granules. […] If a tumor stains for gastrin or secretes gastrin but does not produce symptoms of Zollinger-Ellison syndrome, it should not be considered a gastrinoma.

• #59 Gastroenterology Education and CPD for trainees and specialists » Zollinger Ellison syndrome

  https://www.gastrotraining.com/category/gastro-duodenal/zollinger-ellison-syndrome

  More than 90% of gastrinomas can be localized using Octreoscan and/or EUS. CT and MRI also have high sensitivity in localizing the gastrinomas. […] The incidence of ZES is 0.1 to 1% in patients with peptic ulcer disease. If we test, we are going to test more than 1000 people unnecessarily in order to identify one Z-E patient. If we do not test, the worry is Will we delay the diagnosis of Z-E? Most importantly, if we delay the diagnosis will that lead to more advanced disease? There is no good study which has evaluated this.

• #60 Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome – PubMed

  https://pubmed.ncbi.nlm.nih.gov/28776139/

  Zollinger-Ellison syndrome (ZES) results from an ectopic gastrin-secreting tumor leading to peptic ulcer disease, reflux, and chronic diarrhea. […] While early recognition portends an excellent prognosis with 80% survival at 15 years, symptoms are often nonspecific making the diagnosis difficult to establish. Diagnosis involves a series of tests, including fasting gastrin, gastric pH, chromogranin A, and secretin stimulation. Performing these tests in the correct sequence and at the proper time is essential to avoid inaccurate results. […] Tumor localization is equally nuanced. Although providers have classically used 111indium-radiolabeled octreotide with somatostatin receptor scintigraphy to evaluate tumor size and metastases, recent studies have shown superior results with newer imaging modalities. In particular, 68gallium (68Ga)-labeled somatostatin radiotracers (i.e., 68Ga-DOTATOC, 68Ga-DOTANOC and 68Ga-DOTATATE) used with positron emission tomography/computed tomography can provide excellent results. Endoscopic ultrasound is another useful modality, particularly in patients with ZES in the setting of multiple endocrine neoplasia type 1. This review aims to provide clinicians with an overview of ZES with a focus on both clinical presentation and the proper utilization of the various biochemical and imaging tests available.

• #61 Zollinger-Ellison syndrome | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/zollinger-ellison-syndrome?lang=us

  Zollinger-Ellison syndrome (ZES) is a clinical syndrome that occurs secondary to elevated gastrin levels produced by a gastrinoma. […] Diagnosis is often delayed by 5-7 years after the onset of symptoms. […] Increased gastrin levels in fasting patients (but not specific, and some data suggest that clinical assays may be unreliable). […] Surgery plays a vital role. […] Death from complications of Zollinger-Ellison syndrome (e.g. perforation, hemorrhage) can occur. […] Diagnosis of Zollinger-Ellison syndrome: increasingly difficult.

• #62 Zollinger-Ellison syndrome – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/408

  Zollinger-Ellison syndrome (ZES) is a condition caused by gastrin-producing neuroendocrine tumour that causes gastric acid hypersecretion and peptic ulcer disease. […] Common presentation includes refractory peptic ulcer disease, abdominal pain, diarrhoea, and gastro-oesophageal disease. […] Zollinger-Ellison syndrome is a condition caused by a gastrin-secreting tumour that causes hypersecretion of gastric acid leading to ulcer disease. It most commonly presents with abdominal pain (70% to 100% of patients), diarrhoea (37% to 73% of patients), and gastro-oesophageal reflux disease (44% of patients). […] Key diagnostic factors: presence of risk factors, refractory or recurrent peptic ulcer disease, diarrhoea. […] Other diagnostic factors: epigastric abdominal pain, gastro-oesophageal reflux disease, steatorrhoea, gastrointestinal bleed, bone pain.

• #63 Zollinger-Ellison Syndrome (ZES) – Gastric Diseases – Gastrointestinal Diseases – Gastroenterology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.4.70.2.

  Zollinger-Ellison syndrome (ZES) is characterized by gastrin-secreting tumors (gastrinomas), which are most frequently located in the duodenum or pancreas, rarely in adjacent lymph nodes. […] ZES should be suspected in the setting of multiple, refractory, or atypically distal PUD, diarrhea, or MEN 1 history. […] Diagnosis is based on the characteristic clinical features, typical laboratory findings, and location of the tumor confirmed using imaging studies.

• #64 Zollinger-Ellison Syndrome: Symptoms and Treatment

  https://patient.info/doctor/zollinger-ellison-syndrome

  An initial upper gastrointestinal endoscopy with careful inspection of the duodenum is recommended, followed by CT or by MRI and somatostatin receptor scintigraphy (SRS). […] If ZES is confirmed, other features of MEN1 should be sought, including a family history. […] Once the diagnosis has been made, management is within tertiary centres by multidisciplinary teams. […] Management includes medical suppression of gastric acid production and surgical resection of primary tumour for the prevention of malignant transformation and metastatic complications. […] Postoperative surveillance involves measurement of gastrin level, with imaging if an elevation of gastrin levels is detected.

• #65 Zollinger-Ellison Syndrome: Symptoms and Treatment

  https://patient.info/doctor/zollinger-ellison-syndrome

  An initial upper gastrointestinal endoscopy with careful inspection of the duodenum is recommended, followed by CT or by MRI and somatostatin receptor scintigraphy (SRS). […] If ZES is confirmed, other features of MEN1 should be sought, including a family history. […] Once the diagnosis has been made, management is within tertiary centres by multidisciplinary teams. […] Management includes medical suppression of gastric acid production and surgical resection of primary tumour for the prevention of malignant transformation and metastatic complications. […] Postoperative surveillance involves measurement of gastrin level, with imaging if an elevation of gastrin levels is detected.

• #66 Zollinger–Ellison Syndrome

  https://neuroendocrine.org.au/zollinger-ellison-syndrome/

  In addition to imaging, other tests can help confirm the presence of gastrinomas, such as: Secretin Stimulation Test: Measures the increase in gastrin levels after administration of secretin, Endoscopic Ultrasound (EUS): Allows for detailed imaging and biopsy of pancreatic and duodenal lesions. […] ZollingerEllison Syndrome is a complex condition that requires a multidisciplinary approach for effective management. Early diagnosis, appropriate medical and surgical treatments, and ongoing supportive care are essential to improving patient outcomes.

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