Zollinger-Ellison syndrome screening: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{CMG}} {{AE}} {{MJK}}; {{ARK}} | {{CMG}} {{AE}} {{MJK}}; {{ARK}} | ||
{{Zollinger-Ellison syndrome}} | {{Zollinger-Ellison syndrome}} | ||
==Overview== | ==Overview== | ||
The single best screening test for Zollinger-Ellison syndrome (ZES) is by measuring fasting serum gastrin levels. Other tests such as provocative tests include, the secretin stimulation test, calcium stimulation test, secretin-plus-calcium stimulation tests, bombesin test, and protein meal test. | The single best screening test for Zollinger-Ellison syndrome (ZES) is by measuring fasting [[serum]] [[gastrin]] levels. Other tests such as provocative tests include, the [[secretin]] stimulation test, [[calcium]] stimulation test, secretin-plus-calcium stimulation tests, [[bombesin]] test, and protein meal test. | ||
==Screening== | ==Screening== | ||
*The single best screening test for Zollinger-Ellison syndrome (ZES) is by measuring fasting serum gastrin levels. | *The single best screening test for Zollinger-Ellison syndrome (ZES) is by measuring fasting [[serum]] [[gastrin]] levels. | ||
*At the time of the test, it is advised not to take gastric antisecretory medications. | *At the time of the test, it is advised not to take [[gastric]] antisecretory medications. | ||
*Serial multiple measurements on different days have to be performed because, fasting gastrin levels can fluctuate from day to day and can appear to be normal. | *Serial multiple measurements on different days have to be performed because, fasting [[gastrin]] levels can fluctuate from day to day and can appear to be normal. | ||
*Normal levels of serum gastrin in untreated ZES are extremely rare (<1%) | *Normal levels of serum gastrin in untreated ZES are extremely rare (<1%) | ||
*Multiple endocrine neoplasia-type 1 (MEN 1) syndrome should be suspected if serum calcium levels are elevated. | *[[Multiple endocrine neoplasia type 1|Multiple endocrine neoplasia-type 1]] (MEN 1) syndrome should be suspected if serum [[calcium]] levels are elevated. | ||
:*Gastric acid secretory test: 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 acid secretory test:''' 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. | ||
:*Basal gastric secretory volume greater than 140 mL in patients with no prior gastric acid–reducing surgery has a high sensitivity and specificity. | :*Basal gastric secretory volume greater than 140 mL in patients with no prior gastric acid–reducing surgery has a high sensitivity and specificity. | ||
:*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. | :*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. | ||
*'''Provocative tests:''' <ref name="pmid17108779">{{cite journal |vauthors=Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT |title=Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features |journal=Medicine (Baltimore) |volume=85 |issue=6 |pages=331–64 |year=2006 |pmid=17108779 |doi=10.1097/MD.0b013e31802b518c |url=}}</ref> | *'''Provocative tests:''' <ref name="pmid17108779">{{cite journal |vauthors=Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT |title=Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features |journal=Medicine (Baltimore) |volume=85 |issue=6 |pages=331–64 |year=2006 |pmid=17108779 |doi=10.1097/MD.0b013e31802b518c |url=}}</ref> | ||
:*Secretin stimulation test <ref name="pmid23851430">{{cite journal |vauthors=Shah P, Singh MH, Yang YX, Metz DC |title=Hypochlorhydria and achlorhydria are associated with false-positive secretin stimulation testing for Zollinger-Ellison syndrome |journal=Pancreas |volume=42 |issue=6 |pages=932–6 |year=2013 |pmid=23851430 |pmc=3712291 |doi=10.1097/MPA.0b013e3182847b2e |url=}}</ref> | :*[[Secretin]] stimulation test <ref name="pmid23851430">{{cite journal |vauthors=Shah P, Singh MH, Yang YX, Metz DC |title=Hypochlorhydria and achlorhydria are associated with false-positive secretin stimulation testing for Zollinger-Ellison syndrome |journal=Pancreas |volume=42 |issue=6 |pages=932–6 |year=2013 |pmid=23851430 |pmc=3712291 |doi=10.1097/MPA.0b013e3182847b2e |url=}}</ref> | ||
:*Calcium stimulation test | :*[[Calcium]] stimulation test | ||
:*Secretin-plus-calcium stimulation tests | :*Secretin-plus-calcium stimulation tests | ||
:*Bombesin test | :*[[Bombesin]] test | ||
:*Protein meal test | :*Protein meal test | ||
*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. | *[[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. | ||
*'''Evaluation of a patient with suspected gastrinoma:''' | *'''Evaluation of a patient with suspected gastrinoma:''' | ||
:*Step 1: Fasting gastrin level are evaluated. A minimum of 3 fasting levels of gastrin on different days are measured. | :*Step 1: Fasting gastrin level are evaluated. A minimum of 3 fasting levels of gastrin on different days are measured. | ||
| Line 28: | Line 29: | ||
:*Step 3: Perform a provocative test. The secretin stimulation test is the preferred test. | :*Step 3: Perform a provocative test. The secretin stimulation test is the preferred test. | ||
:*Step 4: Perform somatostatin receptor scintigraphy (SRS). | :*Step 4: Perform somatostatin receptor scintigraphy (SRS). | ||
:*Step 5: Perform imaging studies to stage and localize the gastrinoma. | :*Step 5: Perform imaging studies to stage and localize the [[gastrinoma]]. | ||
:*Step 6: Determine if patient is a surgical candidate for tumor resection. | :*Step 6: Determine if patient is a surgical candidate for tumor resection. | ||
Revision as of 01:57, 16 August 2017
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]; Aravind Reddy Kothagadi M.B.B.S[3]
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Overview
The single best screening test for Zollinger-Ellison syndrome (ZES) is by measuring fasting serum gastrin levels. Other tests such as provocative tests include, the secretin stimulation test, calcium stimulation test, secretin-plus-calcium stimulation tests, bombesin test, and protein meal test.
Screening
- The single best screening test for Zollinger-Ellison syndrome (ZES) is by measuring fasting serum gastrin levels.
- At the time of the test, it is advised not to take gastric antisecretory medications.
- Serial multiple measurements on different days have to be performed because, fasting gastrin levels can fluctuate from day to day and can appear to be normal.
- Normal levels of serum gastrin in untreated ZES are extremely rare (<1%)
- Multiple endocrine neoplasia-type 1 (MEN 1) syndrome should be suspected if serum calcium levels are elevated.
- Gastric acid secretory test: 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.
- Basal gastric secretory volume greater than 140 mL in patients with no prior gastric acid–reducing surgery has a high sensitivity and specificity.
- 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.
- Provocative tests: [1]
- 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.
- Evaluation of a patient with suspected gastrinoma:
- Step 1: Fasting gastrin level are evaluated. A minimum of 3 fasting levels of gastrin on different days are measured.
- Step 2: Gastric acid secretory studies which reveal highly suggestive findings of gastrinoma:
- Basal acid output (BAO) value of greater than 15 mEq/h (OR) [3]
- Gastric volume of greater than 140 mL and pH of less than 2.0 .
- Step 3: Perform a provocative test. The secretin stimulation test is the preferred test.
- Step 4: Perform somatostatin receptor scintigraphy (SRS).
- Step 5: Perform imaging studies to stage and localize the gastrinoma.
- Step 6: Determine if patient is a surgical candidate for tumor resection.
References
- ↑ Berna MJ, Hoffmann KM, Long SH, Serrano J, Gibril F, Jensen RT (2006). "Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features". Medicine (Baltimore). 85 (6): 331–64. doi:10.1097/MD.0b013e31802b518c. PMID 17108779.
- ↑ Shah P, Singh MH, Yang YX, Metz DC (2013). "Hypochlorhydria and achlorhydria are associated with false-positive secretin stimulation testing for Zollinger-Ellison syndrome". Pancreas. 42 (6): 932–6. doi:10.1097/MPA.0b013e3182847b2e. PMC 3712291. PMID 23851430.
- ↑ Roy PK, Venzon DJ, Feigenbaum KM, Koviack PD, Bashir S, Ojeaburu JV, Gibril F, Jensen RT (2001). "Gastric secretion in Zollinger-Ellison syndrome. Correlation with clinical expression, tumor extent and role in diagnosis--a prospective NIH study of 235 patients and a review of 984 cases in the literature". Medicine (Baltimore). 80 (3): 189–222. PMID 11388095.