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25 Cards in this Set
- Front
- Back
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What is Zollinger-Ellison Syndrome?
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Gastrinoma
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What is a gastrinoma?
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non beta islet cell tumor of pancreas (or other locale) that produces gastrin, causing gastric hypersecretion of HCl acid, resulting in GI ulcers
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Incidence of Zollinger Ellison Syndrome?
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1/1000 pts with peptic ulcer disease, but 2% with pts who have recurrent ulcers
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What is the syndrome associated with Z-E Syndrome?
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MEN I syndrome
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What % of pts with ZE have MEN I syndrome?
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25%
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What % of pts with MEN I syndrome will have ZE?
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about 50%
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Signs and symptoms of ZE?
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peptic ulcers
diarrhea weight loss abdominal pain |
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What causes the diarrhea in ZE?
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massive acid hypersecretion and destruction of digestive enzymes
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Signs of ZE?
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peptic ulcer disease: epigastric pain, hematemesis, melena, hematochezia, GERD, diarrhea, recurrent ulcers, ulcers in UNUSUAL LOCATIONS (eg. prox jejunum)
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Possible complications of ZE?
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GI hemorrhage/perforation, gastric outlet obstruction/stricture, metastatic disease
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What is the differential dx of increased gastrin?
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postvagotomy
gastric outlet obstruction antral G-cell hyperplasia/hyperfunction pernicious anemia atrophic gastritis short gut syndrome renal failure H2 blocker, omeprazole (normally pH<2 blocks gastrin) |
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What pts should have gastrin checked?
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pts with recurrent ulcers, ulcers in unusual spots (eg. jejunum), or refractory to med mgmt, before any operation for ulcer
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What lab tests should be performed for ZE?
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fastin gastrin level
postsecretin challenge gastrin level calcium (MEN I screen) chem 7 |
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What are normal and ZE associated gastrin levels?
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NL fasting = 100 pg/ml
ZES fasting = 200-1000 pg/ml basal acid secretion (ZES > 15mEq/hr, nl<10mEq/hr) |
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What is the secretin stimulation test?
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ZE pts have paradoxical increase in gastrin when IV secretin is administered; NL pts have decreased gastrin
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What tests evaluate ulcers?
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EGD
UGI |
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What tests are used to localize ZE tumor?
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abdominal CT
MRE octreotide scans selective angiography selective venous sampling for gastrin |
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What is most common site for ZE?
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pancreas
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What is most common non-pancreatic site for ZE?
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duodenum
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What is the gastrinoma triangle?
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1. cystic duct
2. junction of the 2nd and 3rd portions of the duodenum 3. neck of the pancreas (90% of gastrinomas are in this triangle) |
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What is med mgmt for ZE?
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H2 blockers
omeprazole somatostatin |
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What % have malignant tumors in ZE?
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67%
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What is most common site of metastasis in ZE?
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liver
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What is treatment of pts with liver mets in ZE?
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excision
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What is surgical option if ZE is in duodenum and head of pancreas and is too large, or too many lymph mets for resection?
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Whipple procedure
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