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25 Cards in this Set

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