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Review Question - QID 101082

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QID 101082 (Type "101082" in App Search)
A 41-year-old female complains of frequent diarrhea and abdominal pain between meals. Endoscopy reveals a duodenal ulcer distal to the duodenal bulb. CT scan of the abdomen demonstrates a pancreatic mass, and subsequent tissue biopsy of the pancreas reveals a malignant islet cell tumor. Which of the following hormones is likely to be markedly elevated in this patient:

Gastrin

58%

173/298

Cholecystokinin

13%

40/298

Secretin

9%

26/298

Vasoactive intestinal peptide

17%

51/298

Motilin

1%

2/298

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The patient is suffering from Zollinger-Ellison (ZE) syndrome due to a pancreatic gastrinoma. Excess gastrin secretion leads to excess gastric acid production in the stomach, often resulting in peptic ulcer disease.

Atypical ulcers, such as a duodenal ulcer distal to the duodenal bulb (the first part of the duodenum), multiple ulcers, and ulcers refractory to therapy are all suggestive of ZE syndrome. Diarrhea due to acidic inactivation of pancreatic enzymes may also be present in patients with ZE syndrome. Serum gastrin levels are dramatically increased in patients with ZE syndrome.

Macaron reviews endocrine-secreting pancreatic tumors. Examples include, insulinoma, glucagonoma, gastrinoma (ZE syndrome), VIPoma, somatostatinoma and others. Hormones are produced by specific tumor cell types and cause dramatic clinical presentations.

Oberg reviews the pathophysiology and clinical presentation of pancreatic endocrine tumors, including gastrinomas. Ectopic gastrin secretion from pancreatic endocrine tumors or duodenal tumors (gastrinomas) cause ZE syndrome. Excessive acid secretion leads to peptic ulcers and gastric esophagus reflux disease (GERD).

Illustration A shows an endoscopy image of multiple small ulcers in the distal duodenum in a patient with Zollinger–Ellison syndrome.

Incorrect answers:
Answer 2: Cholecystokinin regulates gallbladder contraction, gastric emptying, and pancreatic secretion. It is secreted by I cells in the duodenum and jejunum in response to fatty acids and amino acids.
Answer 3: Secretin is secreted by duodenal S cells. It decreases gastric acid secretion and increases pancreatic bicarbonate secretion.
Answer 4: Vasoactive intestinal peptide increases intestinal water and electrolyte secretion and prompts relaxation of intestinal smooth muscle. A VIPoma could cause watery diarrhea, hypokalemia, and hypochlorhydria or achlorhydria, but does not usually cause abdominal pain or ulcers.
Answer 5: Motilin is secreted by the small intestine and is an important regulator of peristalsis.

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