Curriculum · Gastroenterology
Zollinger-Ellison syndrome (gastrinoma)
What it is
Zollinger-Ellison syndrome is a gastrin-secreting tumor of non-beta islet cells, the islet of Langerhans, and it is also set down as a gastrin-secreting cancer of the pancreas.
The chain runs one way and explains every feature: the tumor secretes gastrin, gastrin acts on the parietal cell, the parietal cell pours out hydrochloric acid, and the acid makes the ulcer. ZES is a rare but important cause of refractory or multiple PUD.
How it presents
The clinical list is abdominal pain, chronic diarrhea, dyspepsia, weight loss and GI bleeding.
Two of those together are what should raise it: multiple refractory ulcers with persistent diarrhea. Refractory duodenal ulcers and diarrhea is suggestive of ZES.
The syndrome it belongs to
ZES is associated with multiple endocrine neoplasia, MEN syndrome, and with MEN1, and that association is half the topic.
MEN-I is the three Ps: parathyroid, pituitary and pancreatic tumors, and with them hyper-Ca. So a hyper-Ca beside a refractory ulcer is not a separate problem; it comes from the parathyroid tumor of MEN-I.