Zollinger-Ellison syndrome
Meaning of Zollinger-Ellison syndrome: Ulcers that keep coming back in unusual places, traced to a small hormone-producing tumor that leaves the stomach permanently switched on.
Definition of Zollinger-Ellison syndrome
Zollinger-Ellison syndrome is the set of problems caused by a tumor that releases the hormone gastrin without restraint. The excess gastrin drives the stomach to pour out acid, producing ulcers that are often multiple, resistant to healing and found further along the bowel than usual, together with diarrhea and heartburn. The tumor, a gastrinoma, most often sits in the wall of the duodenum or in the pancreas.
Where does the tumor actually sit?
Older descriptions place it in the islet cells of the pancreas, and that is not where most are found. The majority arise in the wall of the duodenum, the first stretch of small bowel, where they can be small enough to be missed at surgery. The rest lie in the pancreas or in nearby lymph nodes, within a region surgeons call the gastrinoma triangle. Gastrin producing cells are not a normal part of the adult pancreas at all, which makes the pancreatic tumors the more surprising of the two.
Why does excess gastrin cause so much trouble?
Gastrin is the normal signal telling the stomach to make acid, and it also drives the acid producing cells to multiply. A tumor releasing it continuously does both at once. Acid output climbs and stays high, so a peptic ulcer heals poorly and returns after it has healed. The flood of acid passing on into the small bowel also inactivates the pancreatic enzymes that digest fat, which is why loose greasy stools and weight loss accompany the ulcers. Heartburn from reflux is common for the same reason.
What points to it rather than an ordinary ulcer?
| Feature | Ordinary peptic ulcer | Zollinger-Ellison |
|---|---|---|
| Number | Usually one | Often several |
| Site | Stomach or the first part of the duodenum | May extend well beyond that point |
| Response to standard treatment | Heals | Persists or recurs |
| Diarrhea | Uncommon | Frequent, sometimes the first complaint |
Most gastrinomas are capable of spreading, and the liver is where that matters for survival. A minority occur as part of an inherited condition, multiple endocrine neoplasia type 1, in which tumors of the parathyroid and pituitary glands appear as well, and those cases tend to involve several small tumors rather than one.
Also known as
Used in a sentence
Zollinger-Ellison syndrome was considered after multiple duodenal ulcers recurred despite treatment.
Good to know
Most gastrinomas arise in the wall of the duodenum rather than in the pancreatic islets, and acid-suppressing drugs raise gastrin levels on their own, which complicates interpretation of a blood gastrin result.