NEJM case: Zollinger–Ellison syndrome

Zollinger–Ellison syndrome, also known as Zollinger–Ellison syndrome, is a chronic refractory ulcer
of the upper gastrointestinal tract caused by non-β islet cell tumor or antral G-cell proliferation that occurs in the pancreas.
The peptic ulcer caused by the former is called Zhuolig syndrome type II, while the peptic ulcer caused by the latter is called type
I.
In 1955, Zollinger and Ellison first reported two such cases in 1956, when Eiseman and Maynard proposed that the syndrome be called Zollinger syndrome
.

Later, with the deepening of the understanding of this disease, many other names such as gastrinoma, pancreatic ulcer, primary gastrin hypertrophosis pancreatic non-β cell tumor, etc
.
have appeared.
Gastric juice stimulating tumor secretes a large amount of gastric juice stimulating gastric juice hormone, constantly stimulating gastric parietal cells to secrete a large amount of gastric acid, and finally causing gastroduodenal mucosal damage, erosion and ulceration, because it is a tumor, this process is always going on, so the treatment of general peptic ulcer drugs is difficult to get good effect
.

For the small intestine, gastric juice stimulating hormone can inhibit its absorption of water and electrolytes and promote the increase of small intestinal peristalsis, so patients with gastric juice protoma may have watery diarrhea, and gastric juice can also promote the increase of pancreatic exocrine, and also increase the secretion of
bile duodenal fluid.
These have strengthened the factors of diarrhea, but the most important is the continuous large-scale secretion of tumors, resulting in serious results
such as refractory ulcers with bleeding and perforation.

One case
was reported in the latest issue of the New England magazine.

A 75-year-old man presents to the emergency department with epigastric pain, watery diarrhoea, and weight loss for 6 months
.
Physical examination may reveal epigastric tenderness
.
Esophagogastroduodenoscopy shows severe esophagitis (figure A), antral erosions, and duodenal ulcer (figure B).

Gastric pH below 2 and fasting serum gastrin levels above 1000 pg/mL (reference range, 13-115) establish the diagnosis of Zollinger-Ellison syndrome, a syndrome
of gastric acid hypersecretion caused by gastrinoma.
With initiation of proton-pump inhibitor therapy, patients experience less
frequent episodes of abdominal pain and diarrhea.
A computed tomography scan with positron emission tomography found a tumor
in the fourth part of the duodenum.
Procedures with curative intent were performed, including segmental duodenectomy and lymphadenectomy (figure C; Arrows indicate tumors).

Histopathologic analysis shows a well-differentiated neuroendocrine tumour within the lymph node, and immunohistochemical staining is positive for gastrin (figure D).

At 3 months postoperatively, fasting serum gastrin levels were 44 pg/mL, and esophagogastroduodenoscopy was normal
.
The dose of the proton-pump inhibitor was gradually reduced, and the patient's symptoms did not recur
after discontinuation of the proton-pump inhibitor.

Original source:

The Zollinger–Ellison Syndrome.
N Engl J Med 2022; 387:1699 DOI: 10.
1056/NEJMicm2203797