What is Zollinger-Ellison Syndrome?

QUESTION: At first it was thought my husband was suffering from a stomach
ulcer. Now the doctor informs us that he has a disease with the strange name
of Zollinger-Ellison, but that the treatment is the same. What does this all
mean?
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ANSWER: The strange name comes from the discoverers of this syndrome, which
indeed closely resembles a stomach ulcer. It develops rapidly with pain that
is difficult to control, ulcer formation and diarrhea as its principle
symptoms; but the ulcers are found to be in different locations in the stomach
than are usually found with simple peptic ulcers. Even with tests performed
correctly, including x-rays and endoscopy (looking at the stomach through a
special telescope-like tube) this syndrome cannot be distinguished from
ordinary ulcer in more than 50% of the patients. Its cause is a tumor of the
pancreas or wall of the duodenum called a gastrinoma. These tumors result in
the increased secretion of gastrin into the blood which stimulates the cells
of the stomach to produce massive amounts of gastric acid. If a sensitive
blood test for gastrin shows that there are increased amounts, Z-E syndrome
can be diagnosed. The tumors, however, are hard to find. Arteriography,
which may show circulation to the tumor, is the best test but discovers them
in less than one case in two. Sometimes ultrasound can be used, but it too
detects tumors in only 20-30% of the patients. The good news is that the same
medications that block acid formation in peptic ulcer (called H2 blockers)
work very well for Z-E. However since a surgical cure is possible, an
exploratory operation should be considered to locate and remove the tumor.