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What is Zollinger-Ellison Syndrome (ZES)?

An endoscopy might identify Zollinger-Ellison Syndrome.
Extreme stomach discomfort may be a sign of ZES.
Several abdominal organs, including the pancreas and duodenum.
Article Details
  • Written By: Tricia Ellis-Christensen
  • Edited By: O. Wallace
  • Last Modified Date: 06 October 2014
  • Copyright Protected:
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Zollinger-Ellison Syndrome (ZES) is a very rare condition that causes both tumors and ulcers to form in the stomach and intestine. Tumors may also form on the liver or pancreas. Left untreated, some tumors can metastasize and spread to other parts of the body. ZES is also associated with extreme stomach discomfort. Treatment can reduce discomfort and prevent tumors from spreading.

Researchers know very little about the causes of ZES. However, they do know that ZES causes the gut to produce far too much acid. This in turn leads to the development of ulcers, and usually more than one ulcer exists.

Symptoms of ZES include extreme pain in the upper abdomen. Some patients describe this as a burning or gnawing sensation. Diarrhea or evidence of bleeding by producing black bowel movements is common. Appetite tends to be affected by significant pain, and patients may lose weight quickly. ZES may also cause ongoing nausea or vomiting.

ZES usually starts because a tumor has formed. Tumor location is usually the pancreas or duodenum. Several tumors may be present and are occasionally malignant. Usually the tumors themselves are made up of cells that force the body to produce additional acids, sometimes called gastric juices. The acids then begin to deteriorate the lining of the stomach, creating many small ulcers.

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25% of ZES cases occur with a condition called multiple endocrine neoplasia (MEN), which also causes tumors on the pituitary glands. MEN may also result in tumors on the thyroid gland, which can cause a hyperthyroid or hypothyroid state.

Diagnosis of ZES is usually made through complete physical exam, blood tests, and endoscopy. In an endoscopy, a small tube is inserted via the mouth, into the abdomen to look for the presence of ulcers and multiple tumors. Ultrasound may also be used to pinpoint the locations of tumors or ulcers.

Often the tumors and ulcers are located in places difficult to remove, yet removal of the tumors is important to prevent cancer. Once tumors and ulcers are removed, the follow-up treatment consists of medication to block acid from forming. However, tumors frequently recur with this condition, so a return of significant pain in the upper abdomen may mean future surgery.

ZES remains difficult to treat and because it is rare, resulting in only about one in a million cases per year, it is also difficult to study. Unlike the ulcers formed by bacteria, ulcers in ZES cases cannot be treated with antibiotics. Treatment is likely to include at least yearly check-ups, so quality of life may be somewhat affected. However, newer and stronger antacid medications have significantly improved quality of life for those with ZES.

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Discuss this Article

anon167350
Post 10

My wife had a diagnostic of ZES in 1995. After wo surgeries I found in Johns Hopkins Hospital not a treatment, but a radiofarmaco called Indium 111, and we found three metastasis in two lymph nodes and in the pancreas head. Nothing happened again, after 12 years. (Marco-Brazil)

anon101533
Post 9

I was having episodes in Oct 2003 and finally diagnosed with ZES and had surgery in Oct 2004. The gastronomas were located on my pancreas and that thing below the stomach that starts with a "Du" that I will not attempt to spell. I signed up for a whipple but the surgeon was able to scoop up the tumors without performing the whipple.

In 2008 I had a recurrence of ZES. It was in my pancreas, liver, lymph nodes and sternum. Surgery was not an option. Instead I was given a drug called Sandostatin. It does not treat the symptoms of ZES like the PPIs but the cause which is the gastronomic.

I take Sandostatin once a month and that's it. No H2s and no PPIs. The gastronomas have shrunk to almost nothing. My gastrin level was 2400 before surgery to less than 100 after surgery, to something above 500 during my relapse. With the sandostatin my gastrin level is in the 60s. Not bad!

I am hoping to get over the 10 year mark and yes, my gastrinomas are cancerous.

My suggestion is to manage your own health. Go get evaluated at a second hospital such as MD Anderson, Walter Reed, Mayo Clinic, or any other hospital that performs miracles daily. --Regards, Gus.

anon71929
Post 8

I was diagnosed 12 years ago. first I was taking h2 receptor antagonist - now on proton pump inhibitor (prilosec) 60 mg a day. All has been well. I have had some issues when I forget to take the medication. other than that, just keep following up with GI. Also have had parathyroidectomy - which has proven fine - but recommend others should seek out Dr. Norman located on FLA.

anon59616
Post 7

Hi Anon: My father just had his operation and they removed the tumor and the positive lymph nodes from his Liver. what would you like to know? Donnajo the above recipient know smore then I do as she has it herself. I know it's heredity and mother and father passed on the gene. My father and I are going to go for genetic counseling. danielle

anon59428
Post 6

My sister was recently diagnosed with ZES. I have never heard of this condition before her diagnosis. I can tell you that she is really suffering. Any words of wisdom would be greatly appreciated. A.T.

dmazza
Post 4

Hi donnajo,

My mother and I are completely overwhelmed with this news. Although a Dr. last year suspected that he may have had it and dropped the ball from there.

We since got a new Dr. and were just told that we didn't even need the secretion test done because he's gastrin levels were over 1,000.

He is scheduled for a few ultra sounds with X-rays before they operate. My poor father, as you know what it is like to have it, is very very weak and has lost over 25 lbs since last year.

Were you operated on? Were there tumors? Malignant or benign? What is your treatment? Do you still live a normal healthy lifestyle? Because what and how my father looks is not healthy. Do you have the *men* 1 that goes along with it. Is this hereditary?

I am so sorry for all the questions? I am very upset and confused.

How are you being treated now?

Danielle

Donnajo
Post 3

Dmazza I would love to tell you my experience with Zes and answer any questions if I can.-Donnajo

dmazza
Post 2

Is there anyone out there that has Zollinger Ellison Syndrome? My father was just told he has it. I would love to talk to someone who has it.

Donnajo
Post 1

I was diagnosed with Zollinger Ellison Syndrome 5 years ago. I have had 2 surgeries. I was put on ultram for the pain.

My question, is there any alternative medication that I can take instead for the discomfort?

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