What is Zollinger-Ellison syndrome?
Zollinger-Ellison syndrome is a condition in which one or more tumors called gastrinomas form and oversecrete (produce too much of) a hormone called gastrin. High levels of gastrin cause the stomach to overproduce gastric acid, which can lead to peptic ulcers.
A peptic ulcer is a breakdown of the mucosal barrier in the lining of the stomach or the first part of the small intestine (the duodenum). This breakdown causes deep penetrating ulcers that can sometimes bring about pain, bleeding, nausea, dyspepsia (painful digestion), unintended weight loss and/or diarrhea.
Symptoms and Causes
What causes Zollinger-Ellison syndrome?
The majority (80 percent) of gastrinomas are sporadic (random), but 20 to 30 percent occur in association with a genetic (inherited) disorder called multiple endocrine neoplasia type 1 (MEN1). Zollinger-Ellison syndrome is very rare, and occurs most often in men aged 25 to 50.
What are the symptoms of Zollinger-Ellison syndrome?
The signs and symptoms of Zollinger-Ellison include:
- Pain in the upper abdomen
- Bloating and burping
- Gastroesophageal reflux (backup of stomach contents into the esophagus [food tube] that causes pain or a burning feeling)
- Loss of appetite and unintended weight loss
- Nausea and vomiting
- Steatorrhea (increased levels of fats within the stool)
Diagnosis and Tests
How is Zollinger-Ellison syndrome diagnosed?
If a thorough history and physical examination suggest that the patient may have Zollinger-Ellison syndrome, testing may include one or more of the following:
- Blood tests to look for abnormal levels of gastrin
- Imaging tests, such as a specialized ultrasound called EUS, CT scan, MRI imaging test, or a specialized scan called somatostatin receptor scintigraphy (sometimes called an Octreotide scan)
- Upper endoscopy: A physician uses an endoscope (a long, thin, flexible instrument with a camera) to examine the inside of the upper digestive system, including the esophagus, stomach, and first portions of the small intestines
Management and Treatment
How is Zollinger-Ellison syndrome managed or treated?
The treatment for Zollinger-Ellison syndrome is to normalize stomach acid production and heal peptic ulcerations. Treatment depends on the tumor’s size, location, grade and staging (how advanced it is), and includes:
- Medication: Drugs called proton pump inhibitors reduce the production of stomach acid and help heal ulcers.
- Surgery to remove gastrinomas
- Chemotherapy: Anticancer drugs to shrink gastrinomas and lower the levels of gastrin in the blood.
What complications are associated with Zollinger-Ellison syndrome?
Complications of Zollinger-Ellison syndrome are related to the overproduction of stomach acid and to the severity of the peptic ulcers. Severe peptic ulcers can:
- Penetrate to an artery, causing internal bleeding;
- Cause swelling, resulting in an obstruction (blockage) in the path of food leaving the stomach;
- Cause an intestinal perforation (hole in the wall of the stomach and or duodenum).
The following complications of Zollinger-Ellison syndrome can be life-threatening. Seek immediate medical help if you have these symptoms:
- Black or bloody stools
- Chest pain
- Symptomatic anemia (fatigue, lightheadedness, dizziness, low blood pressure, fast heart rate, etc.)
- Severe, sudden and/or persistent stomach pain
- Vomit that looks like coffee grounds or has blood in it
What are the risk factors for Zollinger-Ellison syndrome?
People at higher risk for Zollinger-Ellison syndrome include males ages 25–50 and people who have a parent with the disorder. Zollinger-Ellison syndrome cannot be prevented.
Outlook / Prognosis
What is the prognosis (outlook) for people who have Zollinger-Ellison syndrome?
The outlook for people with Zollinger-Ellison syndrome depends on the person. The condition can be cured if the gastrinoma is successfully removed with surgery. If surgery is not possible, in some cases Zollinger-Ellison syndrome can be managed medically. People with Zollinger-Ellison syndrome can live active lives for many years after diagnosis.
When should I call the doctor about Zollinger-Ellison syndrome?
Contact your doctor if you are having symptoms of Zollinger-Ellison syndrome. Some symptoms, such as black or bloody stools or chest pain, may be life-threatening. Seek immediate medical attention if you have these symptoms.
What questions should I ask my doctor about Zollinger-Ellison syndrome?
If you have Zollinger-Ellison syndrome, you may want to ask your doctor:
- Do I have symptoms that suggest Zollinger-Ellison syndrome?
- What are my diagnostic options?
- What are my treatment options?
- What signs of complications should I look out for?
- How will Zollinger-Ellison syndrome affect my job or lifestyle?
- Should my family consider genetic testing?
- Are there focus groups or support groups in our area for people with this syndrome?
- When can I go back to my regular activities?
- What kind of follow up/surveillance should I have?