Zollinger-Ellison Syndrome
What is Zollinger-Ellison Syndrome?
Zollinger-Ellison Syndrome is a rare condition caused by tumors that produce too much gastrin. Gastrin is a hormone that tells your stomach to make acid. When your body makes too much gastrin, your stomach produces dangerous amounts of acid.
These tumors are called gastrinomas. They usually form in the pancreas or the first part of the small intestine. Most gastrinomas are small, but about half are cancerous and can spread. The excess acid damages your stomach and intestinal lining, creating painful ulcers.
This condition affects about 1 to 3 people per million each year. Most people are diagnosed between ages 20 and 50. About 25% of cases happen as part of a genetic condition called Multiple Endocrine Neoplasia type 1.
Symptoms
- Severe abdominal pain that feels like burning in the upper belly
- Diarrhea that may be watery and persistent
- Nausea and vomiting
- Blood in stool or black, tarry stools
- Unexplained weight loss
- Loss of appetite
- Heartburn that does not respond to usual treatments
- Stomach ulcers that keep coming back
- Bleeding from ulcers in the digestive tract
Some people experience only mild heartburn or indigestion early on. Others develop severe ulcers before getting diagnosed. Symptoms can come and go, making diagnosis challenging.
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Causes and risk factors
Zollinger-Ellison Syndrome is caused by tumors called gastrinomas that produce excess gastrin. These tumors develop in the pancreas about 70% of the time. The remaining 30% form in the duodenum, the first part of the small intestine. Doctors do not know exactly why gastrinomas form in most people.
About 1 in 4 cases occur as part of Multiple Endocrine Neoplasia type 1, a genetic disorder. This inherited condition causes tumors in multiple hormone-producing glands. People with a family history of this genetic syndrome have higher risk. Other risk factors remain unclear because the condition is so rare.
How it's diagnosed
Doctors diagnose Zollinger-Ellison Syndrome using blood tests and imaging. A fasting gastrin test measures the amount of gastrin in your blood. Levels above 1,000 picograms per milliliter strongly suggest this condition. Your doctor may also perform a secretin stimulation test, where they inject secretin and measure how your gastrin levels respond.
Additional tests help locate the tumors. These include CT scans, MRI, endoscopic ultrasound, and specialized nuclear medicine scans. An upper endoscopy lets doctors see ulcers in your stomach and small intestine. Talk to a doctor about specialized testing if you have persistent ulcers or severe acid symptoms that do not improve with treatment.
Treatment options
- Proton pump inhibitors like omeprazole or lansoprazole to reduce stomach acid production
- Higher doses of acid reducers than typically used for regular ulcers
- Surgery to remove tumors when possible
- Medications to slow tumor growth if cancer has spread
- Regular monitoring with blood tests and imaging
- Treatment for Multiple Endocrine Neoplasia type 1 if present
- Nutritional support to address weight loss and diarrhea
- Small, frequent meals to reduce acid stimulation
Frequently asked questions
The main symptom is severe, recurring stomach ulcers that do not respond well to treatment. Most people experience intense burning pain in the upper abdomen. Many also have persistent diarrhea and heartburn that does not improve with standard medications.
This condition affects only 1 to 3 people per million each year. It accounts for less than 1% of all peptic ulcer cases. Most people are diagnosed between ages 20 and 50, though it can occur at any age.
A fasting gastrin blood test is the primary screening tool. Gastrin levels above 1,000 picograms per milliliter strongly suggest this condition. Your doctor may also order a secretin stimulation test, which shows how gastrin levels respond to an injected hormone.
About 50% of gastrinomas are cancerous and can spread to other organs. The other half are benign but still cause serious problems by producing excess gastrin. Even benign tumors require treatment to control acid production and prevent complications.
Surgery to remove the tumor can cure the condition if the gastrinoma is small and has not spread. However, tumors are often hard to locate or have already spread when diagnosed. In these cases, medications can control acid production and manage symptoms effectively for many years.
About 25% of cases occur as part of Multiple Endocrine Neoplasia type 1, an inherited genetic disorder. People with this condition develop tumors in multiple hormone-producing glands. The remaining 75% of cases occur randomly without a known genetic cause.
Untreated cases can lead to severe complications including bleeding ulcers, intestinal perforation, and scarring. The excess acid can damage your esophagus, stomach, and small intestine. If the tumor is cancerous, it may spread to the liver or other organs.
Regular ulcers are usually caused by infection or medication use and respond to standard treatment. Zollinger-Ellison ulcers are caused by tumors producing too much gastrin. These ulcers tend to be more severe, occur in unusual locations, and keep coming back despite treatment.
Proton pump inhibitors are the main treatment and reduce stomach acid production. Common ones include omeprazole, lansoprazole, and pantoprazole. Patients with this condition need much higher doses than people with regular ulcers, often taken multiple times per day.
Yes, regular monitoring is essential even with treatment. Your doctor will check gastrin levels and perform imaging tests to watch for tumor growth or spread. Most people need blood tests every few months and imaging annually. This helps catch any changes early and adjust treatment as needed.