Zollinger Ellison awareness

Overview

Zollinger-Ellison syndrome (ZES) is a rare condition where one or more tumors form in the pancreas or the upper part of the small intestine (duodenum). These tumors, called gastrinomas, release too much of a hormone called gastrin. High levels of gastrin cause the stomach to produce far more acid than normal, leading to severe and recurring peptic ulcers (sores in the lining of the stomach or duodenum) and other digestive problems.

No, Zollinger-Ellison syndrome is very rare. It affects only a small number of people worldwide, so most doctors will only see a few cases in their career.

ZES can occur at any age, but it is most often diagnosed between ages 30 and 50. It affects men and women roughly equally. If the condition is linked to MEN1, it may appear at a younger age and affect multiple family members.

Symptoms

Common symptoms

• Burning or gnawing pain in the upper abdomen (stomach area), often worse when the stomach is empty
• Chronic diarrhea, sometimes watery or greasy
• Heartburn (acid reflux) that does not get better with typical remedies
• Nausea and vomiting
• Loss of appetite and unintended weight loss
• Feeling bloated or full quickly after eating

Symptoms in children

• Symptoms in children are similar to adults but may be mistaken for other conditions. Watch for persistent abdominal pain, poor growth, frequent vomiting, and severe diarrhea.
• If the condition is linked to MEN1, children may also have other hormone-related issues like overactive parathyroid glands.

Symptoms in older adults

• Older adults with ZES may have more subtle symptoms, such as general weakness, anemia from chronic bleeding, or weight loss that is not explained.
• They are also more likely to develop complications like stomach bleeding or perforation because of other age-related health conditions.

Causes

Main causes

• The main cause is a gastrinoma — a tumor that usually starts in the pancreas or duodenum and produces excess gastrin hormone.
• In a majority of cases, the cause is sporadic (unknown). In about 20-30% of cases, ZES is part of a genetic condition called multiple endocrine neoplasia type 1 (MEN1), caused by a mutation in the MEN1 gene.

Risk factors

• Having a family history of MEN1 or Zollinger-Ellison syndrome increases the risk.
• Being diagnosed with MEN1 is the strongest known risk factor for developing a gastrinoma.

When to see a doctor

See a doctor urgently if:

• If you have severe abdominal pain, vomiting blood, or notice black or bloody stools, call emergency services immediately.
• If you are losing weight without trying, or have persistent diarrhea that is making you dehydrated, see a doctor urgently.

Book a routine appointment if:

• Make an appointment if you have ongoing heartburn, abdominal pain, or diarrhea that has not improved with lifestyle changes or over-the-counter treatments after a few weeks.
• If you have a family history of MEN1 or ZES, ask your doctor about screening even if you have no symptoms.

Diagnosis

Diagnosis starts with a thorough review of your symptoms, medical history, and family history. Your doctor will order blood tests to check for high levels of gastrin. Other tests help find the location of the tumor and rule out other conditions.

Tests that may be done

• Blood test for fasting gastrin level — a high level suggests ZES
• Gastric acid analysis — to measure how much acid your stomach is producing
• Imaging tests like CT scan, MRI, or endoscopic ultrasound to locate gastrinomas
• Endoscopy — a thin tube with a camera to look at the stomach and duodenum for ulcers
• Secretin stimulation test — to see how gastrin levels change after a special injection

What to expect at your appointment

Diagnosing ZES can take time because it is rare. You may see a gastroenterologist (a doctor specializing in digestive diseases). The tests are generally done as an outpatient. If a tumor is found, your doctor will also check for MEN1 by asking about other hormonal symptoms and possibly doing genetic testing.

Treatment

Treatment for Zollinger-Ellison syndrome aims to reduce stomach acid and control or remove the gastrinoma. Your doctor will create a plan based on the size and location of the tumor, whether it has spread, and whether you have MEN1.

Self-care at home

• Take all medications exactly as prescribed — never stop or change them without talking to your doctor.
• Avoid known triggers for acid, such as spicy foods, alcohol, and smoking.
• Keep a symptom diary to help your doctor adjust your treatment.
• Learn to recognize signs of ulcers or complications so you can seek help quickly.

Medical treatments

The main medical treatment is medicine that reduces stomach acid production. These are called proton pump inhibitors (PPIs) and are taken by mouth, usually in high doses. For most people, these medicines control symptoms and let ulcers heal. If the tumor cannot be removed or has spread, additional treatments such as injections to block the effects of gastrin or targeted therapies may be considered. Your doctor will explain the best options for your situation.

When is surgery considered?

If the gastrinoma is found in a single spot and has not spread to other organs, surgery to remove it may be possible. This can sometimes cure the condition. Surgery is often recommended for people without MEN1. If MEN1 is present, surgery is still an option but may be more complex because of other tumors.

Living with this condition

Living with ZES means managing stomach acid and watching for symptoms. Most people can lead a normal life with the right treatment. You will need regular follow-ups with your gastroenterologist, including blood tests and imaging to check the tumor status.

Lifestyle tips

• Eat smaller, more frequent meals to avoid overwhelming your stomach with acid.
• Avoid foods and drinks that trigger your symptoms, such as citrus, tomatoes, spicy dishes, alcohol, and caffeine.
• Stop smoking — it irritates the stomach and increases acid.
• Manage stress with relaxation techniques like deep breathing, meditation, or gentle exercise.

Diet and exercise

A balanced diet that is gentle on the stomach is best. Include lean proteins, cooked vegetables, whole grains, and low-acid fruits like bananas and melons. Avoid very hot or cold foods. Regular, moderate exercise like walking or swimming can help digestion and reduce stress, but listen to your body and rest if you feel unwell.

Mental health and emotional wellbeing

Living with a chronic, rare condition can be emotionally challenging. You may feel anxious about the tumor or frustrated with ongoing symptoms. It is normal to feel this way. Talking to a counselor or joining a support group for people with rare digestive diseases can help. If you ever feel overwhelmed or have thoughts of harming yourself, reach out to a crisis helpline or your doctor immediately.

Prevention

Zollinger-Ellison syndrome cannot be prevented because it is caused by a tumor that develops for genetic or unknown reasons. If you have MEN1, regular screening can help find gastrinomas early, which may prevent complications.

Screening programmes

If you have a family history of MEN1 or ZES, ask your doctor about genetic counseling and screening tests to detect tumors early, even before symptoms appear.

Complications

If left untreated

• Severe, recurring peptic ulcers that may bleed or perforate (create a hole in the stomach or intestine)
• Chronic bleeding from ulcers can lead to anemia (low red blood cells)
• Blockage in the stomach or intestine due to ulcer scarring
• Spread of the gastrinoma to other organs, such as the liver, which can be life-threatening
• Malnutrition and weight loss from chronic diarrhea and poor absorption of nutrients

Long-term outlook

With proper treatment, most people with Zollinger-Ellison syndrome can control their symptoms and prevent complications. The outlook depends on whether the tumor can be removed. If the tumor is found early and removed, the condition can sometimes be cured. If the tumor has spread, treatments are available to manage it, and many people live for years with a good quality of life. Regular follow-up care is essential.

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