Zollinger Ellison Syndrome

Zollinger-Ellison syndrome is a rare condition where tumors, called gastrinomas, grow in the pancreas or small intestine. These tumors release a lot of a hormone called gastrin. Gastrin's job is to tell the stomach to make more acid. This extra stomach acid can create sores called peptic ulcers. The excess gastrin also often causes problems like diarrhea, stomach pain, and other uncomfortable symptoms.

This condition can develop at any age, but most people are diagnosed between the ages of 20 and 50.

Treatment usually involves taking medications to reduce stomach acid and help heal the ulcers. In some cases, surgery to remove the tumors is also needed.

Zollinger-Ellison syndrome can cause a variety of digestive problems. Common symptoms include:

• Stomach pain: This can range from a dull ache to sharp pain.
• Diarrhea: Frequent bowel movements can be a symptom.
• Upper abdominal discomfort: A burning, aching, or simply uncomfortable feeling in your upper stomach area. Think of the area just below your ribs.
• Acid reflux and heartburn: This is when stomach acid flows back up into your esophagus, causing a burning sensation in your chest.
• Burping: Frequent belching or burping can be a sign.
• Nausea and vomiting: Feeling sick to your stomach and throwing up.
• Digestive bleeding: This can sometimes cause blood in your stool or vomit. It's important to note that this can be a serious symptom, and you should seek medical attention immediately if you see blood.
• Unintentional weight loss: Losing weight without trying can be a sign of a problem.
• Loss of appetite: Not feeling hungry as much as usual.

If you experience a persistent burning or aching pain in your upper stomach, especially if it's accompanied by nausea, vomiting, and diarrhea, it's crucial to see a doctor. This is especially important if you've been using over-the-counter medications to reduce stomach acid, such as omeprazole (Prilosec, Zegerid), cimetidine (Tagamet HB), or famotidine (Pepcid AC). These medications can mask the symptoms of Zollinger-Ellison syndrome, potentially delaying diagnosis and proper treatment. It's important to be open and honest with your healthcare provider about all medications you are taking, both prescription and over-the-counter.

If you have a persistent, sharp pain in your upper stomach that won't go away, see a doctor right away. This is especially important if you're also experiencing nausea, vomiting, or diarrhea.

Let your doctor know if you've been taking any over-the-counter medications to reduce stomach acid. These include medicines like omeprazole (sometimes called Prilosec or Zegerid), cimetidine (Tagamet HB), or famotidine (Pepcid AC). These medicines can hide the symptoms of a problem, which might make it harder for your doctor to find out what's wrong. So, telling them about these medications is crucial for getting an accurate diagnosis.

Zollinger-Ellison syndrome is a condition where tumors form in the pancreas, the duodenum (part of the small intestine connected to the stomach), or sometimes nearby lymph nodes. These tumors are made of cells that produce excessive amounts of a hormone called gastrin. This is why they're also known as gastrinomas.

The pancreas, located behind the stomach, plays a vital role in digestion. It makes enzymes to break down food and hormones like insulin, which helps control blood sugar. The duodenum is where digestive juices from the pancreas, liver, and gallbladder combine to complete most of the digestive process.

In Zollinger-Ellison syndrome, the extra gastrin causes the stomach to produce far too much acid. This excess acid is the primary cause of peptic ulcers, and can also lead to diarrhea. Importantly, these gastrinomas are often cancerous, although they typically grow slowly. If cancerous, the cancer can spread to nearby lymph nodes or the liver.

The exact cause of Zollinger-Ellison syndrome is unknown. However, it's sometimes linked to a genetic condition called multiple endocrine neoplasia type 1 (MEN 1). People with MEN 1 tend to develop tumors in more than one gland in their body, including the parathyroid glands and, sometimes, the pituitary gland. About one-quarter of people with gastrinomas have them because of MEN 1. These patients may also have tumors in other parts of their bodies.

If you have a close family member, like a brother, sister, or parent, who has Multiple Endocrine Neoplasia type 1 (MEN1), you're at a higher risk of developing Zollinger-Ellison syndrome. This means you have a greater chance of getting this condition. MEN1 is a genetic disorder that causes tumors to grow in different parts of the endocrine system. Zollinger-Ellison syndrome is a specific type of tumor that affects the stomach and can lead to ulcers. So, having a relative with MEN1 increases your chances of getting this stomach-related problem.

Diagnosing Zollinger-Ellison Syndrome: A Step-by-Step Guide

Zollinger-Ellison syndrome (ZES) is a condition where tumors in the pancreas or duodenum produce too much gastrin, a hormone that controls stomach acid production. This excess gastrin can lead to ulcers and other digestive problems. Doctors use a combination of tests to diagnose ZES, and here's how it typically works:

1. Medical History and Blood Tests:

The process begins with a conversation about your symptoms and medical history. This helps the doctor understand your overall health and possible causes of your issues. A blood sample is then analyzed. One key test looks for high levels of gastrin. While high gastrin can suggest ZES, it's not always the case. Other conditions, like a lack of stomach acid production or past gastric surgery, can also cause high gastrin levels. Taking medications to reduce stomach acid can also elevate gastrin. For the blood test, you'll need to fast beforehand, and possibly stop taking acid-reducing medications for a period of time. Sometimes, the blood test is repeated to get a more accurate reading because gastrin levels can fluctuate.

2. Secretin Stimulation Test:

A secretin stimulation test further investigates the gastrin issue. Secretin is a hormone that regulates stomach acid. First, your gastrin levels are measured. Then, you'll receive a secretin injection. Your gastrin levels are measured again. If you have ZES, your gastrin levels will increase significantly in response to the secretin.

3. Upper Gastrointestinal Endoscopy:

This test involves a thin, flexible tube (endoscope) with a camera and light at the end. The endoscope is passed down your throat and into your stomach and small intestine (duodenum). This lets the doctor look for ulcers and other abnormalities. A small tissue sample (biopsy) might be taken for further examination. The biopsy helps determine if gastrin-producing tumors are present. The test also helps the doctor assess if your stomach is producing adequate acid. If your stomach is producing acid and your gastrin levels are high, the diagnosis of ZES becomes more likely. You will need to fast after midnight the night before this procedure.

4. Endoscopic Ultrasound:

A specialized endoscope fitted with an ultrasound probe is used. This combination makes it easier to pinpoint tumors in the stomach, small intestine, or pancreas. The doctor might also take a tissue sample during this procedure. Fasting is also required.

5. Imaging Tests:

Imaging techniques, like CT scans, MRI scans, and a special type of scan called Ga-DOTATATE PET-CT, can help pinpoint the location and size of any tumors. These tests create detailed images of the digestive system.

Important Note: These tests are often used in combination. The specific tests and their order might vary depending on your individual case. Your doctor will determine the best approach to diagnose ZES accurately.

Zollinger-Ellison Syndrome Treatment

Zollinger-Ellison syndrome (ZES) involves tumors that produce too much gastrin, a hormone that leads to excess stomach acid. This excess acid causes ulcers and other problems. Treatment focuses on both removing or controlling the tumors and managing the acid buildup.

Treating the Tumors:

Removing the tumors (called gastrinomas) is often necessary. Finding these tumors can be challenging because they are frequently small and hidden. A skilled surgeon is crucial for this procedure.

• Surgery: If you have just one tumor, surgery to remove it is often the first choice. However, if you have many tumors or if the tumors have spread to your liver, surgery might not be an option. Even with multiple tumors, the surgeon might still recommend removing a large one if possible.
• Other Treatments for Tumors: If surgery isn't an option or isn't completely effective, doctors might use other methods to manage the tumors:
  • Debulking: If tumors are in the liver, a procedure called debulking may be used to remove as much of the tumor as possible.
  • Embolization: This involves cutting off the blood supply to the tumor to try and shrink or destroy it.
  • Radiofrequency Ablation: This uses heat to destroy the cancer cells.
  • Injections: Drugs can be injected into the tumor to relieve symptoms.
  • Chemotherapy: This is used to try to slow the growth of the tumors.
  • Liver Transplant: In some very advanced cases, a liver transplant may be considered.

Managing Excess Stomach Acid:

Controlling the excessive stomach acid is essential to manage the symptoms.

• Proton Pump Inhibitors (PPIs): These are the most common and often the first-line treatment for controlling acid production. PPIs work by blocking the "pumps" in the stomach cells that produce acid. They are very effective in reducing acid. Common examples include lansoprazole (Prevacid), omeprazole (Prilosec), pantoprazole (Protonix), rabeprazole (Aciphex), and esomeprazole (Nexium). It's important to talk to your doctor about the potential long-term risks of taking PPIs.
• Octreotide: This medicine is similar to a natural hormone and can sometimes help reduce the effects of gastrin, which in turn helps control the acid production.

Important Considerations:

The best treatment plan will depend on the specific situation, including the size, number, and location of the tumors. It's crucial to discuss all options and potential side effects with your healthcare provider to create a personalized treatment strategy.

Further Information:

If you need more information on specific treatments like chemotherapy, liver transplant, or radiofrequency ablation, please ask your doctor.