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All About Zollinger-Ellison Syndrome; From symptoms to treatment

Study guide

Zollinger-Ellison syndrome (ZES) is a rare disease in which the pancreas (Pancreas) Or دوازدهه (Upper part and the first part of the small intestine) One or more tumors develop. These tumors, called gastrinomas, are hormones. Gastrin They secrete a lot. Because gastrin causes the stomach to secrete too much acid, 90% of patients with Zollinger-Ellison syndrome develop gastric and duodenal ulcers. In the following, we will examine the symptoms, cause, methods of diagnosis and treatment of Zollinger-Ellison syndrome.

Zollinger-Ellison syndrome can occur at any age; But usually people in their 20s and 60s are diagnosed with the disease.

Symptoms of Zollinger-Ellison syndrome

Of course, it is not the case that Zollinger-Ellison syndrome always has symptoms; However, the following symptoms may indicate that you have the disease:

  • Weakness.
  • Diarrhea;
  • Belching;
  • Fatigue;
  • stomach ache;
  • Decreased appetite;
  • Unwanted weight loss;
  • Nausea and vomiting;
  • Gastrointestinal bleeding;
  • Gastric reflux and heartburn;
  • Burning, pain or discomfort in the upper abdomen.

Cause of Zollinger-Ellison syndrome

The exact cause of this disease is unknown; But the sequence of events in which it occurs is clear. This syndrome begins when one or more tumors (gastrinoma) form in the pancreas, duodenum, or other areas, such as the lymph nodes adjacent to the pancreas.

The pancreas is located in the back and below the stomach. This gland produces enzymes necessary for digestion. Of course, the pancreas also secretes other hormones, such as insulin. Insulin is a hormone that is involved in controlling blood sugar. The digestive juices secreted by the pancreas, liver, and gallbladder combine in the duodenum. The duodenum is part of the small intestine near the stomach. Digestion reaches its peak in the twelfth decade.

Zollinger-Ellison syndrome causes gastrointestinal ulcers.

Tumors in Zollinger-Ellison syndrome are made up of cells that secrete large amounts of the hormone gastrin. Increased gastrin secretion causes the stomach to produce too much acid. Excess acid also causes gastrointestinal ulcers and sometimes diarrhea. These tumors are usually cancerous (malignant). Although these tumors grow slowly, the cancer can spread to other parts of the body, including the liver, adjacent lymph nodes, spleen, bones, or skin.

Relationship between Zollinger Ellison syndrome and MEN 1

Zollinger-Ellison syndrome is sometimes caused by an inherited condition called “Multiple endocrine neoplasia, type 1 (MEN 1)”. Patients with MEN 1 tumors in Parathyroid glands Have and may be in the gland Pituitary Also have tumors. About 25% of people with gastrinoma have MEN 1. These patients may also have tumors in the pancreas and other organs.

If a blood relative or sibling (sibling or parent) has MEN 1, you are more likely to have Zollinger-Ellison syndrome.

Methods of diagnosis of Zollinger-Ellison syndrome

The doctor can diagnose the disease using the following methods:

1. Medical history review

Your doctor will ask about your signs and symptoms and review your medical history.

۲. blood test

If your doctor suspects Zollinger-Ellison syndrome, he or she will use a blood test to diagnose high levels of gastrin (the hormone secreted by gastrinoma). Although high levels of gastrin may be a sign of tumors in the pancreas or duodenum, other diseases can also cause an increase in gastrin levels; For example, if the stomach does not produce acid or if acid-lowering drugs such as Proton pump inhibitors Consume, gastrin levels may rise.

You should not eat anything before this test, and you may need to stop taking stomach-lowering medications to get an accurate measure of your gastrin. Of course, the level of gastrin fluctuates and this test may be repeated several times. Sometimes your doctor will also do a Secretin Stimulation Test. In this test, the doctor first measures the level of gastrin and then the hormone Secret Injects and re-measures the level of gastrin. If you have Zollinger Ellison, your gastrin levels will rise.

Your doctor may order tests to measure the amount of acid produced in your stomach.

3. Upper gastrointestinal endoscopy

Use of endoscopy to diagnose Zollinger-Ellison syndrome

The doctor inserts a thin, flexible tube into the throat after spraying the anesthetic or injecting the anesthetic. This narrow tube, which is equipped with lights and a video camera, is called an endoscope. The endoscope is inserted through the throat into the esophagus, stomach and duodenum. Using an endoscope, the doctor can remove a sample tissue (biopsy) from the duodenum to check for gastrin-producing tumors. You should not eat anything from midnight before the endoscopy.

4. Endoscopic Ultrasound

In this procedure, the doctor examines the stomach, duodenum, and pancreas with an endoscope equipped with an ultrasound probe. Using this probe, more accurate examination can be done and tumors can be detected more easily. The doctor can also remove the sample tissue with an endoscope. You should not eat anything from midnight before the test and you will be numb or drowsy during the test.

5. Imaging experiments

Your doctor may use imaging techniques, such as nuclear scans, called somatostatin receptor scintigraphy. In this test, radioactive tracers are used to find tumors.

Other imaging techniques such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) are also helpful. CT scans can also be used to diagnose Zollinger-Ellison syndrome. CT scan is a special type of X-ray that produces cross-sectional images of the body. PET scans to locate tumors and Octreotide scans to find neural tumor cells; Endocrine (Neuroendocrine) is used.

Despite all these tests, gastrinoma is difficult to diagnose.

Treatment of Zollinger-Ellison syndrome

Treatment for Zollinger-Ellison syndrome depends on whether the gastrinoma Single (Sporadic) or part of MEN I syndrome is inherited. While the latter is usually treated only by reducing acid production, acid reduction and tumor resection surgery are used to treat recurrent gastrinoma. Analogues Somatostatin Like Octreotide, which suppresses hormone production, they are also very helpful in controlling symptoms.

1. Treatment of tumors

Skilled surgery can remove tumors of Zollinger-Ellison syndrome because the tumors are usually small and difficult to find. If you have only one tumor, your doctor can remove it surgically; But if you have several tumors or the tumors have spread to the liver, surgery cannot be used. However, in some cases, despite a few tumors, your doctor may recommend removing a large tumor.

Your doctor will sometimes recommend other methods to control tumor growth, including:

  • Liver Transplant;
  • Injecting drugs into the tumor to reduce the symptoms of cancer;
  • Use of chemotherapy to slow the growth of the tumor;
  • Removal of liver tumor, as far as possible (volume reduction surgery or Debulking);
  • Tumor removal by cutting off blood flow (embolization) or using heat to kill cancer cells (Delete with radio frequency).

۲. Excess acid treatment

Excess acid production can always be controlled. Drugs called proton pump inhibitors are the first step in treatment. These drugs are effective drugs for reducing acid production in Zollinger-Ellison syndrome. Proton pump inhibitors are powerful drugs that reduce acid production and heal wounds by blocking the function of small “pumps” within acid-secreting cells. Prescription drugs usually include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), robprazole (Aciphex), dexilansoprazole (Dexilant), and esamprazole (Nexium).

According to Food and Drug AdministrationProlonged use of prescription proton pump inhibitors, especially in people over 50 years of age, increases the risk of pelvic, wrist, and spinal fractures. Of course, this risk is small and the benefits of these drugs in reducing acid are insignificant. Medicine Octreate (Sandostatin), a similar drug The hormone somatostatin, Can neutralize the effects of gastrin and is useful for some people.

If the disease is metastatic, a combination of treatments may be used, including surgery, chemotherapy, medication, or targeted radiation therapy.

going to the doctor

See your doctor if you have persistent burning or pain in your upper abdomen, especially if you have nausea, vomiting, and diarrhea. Tell your doctor if you have been taking over-the-counter anticoagulants such as omeprazole (Prilosec, Zegerid), cimetidine (Tagamet HB) or famotidine (Pepcid AC) for a long time. These drugs can mask the symptoms of the disease and delay the diagnosis.

What doctor should we go to?

Although your symptoms may prompt you to see a GP, your GP will probably refer you to a gastroenterologist for diagnosis and treatment of Zollinger-Ellison syndrome. You may also be referred to an oncologist. Is a medical oncologist who specializes in treating cancer.

What should we do?

Do the following when or before seeing your doctor:

  • Write down your questions to the doctor;
  • Make a list of all the medicines you are taking (even vitamins or supplements);
  • Make a note of all the symptoms you see, even those that seem to be irrelevant;
  • Write down important personal information such as stress or recent changes in your life. Keep notes of what you learned from your family history;
  • Tell your doctor what medications you are taking. Some acid-lowering drugs, such as proton pump inhibitors or H-2 antagonists, can alter the results of some tests to diagnose Zollinger-Ellison syndrome. However, do not stop taking these drugs without consulting your doctor.
After treatment for Zollinger-Ellison syndrome, you should also see your doctor regularly to check for the possibility of recurrence and recurrence of the disease.

Concluding remarks

Gastrinoma usually grows slowly and is not always malignant. The 5-year survival rate depends on whether the tumors are cancerous or whether they have spread. If the tumors have not spread to the liver, the 5-year survival rate can be as high as 90%. 20 to 25% of patients in whom gastrinoma is surgically removed completely recover.

Warning! This article is for educational purposes only and you should consult your doctor or specialist to use it. more information





All About Zollinger-Ellison Syndrome; From symptoms to treatment

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