Zollinger-Ellison syndrome
Definition:
Zollinger-Ellison syndrome is a complex condition in which one or more
tumors form in your pancreas or the upper part of your small intestine
(duodenum). These tumors, called gastrinomas, secrete large amounts of
the hormone gastrin, which causes your stomach to produce too much acid.
The excess acid, in turn, leads to peptic ulcers.
Zollinger-Ellison syndrome (ZES) is rare. The disease may occur at any time in life, but people are usually diagnosed between ages 30 and 50. Medications to reduce stomach acid and heal the ulcers is the usual treatment for Zollinger-Ellison syndrome.
Symptoms:
Signs and symptoms of Zollinger-Ellison syndrome may include:
See your doctor if you have a persistent, burning, aching or gnawing pain in your upper abdomen, especially if you've also been experiencing nausea, vomiting and diarrhea.
Tell your doctor if you've used over-the-counter acid-reducing medications such as omeprazole (Prilosec), cimetidine (Tagamet), famotidine (Pepcid) or ranitidine (Zantac) for long periods of time. These medications may mask your symptoms, which could delay your diagnosis. If you have Zollinger-Ellison syndrome, early detection and treatment are important.
Causes:
The exact cause of Zollinger-Ellison syndrome remains unknown. But the sequence of events that occurs in Zollinger-Ellison syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas, duodenum or the lymph nodes adjacent to your pancreas.
Your pancreas sits behind and below your stomach.
It produces enzymes that are essential to digesting food. The pancreas also produces several hormones, including gastrin, a hormone that controls stomach acid production. Digestive juices from the pancreas, liver and gallbladder mix in the duodenum, the part of the small intestine next to your stomach. This is where digestion reaches its peak.
The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of gastrin, which in turn causes the stomach to produce far too much acid. The excessive acid then leads to peptic ulcers and sometimes to diarrhea.
Besides causing excess acid production, the tumors may be cancerous (malignant). The tumors themselves grow slowly, but the cancer can spread elsewhere — most commonly to nearby lymph nodes or your liver.
Association with MEN I
Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type I (MEN I). People with MEN I have multiple tumors in the endocrine system in addition to pancreatic tumors. They also have tumors in the parathyroid glands and may have tumors in their pituitary glands. About 25 percent of people who have gastrinomas have them as part of MEN I.
Treatments and drugs:
In treating Zollinger-Ellison syndrome, doctors treat the tumors as well as the ulcers. If your doctor can remove the tumors, then ulcer treatment may no longer be needed.
Treatment of tumors
An operation to remove the tumors that occur in Zollinger-Ellison requires a skilled surgeon because the tumors are often small and difficult to locate. If you have just one tumor, your doctor may be able to remove it surgically, but surgery may not be an option if you have multiple tumors or tumors that have spread to your liver. On the other hand, even if you have multiple tumors, your doctor still may recommend removing a single large tumor.
In some cases, doctors advise other treatments to control tumor growth, including:
Treatment of excess acid
Excess acid production can almost always be controlled. Medications known as proton pump inhibitors are the first line of treatment. These are the most effective medications for decreasing acid production in Zollinger-Ellison syndrome. Proton pump inhibitors are powerful drugs that reduce acid by blocking the action of the tiny "pumps" within acid-secreting cells. Commonly prescribed medications include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), rabeprazole (Aciphex) and esomeprazole (Nexium). Long-term use of prescription proton pump inhibitors, especially in people age 50 and older, has been associated with an increased risk of fractures of the hip, wrist and spine, according to the Food and Drug Administration. This risk is small and should be weighed against the acid-blocking benefits of these medications.
Your doctor may also suggest one of several operations to treat peptic ulcers, such as surgery to:
Definition:
Zollinger-Ellison syndrome is a complex condition in which one or more
tumors form in your pancreas or the upper part of your small intestine
(duodenum). These tumors, called gastrinomas, secrete large amounts of
the hormone gastrin, which causes your stomach to produce too much acid.
The excess acid, in turn, leads to peptic ulcers.
Zollinger-Ellison syndrome (ZES) is rare. The disease may occur at any time in life, but people are usually diagnosed between ages 30 and 50. Medications to reduce stomach acid and heal the ulcers is the usual treatment for Zollinger-Ellison syndrome.
Symptoms:
Signs and symptoms of Zollinger-Ellison syndrome may include:
- Abdominal pain
- Diarrhea
- Burning, aching, gnawing or discomfort in your upper abdomen
- Acid reflux and heartburn
- Nausea and vomiting
- Weakness
- Bleeding in your digestive tract
- Unintended weight loss
- Decreased appetite
- Anemia
See your doctor if you have a persistent, burning, aching or gnawing pain in your upper abdomen, especially if you've also been experiencing nausea, vomiting and diarrhea.
Tell your doctor if you've used over-the-counter acid-reducing medications such as omeprazole (Prilosec), cimetidine (Tagamet), famotidine (Pepcid) or ranitidine (Zantac) for long periods of time. These medications may mask your symptoms, which could delay your diagnosis. If you have Zollinger-Ellison syndrome, early detection and treatment are important.
Causes:
The exact cause of Zollinger-Ellison syndrome remains unknown. But the sequence of events that occurs in Zollinger-Ellison syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas, duodenum or the lymph nodes adjacent to your pancreas.
Your pancreas sits behind and below your stomach.
It produces enzymes that are essential to digesting food. The pancreas also produces several hormones, including gastrin, a hormone that controls stomach acid production. Digestive juices from the pancreas, liver and gallbladder mix in the duodenum, the part of the small intestine next to your stomach. This is where digestion reaches its peak.
The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of gastrin, which in turn causes the stomach to produce far too much acid. The excessive acid then leads to peptic ulcers and sometimes to diarrhea.
Besides causing excess acid production, the tumors may be cancerous (malignant). The tumors themselves grow slowly, but the cancer can spread elsewhere — most commonly to nearby lymph nodes or your liver.
Association with MEN I
Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type I (MEN I). People with MEN I have multiple tumors in the endocrine system in addition to pancreatic tumors. They also have tumors in the parathyroid glands and may have tumors in their pituitary glands. About 25 percent of people who have gastrinomas have them as part of MEN I.
Treatments and drugs:
In treating Zollinger-Ellison syndrome, doctors treat the tumors as well as the ulcers. If your doctor can remove the tumors, then ulcer treatment may no longer be needed.
Treatment of tumors
An operation to remove the tumors that occur in Zollinger-Ellison requires a skilled surgeon because the tumors are often small and difficult to locate. If you have just one tumor, your doctor may be able to remove it surgically, but surgery may not be an option if you have multiple tumors or tumors that have spread to your liver. On the other hand, even if you have multiple tumors, your doctor still may recommend removing a single large tumor.
In some cases, doctors advise other treatments to control tumor growth, including:
- Removing as much of a liver tumor as possible (debulking)
- Attempting to destroy the tumor by cutting off the blood supply (embolization) or by using heat to destroy cancer cells (radiofrequency ablation)
- Injecting drugs into the tumor to relieve cancer symptoms
- Using chemotherapy to try to slow tumor growth
- A liver transplant
Treatment of excess acid
Excess acid production can almost always be controlled. Medications known as proton pump inhibitors are the first line of treatment. These are the most effective medications for decreasing acid production in Zollinger-Ellison syndrome. Proton pump inhibitors are powerful drugs that reduce acid by blocking the action of the tiny "pumps" within acid-secreting cells. Commonly prescribed medications include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), rabeprazole (Aciphex) and esomeprazole (Nexium). Long-term use of prescription proton pump inhibitors, especially in people age 50 and older, has been associated with an increased risk of fractures of the hip, wrist and spine, according to the Food and Drug Administration. This risk is small and should be weighed against the acid-blocking benefits of these medications.
Your doctor may also suggest one of several operations to treat peptic ulcers, such as surgery to:
- Stop an ulcer from bleeding
- Relieve an obstruction caused by an ulcer
- Close up the hole (perforation) that an ulcer has made in the wall of your stomach or duodenum
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