What are the other Names for this Condition? (Also known as/Synonyms)

• Gastrinoma Syndrome
• Ulcerogenic Tumor of the Pancreas Syndrome
• ZES (Zollinger-Ellison Syndrome)

What is Zollinger-Ellison Syndrome? (Definition/Background Information) 

• Zollinger-Ellison Syndrome (ZES) is a rare disease of the gastrointestinal system. The syndrome is known to randomly affect individuals between the ages of 30 and 50 years; men appear to be more prone to ZES than women
• Individuals with a history of ulcers in their families bear an increased risk of getting Zollinger-Ellison Syndrome. The syndrome is caused by potentially cancerous small tumors (single or multiple) known as “gastrinomas” on the pancreas or small intestines
• These tumors produce “gastrin,” a hormone that results in an increase in stomach acid in the affected individuals. In a majority of cases, Zollinger-Ellison Syndrome occurs as a consequence of pancreatic endocrine tumors
• Symptoms of Zollinger-Ellison Syndrome include diarrhea, nausea, vomiting, abdominal pain, fatigue, etc. Complications from the disorder could include gastrointestinal bleeding, anemia due to blood loss, jaundice, and weight loss
• A physician might seek to understand the family medical history of a patient and combine that knowledge with blood tests, imaging studies (such as x-ray, endoscopy, and magnetic resonance imaging scans), biopsies, etc., to arrive at an accurate diagnosis of ZES
• Reducing the stomach acid with antacids, surgery to remove tumor(s), chemotherapy, etc., are some common treatment options for Zollinger-Ellison Syndrome.
• Gastrinomas are typically slow-growing tumors. The excess stomach acid in Zollinger-Ellison Syndrome is usually treatable by antacids
• The prognosis depends on whether the tumor is locally invasive or has metastasized. The outcome is generally better for locally invasive tumors when compared to metastatic ones
• At this time, no preventive methods are known for Zollinger-Ellison Syndrome

Who gets Zollinger-Ellison Syndrome? (Age and Sex Distribution)

• Zollinger-Ellison Syndrome randomly affects individuals between 30-50 years of age
• More men than women are reported to be affected by ZES
• All racial and ethnic groups are at risk

What are the Risk Factors for Zollinger-Ellison Syndrome? (Predisposing Factors)

The following are some known risk factors for an individual developing Zollinger-Ellison Syndrome:

• A family history of ulcers or certain type of tumors
• An inherited genetic condition called multiple endocrine neoplasia type 1 (MEN-1) syndrome: MEN-1 is characterized by gastrinomas, increasing susceptibility to Zollinger-Ellison syndrome; about 50% of individuals who inherit MEN-1 are reported to be susceptible to Zollinger-Ellison Syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Zollinger-Ellison Syndrome? (Etiology)

• Zollinger-Ellison Syndrome is caused by gastrinomas. In a majority of cases, the cause for development of gastrinomas is unknown
• A gastrinoma is a tumor that secrets excess gastrin. Gastrin is the major hormonal regulator of gastric acid secretion in the stomach. When an individual eats, gastrin is released into blood. As the acid level rises in the stomach and intestines, the body normally makes less gastrin
• This release of excess amount of acid causes severe ulcers in the duodenum (part of intestine that is attached to the stomach), stomach, and the small intestine. These are generally small and multiple
• This gastrin-secreting tumor could also occur in pancreas (tend to be single), liver, gallbladder, common bile duct, and the ovary

What are the Signs and Symptoms of Zollinger-Ellison Syndrome?

Some of the signs and symptoms associated with Zollinger-Ellison Syndrome include:

• Fatigue
• Vomiting: At times, the vomit could contain blood or could be soft lumpy brown in color
• Bloating and feeling of fullness
• Nausea
• Burning sensation in the stomach that travels up towards the throat (gastroesophageal reflex disease)
• Pain in the abdomen that develops about half an hour after a meal
• The patient usually identifies the area between the breastbone and the navel as source of pain
• Stools might be almost black red in color
• Weight loss
• Diarrhea in over half the patients; very often, diarrhea is the reason for seeking medical care

It is important to be aware that the symptoms may be very similar to those of peptic ulcer disease. Peptic ulcer disease is very common, but ZES is rare disorder.

How is Zollinger-Ellison Syndrome Diagnosed?

Tests used to diagnose Zollinger-Ellison Syndrome include: 

• A physical examination performed by a physician that includes assessing the symptoms and understanding the patient’s history and their family history
• Blood tests to check for gastrin levels: A high level of gastrin could indicate tumors in duodenum and pancreas
• Secretin stimulation test
• Upper endoscopy: It involves inserting a thin tube with a camera at its tip through the mouth into the stomach. This helps the physician to look for ulcers and also remove sample tissue to detect the presence of gastrin-producing tumors. In ZES, patients might have multiple ulcers, which can be seen during the endoscopic procedure
• The tissue taken out during the endoscopic procedure is sent to a pathologist for examination. The pathologist examines the tissue under the microscope to arrive at the diagnosis. Usually, additional special studies will be performed on the tissue by the pathologists.
• Barium x-ray, wherein the patient is made to drink a liquid that has barium suspensions. After consumption, the liquid coats the walls of the esophagus, stomach, and duodenum. An x ray along the GI tract can show the presence of abnormalities, which indicates ulcers
• Somatostatin receptor scintigraphy (SRS) also called OctreoScan: Somatostatin receptor scintigraphy is utilized to detect gastrin-producing tumors in the body. During this procedure, radioactive dye that targets the tumors cells is injected in blood. Then an instrument called gamma camera is used to locate the presence of radioactively labeled tumor cells in the body
• Physicians might recommend imaging techniques, such magnetic resonance imaging (MRI), computerized tomography (CT) scan, or an endoscopic ultrasound to localize the tumor

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Zollinger-Ellison Syndrome?

Some possible complications in an individual with Zollinger-Ellison Syndrome may include:

• Gastrointestinal bleeding
• Blood loss leading to anemia
• Severe ulcers that may lead to the development of a hole in the bowel (called perforation of bowel wall)
• Intestinal obstruction
• Jaundice
• Weight loss
• Intestinal perforation
• Chronic diarrhea 

How is Zollinger-Ellison Syndrome Treated?

• Medications to decrease or control acid production: Acid-suppressive drugs to prevent ulcers caused by excess acid on the stomach lining. For example, histamine (H-2) blockers can reduce the amount of hydrochloric acid released into the stomach.  Also, proton pump inhibitors are quite powerful and suppress acid production. Both these drugs help promote healing
• Surgery: The gastrinoma may be surgically removed to prevent the tumor spread. But sometimes, a surgery on the tumors might not be always possible
• Chemotherapy: In such cases where the tumor is not resectable (removable by surgery), use of chemotherapy to slowdown the growth rate of the tumor may be helpful. This slowing down of tumor growth may decrease the severity of symptoms
• Tumor embolization: This is a surgical procedure wherein an attempt is made to starve the tumor of blood supply by blocking the blood vessel that carries the food to the tumor. This process is called embolization. The cutting-off of blood supply to the tumor to destroy cancer cells is performed using electric current, a process also known as radiofrequency ablation

How can Zollinger-Ellison Syndrome be Prevented?

• Currently, there is no known way to specifically prevent Zollinger-Ellison Syndrome, which is a peptic ulcer disease complication.
• However, people with a predisposed genetic tendency towards peptic ulcers could go for regular checkups and if needed, under medical supervision, can take acid-suppressing drugs to prevent excessive acid production

What is the Prognosis of Zollinger-Ellison Syndrome? (Outcomes/Resolutions)

• The prognosis depends on the extent of spread of tumor in the body. Localized tumors have a better prognosis than widespread disease. In more than half the patients, the gastrin-producing tumors either grow to affect the local tissue (called locally-invasive tumors) or spread to other parts of the body (called metastatic tumor) at the time of diagnosis.
• Due to improved acid-suppressing drugs, the symptoms of acid over-production could potentially be controlled, enabling the patient to live for many years after a tumor is detected
• In about 25% of cases the gastrinomas turn out to be cancerous. One-third of such patients are reported to survive 10 years following diagnosis. In cases where the tumor is slow growing, the prognosis is very good with over 95% surviving 10 years after the diagnosis
• If the tumor has spread to the liver, the prognosis is poor, and can result in decreased life expectancy. Metastasis to the liver could result in uncontrolled tumor growth, which may eventually cause liver failure

Additional and Relevant Useful Information for Zollinger-Ellison Syndrome:

Gastrinoma of pancreas is a malignant tumor of G-cells that produce the gastrin hormone. They are considered to be functionally-active tumors, because they secrete gastrin

The following article link will help you understand gastrinoma of pancreas:

http://www.dovemed.com/diseases-conditions/gastrinoma-pancreas/