Causes
These tumors, called gastrinomas, secrete large amounts of the hormone gastrin, which causes your stomach to produce too much acid. The excess acid then leads to peptic ulcers, as well as to diarrhea and other symptoms. Zollinger-Ellison syndrome (ZES) is rare.
Symptoms
Zollinger-Ellison syndrome. Overview. Zollinger-Ellison syndrome is a rare condition in which one or more tumors form in your pancreas or the upper part of your small intestine (duodenum).
Complications
The gastrinomascaused by ZES secrete the hormone gastrin. Because gastrin creates excessive stomachacid, 90 percent of patients with ZES develop stomach and duodenal ulcers. What Are the Complications of Zollinger-Ellison Syndrome? A person who has Zollinger-Ellison syndrome may have only one gastrinoma or may have several.
What causes stomach ulcers with Zollinger-Ellison syndrome?
There is not a genetic test specifically for Zollinger-Ellison syndrome (ZES), which usually occurs sporadically as a result of a tumor that secretes gastrin (a gastrinoma). However, genetic testing is available for multiple endocrine neoplasia type 1 (MEN1), which is a genetic condition present in about 25-30% of people with ZES.
What is Zollinger-Ellison syndrome?
What are the complications of Zollinger-Ellison syndrome (ZES)?
Is there a genetic test for Zollinger-Ellison syndrome (ZES)?
How do you know if you have Zollinger-Ellison syndrome?
How is Zollinger-Ellison syndrome diagnosed? If you have the symptoms associated with ulcers or ZES, your healthcare provider may measure your stomach acid levels. He or she may also give you a blood test to measure your level of the hormone gastrin, to see whether your body makes too much.
Can Zollinger-Ellison syndrome be cured?
The outlook for people with Zollinger-Ellison syndrome depends on the person. The condition can be cured if the gastrinoma is successfully removed with surgery. If surgery is not possible, in some cases Zollinger-Ellison syndrome can be managed medically.
How long can you live with Zollinger-Ellison syndrome?
The five-year survival rate depends on whether tumors are cancerous and if they've spread. If they have not spread to the liver, the 5-year survival rate may be 90%.
What causes Gastrinoma?
What causes gastrinomas? Gastrinomas arise due to uncontrolled division and replication of cells in the gut called G cells, which produce the hormone gastrin. Under normal conditions gastrin can increase stomach acid and help with the digestion of food.
When should you suspect Zollinger-Ellison syndrome?
A medical professional may suspect Zollinger-Ellison syndrome if blood drawn after the infusion shows an elevated gastrin level. Once a diagnosis is made, localizing the tumor is important. Most Gastrinoma are small lesions and therefore, localizing the tumor may be difficult.
Can endoscopy detect Zollinger-Ellison syndrome?
Upper GI endoscopy may show signs of Zollinger-Ellison syndrome or its complications. Doctors may also order this test to look for the tumors that cause Zollinger-Ellison syndrome.
How do you get rid of Zollinger-Ellison syndrome?
TreatmentRemoving 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.More items...•
What causes too much acid in the stomach?
There are several causes of high stomach acid. Examples include H. pylori infection, Zollinger-Ellison syndrome, and rebound effects from medication withdrawal. If left untreated, high stomach acid can lead to complications like ulcers or GERD.
Is Zollinger-Ellison syndrome hereditary?
However, in approximately 25 percent of affected individuals, ZES occurs in association with the genetic syndrome known as multiple endocrine neoplasia type 1 (MEN-1). In most patients, MEN-1 is inherited as an autosomal dominant genetic condition.
Are all gastrinomas cancerous?
Gastrinomas can be either benign or malignant. More than 60 percent of gastrinomas are cancerous, according to the Center for Pancreatic and Biliary Diseases.
How long can you live with gastrinoma?
5 or 10 years is a common time point to measure survival. But some people live much longer than this. 5 or 10 year survival is the number of people who have not died from their cancer within 5 or 10 years after diagnosis.
Can gastrinomas be cured?
Surgery is the main treatment for gastrinoma and it's usually the only treatment that can cure it. But surgery isn't always possible. Some gastrinomas may have already started to spread when they are diagnosed. You might have treatment to control your symptoms if you can't have surgery to try to cure your gastrinoma.
What is the best treatment for Zollinger-Ellison syndrome?
Medications known as proton pump inhibitors are the first line of treatment. These are 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.
Are gastrinomas cancerous?
Gastrinomas occur as single tumors or several tumors. One half to two thirds of single gastrinomas are cancerous (malignant) tumors. These tumors often spread to the liver and nearby lymph nodes. Many people with gastrinomas have several tumors as part of a condition called multiple endocrine neoplasia type I (MEN I).
Why is my stomach producing too much acid?
There are several causes of high stomach acid. Examples include H. pylori infection, Zollinger-Ellison syndrome, and rebound effects from medication withdrawal. If left untreated, high stomach acid can lead to complications like ulcers or GERD.
What are the symptoms of high stomach acid?
Signs and Symptoms of Excess Stomach AcidHeartburn.A sour taste in your mouth.Bad breath.Recurrent cough or hiccups.Hoarse voice.Bloating.Nausea.Diarrhea.
Where does Zollinger-Ellison syndrome form?
The extra acid causes peptic ulcers to form in the duodenum and elsewhere in the upper intestine. The tumors seen with Zollinger-Ellison syndrome are sometimes cancerous and may spread to other areas of the body.
How common is Zollinger-Ellison syndrome?
Zollinger-Ellison syndrome is rare. Only 0.5 to 3 out of every 1 million people are diagnosed with Zollinger-Ellison syndrome each year. 1
What tests do doctors use to diagnose Zollinger-Ellison syndrome?
Doctors may order the following tests to diagnose Zollinger-Ellison syndrome and find the tumors that cause this condition.
How do doctors prevent and treat the complications of Zollinger-Ellison syndrome?
Treating Zollinger-Ellison syndrome reduces the amount of acid in the stomach, which can help prevent or treat complications related to peptic ulcers or acid reflux.
What causes gastrinomas in Zollinger-Ellison syndrome?
In most cases of Zollinger-Ellison syndrome, experts don’t know what causes the gastrinomas to form. In about 20% to 25% of cases, a rare genetic disorder called MEN1 causes gastrinomas to form. 3
How to check for elevated gastrin levels?
The health care provider may use blood tests to check for an elevated gastrin level. A technician or nurse draws a blood sample during an office visit or at a commercial facility and sends the sample to a lab for analysis. A health care provider will ask the person to fast for several hours prior to the test and may ask the person to stop acid-reducing medications for a period of time before the test. A gastrin level that is 10 times higher than normal suggests Zollinger-Ellison syndrome. 2
Can Zollinger-Ellison syndrome cause peptic ulcers?
The extra stomach acid caused by Zollinger-Ellison syndrome can lead to peptic ulcer disease or GERD. Without treatment, these diseases can lead to complications such as
What is Zollinger-Ellison syndrome?
Zollinger-Ellison syndrome (ZES) is a rare digestive disorder. If you have ZES, you likely have one or more tumors in the first part of the small intestine, the pancreas, or both. These tumors, called gastrinomas, release the hormone gastrin. This causes the stomach to release too much acid. Stomach acid is needed to break down food. But, too much acid can cause painful peptic ulcers inside the lining of your stomach and intestine. While gastrinoma tumors do cause health problems, they are typically not cancerous tumors.
How is Zollinger-Ellison syndrome diagnosed?
If you have the symptoms associated with ulcers or ZES, your health care provider may measure your stomach acid levels. He or she may also give you a blood test to measure your level of the hormone gastrin, to see whether your body makes too much. Your healthcare provider may order imaging tests to look for tumors.
What causes a zes in the stomach?
ZES is caused by tumors, called gastrinomas. The tumors cause the release of too much stomach acid. The extra acid can cause painful peptic ulcers inside the lining of your stomach and intestines.
What are the symptoms of ZES?
They include: Nausea. Vomiting. Weight loss. Diarrhea. Abdominal pain, sometimes burning in nature. Severe heartburn (GERD or gastroesophageal reflux disease) Intestinal bleeding (such as black or tarry stool, or blood in the stool)
What is ZES syndrome?
In about 25-30% of people with ZES, it is associated with an inherited condition called multiple endocrine neoplasia type 1 (MEN1). [3]
What causes high levels of gastrin in the stomach?
Zollinger-Ellison syndrome (ZES) is a condition in which tumors called gastrinomas in the pancreas and duodenum (part of the small intestine) cause high levels of the hormone gastrin in the blood. High levels of gastrin then cause production of too much stomach acid. Signs and symptoms may include abdominal pain, peptic ulcers, vomiting blood, and diarrhea. [1] [2] The tumors are sometimes cancerous and may spread to other areas of the body. [3] In most cases, the cause of ZES is unknown. However, about 25-30% of gastrinomas are caused by an inherited condition called multiple endocrine neoplasia type 1 (MEN1). Treatment for ZES may include medication to reduce the production of stomach acid, and surgery for peptic ulcers or to remove tumors. [4]
How is MEN1 inherited?
MEN1 is inherited in an autosomal dominant manner and is caused by mutations in the MEN1 gene. Most affected people inherit the mutated gene from a parent, and a few cases result from a new mutation that was not inherited. Unlike most autosomal dominant conditions, both copies of the MEN1 gene must have a mutation to cause symptoms of the condition. The mutation in the second copy of the gene occurs during a person's lifetime. Because of this, most, but not all, people born with an MEN1 gene mutation will develop signs and symptoms of the condition. [6] This phenomenon is known as reduced penetrance. A person with an MEN1 gene mutation has a 50% (1 in 2) risk to pass on the mutated gene to each child.
Is there a genetic test for Zollinger-Ellison syndrome?
There is not a genetic test specifically for Zollinger-Elli son syndrome (ZES), which usually occurs sporadically as a result of a tumor that secretes gastrin (a gastrinoma). However, genetic testing is available for multiple endocrine neoplasia type 1 (MEN1), which is a genetic condition present in about 25-30% of people with ZES.
Who is at higher risk for Zollinger-Ellison syndrome?
People at higher risk for Zollinger-Ellison syndrome include males ages 25–50 and people who have a parent with the disorder. Zollinger-Ellison syndrome cannot be prevented.
What tests are done to determine if you have Zollinger-Ellison syndrome?
If a thorough history and physical examination suggest that the patient may have Zollinger-Ellison syndrome, testing may include one or more of the following: Blood tests to look for abnormal levels of gastrin.
What is a peptic ulcer?
A peptic ulcer is a breakdown of the mucosal barrier in the lining of the stomach or the first part of the small intestine ( the duodenum ). This breakdown causes deep penetrating ulcers that can sometimes bring about pain, bleeding, nausea, dyspepsia (painful digestion), unintended weight loss and/or diarrhea.
What is the name of the condition where one or more tumors form and produce too much of a hormone called gas?
Zollinger-Ellison Syndrome. Zollinger-Ellison syndrome is a condition in which one or more tumors called gastrinomas form and produce too much of a hormone called gastrin, which can lead to peptic ulcers. Symptoms include pain in the upper abdomen and nausea. Treatments include medication or surgery to remove the gastrinomas.
How do you know if you have Zollinger-Ellison?
The signs and symptoms of Zollinger-Ellison include: Pain in the upper abdomen. Bloating and burping. Diarrhea. Gastroesophageal reflux (backup of stomach contents into the esophagus [food tube] that causes pain or a burning feeling) Loss of appetite and unintended weight loss. Nausea and vomiting.
What is the term for increased fats in stool?
Steatorrhea (increased levels of fats within the stool).
Is Zollinger-Ellison syndrome life threatening?
The following complications of Zollinger-Ellison syndrome can be life-threatening. Seek immediate medical help if you have these symptoms:
What causes a ZES?
Experts aren’t exactly sure what causes ZES. In most cases, the disorder appears for no obvious reason. In 25 percent of cases, it’s association with an inherited, tumor-causing condition called multiple endocrine neoplasia type 1 (MEN1). More research is needed to better understand this condition.
Is ZES a serious disease?
ZES is a rare but serious disease. Most ZES tumors grow slowly, and medications are very effective in reducing gastric acid and ulcer pain. See your healthcare provider if you have digestive issues like diarrhea, heartburn, or abdominal pain lasting more than a few days.
What Are the Complications of Zollinger-Ellison Syndrome?
A person who has Zollinger-Ellison syndrome may have only one gastrinoma or may have several. Approximately 25% to 30% of ZES patients also have a genetic (inherited) disorder known as "multiple endocrine neoplasia type 1," which also causes tumors in the pituitary and parathyroid glands .
How Is Zollinger-Ellison Syndrome Treated?
ZES is treated by reducing the amount of acid your stomach produces. Medications called proton pump inhibitors are usually prescribed. These drugs, which include dexlansoprazole ( Dexilant ), esomeprazole ( Nexium ), lansoprazole ( Prevacid ), omeprazole ( Prilosec, Zegerid ), pantoprazole ( Protonix ), and rabeprazole ( Aciphex ), curb the production of stomach acid and allow the ulcers to heal.
How to treat a ZES?
While the latter is usually treated with acid suppression alone, sporadic gastrinomas are treated with acid suppression and surgical removal of the tumor. Somatostatin analogs such as octreotide, which suppresses hormone production, are also very good at controlling symptoms.
Can a ZES spread to other parts of the body?
These malignant gastrinomas can spread to other parts of the body, including the liver, lymph nodes, spleen, bones, or skin.
What causes ZES?
The symptoms associated with ZES are caused by the presence of high levels of stomach acid, high levels of gastrin, or due to ulcers that form because of the disorder.
What causes ulcers in the stomach?
The excess stomach acid can cause the development of painful ulcers in the gastrointestinal tract. This rare condition is found in approximately one in one million people 1 and was first identified in 1955 by Drs. Robert Zollinger and Edward Ellison in Columbus, Ohio. 2
Can surgery cure Zollinger-Ellison syndrome?
Yes, surgery is the only way to cure Zollinger-Ellison syndrome. It is estimated that approximately 20% to 30% of ZES cases can be cured. 3
Is ZES a slow growing disease?
A diagnosis of ZES can be concerning, but it is usually a slow-growing disease and is not life-threatening if treated early. If you notice changes in the stool or the development of burning or gnawing pain in the abdomen or chest, seek medical treatment to evaluate these symptoms.
How does Zollinger-Ellison syndrome affect the patient?
Zollinger-Ellison syndrome could impact the patient's quality of life and increase mortality if it is not recognized and treated. This activity covers the proper evaluation by the interprofessional team to provide effective treatment plans to lower mortality. This activity reviews the evaluation and management of Zollinger-Ellison syndrome and the role of the interprofessional team in managing patients with this condition.
What causes peptic ulcers?
The etiology of peptic ulcers includes the overuse of NSAIDs such as ibuprofen and aspirin. A clinician may not be able to distinguish abdominal pain from excess acids and ulcers from PUD and ZES, but ZES causes more diarrhea and esophageal disease.[5] Helicobacter pyloriproduce urease which acts near G cells and causes urea hydrolysis and alkalization, affecting D cells and diminishing somatostatin cell release secondary to inflammation. Gastric outlet obstruction causes antral distention which activates parietal cells via acetylcholine.[8] Patients with chronic kidney disease or end-stage renal disease and undergoing dialysis have elevated serum gastrin. Gastrin clears through the kidney, and renal insufficiency will prevent the kidney from working properly. One study revealed an increase in gastric cell growth in a rat model with uremia resulted from proton back-diffusion in the stomach causing less mucosal barrier protection. [8][17][18][19] Gastric outlet obstruction causes distension of the antrum which activates parietal cells via acetylcholine and results in hypergastrinemia. [8]
What causes a zes in the stomach?
ZES is caused by gastrinoma which causes trophic effects of gastrin hypersecreting gastric acid. It can cause severe abdominal pain from multiple ulcers in the esophagus, stomach, and duodenum, diarrhea, and heartburn. PPIs have been the main therapy to control the symptoms. MEN1 is associated with ZES due to gastrinoma being a part of MEN1. However, the cure rate is slim, and surgical resection is not an ideal option. If surgical exploration is indicated, then managing hyperparathyroidism including subtotal parathyroidectomy must precede actual gastrinoma resection to control symptoms. There is a high propensity of sporadic gastrinoma metastasizing to the liver, lymph nodes, and a surgeon must evaluate distant organs and those that found to have sporadic gastrinoma for surgery. If the disease has already metastasized and the patient is not a surgical candidate, there are multiple options including embolizations and chemotherapy.
What causes gastric acid secretion?
Most common symptoms including abdominal pain, diarrhea, and heartburn are caused by excess gastrin secretion from gastrinoma. [5] [12] An article by Arnold described the trophic effect of excess gastrin as "increased fundic mucosal thickness, increased parietal cell mass, prominent gastric folds, and proliferation of gastric enterochromaffin-like (ECL) cells." [12] Usually, gastric acid secretion is controlled by negative feedback mechanisms by somatostatin released by gastric D cells to maintain gastric acid homeostasis to maintain pH in the stomach properly.[8] However, due to unopposed gastrin release by the neuroendocrine tumor, gastrinoma results in severe PUD because of excess gastric acid secretion to the post-bulbar regions of the duodenum from the esophagus via the trophic effect of gastrin on ECL and parietal cells. [8][13][14]
How to diagnose ZES?
To perform laboratory tests to diagnose ZES accurately, patients must stop the use of PPIs for at least 1 week, and cease using H2-receptor antagonist for 48 hours . [4][5] An initial test involves measuring fasting serum gastrin levels. This test has 99% sensitivity, and patients with gastrinoma will have a gastrin level greater than 100 pg/ml. The test is diagnostic if the level is greater than 1000 pg/ml.[4][5][10] However, the fasting gastrin level can normalize after parathyroidectomy in patients with MEN1 and gastrinoma resection even though the ZES is not fully cured.[5] Basal acid output test has 98% sensitivity and a level greater than 5 mEq per hour if the patient had gastrinoma resection or greater than 15 mEq per hour for those who did not. Gastric pH would be less than equal to 2. One can perform a secretin stimulation test which measures fasting serum gastrin level at 2 to 15 minutes after intravenous secretin administration. A level greater than or equal to 200 pg/ml indicates ZES and has 85% to 87% sensitivity. [4][5][10]
When was ZES first discovered?
In 1955 , ZES made first its appearance in Annals of Surgery as a case series compiled by two surgeons from Ohio State University, Dr. Robert M. Zollinger and Edwin H. Ellison. Both cases had ulcers at the upper jejunum with excess gastric acid production refractory to medical therapy and surgical therapy including gastrectomy. The ultimate finding that led to ZES with hypersecretion, hyperacidity, and recurrent peptic ulceration was a non-beta cell islet tumor of the pancreas.[7] The pioneer case series induced enough interest to lead to the publication of multiple studies that recognize the connections between gastrinoma and ZES.
What is ZES in GERD?
Zollinger-Ellison syndrome (ZES) is a group of symptoms comprised of severe peptic ulcer disease, gastroesophageal reflux disease (GERD), and chronic diarrhea caused by a gastrin-secreting tumor of the duodenum or pancreas (gastrinoma triangle) that results in increased stimulation of acid-secreting cells of the stomach.[1][2] Gastrinoma is a functional neuroendocrine tumor that secretes gastric acid which causes ZES.[3] The earlier misconception was that the location of gastrinoma is in the pancreas. However, gastrinomas occur in the duodenum more than the pancreas by three times especially in the first portion of the duodenum.[4] There are other non-neuroendocrine tumors secreting gastrin, but not adequate amounts to cause significant symptoms.[1] Gastrinoma causing ZES occurs sporadically in about 80% of cases and is reported to be 20% to 25% as multiple endocrine neoplasia type 1 (MEN1) from multiple reports in the literature.[4][5] Approximately 50% of patients with MEN1 have ZES; therefore, MEN1 must be included in a workup if ZES is highly suspicious.[4] It takes an average of 8 years from the start of symptoms to diagnosis due to the widespread use of proton pump inhibitors (PPIs).[4][5] One study of two referral centers in Italy and the United States showed a 62% decrease in referrals and diagnosis of ZES compared to a time when PPIs were used less frequently.[5][6]
