- Zollinger-Ellison Syndrome – NORD (National Organization for Rare Disorders)
- Zollinger-Ellison Syndrome: Background, Pathophysiology, Etiology
- Zollinger-Ellison Syndrome (ZES)
- WHAT ARE THE SYMPTOMS OF ZOLLINGER-ELLISON SYNDROME?
- WHAT CAUSES ZOLLINGER-ELLISON SYNDROME?
- HOW IS ZOLLINGER-ELLISON SYNDROME DIAGNOSED?
- HOW IS ZOLLINGER-ELLISON SYNDROME TREATED?
- WHAT ARE THE COMPLICATIONS OF ZOLLINGER-ELLISON SYNDROME?
- WHO IS AT RISK FOR ZOLLINGER-ELLISON SYNDROME?
- NEXT STEPS
- Learn the Symptoms, Causes, Diagnosis of Zollinger-Ellison Syndrome
- What Is Zollinger-Ellison Syndrome?
- Zollinger-Ellison Syndrome/Gastrinoma
- How are Gastrinoma’s diagnosed?
- How are Gastrinoma’s treated?
Zollinger-Ellison Syndrome – NORD (National Organization for Rare Disorders)
When possible (e.g., if there is no evidence of metastasis of a single tumor), complete surgical removal of the gastrinoma may be considered the optimal treatment for ZES. Evidence suggests that complete and curative removal of gastrinoma is possible in approximately 20 to 30 percent of individuals with ZES.
Various advanced imaging techniques may be used before surgery to help localize and characterize the gastrinoma and exclude metastatic disease (e.g., endoscopic ultrasound, nuclear medicine studies, abdominal ultrasounds, computerized tomography [CT] scanning, abdominal angiography).
Recent advances in imaging have led to 68G Ga-Dotatate PET CT replacing somatostatin scintigraphy for localization of neuroendocrine tumors including those causing ZES. This test has a high sensitivity and specificity. Gastrinomas are slow growing and hence 18F-FDG PET/CT is not commonly used for initial evaluation.
Due to slow metabolic activity of gastrinoma in initial stages, they are not typically avid on 18F-FDG PET/CT. In contrast to FDG PET/CT, 68Ga-Dotatate PET demonstrates a high uptake because neuroendocrine tumors express significant numbers of somatostatin 2 receptors. In addition, in some patients, certain imaging techniques (e.g.
, intraoperative endoscopic transillumination or ultrasound) may be used during surgical exploration to aid in the localization and possible removal of tumors. Recent studies have shown that intraoperative localization may be facilitated by the intravenous administration of indocyanine- green (ICG) 0.
1 mg/kg and within one minute of injection examination of the pancreas and duodenum with near infrared fluorescence visualization.
Surgery is the only treatment shown to potentially cure gastrinoma. Biochemical cure of sporadic gastrinoma is reported in 30% to 50% of patients. However, recurrence has been documented in nearly one-third of patients. The average time to recurrence is 5 to 10 years.
Regardless of achieving biochemical cure, complete resection of all tumors is associated with improved survival.
The 10-year disease-specific survival in patients having complete resection of sporadic gastrinoma is 85% compared to 40% for patients having incomplete resection and 25% for those having no resection.
In some studies, MEN-1 patients operated upon with a curative intent, cure was achieved in only 6% of patients.
However, the 10-year survival with gastrinoma in MEN-1 with complete resection was 90%, compared with only 45% for patients having an R2 resection or no resection.
As incomplete resections do not increase survival, MEN -1 patient with extensive metastatic disease or loco-regional spread that precludes complete resection receive little benefit from surgical resection and these patients are typically not offered surgery.
Pancreatic gastrinoma survival fairs well when compared to other pancreatic neuroendocrine tumors (PNETs). It has been reported that between histologic subtypes, there were significant differences in sex and age, and in tumor size, grade, location, and stage.
Median survival time for insulinomas was 12.7 years; gastrinomas, 10.2 years; glucagonomas, 7.7 years; VIPomas, 7.9 years; and mixed tumors, 3.4 years. Multivariate analysis has shown that histology (insulinoma, gastrinoma, and VIPoma; p = .
009), absence of distant metastases (p = .002), age < 50 years (p = .001), surgical intervention (p = .001), and stage I/II disease (p = .011) were associated with prolonged survival.
Rarely, in severe cases in which other therapy is ineffective, surgical removal of the stomach (gastrectomy) may be considered.
Initial treatment commonly includes the use of certain medications called proton pump inhibitors, such as omeprazole. Such medications may reduce stomach acid production, relieve symptoms, and promote ulcer healing. In some patients, another type of acid-suppressing medication called H2 blockers may also be used, such as cimetidine or ranitidine.
In 2006, the FDA approved AstraZeneca’s proton pump inhibitor Nexium for management of acid hypersecretion in patients with ZES.
The FDA approved a proton pump inhibitor called Protonix (pantoprazole sodium), in the form of delayed-release tablets, for the long-term treatment of individuals with ZES. Protonix is marketed in the United States by Wyeth Pharmaceuticals.
Due to the effectiveness of the medications discussed above, serious complications associated with ulcers may often be prevented. However, some affected individuals may remain undiagnosed until developing such complications (e.g., perforation or obstruction). These complications are considered medical emergencies that require immediate treatment, potentially including surgery.
In some affected individuals with aggressively invasive gastrinoma, recommended treatment may include the use of certain anticancer drugs (chemotherapy) to help reduce tumor mass and blood gastrin levels.
Management of Metastatic Disease
Patients with malignant gastrinoma of the pancreas and duodenum not infrequently present with liver metastases. In these situations, it is essential for the surgeon to work collaboratively with a multidisciplinary team.
Liver directed therapy for patients with unresectable liver only or liver-dominant metastases, who present with symptomatic disease is beneficial for those with >25% liver burden. Transarterial chemoembolization (TACE), radionuclide-laden spheres (Yittrium-90) or local ablative therapy (radiofrequency or microwave ablation) are effective liver directed therapies.
These modalities will not cure the patient, but they may provide effective cytoreduction of liver metastases, alleviate symptoms attributable to metastatic disease, and possibly extend survival. However, these treatments have not been compared to one another or to best supportive care.
Hence, these patients are best served in the context of a multidisciplinary team and perhaps in the framework of a clinical trial.
Systemic Treatments/Cytotoxic Chemotherapy
Trials using chemotherapeutic drugs including doxorubicin, streptozocin, 5-fluorouracil (5-FU), temozolomide, and dacarbazine have established cytotoxic effects in PNETs. There have been limited numbers of patients with gastrinoma treated in these trials.
Capecitabine and temozolomide have been shown to have a high and durable response in PNETs in a small study with 70% of patients demonstrating a radiographic response with median progression free survival of 18 months. Given this radiographic response this regimen has also been reported in the neoadjuvant setting. Response rates specific to gastrinoma have not been reported.
Everolimus and sunitinib are both FDA approved treatments for advanced pancreatic neuroendocrine tumors.
A randomized controlled trial of everolimus, an oral inhibitor of mammalian target of rapamycin (mTor) showed an increase in progression free survival from 4.6 months to 11.0 months.
Sunitinib, a multi-targeted tyrosine kinase inhibitor has also been shown to increase progression free survival to 11.4 months from 5.5 months and increase in overall survival in patients with metastatic unresectable disease.
Both octreotide and l long acting octreotide (LAR) have been shown to prolong progression free survival however overall survival has not been significantly increased. These drugs are thought to stabilize tumor growth in addition to relieving symptoms associated with functional tumors.
Peptide receptor radionuclide therapy (PRRT) was approved by the FDA for treatment of neuroendocrine tumors in 2017.
PRRT is a molecular therapy that uses octreotide combined with a small amount of radioactive material creating a radiopeptide. This is then injected into the bloodstream.
The NETTER-1 study, a large phase III randomized clinical trial showed lutetium 177 (177Lu) dotatate improved progression free survival a median of 33 months when compared with LAR.
Genetic counseling is recommended for individuals with ZES and their families.
Zollinger-Ellison Syndrome: Background, Pathophysiology, Etiology
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Zollinger-Ellison Syndrome (ZES)
Zollinger-Ellison syndrome (ZES) is sometimes known as gastrinoma, pancreatic ulcerogenic tumor syndrome or Z-E syndrome.
ZES is a rare condition that is usually diagnosed in patients between the ages of 20 and 60.
This disease causes a tumor or multiple tumors to form in the upper part of your small intestine (duodenum), the lymph nodes next to your pancreas, or in your pancreas.
These tumors cause your stomach to produce too much acid which can lead to ulcers, diarrhea, and more. In addition, these tumors are cancerous, or have the potential to become cancerous, and the cancer can spread if not treated or removed.
At Loma Linda University Health, we are uniquely qualified to treat Zollinger-Ellison syndrome. We treat more pancreatic disorders than any other center in the region.
WHAT ARE THE SYMPTOMS OF ZOLLINGER-ELLISON SYNDROME?
Signs and symptoms of Zollinger-Ellison syndrome may include:
- Stomach pain
- Decreased appetite
- Feeling full too soon
- Nausea and vomiting
- Burning, aching, or gnawing in the upper abdomen
- Heartburn and acid reflux
- Unintended weight loss
- Bleeding in your digestive tract
- Black or tarry stool
- Blood in the stool
WHAT CAUSES ZOLLINGER-ELLISON SYNDROME?
The cause of Zollinger-Ellison syndrome is unknown. However, in approximately 25% of those affected, ZES occurs in association with a genetic syndrome known as multiple endocrine neoplasia type 1 (aka MEN-1).
HOW IS ZOLLINGER-ELLISON SYNDROME DIAGNOSED?
Diagnosis of Zollinger-Ellison syndrome starts with a medical exam and health history. Doctors may order lab work, including a blood test to check for high levels of a certain hormone that is associated with ZES.
Patients should tell the doctor if they have used over-the-counter, acid-reducing medications. Often, these medications may mask the symptoms of ZES. This could lead to a misdiagnosis or delay the diagnosis.
HOW IS ZOLLINGER-ELLISON SYNDROME TREATED?
At Loma Linda University, our Gastroenterology and Hepatology department provides state-of-the-art, patient-centered care for those suffering from digestive disorders such as Zollinger-Ellison syndrome. Zollinger-Ellison syndrome is typically treated with medication, surgery or a combination of the two.
Treatment of Excess Acid Production
Medications known as proton pump inhibitors are powerful drugs that reduce acid by blocking the action of the tiny “pumps” within acid-secreting cells, and they are the first line of treatment.
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.
Treatment of Tumors
An operation to remove the tumors that occur in Zollinger-Ellison may be necessary. Our board-certified specialists have the skills to locate even the smallest of tumors even though they are often difficult to find.
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 may still recommend removing a single large tumor.
Doctors may advise other treatments to control tumor growth, including:
- Debulking – In this procedure, the doctor removes as much of a liver tumor as possible.
- Embolization or radiofrequency ablation – This treatment attempts to destroy the tumor by cutting off the blood supply or by using heat to destroy cancer cells.
- Injecting medications – Drugs are injected into the tumor to relieve cancer symptoms.
- Chemotherapy – The goal of chemotherapy is to slow tumor growth.
- Enucleation – Many small ulcers in the pancreas may be treated by “scooping out” alone. This is a procedure of choice for patients that have small tumors (less than 1cm) where the tumor is located on the surface of the pancreas.
- Resection of the pancreas – In patients with large tumors, a distal pancreatectomy or a Whipple operation may be performed, depending on where the tumor is located in the pancreas.
- Duodenal exploration – Tumors often occur in the wall of the duodenum (first part of the intestine). Therefore, opening the duodenum and carefully feeling it to remove any tumors in this area is important.
- Lymph nodes – In some patients, the tumor may be located in the lymph glands outside the pancreas. When this happens, careful palpation and removal of these glands is important at the time of surgery.
- Liver transplant – In some cases, a patient may need to undergo a liver transplant.
In addition, genetic counseling may be beneficial for patients and their families.
WHAT ARE THE COMPLICATIONS OF ZOLLINGER-ELLISON SYNDROME?
If Zollinger-Ellison syndrome is left untreated, complications may include continued pain, diarrhea, and weight loss. If ZES is left untreated, further complications include the possible spread of cancer and the need for more complex and unpleasant treatments that may have been avoided. Ultimately, failing to seek treatment for ZES can be fatal.
WHO IS AT RISK FOR ZOLLINGER-ELLISON SYNDROME?
Anyone can develop Zollinger-Ellison syndrome. However, in about 25% of those affected by ZES, it may be caused by an inherited condition called multiple endocrine neoplasia type 1. This is also known as MEN-1.
Those who have a parent, sibling, or another blood relative with MEN-1 may be at greater risk for developing ZES.
Seek medical intervention. If you are experiencing symptoms those associated with ZES, seek medical intervention right away. Being proactive is important, and it starts with a medical evaluation. To request an evaluation at Loma Linda University Health, contact this department or schedule the appointment through MyChart.
Learn the Symptoms, Causes, Diagnosis of Zollinger-Ellison Syndrome
Zollinger-Ellison Syndrome (ZES) is a rare disorder that causes one or more tumors to form in the pancreas or the upper part of the small intestine called the duodenum. It can also cause ulcers to develop in the stomach and the duodenum.
The tumors are called gastrinomas, and they secrete a large amount of the hormone gastrin. This then causes excessive production of stomach acid, which can lead to peptic ulcers. The tumors are cancerous in 50 percent of the cases.
The ulcers caused by ZES are less responsive to treatment than ordinary peptic ulcers. What causes people with ZES to develop tumors is unknown, but approximately 25 percent of ZES cases are associated with a genetic disorder called multiple endocrine neoplasia.
Zollinger-Ellison syndrome is rare, and though it may occur at any age, people between the ages of 30 and 60 are more ly to develop it. Also, of all the people who suffer from a peptic ulcer, only a tiny percentage of those people will have Zollinger-Ellison.
The symptoms of ZES include:
- Gnawing, burning pain in the abdomen
- This pain is usually located in the area between the breastbone and the navel.
- The sensation of pressure, bloating, or fullness
- This pain usually develops 30 to 90 minutes after a meal and is often relieved by antacids.
- Pain or burning sensation in the abdomen that travels up toward the throat
- The vomit may contain blood or resemble coffee grounds.
- Stools may be foul-smelling.
- Black, tarry stools
- Blood in the stools will turn them dark red or black, and make them tarry or sticky.
- Weight loss
Zollinger-Ellison syndrome is caused by a tumor or tumors in the pancreas and the duodenum. These tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause overproduction of stomach acid. This increase in acidity can lead to the development of peptic ulcers in the stomach and duodenum.
Blood Test: A blood test is performed to see whether there is an increased gastrin level in the blood. An elevated level of gastrin may indicate tumors in the pancreas or duodenum.
Barium X-ray: The patient drinks a liquid that contains barium, which will coat the walls of the esophagus, stomach, and duodenum. X-rays are then taken. The doctor will then view the X-rays, looking for signs of ulcers.
Upper Endoscopy: The doctor examines the inside of the esophagus, stomach, and duodenum with an instrument called an endoscope, a thin, flexible, lighted tube with a lens.
The endoscope is inserted through the mouth and down the throat, and into the stomach and duodenum.
The doctor can look for ulcers, and can also remove a tissue sample, called a biopsy, for examination in the laboratory to identify if there is the presence of gastrin-producing tumors.
Imaging Techniques: A doctor may use a computerized tomography (CT) scan, a magnetic resonance imaging (MRI) scan, and ultrasound, or a nuclear scan in an effort to pinpoint where tumors may be located.
- A CT scan is a diagnostic test that uses X-rays aided by computer technology. The X-ray beams are taken from many different angles to create cross-sectional images of the patient's body. Then a computer assembles these images into a three-dimensional picture that can display organs, bones, and tissues in great detail.
- With an MRI scan, magnetic signals are used rather than X-rays to create images of the human body. These images show the differences between types of tissues.
- An ultrasound sends out high-frequency sound waves that go into the area being examined and bounce back when they hit an organ. This is processed by a computer, which produces a map of the area being scanned.
- With a nuclear scan, radioactive substances are introduced into the body that permits a gamma camera to detect tumors.
In 50 percent of cases of Zollinger-Ellison, the tumors are cancerous (malignant). If the tumors are cancerous, there is a risk that cancer will spread to the liver, the lymph nodes near the pancreas and small intestine.
Other complications of Zollinger-Ellison Syndrome:
- Severe ulcers that may lead to the development of a hole in the bowel
- Blood loss
- Chronic diarrhea
- Weight loss
The treatment of Zollinger-Ellison syndrome focuses on two areas: Treating the tumors and treating the ulcers.
Surgery is often performed if there is only one tumor. If tumors are in the liver, a surgeon will remove as much of a liver tumor as possible (debulking).
When surgery on tumors isn't possible, other treatments are used:
- Attempting to destroy the tumor by cutting off the blood supply (embolization).
- Attempting to destroy cancer cells by using an electric current (radio-frequency ablation).
- Injecting drugs into the tumor to relieve cancer symptoms.
- Using chemotherapy to try to slow tumor growth.
- Proton Pump Inhibitors: These are the most effective medications for Zollinger-Ellison syndrome. Proton pump inhibitors are quite powerful and suppress acid production and promote healing. Examples of proton pump inhibitors include Prilosec, Prevacid, Nexium, Aciphex, and Protonix.
- Acid Blockers: These are also called histamine (H-2) blockers. These medications reduce the amount of hydrochloric acid released into the digestive tract. This helps to relieve ulcer pain and encourage healing. Acid blockers work by keeping histamine from reaching histamine receptors. Histamine receptors signal acid-secreting cells in the stomach to release hydrochloric acid. Examples of acid blockers include Tagamet, Pepcid, and Axid. Acid blockers don't work as well as proton pump inhibitors do, and may not be prescribed. For those people who do use acid blockers, they need high, frequent doses for them to be effective.
As stated above, in 50 percent of cases of Zollinger-Ellison, the tumors are cancerous. Early diagnosis and surgical invention can achieve a cure rate of only 20 to 25 percent. However, gastrinomas are slow-growing, and patients may live for many years after a tumor is discovered. Acid-suppressing medications are very effective at controlling the symptoms of acid overproduction.
For a diagnosis, you need to see your healthcare provider. At this time all aspects of this condition, along with treatment options and ongoing care and monitoring of the condition, should be discussed with your doctor. After diagnosis, if you should experience and new or worsening symptoms, these should be reported to your doctor.
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National Organization for Rare Disorders (NORD). Zollinger-Ellison Syndrome.
National Institute of Diabetes and Digestive and Kidney Diseases. Zollinger-Ellison Syndrome. December 2013
What Is Zollinger-Ellison Syndrome?
Zollinger-Ellison syndrome (ZES) is a disease of the gastrointestinal system. People who have ZES develop tumors known as gastrinomas in the pancreas and duodenum (the first section of the small intestine). The gastrinomas caused by ZES secrete the hormone gastrin. Because gastrin creates excessive stomach acid, 90 percent of patients with ZES develop stomach and duodenal ulcers.
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.
Another complication of ZES is that more than half of single gastrinomas are malignant (cancerous). These malignant gastrinomas can spread to other parts of the body, including the liver, lymph nodes, spleen, bones, or skin.
People who have Zollinger-Ellison syndrome don't always have symptoms. When symptoms do occur, they include:
If your doctor suspects that you have ZES, he or she will perform a blood test to look for high levels of gastrin (the hormone secreted by gastrinomas). They may also perform tests to measure how much acid your stomach is producing.
Your doctor may examine you for gastrinomas by performing an endoscopy. This procedure is done with a flexible, lighted tube (an endoscope) that looks at your esophagus, stomach and duodenum. This is often done with endoscopic ultrasound to see the tumor.
Other tests your doctor might perform include a CT scan, a special type of X-ray that provides cross-sectional images of the body, a PET scan to locate tumors, and an octreotide scan to look for neuroendocrine tumor cells.
Despite these tests, gastrinomas can be difficult to find.
ZES is treated by reducing the amount of acid your stomach produces. Medications called proton pump inhibitors are usually prescribed.
These drugs, which include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), dexlansoprazole (Dexilant ), esomeprazole (Nexium), and rabeprazole (Aciphex), curb the production of stomach acid and allow the ulcers to heal.
Treatment of ZES depends on whether the gastrinoma is sporadic or part of the inherited MEN I syndrome. 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.
If there is metastatic disease, you may be offered a combination of therapies including surgery, chemotherapy, or targeted drug therapy or radiation.
Gastrinomas tend to grow slowly and are not always malignant. 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%. If surgery removes the gastrinoma, 20%-25% of patients are completely cured.
If you have been treated for ZES, you should see your doctor on a regular basis to determine if the gastrinomas recur.
National Digestive Diseases Information Clearinghouse.
National Institutes of Health.
Harrison's Principles of Internal Medicine-13th Edition (1994).
Feldman: Sleisenger and Fordtran's Gastrointestinal and Liver Disease-7th Edition (2002).
© 2018 WebMD, LLC. All rights reserved.
Zollinger-Ellison syndrome is a rare disorder characterized by the development of a tumor called a Gastrinoma found in the pancreas and/or duodenum. Gastronoma’s secrete excessive levels of gastrin, a hormone that stimulates production of acid by the stomach.
Normally, the body releases small amounts of gastrin after eating, which triggers the stomach to make gastric acid that helps break down food and liquid in the stomach. The extra acid causes peptic ulcers to form in the duodenum and elsewhere in the upper intestine.
Although anyone can get Zollinger-Ellison syndrome, the disease is more common among men 30 to 50 years old. The prevalence of the disease occur in approximately 10 per million of the population.1 Also a child who has a parent with MEN1 is also at increased risk for Zollinger-Ellison syndrome.2
Medical researchers are still studying the exact cause of Zollinger-Ellison syndrome. About 25 to 30 percent of Gastrinoma’s are caused by an inherited genetic disorder called multiple endocrine neoplasia type 1 (MEN1).
1 MEN1 causes hormone-releasing tumors in the endocrine glands and the duodenum. Symptoms of MEN1 include increased hormone levels in the blood, kidney stones, diabetes, muscle weakness, weakened bones, and fractures.
Zollinger-Ellison syndrome signs and symptoms are similar to those of peptic ulcers. A dull or burning pain felt anywhere between the navel and midchest is the most common symptom of a peptic ulcer.
Other symptoms Zollinger-Ellison syndrome include:
• diarrhea• mild to severe abdominal pain• steatorrhea (increased amounts of fat in the stools)• bloating• burping• nausea• vomiting• weight loss
• poor appetite
Some people with Zollinger-Ellison syndrome have only diarrhea, with no other symptoms. Others develop gastroesophageal reflux (GER), which occurs when stomach contents flow back up into the esophagus.
How are Gastrinoma’s diagnosed?
A diagnosis of a Gastrinoma should be considered in a patient with peptic ulcers that recur frequently and are resistant to treatment. A medical professional may suggest a biochemical study called the Secretin Stimulated blood test to check for an elevated gastrin level after an infusion of secretin.
Secretin is a hormone that causes Gastrinoma’s to release more gastrin. A technician or nurse places an intravenous (IV) needle in a vein in the arm to give an infusion of secretin.
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.
Some of the tests that are performed to localize the tumor include a CT scan, octreotide scan and MRI and an endoscopic ultrasound.
An experienced surgeon will detect these tumors at surgery even when they are not visible on preoperative imaging tests.
For a listing of NPF Centers who perform Gastrinoma Testing, please click HERE.
How are Gastrinoma’s treated?
Treatment of choice for Gastrinoma is to remove the surgically where possible. Peptic ulcers must be aggressively treated and controlled prior to surgery.
The type of surgery for Gastrinoma’s depends on the location of the tumor. Since these tumors may frequently occur at more than one spot in the pancreas and the surrounding tissues more than one procedure may be required.
The following operative procedures may be utilized to treat Gastrinoma’s.
• Enucleation: Many small Gastrinoma’s in the pancreas may be treated by enucleation alone. This is a procedure of choice for patients that have small tumors (less than 1cm) where the tumor is located on the surface of the pancreas.
• Resection of the pancreas: in patients with large tumors a distal pancreatectomy or a Whipple operation may be indicated depending on where the tumor is located in the pancreas.
• Duodenal exploration: Gastrinomas often occur in the wall of the duodenum (first part of the intestine) and therefore opening duodenum and carefully feeling it to remove any tumors in this area is important.
• Lymph nodes: In some patients the tumor may be located in the lymph glands outside the pancreas therefore careful palpation and removal of these glands is important at the time of surgery
In some affected individuals with aggressively invasive Gastrinoma, recommended treatment may include the use of certain anticancer drugs (chemotherapy) to help reduce tumor mass and blood gastrin levels.
Genetic counseling may be of benefit for affected individuals and their families. Other treatment for this disease is symptomatic and supportive.
1 Metz DC. Diagnosis of the Zollinger-Ellison syndrome. Clinical Gastroenterology and Hepatology. 2012;10(2):126–130.
2 Del Valle J. Zollinger-Ellison syndrome. In: Yamada T, ed. Textbook of Gastroenterology. 5th ed. Hoboken, NJ: Blackwell Publishing; 2009: 982–1002.