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Source: In partnership with Genetic and Rare Diseases Information Center, funded by the National Center for Advancing Translational Sciences, and the National Human Genome Research Institute

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Zollinger-Ellison syndrome

overview Question

What is Zollinger-Ellison syndrome?

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. The tumors are sometimes cancerous and may spread to other areas of the body. 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.

Date Modified: 2014-01-14T10:57:00

basic Questions

What causes Zollinger-Ellison syndrome?

In most people with Zollinger-Ellison syndrome (ZES), the cause is not known. However, in about 25-30% of cases, it occurs with an inherited condition called multiple endocrine neoplasia type 1 (MEN1). This condition is caused by changes (mutations) in the MEN1 gene and is inherited in an autosomal dominant manner. Most, but not all, individuals with an MEN1 gene mutation will develop symptoms of multiple endocrine neoplasia type 1.

The MEN1 gene normally regulates the body's production of a protein thought to play a role in preventing the development of tumors (a tumor suppressor gene). Mutations that affect the function of this gene therefore affect the body's ability to prevent the growth of tumors, thus leading the the signs and symptoms of multiple endocrine neoplasia type 1.

Date Modified: 2014-01-14T11:42:00

Is Zollinger-Ellison syndrome inherited?

Zollinger-Ellison syndrome (ZES) usually occurs sporadically, for unknown reasons in a person with no history of the condition in the family. In about 25-30% of people with ZES, it is associated with an inherited condition called multiple endocrine neoplasia type 1 (MEN1).

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. 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.

Date Modified: 2014-01-14T12:03:00

Is genetic testing available for 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.

People who have a single MEN1-related tumor (such as one gastrinoma) and no family history of multiple endocrine neoplasia type 1 are rarely born with MEN1 gene mutations. Generally, the chance of detecting a MEN1 gene mutation increases in people with more main tumors (parathyroid, pancreatic, and pituitary).

Genetic testing is most informative for asymptomatic family members when the affected family member has genetic testing first. The person with symptoms of MEN1 typically first has sequencing analysis of the gene to look for changes; if no mutation is found, they may have duplication/deletion analysis (a test that detects extra or missing parts of the gene).

Predictive testing for at-risk, asymptomatic, adult family members requires first finding the disease-causing mutation in the affected family member. It is important to note that in people with a MEN1 gene mutation, the chance to develop symptoms by age 20 is over 50%. The chance to develop symptoms by age 40 is 95%. Therefore, as a person ages and does not have signs or symptoms, the chance to have a MEN1 gene mutation decreases significantly.

The Genetic Testing Registry (GTR) provides information about the genetic tests for multiple endocrine neoplasia type 1. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

Date Modified: 2014-01-14T15:35:00


Exocrine pancreatic insufficiency



Abnormality of the gastric mucosa

Abnormality of the thyroid gland


Multiple lipomas

Adenoma sebaceum

Adrenocortical adenoma

Autosomal dominant inheritance

Cafe-au-lait spot

Carcinoid tumor

Confetti-like hypopigmented macules




Growth hormone excess



Parathyroid adenoma

Peptic ulcer

Pituitary adenoma

Pituitary prolactin cell adenoma

Subcutaneous lipoma

Zollinger-Ellison syndrome

medical Products

Synthetic human secretin

Trade Name: ChiRhoStim™

Indication: For use in the diagnosis of gastrinoma associated with Zollinger-Ellison syndrome.

Synthetic porcine secretin

Trade Name: Synthetic porcine secretin

Indication: For use in secretin stimulation testing for: Stimulation of pancreatic secretions to facilitate the identification of the ampulla of Vater and accessory papilla during endoscopic retrograde cholangio-pancreatography (ERCP)

FDA Approved Medicines

  • FDA approved secretin, sold as SecreFlo, manufactured by Repligen as to aid in the diagnosis of pancreatic dysfunction and gastrinoma ( Gastrinoma | Pancreatic Cancer ) in year 2002.