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Zollinger-Ellison syndrome

Other Names for this Disease
  • Gastrinoma
  • Pancreatic ulcerogenic tumor syndrome
  • Z E syndrome
  • ZES
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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).[1]

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.[2] 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.
Last updated: 1/14/2014

  1. Zollinger-Ellison Syndrome. National Digestive Diseases Information Clearinghouse (NDDIC). December 24, 2013; Accessed 1/14/2014.
  2. Multiple endocrine neoplasia. Genetics Home Reference. August, 2013; Accessed 1/14/2014.