Zollinger-Ellison syndrome

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What you need to know about Zollinger-Ellison syndrome

Zollinger-Ellison syndrome (ZES) is a rare condition characterized by the development of gastrin-secreting tumors called gastrinomas. These tumors typically arise in the pancreas or duodenum (the first part of the small intestine).

Gastrinomas lead to excessive production of gastrin, a hormone that stimulates the stomach to produce acid. As a result, individuals with Zollinger-Ellison syndrome experience overproduction of stomach acid, which can lead to peptic ulcers in the stomach and small intestine. These ulcers can cause symptoms such as abdominal pain, heartburn, nausea, vomiting, and gastrointestinal bleeding.

How to talk to your doctor about Zollinger-Ellison syndrome

Preparation:
- Make a list of symptoms, including their frequency and severity.
- Bring relevant medical records, such as past test results.
Open Communication:
- Describe your symptoms honestly and openly.
- Share any concerns or questions you may have about your condition.
Ask Questions:
- Inquire about the diagnostic process for ZES.
- Discuss potential complications and treatment options.
- Seek clarification on how ZES may impact your daily life and any necessary lifestyle changes.
Medical History:
- Provide details about your medical history, including past conditions, surgeries, and medications.
- Mention any family history of endocrine disorders or cancers.
Follow-Up:
- Schedule any recommended tests or consultations promptly.
- Stay engaged in your healthcare and advocate for yourself by seeking further information or clarification as needed.
Note-Taking:
- Consider taking notes during your appointment or bringing a trusted companion to help remember important details.
Treatment Goals:
- Discuss your treatment goals and preferences with your doctor.
- Work together to develop a treatment plan that aligns with your individual needs and priorities.

Types

Zollinger-Ellison syndrome is primarily characterized by the presence of gastrin-secreting tumors called gastrinomas. These tumors are typically found in the pancreas or duodenum. Gastrinomas can be sporadic (occurring without any apparent underlying cause) or associated with multiple endocrine neoplasia type 1 (MEN-1), a genetic disorder that predisposes individuals to the development of tumors in multiple endocrine glands.

Causes

The exact cause of Zollinger-Ellison syndrome is not fully understood. In sporadic cases, the cause of gastrinoma formation is unknown. However, in cases associated with MEN-1, a mutation in the MEN1 gene is responsible for the development of gastrinomas and other endocrine tumors.

Symptoms

The symptoms of Zollinger-Ellison syndrome are primarily due to the excessive production of stomach acid caused by gastrinomas. Common symptoms include:

Abdominal pain: This pain can range from mild to severe and may be persistent or intermittent.
Heartburn: A burning sensation in the chest or upper abdomen, often worsened by lying down or bending over.
Nausea and vomiting: These symptoms may occur due to irritation of the stomach lining.
Gastrointestinal bleeding: Bleeding from peptic ulcers can cause symptoms such as black, tarry stools or vomiting blood (hematemesis).
Weight loss: Chronic inflammation and ulcers in the digestive tract can lead to a decreased appetite and unintended weight loss.

Diagnosis

Diagnosing Zollinger-Ellison syndrome involves a combination of clinical evaluation, laboratory tests, imaging studies, and endoscopic examinations. Common diagnostic procedures include:

Blood tests: Measurement of gastrin levels in the blood. Elevated gastrin levels are indicative of Zollinger-Ellison syndrome.
Imaging studies: CT scans, MRI, or endoscopic ultrasound (EUS) may be used to locate tumors in the pancreas or duodenum.
Endoscopic examinations: Upper endoscopy allows direct visualization of ulcers in the stomach and duodenum, as well as biopsy samples for histological examination.

Treatment

Treatment of Zollinger-Ellison syndrome aims to control symptoms, reduce stomach acid production, and manage complications. Treatment options include:

Proton pump inhibitors (PPIs): These medications are the mainstay of treatment and effectively reduce stomach acid production, promoting healing of ulcers and alleviating symptoms.
Surgery: Surgical removal of gastrinomas may be considered, especially if they are localized and causing severe symptoms or complications.
Chemotherapy: In cases of metastatic gastrinomas or those not amenable to surgical removal, chemotherapy may be used to slow tumor growth and alleviate symptoms.
Supportive care: Nutritional support and management of complications such as gastrointestinal bleeding are important components of treatment.