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Zollinger-Ellison Syndrome - Causes, Symptoms, Diagnosis, and Treatment

Published on Aug 25, 2022 and last reviewed on Aug 30, 2022   -  5 min read

Abstract

Zollinger-Ellison syndrome is a rare digestive system disorder in which one or more tumors (benign and malignant) are formed in the pancreas.

Contents

Introduction:

Zollinger-Ellison syndrome is a condition in which one or more tumors are found in the pancreas or upper part of the small intestine. These tumors are called Gastrinomas. Gastrinomas cause excess production of hormones known as “Gastrin.” This hormone regulates the level of gastric acid in the stomach, which helps in the digestion of food. Excess levels of gastric acid cause rupture of the inner lining of the stomach leading to the formation of peptic ulcers.

Peptic ulcers are sores that are formed due to erosion of the inner lining of the stomach.

What Is Zollinger-Ellison Syndrome?

A condition is called a syndrome when a set of symptoms appear together and suggest the presence of a certain disease. Zollinger-Ellison syndrome is characterized by the development of one or more tumors called Gastrinomas. Gastrinomas develop on the duodenum or pancreas, which are both benign and malignant. These tumors cause excess production of gastric acid that leads to rupture of the inner lining of the stomach and cause peptic ulcers. Excess gastric acid causes diarrhea and other health problems like peptic ulcer disease and gastroesophageal reflux disease (GERD).

What Causes Zollinger-Ellison Syndrome?

Research studies are still being conducted to reach the etiology of the cause of Zollinger-Ellison syndrome. Zollinger-Ellison syndrome is caused by multiple tumors called “Gastrinoma.” Gastrinomas develop in the pancreas or duodenum. The pancreas produces digestive juices and gastrin that help in the digestion of food. It also produces other hormones, including insulin. Insulin helps in regulating blood glucose levels.

Gastrinomas are the tumor masses in the pancreas, leading to excess production of the digestive juice, gastrin. Due to its acidic nature, tumors often become malignant. This syndrome is also known to be associated with a hereditary genetic disorder known as multiple endocrine neoplasia 1 (MEN 1). MEN 1 also produces a few hormones that are responsible for the formation of gastrinomas in the duodenum.

Who Is at Risk of Zollinger-Ellison Syndrome?

It is more prevalent in men than in women. It is found in people between the ages of 30 and 40. A family history of multiple endocrine neoplasias predisposes a person to develop Zollinger-Ellison syndrome. Persons with MEN 1 condition are more likely to develop ZES.

What Are the Symptoms of Zollinger-Ellison Syndrome?

Gastrinomas produce excess gastrin, due to which the patient might develop gastroesophageal reflux disease. The symptoms of this syndrome might be similar to that of GERD and peptic ulcer disease.

Sometimes, diarrhea could be the only symptom without any other symptoms.

How Can Zollinger-Ellison Syndrome Be Diagnosed?

What Is the Treatment for Zollinger-Ellison Syndrome?

Surgery is the best treatment option when the gastrinomas are non-malignant. Surgeries that can be chosen depending on the diagnosis are as follows:

  1. Enucleation: This surgery can be chosen when gastrinoma is small and located on the surface of the pancreas.

  2. Resection of the Pancreas: When the tumor is large, Whipple operation- removal of the head of the pancreas is advised depending on the location of gastrinoma.

  3. Duodenal Exploration: When the tumor is located on the wall of the duodenum, careful exploration and removal of the tumor can be performed.

  4. Lymph Nodes: If the tumor has become metastatic and reached the lymph nodes, careful palpation of the lymph nodes and their removal during surgery is advised.

Patients with Zollinger-Ellison syndrome are best cured with the usage of proton pump inhibitors (PPIs). PPI’s reduce the levels of gastrin which reduces the symptoms and allows healing. PPIs have very few side effects and are hence commonly prescribed medication. Few patients may need to take PPIs forever. Long-term use of PPIs may cause headaches, stomach upset, and deficiency of Vit B12. Some patients have been prescribed H2 blockers that reduce gastrin production. H2 blockers that are widely prescribed are Ranitidine and Cimetidine. In the case of malignant or invasive gastrinomas, chemotherapy- the use of anticancer drugs is advised.

When the tumor has spread to the liver, this treatment is best considered to alleviate the symptoms and expand the patient's life. This approach is also known as liver-directed therapy. Liver-directed therapy will not cure the disease but only help reduce the symptoms. Some of the liver-directed therapies are:

  1. Transarterial Chemoembolization (TACE): It is a combination of chemotherapy and embolization. Embolization is the process of blocking blood flow to cancer, so the cancer cells die.

  2. Radionuclide-Laden Spheres: This therapy uses tumor-targeting nanoscale and microscale carriers that selectively carry nucleotides to the tumor area, improving the outcome of cancer diagnosis and treatment.

  3. Local Ablative Therapy: It is a minimally invasive procedure to destroy cancer cells either by surgery, radio ablation, or cryoablation.

Though these medications have cured a lower number of patients with Zollinger-Ellison syndrome, they have shown a 70% reduction of the disease radiographically. Some of the medications are Temozolomide and Capecitabine. Due to its effectiveness, this treatment option is also considered neoadjuvant therapy.

The food and drug administration (FDA) has approved the use of Everolimus and Sunitinib for advanced pancreatic neuroendocrine tumors.

The Food and Drug Administration (FDA) has approved Peptide receptor radionuclide therapy (PRRT) for the treatment of neuroendocrine tumors. This therapy combines octreotide and a radioactive material which results in the formation of radiopeptide. This radiopeptide is then injected into the bloodstream for the treatment of tumors.

What Is the Prognosis of Zollinger-Ellison Syndrome?

Gastrinomas grow very slowly. Early detection and surgical removal of these tumors increase the life span of the patient.

Conclusion:

It is a rare digestive disorder that causes excess production of stomach acids. When left untreated, it can become life-threatening. Tumors grow very slowly, which gives time for proper treatment planning. People with hereditary tendencies are advised to undergo periodic checkups.

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Last reviewed at:
30 Aug 2022  -  5 min read

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