Zollinger Ellison Syndrome
Zollinger-Ellison syndrome (ZES) is a rare condition that causes tumors called gastrinomas to grow in your pancreas or intestine. These gastrinomas release the hormone gastrin, which triggers your stomach to produce gastric acid. Excessive stomach acid leads to ulcers and other complications, and some gastrinomas may become cancerous.
What is Zollinger-Ellison Syndrome?
Zollinger-Ellison syndrome is an exceedingly rare disorder that affects the digestive system. It arises from the presence of specific tumors called gastrinomas, which can develop in the pancreas or the duodenum (the upper part of the small intestine). Gastrinomas are responsible for the excessive production of gastrin, a hormone that triggers stomach acid production. Elevated gastrin levels lead to an overabundance of stomach acid, resulting in the formation of ulcers in the stomach and duodenum.
Effects of Zollinger-Ellison Syndrome
Individuals with ZES often experience complications related to excessive stomach acid production, including:
- Diarrhea
- Acid reflux
- Ulcers in the stomach and intestines
- Gastrointestinal bleeding
- Scarring in the gastrointestinal (GI) tract
- Blockages due to scar tissue
Approximately 90% of individuals with ZES develop ulcers, and around half of gastrinomas may become malignant, increasing the risk of metastasis.
Who Is Affected by Zollinger-Ellison Syndrome?
Gastrinomas are extremely rare, affecting an estimated 1 to 2 individuals per million annually. Many physicians may never encounter a case of ZES in their practice. The likelihood of developing this condition is higher for individuals with a family history of multiple endocrine neoplasia (MEN) type 1, a rare genetic disorder that causes tumors in various glands. Approximately 25% of ZES cases are associated with MEN type 1. However, the majority of individuals with gastrinomas acquire them for unknown reasons, and ZES is typically diagnosed during middle age.
Causes and Symptoms
The precise cause of ZES lies in the formation of tumors that overproduce gastrin. Some cases result from genetic mutations disrupting normal cell replication control mechanisms. Regardless, excessive gastrin production leads to an excessive amount of gastric acid in the body.
Common symptoms include:
- Burning or gnawing stomach pain
- Acid reflux and heartburn
- Indigestion, particularly after consuming fatty foods
- Oily or fatty stools and chronic diarrhea
Complications may occur as:
- Gastrointestinal bleeding symptoms (blood in vomit or stool)
- Anemia symptoms due to blood loss (pale skin, weakness, fatigue)
- Esophagitis resulting from acid reflux
- Loss of appetite, nausea, vomiting, and weight loss
Diagnosis and Testing
Diagnosing ZES involves a thorough evaluation of an individual’s medical history and symptoms. If ZES is suspected, healthcare providers typically order laboratory tests to measure gastrin and stomach acid levels. Elevated levels may prompt further investigation using various imaging techniques to identify gastrinomas in the digestive system. In some cases, a biopsy may be performed to assess tumor malignancy.
Management and Treatment Options
Medications:
- Proton pump inhibitors (PPIs): Reduce stomach acid by inhibiting the chemical process responsible for acid production, facilitating the healing of ulcers and damaged tissues.
Surgical Intervention:
- Aims to locate and remove gastrinomas to prevent their growth and spread. This can be technically challenging, and not all individuals qualify for surgery.
Cancer Therapies for Malignant Gastrinomas:
- Chemotherapy
- Radiation therapy
- Gastrectomy (stomach removal)
- Liver transplant (for metastatic cancer)
Prognosis
The prognosis for ZES varies based on the presence of malignancy and the effectiveness of treatment. Non-cancerous gastrinomas tend to grow slowly, and effective control of stomach acid may minimize symptoms. For individuals with cancerous gastrinomas, prognosis depends on whether surgical removal is possible. The 10-year survival rate is approximately 90% among those successfully treated with surgery. However, when surgery is not an option or is not curative, the 10-year survival rate drops to around 25%.
Conclusion
Zollinger-Ellison syndrome is a complex condition that requires careful diagnosis and management. Early detection and appropriate treatment can significantly improve the quality of life and prognosis for affected individuals. For more information, consult with a healthcare provider or specialist in gastroenterology.
