From the Guidelines
Zollinger-Ellison syndrome (ZES) should be managed with proton pump inhibitors (PPIs) as the first line of treatment for symptom control, as they can effectively control the symptoms of excessive gastric acid production in the long term. The symptoms of ZES are primarily related to excessive gastric acid production caused by gastrin-secreting tumors called gastrinomas. The most common symptom is severe, recurrent peptic ulcers that may be multiple and occur in unusual locations like the jejunum. Patients typically experience persistent abdominal pain, often burning or gnawing in nature, that may not respond to standard ulcer treatments. Diarrhea affects 30-65% of patients and can be severe, resulting from the excessive acid inactivating pancreatic enzymes and damaging the intestinal mucosa. Other symptoms include heartburn, nausea, vomiting, and weight loss. Gastrointestinal bleeding may occur from ulcerations, manifesting as black, tarry stools or vomiting blood. Some patients experience GERD symptoms that are unusually severe or resistant to treatment.
According to the most recent guidelines, PPIs are the preferred treatment for controlling symptoms in patients with ZES, and somatostatin analogs (SSAs) may be used in uncontrolled cases 1. The diagnosis of ZES requires measuring fasting serum gastrin levels and confirming with secretin stimulation testing, followed by imaging to locate the gastrinomas. In some cases, other treatment options such as everolimus, diazoxide, or peptide receptor radionuclide therapy (PRRT) may be considered for symptom control in functional pancreatic neuroendocrine tumors (Pan-NETs) refractory to SSA.
Key considerations in the management of ZES include:
- Controlling symptoms with PPIs
- Diagnosing and localizing the gastrinoma
- Considering other treatment options for uncontrolled symptoms
- Monitoring for potential complications such as gastrointestinal bleeding or malabsorption. The treatment approach should be individualized based on the patient's specific needs and circumstances, and the use of SSAs or other treatments should be guided by the most recent clinical guidelines and evidence-based recommendations 1.
From the FDA Drug Label
In open studies of 57 patients with pathological hypersecretory conditions, such as Zollinger-Ellison syndrome (ZES) with or without multiple endocrine adenomas, lansoprazole significantly inhibited gastric acid secretion and controlled associated symptoms of diarrhea, anorexia and pain Doses ranging from 15 mg every other day to 180 mg per day maintained basal acid secretion below 10 mEq/hr in patients without prior gastric surgery and below 5 mEq/hr in patients with prior gastric surgery. Lansoprazole is effective in controlling symptoms of Zollinger-Ellison syndrome, including:
- Diarrhea
- Anorexia
- Pain Initial doses are titrated to the individual patient need, and adjustments may be necessary over time 2.
From the Research
Zollinger Ellison Syndrome Symptoms
The symptoms of Zollinger Ellison syndrome include:
- Refractory peptic ulcer disease
- Diarrhea
- Gastric acid hypersecretion associated with a gastrin-secreting tumor 3
- Acid-peptic symptoms 4
Diagnosis and Treatment
The diagnosis of Zollinger-Ellison syndrome is suggested when:
- Plasma gastrin is > 1000 pg/ml
- Basal acid output is > 15 mEq/h or when associated with a pH < 2 3 The treatment is focused on controlling gastric acid hypersecretion and localization of the tumor and its metastases 3.
- Proton pump inhibitors, such as omeprazole, are the most effective antisecretory drugs and can be administered at high dosages 3
- Histamine H2 receptor antagonists can also control gastric acid secretion safely and effectively in patients with Zollinger-Ellison syndrome 5
Management of Gastric Acid Hypersecretion
The management of gastric acid hypersecretion in Zollinger-Ellison syndrome includes:
- Medical treatment with proton pump inhibitors, such as omeprazole, which can effectively control gastric acid output 4, 6
- Surgical approaches to control acid secretion, which are described and discussed in the context of the current place of surgery in the management of acid hypersecretion 5
- Long-term omeprazole maintenance therapy, which can be safely and effectively used to control gastric acid hypersecretion in patients with Zollinger-Ellison syndrome for up to nine years without evidence of tachyphylaxis 6