Zollinger–Ellison Syndrome (ZES) is a rare disorder characterised by the formation of gastrin-secreting tumours, known as gastrinomas, which lead to excessive production of gastric acid. This condition results in severe peptic ulcers, gastrointestinal discomfort, and other significant complications.
Understanding and managing ZES is crucial for improving patient outcomes and quality of life. This article provides a comprehensive overview of Zollinger–Ellison Syndrome, including its pathophysiology, clinical features, diagnostic methods, and treatment options.
Neuroendocrine Cancer Australia (NECA), is dedicated to assisting individuals with Zollinger–Ellison Syndrome. NECA offers a wealth of resources, educational programs, and advocacy efforts aimed at deepening the understanding of NETs, improving patient care, and encouraging research advancements. Patients diagnosed with Zollinger–Ellison Syndrome can engage with NECA’s comprehensive support and information by calling the NET nurse line.
Zollinger–Ellison Syndrome was first described in 1955 by Drs. Robert Zollinger and Edwin Ellison. They identified the syndrome in patients presenting with severe peptic ulcers, gastric hypersecretion, and non-beta islet cell tumours of the pancreas.
Since its discovery, significant advancements have been made in understanding the disease, particularly the role of gastrinomas in its pathogenesis.
Zollinger–Ellison Syndrome is primarily caused by the presence of gastrinomas, which are tumours that secrete excessive amounts of gastrin. Gastrin is a hormone that stimulates gastric acid production by the parietal cells in the stomach.
In ZES, the overproduction of gastrin leads to hypergastrinemia, resulting in increased gastric acid secretion. This excessive acid can cause multiple, recurrent peptic ulcers, particularly in the duodenum and the jejunum.
The exact cause of gastrinomas in Zollinger–Ellison Syndrome is not fully understood. However, these tumours are often associated with a genetic condition known as Multiple Endocrine Neoplasia type 1 (MEN1). MEN1 is an inherited disorder characterised by the development of tumours in multiple endocrine glands, including the pancreas, parathyroid glands, and pituitary glands. Approximately 25-30% of patients with ZES have MEN1.
Gastrinomas are typically small, malignant tumours that can occur in the pancreas, duodenum, or other parts of the digestive tract. They are often slow-growing but can metastasize to the liver, lymph nodes, and other organs. The diagnosis and management of gastrinomas are crucial in controlling the symptoms and complications associated with Zollinger–Ellison Syndrome.
The most common clinical features of Zollinger–Ellison Syndrome are related to the gastrointestinal tract. Patients often present with severe, recurrent peptic ulcers that are resistant to standard ulcer treatments. These ulcers can occur in unusual locations, such as the jejunum.
Common symptoms include:
Excessive gastric acid production brought on by ZES can lead to significant complications, including:
While gastrointestinal symptoms are most common, Zollinger–Ellison Syndrome can also present with extra-gastrointestinal symptoms due to gastrinoma metastasis or associated conditions. These may include:
The diagnosis of ZES involves several steps to confirm hypergastrinemia and identify gastrinomas. Key diagnostic criteria include:
Imaging studies are crucial for locating gastrinomas and assessing their extent. Common imaging modalities include:
In addition to imaging, other tests can help confirm the presence of gastrinomas, such as:
Proton pump inhibitors (PPIs) are the mainstay of medical management for ZES. These medications effectively reduce gastric acid production and help control symptoms and heal peptic ulcers. Commonly used PPIs include omeprazole, lansoprazole, and esomeprazole.
Surgical resection of gastrinomas is often considered when feasible. The goals of surgery include:
Surgical options depend on the tumour’s location and extent and may include:
Other treatment modalities may be employed in managing ZES, including:
Living with Zollinger–Ellison Syndrome (ZES) involves managing a chronic condition that significantly impacts daily life. Patients must navigate a rigorous treatment regimen that typically includes medications to control excessive gastric acid production, such as proton pump inhibitors, and potentially undergo surgical interventions to remove gastrin-secreting tumours.
Regular medical follow-ups and monitoring are crucial to track disease progression and manage complications, such as peptic ulcers and gastrointestinal bleeding. Lifestyle adjustments, including dietary changes and stress management, play a vital role in minimising symptoms and improving overall well-being.
Support from healthcare providers, patient education, and community resources, such as those offered by Neuroendocrine Cancer Australia (NECA), are essential in helping patients and their families cope with the physical and emotional challenges of living with ZES.
Patients with ZES can benefit from dietary and lifestyle changes to manage symptoms and improve overall health. Recommendations include:
Ongoing monitoring is essential for managing ZES and preventing complications. Patients should have regular follow-up visits to:
Supportive care is crucial for improving the quality of life for patients with ZES. Resources include:
Neuroendocrine Cancer Australia (NECA) provides comprehensive support for patients living with Zollinger–Ellison Syndrome (ZES) and their families. NECA offers educational resources to help understand the condition and its management, alongside a dedicated nurse helpline for personalised guidance.
We facilitate connections through support groups, fostering a community of shared experiences and emotional support. NECA also advocates for patient access to the latest treatments and clinical trials, ensuring the best possible care. By providing these resources and support systems, NECA empowers patients and their families to navigate the complexities of living with ZES with confidence and armed with information.
Zollinger–Ellison Syndrome is a complex condition that requires a multidisciplinary approach for effective management. Early diagnosis, appropriate medical and surgical treatments, and ongoing supportive care are essential to improving patient outcomes.
Through continued research and advancements in treatment, the prognosis for individuals with ZES continues to improve. For more information and support, patients are encouraged to contact organisations such as Neuroendocrine Cancer Australia (NECA) for resources and guidance.
Further information and support for people diagnosed with Zollinger–Ellison Syndrome is available by calling the NECA NET nurse line.
