AppId is over the quota
In Zollinger-Ellison syndrome, the primary tumour is located in the pancreas. This type of tumour, however, secretes large quantities of a hormone called "gastrin" which causes the stomach to secrete much more acid than usual - leading to ulcers throughout the gatro-intestinal tract - in the stomach, duodenum or jejunum.
Precise data on the incidence is not available. Zollinger-Ellison syndrome is responsible for a small proportion of peptic ulcers.
This type of tumour (gastrinoma) is usually idiopathic. However, 20% of patients with Zollinger-Ellison syndrome have multiple endocrine neoplasia type 1 syndrome, characterised by other endocrine tumours.
Medical treatment can completely treat the symptoms due to the ulcers.
These tumours are slow growing and generally cause no symptoms for many years, even decades. However, they are not always surgically removable and thus can be fatal, albeit after many years.
Full blood count - may indicate iron deficiency anaemia (consider iron studies).
Patients may experience morbidity due to the ulcers, which can be complicated by massive bleeding or even perforation. The ulcers themselves are easily treated medically.
The tumour itself is a slow growing one as mentioned previously, but it is still malignant, and if not surgically removed has the potential to be fatal - although this may take many years. Unfortunately, up to 50% of patients may not be suitable for surgery due to spread of the tumour (e.g. to the liver or other surrounding organs or structures).
The manifestations of acid-peptic disease can be controlled with anti-ulcer medications. The treatment of choice is firstly, a proton pump inhibitor or secondarily, an H2 antagonist. The dose should be adjusted based on the effect and the basal acid outlet.
In those with advanced liver disease, a liver transplant can provide remarkable relief.
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