Zollinger- Ellison Syndrome Flashcards
Zollinger-Ellison Syndrome
Zollinger-Ellison syndrome is condition characterised by excessive levels of gastrin, usually from a gastrin secreting tumour usually of the duodenum or pancreas. Around 30% occur as part of MEN type I syndrome
Features
multiple gastroduodenal ulcers
diarrhoea
malabsorption
Diagnosis
fasting gastrin levels: the single best screen test
secretin stimulation test
Zollinger-Ellison Syndrome - Diagnosis: Example Question
A 62 year old male presents with a 5 month history of epigastric discomfort and two episodes of black tarry stools in the past 4 weeks. Over the past year, he reports a change in his bowel habit of almost predominantly loose bowel motions with no mucous or blood. He has no past medical history but acknowledges that he does not lead the healthiest lifestyle: he is an active smoker with a 50 pack year history and drinks at least 40 units of alcohol per week. However, he has been actively trying to improve a sedentary lifestyle as a lorry driver by using the gym twice a month, reporting 1.5 stones weight loss over the past 4 months. He had been started on increasing doses of omeprazole by his GP with no improvements in his symptoms. His mother died of head of pancreas adenocarcinoma aged 55. On examination, his abdomen is soft and non-tender with no organomegaly. Abdominal XR were unremarkable. Stool culture revealed no organisms on microscopy. An oesophageal-gastric endoscopy demonstrated multiple gastric and duodenal ulcers, biopsies of which were H Pylori negative. His blood tests are as follows:
Hb 12.8 g/dl Platelets 299 * 109/l WBC 9.4 * 109/l Na+ 139 mmol/l K+ 4.5 mmol/l Urea 7.9 mmol/l Creatinine 60 µmol/l Bilirubin 22 µmol/l ALP 30 u/l ALT 120 u/l γGT 82 u/l Adj calcium 2.22 mmol/l Phos 1.31 mmol/l Random blood glucose 7.1 mmol/l
Which of the following tests would make a definitive diagnosis?
CT chest/abdomen/pelvis with contrast Faecal elastase > Serum gastrin and secretin stimulation Endoscopic retrograde cholangiopancreatography (ERCP) Magnetic resonance cholangiopancreatography (MRCP)
A middle aged male with evidence of upper GI tract bleeding, multiple gastric and duodenal ulcers, weight loss and diarrhoea with no history of NSAID use, family history of multiple endocrine neoplasia syndrome 1 (MEN1) and refractory to proton-pump inhibitor treatment should make you suspicious of Zollinger-Ellison syndrome, characterised by a gastrin secreting tumour leading to peptic ulceration and excess stomach acid secretion. The family history of pancreatic adenocarcinoma is a red herring; chronic pancreatitis, where faecal elastase helps to demonstrate malabsorption, may cause chronic diarrhoea but does not explain widespread peptic ulcerations. MRCP can demonstrate structural abnormalities such as strictures while ERCP allows therapeutic interventions and biopsies within the biliary system.
Diagnosis of Zollinger Ellison syndrome is made by raised levels of gastrin. Secretin stimulation test differentiates between ZES and other causes of raised gastrin: secretin increases gastrin secretion in gastrinomas but inhibits gastrin secretion in gastric G cells. CT chest/abdomen/pelvis with contrast may be useful in locating the gastrinoma but does not make the initial diagnosis.
Zollinger Ellison Syndrome Presentation
A middle aged male with evidence of upper GI tract bleeding, OGD multiple gastric and duodenal ulcers, weight loss and diarrhoea with no history of NSAID use, family history of multiple endocrine neoplasia syndrome 1 (MEN1) and refractory to proton-pump inhibitor treatment should make you suspicious of Zollinger-Ellison syndrome, characterised by a gastrin secreting tumour leading to peptic ulceration and excess stomach acid secretion.
Diagnosis of Zollinger Ellison syndrome is made by raised levels of gastrin.
Secretin stimulation test differentiates between ZES and other causes of raised gastrin: secretin increases gastrin secretion in gastrinomas but inhibits gastrin secretion in gastric G cells.
