Related Subjects:
|Zollinger Ellison syndrome
|Gastrinoma
|VIPomas
|Carcinoid Tumour Syndrome
๐งช A gastrinoma is a rare, gastrin-secreting tumour that causes excessive gastric acid production, often leading to recurrent and severe peptic ulcer disease. It is the main cause of ZollingerโEllison syndrome (ZES). ๐ฅ
๐ About
- ๐งฌ Neuroendocrine tumour secreting excess gastrin, stimulating gastric acid hypersecretion โ peptic ulcers.
- ๐ Usually arises in the pancreas or duodenum within the โgastrinoma triangle.โ
- ๐๏ธ Main cause of ZollingerโEllison syndrome.
๐งฌ Aetiology
- Most cases are sporadic, but ~25% linked to Multiple Endocrine Neoplasia type 1 (MEN-1).
- Excessive gastrin โ uncontrolled gastric acid โ recurrent, multiple, or refractory peptic ulcers.
๐ฉบ Clinical Features
- โก Severe/recurrent peptic ulcer disease (90%).
- ๐ค Abdominal pain and refractory dyspepsia.
- ๐ฉ Diarrhoea and steatorrhoea due to acid inactivation of pancreatic enzymes.
- โ ๏ธ Complications: bleeding, perforation, gastric outlet obstruction.
๐ฌ Investigations
- ๐ Serum gastrin: >1000 pg/mL with high acid output strongly suggests gastrinoma.
- ๐ Secretin stimulation test: paradoxical rise in gastrin confirms diagnosis.
- ๐น EUS, CT/MRI, and Octreoscan to localise tumour and assess metastases.
- ๐งฌ MEN-1 screening: parathyroid & pituitary assessment + genetic testing.
โ๏ธ Management
- ๐ PPIs (high-dose): cornerstone of therapy to control acid hypersecretion.
- ๐ Somatostatin analogues (octreotide) for symptom and tumour control in advanced cases.
- โ๏ธ Surgical resection: potentially curative in localised disease.
- ๐งช Targeted therapy (e.g., sunitinib, everolimus) or chemotherapy for metastatic disease.
- ๐ฉป Vagotomy rarely considered if refractory acid production persists.
๐ Prognosis
- ๐ Localised tumours = favourable prognosis with surgery.
- ๐ฉธ Metastatic disease (esp. liver) โ poorer outcomes, but acid control with PPIs improves quality of life.
๐ Cases โ Gastrinoma
- Case 1 (Sporadic ZES): ๐จโโ๏ธ A 42-year-old man has multiple duodenal ulcers resistant to high-dose PPIs, plus chronic diarrhoea. Serum gastrin 1850 pg/mL; secretin test positive. CT shows a 2 cm duodenal mass.
Management: Surgical resection of tumour + ongoing PPI.
Outcome: Symptoms resolve; no recurrence at 3-year follow-up.
- Case 2 (MEN-1 associated): ๐ฉ A 36-year-old woman with family history of pituitary adenoma presents with recurrent peptic ulcer disease and steatorrhoea. Serum gastrin 2100 pg/mL; Octreoscan shows pancreatic tail lesion + parathyroid hyperplasia.
Management: MEN-1 workup; subtotal parathyroidectomy, high-dose PPI, and octreotide. Tumour surgically debulked but liver metastases found.
Outcome: Good symptom control with PPIs + octreotide. Remains under oncology and endocrinology surveillance.
๐งโโ๏ธ Teaching Commentary
Gastrinomas cause ZollingerโEllison syndrome, classically presenting with multiple, recurrent, or refractory ulcers and diarrhoea. Always suspect in โulcers out of proportionโ to risk factors.
๐ Key points:
โข Consider MEN-1 if young or with family history.
โข Diagnosis = high fasting gastrin + secretin test.
โข Treatment = PPIs to control acid, plus surgery if resectable.
โข Prognosis hinges on metastatic status. Early diagnosis is lifesaving. ๐