A 45-year-old woman presents with episodic watery diarrhoea, flushing, and bronchospasm. 24-hour urinary 5-HIAA is markedly elevated. CT abdomen shows a 2 cm ileal mass with liver metastases. Which syndrome does she have and what is the drug of choice for symptom control?
- A VIPoma (WDHA syndrome); proton pump inhibitor
- B Zollinger-Ellison syndrome; pantoprazole
- C Carcinoid syndrome; octreotide LAR ✓
- D Medullary thyroid carcinoma; tyrosine kinase inhibitor
Explanation
The triad of episodic flushing, diarrhoea, bronchospasm, with elevated urinary 5-HIAA (5-hydroxyindoleacetic acid, a serotonin metabolite) and hepatic metastases from a midgut carcinoid tumour is diagnostic of carcinoid syndrome. Long-acting somatostatin analogues (octreotide LAR 20–30 mg every 4 weeks, or lanreotide) are the first-line agents for symptom control and also have antiproliferative effects (PROMID and CLARINET trials). VIPoma causes profuse watery diarrhoea but no flushing and elevated VIP levels. ZES causes peptic ulceration and elevated gastrin.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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