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Pathology · Neoplasia: carcinoid syndrome
Sample itemA 55-year-old man with an ileal carcinoid tumour and multiple liver metastases has flushing, watery diarrhoea and wheeze. Echocardiography shows tricuspid regurgitation and pulmonary stenosis. Why does the syndrome appear only with liver metastases, and which test confirms it?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Serotonin from hepatic metastases bypasses first-pass hepatic metabolism; urinary 5-HIAA is raised
Gastrointestinal carcinoids drain through the portal vein, where the liver degrades serotonin, so syndrome is absent unless liver metastases drain directly into the hepatic veins. Serotonin is metabolised to 5-hydroxyindoleacetic acid, measured in a 24-hour urine collection. Fibrous plaques on the right-sided valves cause tricuspid regurgitation and pulmonary stenosis because the lungs inactivate serotonin.
Option by option
A. Serotonin from hepatic metastases bypasses first-pass hepatic metabolism; urinary 5-HIAA is raised (correct)
Hepatic metastases release serotonin beyond hepatic metabolism, and its metabolite 5-HIAA is raised in urine.
B. Serotonin from the primary tumour reaches the systemic circulation unchecked; urinary VMA is raised
Serotonin from the primary tumour is cleared in the liver, and VMA is a catecholamine metabolite used for neuroblastoma and phaeochromocytoma.
C. Liver metastases secrete catecholamines into hepatic veins; urinary metanephrines are raised
Catecholamine secretion and metanephrines indicate phaeochromocytoma, which presents with paroxysmal hypertension.
D. Liver metastases release gastrin causing acid hypersecretion; serum gastrin is raised
Gastrin excess describes Zollinger-Ellison syndrome from a gastrinoma, with peptic ulceration.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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