Table 6.3.1
The clinical features of neuroendocrine tumours
Site Clinical features Cell type MEN 1

Pancreatic

Insulinoma

Hypoglycaemia, Whipple’s triad, clammy, sweating, weight gain

β-islet cell

5–10%

VIPoma

Werner–Morrison syndrome, watery diarrhoea

VIP

10%

Glucagonoma

Diabetes mellitus, necrolytic migratory erythyema

5–10%

Somatostatinoma

Gallstones, diabetes mellitus, steatorrhoea

D cells

5–10%

Gastrinoma

Zollinger—Ellison syndrome

G cells

25%

Nonfunctional

Symptoms related to mass effect

Bronchial

Majority nonfunctional, 8% carcinoid syndrome, atypical flushing

Midguta

Majority nonfunctional, 40% develop carcinoid syndrome

Hindguta

Usually nonfunctional, however tumours may secrete somatostatin, other peptide, and occasionally carcinoid syndrome may occur

Site Clinical features Cell type MEN 1

Pancreatic

Insulinoma

Hypoglycaemia, Whipple’s triad, clammy, sweating, weight gain

β-islet cell

5–10%

VIPoma

Werner–Morrison syndrome, watery diarrhoea

VIP

10%

Glucagonoma

Diabetes mellitus, necrolytic migratory erythyema

5–10%

Somatostatinoma

Gallstones, diabetes mellitus, steatorrhoea

D cells

5–10%

Gastrinoma

Zollinger—Ellison syndrome

G cells

25%

Nonfunctional

Symptoms related to mass effect

Bronchial

Majority nonfunctional, 8% carcinoid syndrome, atypical flushing

Midguta

Majority nonfunctional, 40% develop carcinoid syndrome

Hindguta

Usually nonfunctional, however tumours may secrete somatostatin, other peptide, and occasionally carcinoid syndrome may occur

a

Midgut tumours arise from the jejunum to caecum and hindgut encompasses tumours from the ascending colon to rectum.

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