Table 1.

Classification of pancreatic neuroendocrine tumours [2–5, 16]

Tumour	Frequency	Syndrome	Pancreatic	Hormone	Malignant	Anatomical	Typical
name	(%)		islet cell	produced	(%)	location	size
Insulinoma	50	Insulinoma	B cell	Insulin	10–15	Pancreas >90% (head = body = tail)	<2 cm 90%, <1 cm 40%
Gastrinoma	20–30	Zollinger–Ellison syndrome	G cell	Gastrin	60–75	Pancreas 30%–60% a, duodenum 30%–40% a, lymph nodes 10%–15% a, other <5	0.3–3.0 cm
Non-functioning and PPoma	15–30	No syndrome	D1 cell	None or pancreatic polypeptide	60–90	Pancreas (most frequently in the head)	Large
VIPoma	3	Verner–Morrison syndrome; WDHA	D2 cell	Vasoactive intestinal peptide	50–80	Pancreas 90% (usually tail), adrenal 10%	Large
Glucagonoma	Rare	Glucagonoma	A cell	Glucagon	60	Pancreas	2–10 cm
Somatostatinoma	Rare	Somatostatinoma b	D cell	Somatostatin	50–70	Pancreas 56%, jejunum 44%	Large

^a 90% are within the gastrinoma triangle. ^b Somatostatinoma may be associated with NF 1.