Islet Cell Tumor

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Most of the endocrine tissue in the pancreas is located in discrete islands.
Islands of endocrine cells, the islets of Langerhans, are scattered throughout the pancreas.
The endocrine cells secrete:
Insulin.}
Glucagon.}
Somatostatin.}
Pancreatic polypeptide.
Amylin (IAPP).
The only other important condition after diabetus resulting from abnormality in the endocrine component of
the pancreas is islet-cell tumour.
Tumours of the islet cells of the pancreas are examples of neuroendocrine tumours (apudomas).
Most are benign, producing their symptoms through excess secretion of a specific hormone,
usually insulin. The tumours are usually solitary, but in the MEN syndromes most are multiple.

Insulinoma produces hypersecretion of insulin, leading to hypoglycaemic attacks that are often
severe enough to produce coma; pre-coma symptoms include confusion and behavioural disturbance
(sometimes aggressiveness).

Glucagonoma is very rare. The excess secretion of glucagon is often asymptomatic, although
patients may be anaemic and develop an unexplained secondary diabetes. A bizarre charac-teristic
skin rash called necrolytic migratory erythema sometimes occurs.

Very rarely, islet-cell tumours may secrete unusual hormones such as vasoactive intestinal
peptide (VIP) and gastrin (producing the Zollinger-Ellison syndrome of recurrent and
multiple peptic ulcers in stomach and proximal small intestine), or may even produce ACTH.
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