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Endocrine tumours of the pancreas

Endocrine tumours of the pancreas are a very heterogeneous group of rare neoplasms (they represent about 5-10% of all pancreatic tumours) and originate from endocrine cells. They manifest themselves in very different forms, but the fact that they have some biological characteristics in common allows doctors to diagnose and treat them in a similar way.
Endocrine tumours of the pancreas are divided into "functioning" and "non-functioning" tumours. The former, usually smaller, produce an excess of hormonal substances that trigger a series of symptoms depending on the hormone produced in excess. The latter do not produce any hormones, but may become very voluminous.

 

Causes 
As with many other neoplasms, the onset of pancreatic endocrine tumours also depends on various factors, many of which are still unknown. However, researchers have found that about 10% of cases are hereditary and are associated with the Multiple Endocrine Neoplasia Syndrome, a rare disease that causes the onset of tumours on organs or glands of the endocrine system such as the adrenals, pituitary, and parathyroids.

 

Symptoms
In the case of "functioning" endocrine tumours, the symptoms depend on the hormone produced in excess. Below is a list of the main "functioning" tumours and their symptoms:

  • Insulinoma: produces an excess of insulin, the hormone that regulates the level of sugar in the blood. Symptoms: tachycardia, feeling lightheaded, sweating and dizziness.
  • Gastrinoma: produces an excess of gastrin, which in turn increases the production of acid in the stomach. Symptoms: diarrhoea and ulcer formation. 
  • Glucagonoma: produces an excess of glucagon, responsible for diabetes and skin rashes.
  • VIPoma: produces an excess of vasoactive intestinal peptide (VIP), the hormone that controls the absorption and secretion of water in the intestine. Main symptom: diarrhoea. 

In the case of "non-functioning" endocrine tumours, the most frequent symptoms are:

  • Nausea and vomiting
  • Weight loss
  • Jaundice
  • Abdominal pain.

 

Diagnosis
The diagnosis of an endocrine tumour of the pancreas often occurs randomly, when clinical tests are performed for other reasons. 
Sometimes the alarm bells are blood tests, which can highlight high values of one of the previously described hormones or of other substances produced by almost all endocrine tumours (such as a protein called chromogranin A).
To confirm the diagnosis it is still necessary to carry out:

  • an endocrinological evaluation;
  • a surgical evaluation (to evaluate the operability of the disease);
  • specific endocrinological tests;
  • ultrasound, endoscopy, CT (to evaluate the state of the pancreas and possible metastases);
  • scintigraphy.

 

Treatment
Pancreatic endocrine tumours may be treated differently. Specialist care is required as treatment varies considerably depending on the case and can range from simple monitoring, surgical resection, to chemotherapy, radiometabolic or biological treatments.