Neuroendocrine tumours (NETs) of the pancreas originate from differentiated or poorly differentiated endocrine cells that can differentiate into exocrine or endocrine cells of the pancreas. The World Health Organization has proposed the term “neuroendocrine neoplasm” (NEN) for all neuroendocrine tumours of the gastroenteropancreatic system. These tumours are characterised by the appearance of specific tumour markers in the patient’s serum: neuron-specific enolase (NSE), chromogranin A and synaptophysin. The specificity of neuron-specific enolase in diagnosing NET is almost 100%, while the sensitivity of this marker is about 30%.
Pancreatic NETs are rare and account for only 2% of diagnosed pancreatic tumours. The annual number of new cases in the world is 1 per 100,000 inhabitants. They can occur at any age, but are most often diagnosed in people between 50 and 70 years old. People with multiple endocrine neoplasia type I, von Hippel-Lindau syndrome, neurofibromatosis type I and tuberous sclerosis have an increased risk of developing pancreatic NETs, indicating the importance of genetic abnormalities in the pathogenesis of this disease.
Pancreatic NETs, based on their secretory activity, can be functional or non-functional.
Functional pancreatic NETs secrete certain peptide hormones into the patient’s bloodstream, which leads to the appearance of accompanying clinical syndromes (Whipple’s triad, carcinoid syndrome, Zollinger-Ellison syndrome, hypokalemia syndrome). Insulinomas and gastrinomas are the most common pancreatic NETs, while glucagonomas, VIPomas and somatostatinomas occur in significantly smaller numbers.
Non-functioning pancreatic NETs account for almost half of all pancreatic NETs. They are characterised by the absence of secretory activity. They are manifested by symptoms associated with a local compressive effect on the surrounding structures (obstructive icterus, abdominal pain with back propagation) and distant metastases.
The diagnosis of pancreatic NETs is made using laboratory analysis of blood and urine (detection of peptides and amines in functional tumours and tumour markers specific for NETs), computed tomography (CT), magnetic resonance (MR), positron emission tomography (PET), endoscopic ultrasound and scintigraphy of somatostatin receptors (octreoscan).
Treatment of pancreatic NETs involves surgical and systemic drug treatment.
Surgical treatment is the most important form of therapy because it significantly prolongs the survival of patients. It involves a resection procedure on the pancreas or the so-called enucleation in smaller tumours.
Systemic treatment includes the use of streptozocin, radioactive elements, somatostatin analogues, and alpha interferon. Control of clinical symptoms and suppression of tumour growth are the main goals of this type of treatment.
There is no sure way to prevent the development of pancreatic NETs. Genetic predisposition for their occurrence is a factor that cannot be influenced. Quitting smoking and limiting alcohol consumption are factors that can reduce the risk of pancreatic NETs.
