Pancreatic neuroendocrine tumours (pNETs), also known as pancreatic neuroendocrine neoplasms (pNENs), are a rare type of tumour that originates in the hormone-producing cells of the pancreas. 


While less common than pancreatic adenocarcinoma, pNETs can vary widely in their behavior, ranging from benign to malignant. In general, pNETS have a better prognosis compared to pancreatic adenocarcinoma. With successful treatment, patients can achieve long term cure.

Causes and Risk Factors

The exact cause of pancreatic neuroendocrine tumours remains largely unknown. However, certain risk factors may increase the likelihood of developing these tumours. These risk factors include:

  1. Genetic Syndromes: Individuals with genetic syndromes such as multiple endocrine neoplasia type 1 (MEN1), von Hippel-Lindau syndrome (VHL), neurofibromatosis type 1 (NF1), and tuberous sclerosis complex (TSC) have an elevated risk of developing pancreatic neuroendocrine tumours.
  2. Family History: A family history of pNETs or other neuroendocrine tumours may increase the risk of developing these tumours.
  3. Age: Pancreatic neuroendocrine tumours are more commonly diagnosed in individuals between the ages of 30 and 60, although they can occur at any age.



The symptoms of pancreatic neuroendocrine tumours can vary depending on the size, location, and hormone-producing capabilities of the tumour. Common symptoms may include:

  1. Abdominal Pain: Persistent or intermittent pain in the abdomen, often located in the upper abdomen or back.
  2. Jaundice: Yellowing of the skin and eyes due to obstruction of the bile duct by the tumour.
  3. Weight Loss: Unexplained weight loss, often accompanied by loss of appetite and fatigue.
  4. Hormonal Symptoms: Some pNETs produce hormones that can cause specific symptoms, such as flushing, diarrhoea, wheezing, or rapid heartbeat.



Diagnosing pancreatic neuroendocrine tumours typically involves a combination of imaging studies, laboratory tests, and tissue biopsy. Diagnostic tests may include:

  1. Imaging Studies: Imaging techniques such as computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans can help visualize the tumour and determine its size, location, and spread.
  2. Blood Tests: Blood tests may be performed to measure levels of specific hormones or tumour markers associated with pNETs.
  3. Biopsy: A tissue biopsy may be performed to obtain a sample of the tumour for examination under a microscope to confirm the diagnosis. These are often achieved via endoscopy (Endoscopic Ultrasound-guided biopsy)



The treatment approach for pancreatic neuroendocrine tumours depends on several factors, including the size and location of the tumour, whether it has spread to other parts of the body, and the overall health of the patient. Treatment options may include:

  1. Surgery: Pancreatic surgery may be recommended for localized pNETs that have not spread to other organs.
  2. Medical Therapy: Medications such as somatostatin analogs, targeted therapy, or chemotherapy may be used to shrink tumours, control symptoms, or slow disease progression.
  3. Radiation Therapy: External beam radiation therapy or targeted radiation therapy (such as peptide receptor radionuclide therapy) may be used to destroy cancer cells and relieve symptoms.
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