Pancreatic Neuroendocrine Tumours

Pancreatic neuroendocrine tumours are a rare type of cancer that starts as the organ's cells proliferate. The role of pancreas is to create enzymes and hormones that facilitate the breakdown of meals. Pancreatic neuroendocrine tumours originate from the cells that produce pancreatic hormones. These cells are known as islet cells. Some pancreatic neuroendocrine tumour cells continue to produce hormones. These are known as functional tumours. 

Functional tumours produce high amount of the hormones. There are several kinds of functional tumours, these are insulinomas, glucagonomas, and gastrinomas.The majority of pancreatic neuroendocrine tumours do not overproduce hormones, these tumours are called as Non-functional tumours.

Insulinoma

• Most of these tumours are benign.
• The patient will present with the features of Whipple’s triad. The triad is Blood sugar<54mg/dl, Immediate relief of symptoms with IV glucose or sugar intake and Symptoms of hypoglycemia.
• Clinical features:
  • Frequent eating and weight gain.
  • Rage attacks and emotional liability.
  • Decreased Concentration span
  • Sympathomimetic features like Fine tremors, Diaphoresis and Palpitations.

Glucagonoma

• The patient will present with 3Ds features- Diarrhea, Diabetes and Dermatological features.
• The dermatological features are unique and will present as red rash, bullae and erosions. It is also called as Migratory necrolytic erythema. The lesions are mainly present in peri-orofacial sites.
• Weight loss

Somatostatinomas

• This tumour produces excess of somatostatin that leads to inhibition of production of insulin.
• It is found in CNS and GIT.
• The clinical features are:
  • Hypochlorhydria
  • Diabetes Mellitus
  • Diarrhea
  • Gallbladder disease

VIPoma

• It leads to excessive production of Vasoactive intestinal polypeptide(VIP).
• It is released by F cells of pancreas and the location is pancreas.
• It is present in both GIT/CNS and causes vasodilation.
• The clinical features are:
  • Achlorhydria
  • Hyperglycemia
  • Increase serum  calcium
  • Flushing episodes
  • Hypokalemia

Also read: Myoclonus: Causes, Symptoms, Diagnosis and Treatment

Diagnosis of Neuroendocrine Tumours

The diagnosis of pancreatic neuroendocrine tumours is made using the tests and procedures listed below:

• Blood tests: Blood testing can identify excess hormones or other markers of a pancreatic neuroendocrine tumour. Blood samples can also be used to look for genetic changes that suggest a higher risk of cancerous tumours.
• Urine-based testing: Testing urine can identify breakdown products that come from the metabolism of hormones.
• Imaging tests: The human body is imaged in imaging tests. They can show where and how big neuroendocrine tumours are in the pancreas. Tests that may be conducted include MRIs, CT scans, X-rays, and PET scans (positron emission tomography).
• Imaging examinations can also be performed using nuclear medicine. During these tests, you will be given an injection of a radioactive tracer. The tracer attaches to pancreatic neuroendocrine tumours, causing them to show up in the photographs. To construct the images, PET scans are frequently combined with CT or MRI scans.
• Creating internal images of the pancreas: During an endoscopic ultrasound, a thin, flexible tube called an endoscope which has a camera at its tip is passed down your throat. It passes through the stomach and small intestine. The tube has an ultrasonic tool that lets you take pictures of your pancreas. Other instruments may be placed into the tube to obtain a sample of the tissue.
• Biopsy: the procedure for taking a tissue sample for examination. A biopsy is the procedure of removing a sample of tissue for examination in a lab. Using an endoscopic ultrasound, the tissue can be removed. A tissue sample may occasionally need to be obtained by surgery. The sample is examined in a lab to check for the presence of malignancy. Further specialised testing yields further details about the cancer cells. Your healthcare team uses this information to formulate a plan of care.

Also read: Focal Segmental Glomerulosclerosis

Treatment Of Pancreatic Neuroendocrine Tumours

The type of cells causing cancer, the tumour's dimensions and characteristics, your preferences, and your overall health all influence the treatment plan for a pancreatic neuroendocrine tumour.

Among the options are:

• Surgery: If the pancreatic neuroendocrine tumour is localised, treatment usually involves surgery. When treating malignancies in the organ's tail, a distal pancreatectomy, or removal of the pancreatic tail, may be required. After this process, the pancreatic head is left entire.
• Cancers affecting the head of the pancreas may require pancreaticoduodenectomy, also referred to as the Whipple procedure. During this treatment, the majority or all of the pancreas is removed together with the cancer. If cancer spreads to other parts of the body, surgery can be required to remove it.
• Peptide receptor radionuclide therapy is referred to as PRRT: In PRRT, a medicine that targets cancer cells is injected into a vein along with a small amount of radioactive material. The drug adheres to pancreatic neuroendocrine tumour cells wherever they may be found in the body. Over several days to weeks, the cancer cells are killed by radiation that is directly exposed to them by the drug. One PRRT for the treatment of advanced cancers is lutetium Lu 177 dotatate, or Lutathera.
• Targeted treatment: Targeted treatment refers to the use of drugs that specifically target certain molecules in cancer cells. By stopping these chemicals from working, targeted medicines have the power to destroy cancer cells. Targeted therapy is used to treat some advanced or recurrent pancreatic neuroendocrine tumours.
• Radiofrequency ablation: Radiofrequency ablation is the process of applying energy waves to cancer cells using a specialist probe fitted with tiny electrodes. Radiofrequency ablation is used to kill cancer cells by heating them to a high temperature. The probe can be inserted just under the skin, or the abdomen can be cut.
• Radiation therapy: Radiation therapy uses high-energy beams to kill cancer cells. The energy comes from protons, X-rays, or other sources. For radiation therapy, you are surrounded by a machine while lying on a table. The device directs radiation onto certain body parts.
• Chemotherapy: Chemotherapy uses strong drugs to attack cancer cells. Under some conditions, it is used to treat pancreatic neuroendocrine tumours.

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