A rare cancer type that begins in the pancreas is called pancreatic neuroendocrine tumors. A long and flat organ located behind the stomach is called the pancreas and it produces specific enzymes and hormones that help digest food.
This cancer type commonly begins in the hormone-producing cells of the pancreas called islet cells. Sometimes, pancreatic neuroendocrine tumors are called islet cell cancer.
In some cases, the pancreatic neuroendocrine tumors may continue to produce hormones. In such cases, they are called functional tumors. These tumors make too much of the given hormone. Examples of functional tumors include insulinoma, gastrinoma, and glucagonoma.
If pancreatic neuroendocrine tumors do not make high hormone amounts they are called nonfunctional tumors.
Symptoms
Some people with islet cell cancer do not have any symptoms. Check below some symptoms of pancreatic neuroendocrine tumors:
- Weakness
- Extreme tiredness (fatigue)
- Muscle cramps
- Unusual weight loss
- Indigestion
- Diarrhea
- Skin rash
- Constipation
- Abdominal or back pain
- Jaundice (yellowing of the skin and eyes)
- Dizziness
- Vision changes (such as blurred vision)
- Headaches
- Increased hunger and thirst
If any of the previous symptoms occur, contact your healthcare professional right away.
Causes
This cancer type often happens when DNA changes (mutations) occur. DNA contains instructions that tell cells to grow, multiply, and die. However, mutated DNA gives different instructions to the cells, which leads to a quick multiply. Moreover, the instructions of the changed DNA make abnormal cells live longer than healthy ones. When cancerous cells become too much, they form a mass called a tumor.
In some cases, cancerous tumors may break and spread throughout the body such as the heart, liver, lungs, or others. In such cases, it is called metastatic cancer.
Experts do not fully understand why it happens but in this cancer type DNA changes occur in the hormone-production cells also known as islet cells.
Risk Factors
There are some factors that may elevate your risk of developing pancreatic neuroendocrine tumors. For example:
- Family history – People who have a family history of islet cell cancer are at higher risk of developing it.
- Certain congenital syndromes – Some syndromes present at birth may increase your risk of developing this cancer type. These include multiple endocrine neoplasia, type 1 (MEN 1), von Hippel-Lindau (VHL) disease, neurofibromatosis 1 (NF1), and tuberous sclerosis.
Diagnosis
Healthcare professionals often diagnose this condition by performing the following tests and procedures. Examples include:
- Blood tests – They often perform these tests to check for pancreatic hormone levels and other abnormalities linked with islet cell cancer. They also can show mutated DNA that indicates an elevated risk of tumors.
- Urine tests – These tests can show breakdown products when the body processes hormones.
- Imaging tests – These include CT (computerized tomography), MRI (magnetic resonance imaging), PET (positron emission tomography), and X-ray scans. These tests are used to get detailed images of the pancreas. Moreover, imaging tests may show the location and size of the tumors. Sometimes, your physician may perform imaging with nuclear medicine tests that involve a radioactive tracer that is injected into the body through a vein.
- Endoscopic ultrasound – During this procedure, a small tube with a camera on the end is inserted through your throat to the stomach and small bowel. It has a specific ultrasound tool that produces images of the pancreas. Furthermore, during this test, doctors may get a small sample of the tumor for laboratory testing (biopsy).
- Biopsy – This procedure involves tests to check for cancer cells in the tissue samples taken from the pancreas or tumors.
In addition, your doctor may take tissue samples from different body parts (such as the liver, lymph nodes, and others) if the cancer spreads.
Treatment
Usually, treatments for people with pancreatic neuroendocrine tumors are different among people because it depends on the severity of the cancer, its location, existing health problems, age, and other factors. Check below the most commonly prescribed treatments for people with islet cell cancer:
- Surgery – This treatment option is usually used when cancer has not spread and tumors are present in the pancreas only. If cancer occurs in the pancreas tail, doctors may recommend a surgery called distal pancreatectomy that involves the removal of the tail while the pancreas head remains intact. When the cancer negatively affects the pancreas head, physicians recommend a surgery called the Whipple procedure also known as pancreaticoduodenectomy. In such cases, surgeons will remove the affected area and a part or most of the pancreas.
- PRRT (peptide receptor radionuclide therapy) – This treatment option involves specific medications that target cancerous cells along with a small amount of radioactive material that is usually given intravenously (IV). PRRT sticks to all pancreatic neuroendocrine tumors whether they are located in the body. Therefore, over a few days or weeks, these medicines deliver radiation to the cancer cells, leading to their death. In advanced stages of this cancer type, doctors may recommend Lutetium Lu 177 dotatate.
- Targeted Therapy – Before using this treatment option, doctors may perform certain tests to determine whether this treatment is effective. However, this therapy involves medications that target specific proteins in the cancer cells and by blocking them, it causes cancerous cells to die. In most cases, physicians recommend targeted therapy for people with recurrent or advanced pancreatic neuroendocrine tumors.
- Radiofrequency ablation – This treatment option involves applying energy waves to the cancerous cells by using a specific probe with small electrodes.
- Radiation therapy – This procedure involves powerful energy beams directed right to the cancer cells to destroy them. The energy used during this therapy comes usually from X-rays, protons, or other sources.
- Chemotherapy – It involves strong medications given usually intravenously to destroy cancer cells. However, oral chemotherapy drugs also are available. In most cases, this treatment option is used along with radiation therapy.
Treatment Options if the Cancer Spreads to the Liver
Commonly, if the pancreatic neuroendocrine tumors spread, they negatively affect the liver. In such cases, doctors may recommend additional treatments. For example:
- Liver transplant – During this procedure, surgeons will remove the liver completely and replace it with a healthy one that usually comes from a donor.
- Reducing blood flow to the liver – In some cases, radiation therapy and chemotherapy are recommended by doctors to block the artery (hepatic artery) that supplies the liver with blood. As a result, the cancer may stop growing or slow down.
- Use heat to destroy cancerous cells – An additional radiofrequency ablation may be recommended by doctors to destroy cancer cells in the liver.
Frequently Asked Questions
What are the primary symptoms of pancreatic neuroendocrine tumors?
These include:
- Hormonal imbalances
- Digestive problems
- Unusual weight loss
- Jaundice
- Abdominal pain
- Reduced appetite
- Blurred vision
- Constipation
Do not hesitate to see a doctor if any of the symptoms above occur.
What are the potential complications of pancreatic neuroendocrine tumors?
Check some complications that may occur in people with this cancer type:
- Insulinomas
- Gastrinomas
- Glucagonomas
- VIPomas
- Somatostatinomas
This article does not contain all possible complications of islet cell cancer. For more details, discuss it with your healthcare professional.
What is the most effective treatment for people with pancreatic neuroendocrine tumors?
The primary treatment for this cancer type, especially if you have more than one tumor, is surgery. During this procedure, surgeons will remove the tumors. The surgery type depends on the location and size of the tumors. However, they can appear in the head, body, or tail of the pancreas. If you have additional questions, ask your healthcare professional.
