Description

Pancreatic neuroendocrine tumors are a rare type of cancer that begins as a growth of the cells in the pancreas. The pancreas is a long, flat gland that lies behind the stomach. Makes hormones and enzymes which help to digest food.

Pancreatic neuroendocrine tumors start of the hormone-producing cells in the pancreas. These cells are called islet cells. Another term for pancreatic neuroendocrine tumor of islet cell cancer.

Some of pancreatic neuroendocrine tumor cells keep making hormones. These are known as functional tumors. Functional tumors create too much of the hormone. Examples of the functional tumors including insulinoma, gastrinoma, and glucagonoma.

The majority of pancreatic neuroendocrine tumors do not produce an excessive amount of hormones. Tumors that do not produce an excess of hormones are called tumors non-functional.

Symptoms

Pancreatic neuroendocrine tumors sometimes do not cause symptoms. When they do, the symptoms may include:

  • Heartburn.
  • The weakness.
  • Fatigue.
  • Muscle cramps.
  • Indigestion.
  • The diarrhea.
  • The loss of weight.
  • Rash on the skin.
  • The constipation.
  • Pain in the abdomen or in the back.
  • Yellowing of the skin and the whites of the eyes.
  • The dizziness.
  • Blurred vision.
  • Headaches.
  • Increased thirst and hunger.

When to see a doctor

Make an appointment with a health care professional if you have any symptoms that worry you.

Causes

Pancreatic neuroendocrine tumors occur when cells in the pancreas develop changes in their DNA. A cell's DNA contains the instructions that tell the cell what to do. The changes, which the doctors called mutations, indicate cells that multiply rapidly. The changes that we're going to the cells to continue living when healthy cells would die as part of their natural life cycle. This makes many of the cells. The cells may form a mass called a tumor. Sometimes, the cells can break away and spread to other organs, like the liver. When cancer spreads, it is called metastatic cancer.

In pancreatic neuroendocrine tumors, changes in DNA occur in hormone-producing cells called islet cells. It is not clear what causes the changes that lead to cancer.

Risk factors

The factors that are associated with an increased risk of pancreatic neuroendocrine tumors are:

  • A family history of pancreatic neuroendocrine tumors. If a family member was diagnosed with a neuroendocrine tumor of the pancreas, the greater your risk is.
  • Syndromes present at birth, which increase the risk of tumors. Some of the syndromes that are passed from parents to children can increase the risk of pancreatic neuroendocrine tumors. Examples of these include multiple endocrine neoplasia type 1 (MEN 1), von Hippel-Lindau (VHL) disease, neurofibromatosis 1 (NF1) and tuberous sclerosis. These hereditary syndromes are caused by changes in the DNA. These changes enable cells to grow and divide more than necessary.

There is no way to prevent pancreatic neuroendocrine tumors. If you develop this type of cancer, not to do anything because of it.

Diagnosis

Tests and procedures used to diagnose pancreatic neuroendocrine tumors are:

  • Blood tests. Blood tests may show an excess of hormones or other signs of a neuroendocrine tumor of the pancreas. Blood samples can also be used to identify changes in the DNA that indicate an increased risk of these tumors.
  • Urine tests. A urine test can show the products of degradation that occur when the body processes hormones.
  • Imaging tests.Imaging tests make pictures of the body. You can show the location and size of a neuroendocrine tumor of the pancreas. The tests may include X-rays,magnetic resonance imaging,CTand positron emission tomography, also called PET scan. Image could also be done with nuclear medicine tests. These tests involve the injection of a radioactive tracer in your body. The tracer attaches to the pancreatic neuroendocrine tumors, in a way that is clearly shown in the images. The photos are often made with aPETscan that combines withCTorMRI.
  • The creation of images of your pancreas from the inside of your body. During an endoscopic ultrasound, a thin, flexible tube with a camera on the end, called an endoscope, is passed by the throat. It goes to your stomach and small intestine. The tube has a special type of ultrasound tool to create images of your pancreas. Other tools can be passed through the tube to collect a sample of tissue.
  • The removal of a sample of tissue for testing, also called a biopsy. A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue may be removed during an endoscopic ultrasound. Sometimes surgery is necessary to obtain a tissue sample. The sample is analyzed in a laboratory to see if it is cancer. Other special tests to give more details on the cancer cells. Your health care team uses this information to make a treatment plan.
  • The collection of cells from other areas for testing. If the cancer has spread to the liver, lymph nodes, or in other places, a needle can be used to collect cells for testing.

Imaging tests. Imaging tests make pictures of the body. You can show the location and size of a neuroendocrine tumor of the pancreas. The tests may include X-rays, magnetic resonance imaging , computed tomography and positron emission tomography, also called PET scan.

Image could also be done with nuclear medicine tests. These tests involve the injection of a radioactive tracer in your body. The tracer attaches to the pancreatic neuroendocrine tumors, in a way that is clearly shown in the images. The pictures are often made with PET combined with CT or MRI .

Treatment

The treatment for a neuroendocrine tumor of the pancreas depends on the types of cells involved in cancer, the degree and characteristics of your cancer, your preferences and your overall health.

The options may include:

  • Surgery.If the neuroendocrine tumor of the pancreas is only in the pancreas, treatment usually includes surgery. For cancers in the tail of the pancreas, the surgery may involve the removal of the tail of the pancreas, called a distal pancreatectomy. This surgery leaves the head of the pancreas intact. Cancers that affect the head of the pancreas may require the Whipple procedure, also called a pancreaticoduodenectomy. This surgery involves the removal of the cancer and part or most of the pancreas. If the cancer spreads to other parts of the body, surgery may be an option to remove it from those locations.
  • Peptide receptor radionuclide therapy, also called PRRT.PRRTcombines a drug that targets cancer cells with a small amount of a radioactive substance is injected into a vein. The medicine is pasted to the pancreatic neuroendocrine tumor cells of any part of the body. Over days to weeks, the medicine delivers radiation directly to the cancer cells, causing their death. OnePRRT, lutetium-177 Lu-dotatate (Lutathera), is used to treat advanced cancers.
  • The targeted therapy. Targeted therapy uses drugs that attack specific chemicals in cancer cells. By blocking these chemicals, specific treatments can cause cancer cells to die. Targeted therapy is used to treat certain advanced or recurrent pancreatic neuroendocrine tumors.
  • Radiofrequency ablation. Radiofrequency ablation involves the application of wave energy to cancer cells using a special probe with tiny electrodes. Radiofrequency ablation causes the cancer cells to heat up and die. The probe can be inserted directly in the skin or through an incision in the abdomen.
  • The radiation therapy. Radiation therapy uses high-energy rays to kill cancer cells. The energy comes from the X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.
  • Chemotherapy. Chemotherapy uses strong drugs to kill cancer cells. It is used in certain situations for the treatment of pancreatic neuroendocrine tumors.

Surgery. If the neuroendocrine tumor of the pancreas is only in the pancreas, treatment usually includes surgery. For cancers in the tail of the pancreas, the surgery may involve the removal of the tail of the pancreas, called a distal pancreatectomy. This surgery leaves the head of the pancreas intact.

Cancers that affect the head of the pancreas may require the Whipple procedure, also called a pancreaticoduodenectomy. This surgery involves the removal of the cancer and part or most of the pancreas.

If the cancer spreads to other parts of the body, surgery may be an option to remove it from those locations.

Peptide receptor radionuclide therapy, also called PRRT. PRRT combines a drug that targets cancer cells with a small amount of a radioactive substance is injected into a vein. The medicine is pasted to the pancreatic neuroendocrine tumor cells of any part of the body. Over days to weeks, the medicine delivers radiation directly to the cancer cells, causing their death.

One PRRT , lutetium-177 Lu-dotatate (Lutathera), is used to treat advanced cancers.

The treatment for the cancer that spreads to the liver

Pancreatic neuroendocrine tumors are most often spread to the liver. Several treatments exist for this, including:

  • Removal of part of the liver. A surgeon may remove part of the liver that he has cancer. The rest of the liver can take over the part which has been removed. In certain situations, it may not be possible to remove the entire liver and replace it with a liver from a donor. This procedure is called a liver transplant.
  • The deceleration of the flow of blood to the liver. The deceleration of blood flow through the main artery of the liver, called the hepatic artery, can slow or stop the growth of cancer. Other vessels in the liver to provide enough blood to the rest of the liver to continue working. Often, chemotherapy drugs, or radioactive beads are used to block the artery. These methods slow down the blood flow and provide treatment directly to cancer cells in the liver.
  • The destruction of the cancer cells with heat. Radiofrequency ablation is a procedure that uses waves of energy to heat the cancer cells and cause them to die. It makes use of a special probe with tiny electrodes that are inserted through the skin and into the liver. Waves of energy that pass through the tube and cause the tissue around it to heat.

Coping and support

With time, you will find what helps you to deal with the uncertainty and distress of a cancer diagnosis. Until then, you may find that it helps to:

  • Learn enough about your cancer to make decisions about your care. Ask your health care team about your cancer, including your test results, treatment options, and, if you like, your prognosis. As you learn more about cancer, you can be more confident in making treatment choices.
  • Keep friends and family close. Keep your close relationships strong will help you deal with your cancer. Friends and family can provide the practical support you need, such as help to take care of your house if you're in the hospital. And that you can serve as emotional support when you feel overwhelmed by the cancer.
  • Find someone to talk to.Find a good listener who is willing to listen to you talk about your hopes and fears. This can be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group may also be useful. Ask your health care team about support groups in your area. Other sources of information are the National Cancer Institute, the American Cancer Society, the North American Neuroendocrine Tumor Society and the Neuroendocrine Tumor Research Foundation.

Find someone to talk to. Find a good listener who is willing to listen to you talk about your hopes and fears. This can be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group may also be useful.

Ask your health care team about support groups in your area. Other sources of information are the National Cancer Institute, the American Cancer Society, the North American Neuroendocrine Tumor Society and the Neuroendocrine Tumor Research Foundation.

Preparing for your appointment

Make an appointment with a health care professional if you have any symptoms that worry you. If you are diagnosed with cancer, it is likely that you will be referred to a specialist. Often this is a doctor who specializes in the treatment of cancer is called an oncologist.

Here's some information to help you prepare for your appointment.

What you can do

When you make the appointment, ask if there is something that you need to do in advance, such as fasting before a specific test.

Collect the medical records that relate to your condition and bring them to your appointment. If you are seeing a new team of health care, we ask that all files and other information, such as sheets of glass containing the tissue samples are sent to your new computer.

Make a list of:

  • Its symptoms, including those that may not appear to be related to the reason for your appointment.
  • Key personal information, including major stresses, recent life changes and family medical history.
  • All medications, vitamins or supplements that you are taking, including the dosage.
  • Questions to ask your health care team.

Consider bringing a family member or friend to help you remember the information they give you.

Pancreatic neuroendocrine tumor, some basic questions to ask include:

  • What is likely causing my symptoms?
  • Other that the most likely cause, what are other possible causes of the symptoms?
  • What tests do I need?
  • What is the best course of action?
  • What are the alternatives to the primary approach you're suggesting?
  • What will happen if I don't have surgery or other medical treatments for my condition?
  • I have other health conditions. How can I best manage them together?
  • There are restrictions that must be followed?
  • You should see a specialist?
  • Are there brochures or other printed material I can have? What sites do you recommend?

Do not hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer common questions, such as:

  • When did your symptoms begin?
  • The symptoms been continuous or occasional?
  • How severe are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • What else should I know about you that will help me to make recommendations about your care?
Symptoms and treatment of Pancreatic neuroendocrine tumors