Description

Neuroendocrine tumors are cancers that begin in specialized cells called neuroendocrine cells. Neuroendocrine cells have traits similar to those of nerve cells and hormone-producing cells.

Neuroendocrine tumors are rare and may occur in any part of the body. Most neuroendocrine tumors occur in the lungs, appendix, small intestine, rectum and pancreas.

There are many types of neuroendocrine tumors. Some grow slowly and some grow very quickly. Some neuroendocrine tumors excess production of hormones (functional neuroendocrine tumors). Others do not the release of hormones or of not knowing enough to cause symptoms (non-functional neuroendocrine tumours).

The diagnosis and treatment of neuroendocrine tumors depend on the type of tumor, its location, if it produces an excess of hormones, how aggressive it is, and whether it has spread to other parts of the body.

Symptoms

Neuroendocrine tumors do not always cause signs and symptoms in the beginning. The symptoms that you may experience will depend on the location of the tumor and whether it produces an excess of hormones.

In general, neuroendocrine tumors of the signs and symptoms may include:

  • The pain of a growing tumor
  • A growing lump can be felt under the skin
  • Feeling unusually tired
  • Losing weight without trying

Neuroendocrine tumors that produce excess hormones (functional tumors) can cause:

  • Redness of the skin.
  • Diarrhea
  • Frequent need to urinate
  • Increased thirst
  • Dizziness
  • Tremors
  • Skin rash

When to see a doctor

Make an appointment with your doctor if you have any persistent signs and symptoms that worry you.

Causes

The exact cause of neuroendocrine tumors is not known. These cancers start in neuroendocrine cells have traits similar to those of nerve cells and hormone-producing cells. The neuroendocrine cells are found throughout the body.

Neuroendocrine tumors begin when the neuroendocrine cells develop changes (mutations) in the DNA . The DNA inside a cell contains the instructions that tell the cell what to do. The changes, to tell the neuroendocrine cells that multiply quickly and form a tumor.

Some neuroendocrine tumors grow very slowly. Others are aggressive cancers that invade and destroy normal body tissues or spread (metastasize) to other parts of the body.

Risk factors

The risk of neuroendocrine tumors is higher in people who inherit genetic syndromes that increase the risk of cancer. Examples include:

  • Multiple endocrine Neoplasia type 1 (MEN 1)
  • Multiple endocrine Neoplasia type 2 (MEN 2)
  • The Von Hippel-Lindau disease
  • Tuberous sclerosis
  • Neurofibromatosis

Diagnosis

The tests and procedures that may be subjected to diagnose a neuroendocrine tumor depend on where the tumor is located in your body. In general, the tests can include:

  • Physical exam. Your doctor can examine your body to better understand your signs and symptoms. He or she can feel the presence of swollen lymph nodes or to search for signs that a tumor is producing excess hormones.
  • Tests to seek the excess of hormones. Your doctor may recommend a blood test or urine for signs of excess hormones that are sometimes produced by neuroendocrine tumors.
  • Imaging tests. You may undergo imaging tests, such as ultrasound, CT and MRI , to create images of your tumor. For neuroendocrine tumors, images are sometimes created using positron emission tomography (PET) with a radioactive tracer is injected into a vein.
  • The procedures for removing a sample of cells for testing (biopsy). To collect the cells, the doctor may insert a long, thin tube with a light and camera on the end into your lungs (bronchoscopy), the esophagus (endoscopy) or the rectum (colonoscopy), depending on your situation. Sometimes, the collection of a tissue sample requires surgery.

If there is a risk that your neuroendocrine tumor has spread to other parts of your body, you may have additional tests to determine the extent of the cancer.

Treatment

The options of treatment for your neuroendocrine tumor will depend on the type of tumor, its location, and if you are experiencing the signs and symptoms of excess hormones produced by the tumor.

In general, the neuroendocrine tumor treatment options may include:

  • Surgery. Surgery is used to remove the tumor. When possible, surgeons work to remove all of the tumor and part of the healthy tissue that surrounds it. If the tumor cannot be removed completely, it can help to remove as much as possible.
  • Chemotherapy. Chemotherapy uses strong drugs to kill tumor cells. It can be given through a vein in the arm or take it in pill form. Chemotherapy may be recommended if there is a risk that your neuroendocrine tumor may recur after surgery. Could also be used for advanced tumors that cannot be removed with surgery.
  • Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present in tumor cells. By blocking these abnormalities, targeted drug treatments can cause tumor cells to die. Targeted drug therapy is usually combined with chemotherapy for neuroendocrine tumors advanced.
  • Peptide receptor radionuclide therapy (PRRT). PRRT combines a drug that acts on cancer cells with a small amount of a radioactive substance. Allows the radiation to be delivered directly to the cancer cells. One PRRT of drugs, lutetium-177 Lu-dotatate (Lutathera), is used for the treatment of neuroendocrine tumors advanced.
  • Medications to control the excess of hormones. If your neuroendocrine tumor releases the excess of hormones, your doctor may recommend medications to control their signs and symptoms.
  • The radiation therapy. Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill tumor cells. Some types of neuroendocrine tumors may respond to radiation therapy. It might be recommended if surgery is not an option.

Other treatments may be available to you depending on your particular situation and your specific type of neuroendocrine tumor.

Coping and support

Being diagnosed with a neuroendocrine tumor can be stressful and overwhelming. With time, you will find ways to cope with the distress and uncertainty. Until then, you may find that it helps to:

  • Learn enough about your diagnosis in order to make decisions about your care. Ask your doctor about your neuroendocrine tumor, including your treatment options and, if you like, your prognosis. As you learn more about the type of neuroendocrine tumor that you have, you can be more confident in making treatment choices.
  • Turn to family and friends for support. Stay connected to your family and friends for support. It can be difficult to talk about your diagnosis, and it is likely to get a range of reactions to share the news. But talking about your diagnosis and transmit information about your cancer can help you to deal with, and the offers of practical assistance which is often the result.
  • Connect with other people with neuroendocrine tumors. Consider joining a support group, either in your community or on the internet. A support group of people with the same diagnosis can be a source of useful information, practical tips and encouragement.

Preparing for your appointment

Start by making an appointment with your family doctor if you have any of the signs or symptoms that worry you. Depending on your particular situation, your doctor may refer you to a specialist.

Because appointments can be brief, and because there is often a large amount of information to go over, it is a good idea to be well prepared. Here's some information to help you to be prepared and know what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there is anything that you need to do in advance, such as restrict your diet.
  • Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • Write down key personal information, including any major stresses or recent life changes.
  • Make a list of all medications, vitamins or supplements you are taking.
  • Consider the possibility of a family member or friend. Sometimes it can be difficult to take in all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write questions to ask their doctor.

Your time with your doctor is limited, so preparing a list of questions will help you to make the most of your time together. The list of questions from most important to least important in case time runs out. For neuroendocrine tumors, some basic questions to ask your doctor include:

  • What type of neuroendocrine tumor do I have?
  • Can you explain why my test results mean?
  • Do you recommend other tests or procedures?
  • What are my treatment options?
  • What side effects are likely to be with each treatment?
  • How will the treatment affect my daily life?
  • What treatment options do you think are the best for me?
  • When do I have to make a decision about my treatment?
  • Should I ask for a second opinion? What will that cost, and will my insurance cover it?
  • Are there brochures or other printed material that I can take with me? What sites do you recommend?

In addition to the questions that you've prepared to ask your doctor, do not hesitate to ask other questions that occur to you.

What to expect from your doctor

Your doctor may ask you a series of questions. Be ready to answer them may allow time later to cover other points you want to address. Your doctor may ask:

  • When did you first begin experiencing symptoms?
  • 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?
Symptoms and treatment of Neuroendocrine tumors