Patient education: Neuroendocrine neoplasms (The Basics)

What are neuroendocrine neoplasms? — 

Neuroendocrine neoplasms ("NENs") are a type of cancer. They are abnormal growths that start in specific types of cells in the body called "neuroendocrine" cells. NENs can release hormones into the body after a signal from the nervous system.

Neuroendocrine cells are found throughout the body. NENs most often form in the digestive tract (figure 1), pancreas (figure 2), or lungs. But they can form in other parts of the body, too.

NENs can grow quickly or slowly. Some release hormones into the bloodstream, which can cause symptoms.

You might also hear the terms "neuroendocrine tumor" ("NET") or "neuroendocrine carcinoma" ("NEC"). These are types of neuroendocrine neoplasms. Your doctor will tell you which type you have.

What are the symptoms of an NEN? — 

Some people have no symptoms. They might learn they have an NEN when they get an X-ray or another test for a different reason.

When symptoms do happen, they depend on the type of NEN and where it is in the body. For example:

●If the NEN is in the digestive tract, it can cause nausea, vomiting, belly pain, or diarrhea. If it is in the pancreas, other symptoms might include yellowing of the skin (called "jaundice"), increased stomach acid, low blood sugar (which can cause sweating or shaking), weight loss, or blood clots.

●If the NEN is in the lung, it can cause coughing, wheezing, or chest pain. Some people cough up blood. It can also cause pneumonia if it blocks part of the lung.

Sometimes, an NEN can cause "carcinoid syndrome." This is when the NEN makes hormones and other substances that get into the blood, causing symptoms. Carcinoid syndrome can cause:

●Episodes of "flushing," which is when the skin gets red or purple and feels hot. This can happen without warning or be triggered by eating certain foods, drinking alcohol, having a bowel movement, stress, or surgery.

●Spider veins (small, swollen veins), usually on the nose, cheek, or upper lip

●Watery diarrhea

●Wheezing (noisy breathing), or trouble breathing

●Swelling of the legs

●Feeling very tired

Will I need tests? — 

Yes. If your doctor suspects you have an NEN, they will do 1 or more of the following tests:

●Blood tests

●Urine tests

●MRI scan, CT scan, or other imaging tests – These create pictures of the inside of the body and can show abnormal growths. For some imaging tests, doctors inject a chemical called a "tracer" into a vein. This can help show where the NEN is in the body.

●Upper endoscopy or colonoscopy – For an upper endoscopy, a doctor puts a thin tube called an "endoscope" in the mouth and down the throat. The tube has a light on the end and a camera that sends images of the GI tract to a TV screen. For a colonoscopy, the doctor puts the tube in through the anus to look inside the colon.

●Biopsy – A doctor removes a small sample of tissue from the growth. Another doctor looks at the sample under a microscope to see if it has cancer.

What is cancer staging? — 

This is a way for doctors to find out if a cancer has spread past the layer of tissue where it began and, if so, how far.

The right treatment for you depends a lot on the type of NEN you have, its stage, and your other medical problems.

How are NENs treated? — 

Treatment might include:

●Surgery – Surgery can remove the cancer.

●Ablation therapy – This is a procedure that can kill cancer cells in the liver. It does not involve surgery. Doctors can do ablation therapy in different ways. They can kill the cancer cells using extreme cold, heat, microwaves, a laser, or radiation therapy.

●Blocking the cancer's blood supply – Doctors can do a procedure called "embolization" to block off the blood vessel that sends blood to the cancer. This keeps the cancer from growing by "starving" it of its blood supply. Sometimes, this procedure is combined with chemotherapy (called "chemoembolization") or radiation (called "radioembolization").

●Hormone-blocking medicines – These block the cancer from making the hormones or substances that cause symptoms.

●Chemotherapy – These are medicines that kill cancer cells or stop them from growing.

●Targeted therapy – Some medicines work only for cancers that have certain characteristics. Your doctor might test your cancer to see if it would respond to these medicines.

●Lutetium Lu 177-Dotatate (brand name: Lutathera) – This is a medicine that delivers radiation directly into the growth to kill the cancer cells. It is given through a thin tube that goes into a vein, called an "IV."

Your doctor might also prescribe medicines to help relieve symptoms like diarrhea.

What else should I do? — 

Follow all your doctor's instructions about visits and tests. It's also important to talk to your doctor about any side effects or problems you have during treatment.

Getting treated involves making many choices, such as what treatment to have. Always tell your doctors and nurses how you feel about a treatment. Any time you are offered a treatment, ask:

●What are the benefits of this treatment? Is it likely to help me live longer? Will it reduce or prevent symptoms?

●What are the downsides of this treatment?

●Are there other options besides this treatment?

●What happens if I do not have this treatment?

More on this topic

Patient education: Carcinoid syndrome (The Basics)