Neuroendocrine tumors, sometimes referred to as neuroendocrine cancers, carcinoid tumors, or NET cancers, are rare tumors that begin in hormone-producing cells found throughout the body.
NETs can start in nearly any organ, but are most common in the following areas:
- Small intestine, rectum, stomach, appendix or esophagus
- Pancreas
- Lungs
NETs can be slow-growing or aggressive. They may or may not grow outside of the body part they started in. Many NETs are found incidentally — or when looking for or treating something else.
Some neuroendocrine tumors cause the affected cells to produce higher than normal amounts of the chemical they’re responsible for making. These NETs are called functional neuroendocrine tumors. Functional NETs may cause symptoms related to excess hormone production.
Other types of NETs do not affect the cells’ function. These are called nonfunctional NETs. More than half of NETs are nonfunctional, and you may not feel any symptoms of your cancer.
Symptoms of Neuroendocrine Tumors
Most people don’t have any signs of a neuroendocrine tumor before they’re diagnosed. Depending on the location of your tumor, you may experience:
- Abdominal pain or cramping
- Diarrhea or other changes in bowel habits
- Red, warm or flushed skin
- Fatigue (tiredness)
- Persistent cough or wheezing
- Shortness of breath
- High blood sugar
- Stomach ulcers
Diagnosing Neuroendocrine Tumors
Most NETs are found when someone is being treated for something else. For example, during a screening colonoscopy or when getting a CT scan.
Once the tumor is identified, Rush can test the tumor to find out exactly what kind of tumor it is — and what treatment you need. A neuroendocrine tumor diagnosis usually involves a biopsy (taking a small piece of the tumor for testing) and blood tests (to check your hormone levels and tumor markers).
If it’s suspected you have a NET, advanced imaging scans can pinpoint the location and type of your tumor. Rush was the first hospital in Chicago to use the 68-Gallium Dotatate PET CT, a precise imaging tool for diagnosing NETs. We also offer the 111-in-pentetreotide single photon emission computed tomography (SPECT).
After these tests, you will get a tumor stage and grade. A cancer’s stage indicates the size of the tumor and if it has spread from its original site.
Grade specifies how abnormal the cancer cells look under a microscope. The more abnormal the cells, the higher the grade. Higher-grade cancers are generally more aggressive than lower-grade cancers.
A NET’s grade and stage play a big role in your treatment plan. Your doctor will discuss your stage and grade with you.
Meet our neuroendocrine tumor care providers
Treatment Options for Neuroendocrine Tumors at RUSH MD Anderson
Because NETs are rare (only 12,000 people in the U.S. will be diagnosed this year), it’s important to find a neuroendocrine tumor specialist. NETs behave differently than other kinds of cancer, and you need a team that is experienced at treating your kind of NET.
The RUSH MD Anderson Cancer Center team has extensive experience treating neuroendocrine tumors. Experts treating you may include medical oncologists, surgeons, radiation oncologists, nuclear medicine physicians, pathologists and nutritionists.
We work with you to understand your treatment goals and find a plan that works for you. Many times, people with NETs can show no evidence of disease after treatment. Sometimes, the cancer cannot be completely removed but can be medicated to slow or stop its growth and watched closely to monitor any changes.
Many people with neuroendocrine tumors need nonsurgical and surgical treatments.
Nonsurgical Treatment for NETs
Most people who have NETs will take some kind of medication to treat their cancer. This may include:
- Somatostatin analogues, or SSAs. They stop excess hormones from being made, reducing the symptoms you may have from NETs. There’s also many nonfunctional NETs that can benefit from this therapy. SSAs come in short- and long-acting formulas.
- Targeted therapy. These medications identify and treat specific cancer cells.
- Peptide receptor radionuclide therapy, or PRRT. This is a method of delivering radiation directly to a tumor. Instead of a beam of energy, a radioactive chemical is combined with another chemical that attaches to a cancer cell. This combined liquid is given in an IV. Rush was the first health system in the state to offer PRRT.
- Chemotherapy. This category of drugs works by killing fast-growing cells.
Surgical Treatment for NETs
Many NETs can be removed with surgery. What type of surgery depends on where your NET first developed and where it has grown.
Because NETs commonly start in the GI tract, you may need part of your bowel or stomach removed. If the NET is on your pancreas, you may need a Whipple procedure (where the head of the pancreas is removed) or a distal pancreatectomy (where the tail and body of the pancreas are removed). Liver resection, or removal of part of the liver, may also be part of your treatment plan.
NETs that start in the lung may require removing part of a lung. This is called a lobectomy.
No matter what kind of surgery you need, surgeons at RUSH MD Anderson will talk through your procedure and answer any questions you have. You will know what kind of recovery time to expect and what complications are possible.
Our experts will perform minimally invasive surgery whenever it’s an option, reducing your pain after surgery and lowering your risk of complications. Some procedures can be performed with the help of a surgical robot, allowing for a high level of precision.
Some tumors can be removed using an endoscope, or tube inserted through the mouth. This avoids the need for surgery.
Liver-Directed Therapies
Sometimes, NETs either start or grow inside the liver. RUSH MD Anderson offers treatments that directly address these NETs, including:
- Liver debulking surgery. Also called cytoreductive surgery, this procedure removes multiple tiny lesions that may form throughout the liver.
- Ablation. This procedure doesn’t require any cuts. During an ablation, extreme heat targets cancer cells.
- Histotripsy. A doctor uses a machine to aim sound waves at tumors or cancer cells.
- Embolization. This treatment uses the blood vessels in your liver as a channel to treat cancer. Transarterial chemoembolization (TACE) injects chemotherapy into the blood vessels that go to your liver, delivering a targeted dose of cancer-killing drugs. Hepatic artery embolization blocks blood flow to the liver, starving your tumor of blood and reducing its size and growth. Yittrium-90 radioembolization uses a small amount of radiation in the blood vessels of your liver to destroy cancer cells.
Getting a Second Opinion from RUSH MD Anderson Cancer Center
Have you been diagnosed with cancer? Consider a second opinion from RUSH MD Anderson. A second opinion can help you explore all your options and begin treatment with confidence and clarity.
RUSH MD Anderson offers easy access to second opinions, including free virtual intake visits with our knowledgeable nurse navigators for anyone in Illinois.
RUSH MD Anderson Excellence in Neuroendocrine Tumors
- A dedicated neuroendocrine tumor team: Because neuroendocrine tumors are rare and complex, it's important to make sure your care team has the necessary expertise and experience. At RUSH MD Anderson, you'll have a team of specialists working together throughout your care. The team includes medical oncologists, surgeons, gastroenterologists, diagnostic and interventional radiologists, nuclear medicine specialists, pathologists, geneticists and endocrinologists. You'll also have a dedicated team of nurses who are readily available to answer your questions and coordinate your appointments.
- Advanced diagnostic technology: The exact location of a neuroendocrine tumor is often hard to pinpoint. Rush was the first hospital in Illinois to offer 68-Gallium DOTATATE PET CT, the most sensitive diagnostic test for neuroendocrine tumors. It can pinpoint tumor location with over 90% accuracy.
- New treatment options: If you are not a good candidate for surgery to treat your neuroendocrine tumor, we offer other effective options. In fact, Rush was the first hospital in Illinois — and one of the first in the nation — to offer peptide receptor radionuclide therapy, commonly called PRRT. This advanced treatment uses targeted radiation to treat gastroenteropancreatic neuroendocrine tumors, or GEP-NETs. It's the one of the most effective therapies available if you have a GEP-NET and aren’t a candidate for surgery.
- Leaders in precision medicine: In some cases, your care team may recommend genetic testing to further tailor your treatment plan. Our neuroendocrine tumor team works closely with our precision oncology team to analyze your genetic and clinical data, which can help predict how your tumor may respond to targeted treatments. This process can pinpoint the treatments that are likely to be most effective against your specific tumor — including some therapies being tested in innovative clinical trials.
FAQs About Neuroendocrine Tumor
Many NETs have no signs or symptoms. You may feel nausea, have diarrhea or see red, inflamed skin.
Doctors aren’t sure what causes neuroendocrine tumors. Most people are not more likely to develop NETs if their parents or immediate family had them. But some rare genetic conditions may increase your risk.
Hormones are chemicals made by your body that carry messages. For example, insulin is a hormone that tells your body to absorb sugars from the blood into your cells. Testosterone and estrogen are hormones that tell your body to develop secondary sex characteristics.
The cells that produce hormones are found all over the body.
NETs are often found when someone is being treated for something else. Once a tumor is found, many tests are done to identify if it is a NET. Tests may include imaging, blood tests and biopsies.
Specialized imaging scans can be used to diagnose NETs. RUSH MD Anderson offers 68-Gallium DOTATATE PET CT to precisely identify where the tumor started. Blood tests (to check for tumor markers and hormone levels) as well as a biopsy may also be needed.
Many NETs are physically removed, either with surgery or a nonsurgical procedure. Most people who have NETs take medication. This may include a somatostatin analogue, targeted therapy or chemotherapy. Radiation (either PRRT or another form) can also treat NETs.
Your specific treatment plan will depend on the location, grade and stage of your NET.
It depends on the location, grade and stage of your NET. Your doctors will discuss all your treatment options with you. They may include medication, radiation, surgery or a nonsurgical procedure.
Yes. Many neuroendocrine tumors are fully removed with surgery and/or other treatment.
Once your tumor has been identified as a neuroendocrine tumor, it’s important to find a NET specialist who is experienced in treating neuroendocrine tumors. This is a rare and complex form of cancer.
Many doctors will work together to treat your NET. Your treatment team may include oncologists, surgeons, gastroenterologists, radiologists, nuclear medicine doctors and pathologists, endocrinologists and geneticists.
PRRT is a medical treatment for NETs. It combines a radioactive chemical with a nonradioactive chemical that takes the radiation directly to cancer cells.
A functional NET produces abnormal amounts of hormones. A nonfunctional NET does not impact a cell’s ability to function normally.
Many NETs have no symptoms. Depending on the location and type of NET, you may have nausea, diarrhea, stomach pain, wheezing and difficulty breathing.
Almost always. If you are not a good candidate for surgery, our team will talk with you about other options that can treat your neuroendocrine tumor.
If a tumor is found, your doctors will run many tests to figure out where the tumor started and what kind of tumor it is. During these tests, you may find out you have a NET. If you do, working with specialists is important because this is a rare and complex form of cancer.
If you have questions about treating your NET or aren’t confident in your care, a second opinion can be important. Second opinions for NETs allow you to ask all your questions, understand all your treatment options and feel confident about your care.
U.S. News & World Report ranked Rush University Medical Center among the best in the nation for cancer care.
