Neuroendocrine Tumor Treatment
Since neuroendocrine tumors (NETs) are rare, finding a care team with experience treating them is especially important. At MD Anderson, we have physicians who specialize in treating neuroendocrine tumors. This gives them incredible experience when designing treatment plans for patients diagnosed with a NET.
Many of these doctors also conduct research in NETs and are working to develop new treatments for these tumors. As a result, MD Anderson has a large number of clinical trials for NET patients. These offer new treatments that may not be available anywhere else.
NET treatment plans
Regardless of location, the preferred treatment for most NETs is surgery to remove the entire tumor. This may cure the patient.
Most surgeries meant to cure NETs are performed on patients whose disease has not metastasized, or spread. In some cases, though, patients whose disease has metastasized can be effectively cured by surgery that removes the primary tumor and the metastases.
For the vast majority of patients, it is not possible to remove the entire tumor through surgery. This may be because it is located in a spot that is hard to reach surgically or because it has spread in such a way that the metastases cannot be removed. In these cases, doctors may still perform surgery to remove part of the tumor. This can help relieve symptoms.
If all of the patient’s tumors cannot be removed through surgery, a complete cure is not possible. Instead, the disease is managed through treatments designed to slow or stop its progression.
NET treatments
The treatments and treatment plans for a NET depend on many factors. These include the location of the tumor, whether it is functional or non-functional, and how advanced the disease is.
Active surveillance
Since NETs can be slow growing, doctors may recommend active surveillance. With this approach, the patient undergoes regular tests, including blood, urine and imaging exams, to see if the tumor is growing. No treatments are given unless and until the disease progresses. This is a popular approach for older patients who might find surgery or other therapies difficult to bear.
Surgery
Many neuroendocrine tumors can be removed with surgery, especially if they are diagnosed before they have metastasized, or spread beyond the main tumor and nearby lymph nodes. If the surgeon cannot remove the entire tumor, only part of it may be removed in order to help treat a patient’s tumor-related symptoms.
The patient’s surgical options will depend on the tumor’s location. The procedures are essentially identical to surgeries for other types of tumors at that site. For example, if a patient has lung cancer, the surgery to treat that tumor is the same, regardless if it is a neuroendocrine tumor or not. To see the surgical options for neuroendocrine tumors at a particular site, learn more about that specific cancer.
Chemotherapy
Chemotherapy works by killing fast-growing cells, including cancer cells. Learn more about chemotherapy.
Targeted Therapy
Traditional chemotherapy works by killing cells that multiply quickly, whether normal or cancerous. Targeted therapy, also called precision medicine, is different. It works by stopping or slowing the growth or spread of cancer.
This happens on a cellular level. Cancer cells need specific molecules (often in the form of proteins) to survive, multiply and spread. These molecules are usually made by the genes that cause cancer, as well as the cells themselves.
Targeted therapies are designed to interfere with, or target, these molecules or the cancer-causing genes that create them. Learn more about targeted therapy.
Radiation therapy
Radiation therapy uses focused beams of energy to destroy cancer cells. There are several ways doctors can deliver this radiation to the tumor. Learn more about radiation therapy.
Peptide radionuclide therapy
Peptide radionuclide therapy is a method of delivering cancer-killing radiation to a tumor. Instead of using a beam of energy, a radioactive chemical is combined with another chemical that attaches to cancer cells. The patient receives this combined liquid through an IV.
Somatostatin analogs
Somostatin analogs are drugs that slow tumor growth and help relieve cancer symptoms. While they were initially used only for functional NETs (NETs that produce hormones) more recent studies show they can work on non-functional NETs as well.
Liver-directed therapies
When NETs metastasize, or spread beyond their primary location, they often move to the liver. Liver directed therapies use a catheter sent through an artery to deliver treatments directly to the metastasis. Treatments include chemotherapy and the use of radioactive beads that can kill cancer cells. Doctors also can use the catheter to heat and kill blood vessels that feed the tumor.
Learn more about neuroendocrine tumors:
NETs can form in any part of the body. Two of the most common locations are the gastrointestingal tract and the lungs.
10 things to know about neuroendocrine tumors
About 12,000 people in the United States will be diagnosed with neuroendocrine tumors — sometimes called carcinoid tumors — each year. But what exactly are these tumors, where are they typically found and are they cancerous?
We spoke with neuroendocrine tumor specialist Daniel Halperin, M.D., to learn more.
What are neuroendocrine tumors, and where are they usually found?
While the name “neuroendocrine” implies that these tumors involve both nerve cells and hormones, they are mainly thought to come from endocrine cells. The “neuro-” is more of a quirk of history.
Neuroendocrine tumors are cancers that can develop anywhere endocrine cells are present. Endocrine cells help regulate various body functions, such as growth, reproduction and metabolism. They are distributed throughout the body, but the most common places for tumors to develop from them are in the:
- lungs
- small intestines
- pancreas
Are all neuroendocrine tumors cancerous?
The short answer is that neuroendocrine tumors are almost always cancerous. But each situation is unique, so it's impossible to capture the entire breadth of this disease in a simple yes or no answer.
Some neuroendocrine tumors are only classified as such because of how they appear under a microscope. But from a clinical standpoint, they almost never grow or spread, whether we do anything about them or not. Those cases only account for about 1% of all neuroendocrine tumors. But it’s important to mention them.
In some of the older systems for classifying neuroendocrine tumors, pathologists would describe them as either “benign” (non-cancerous) or “malignant” (cancerous), based on what the tumor cells looked like under a microscope. Unfortunately, those terms did not always accurately reflect whether the tumor would metastasize and behave aggressively or not, so they have been abandoned.
Today, almost all neuroendocrine tumors are considered malignant. There are just differences in how aggressive they are. Some spread easily. Others do not. But we consider all of them cancer.
What are the most common symptoms of neuroendocrine tumors?
We tend to see neuroendocrine tumor symptoms in two categories:
- Hormonal neuroendocrine symptoms include severe diarrhea, severe gastric ulcers, or uncontrolled blood sugar that responds poorly to treatment. The hormones produced can vary depending on the place in the body where the tumor originates.
- Mechanical neuroendocrine symptoms relate to the function of one part of the body, such as a small bowel obstruction, or pain in a particular place. These symptoms occur because the tumor is physically pushing on another structure.
How are neuroendocrine tumors usually diagnosed?
Most patients fall into one of two groups.
Some seek help for symptoms, such as pain due to metastatic cancer. This most commonly occurs in the liver. So a patient will go to the doctor, thinking they have a gallbladder issue, and it turns out to be a neuroendocrine tumor in the liver. Other patients in this group will exhibit hormonal symptoms, which point doctors in the direction of a tumor as the source.
For others, the cancer is detected purely by chance. Maybe it was picked up during a colonoscopy, or during an X-ray or CT scan for a broken bone. These patients have no symptoms before their diagnosis.
How are neuroendocrine tumors typically treated?
We have more neuroendocrine tumor treatment options than ever before. They fall into two categories.
- Local treatments include surgery. Surgery is most useful when a tumor is just in one place or only one tumor (out of many) is causing a problem. But it may still be appropriate in certain other circumstances, even after a neuroendocrine tumor has spread. Our interventional radiologists have several technologies they can use to control individual tumors, too, particularly in the liver.
- Systemic treatments are treatments we can give that target the cancer wherever it is in the body, even once it has spread. These include conventional chemotherapy, hormone therapy and targeted therapies.
The newest option is a type of systemic radiation called peptide receptor radionuclide therapy. Most neuroendocrine tumors have specific hormone receptors, so instead of giving patients the hormone therapy itself, we can use the hormone to drag the radiation to wherever the cancer is and bind to it, sparing most of the rest of the body from exposure.
This type of therapy was approved by the U.S. Food & Drug Administration in 2018. One study showed it reduced the rate at which neuroendocrine tumors progressed by 80%, so it’s a great option for some neuroendocrine patients.
It's important to note that there’s no “typical” treatment sequence for neuroendocrine tumors, though. There are hundreds of possibilities. And, each patient and care team will determine their specific treatment sequence based on their particular clinical situation as it evolves.
Can neuroendocrine tumors be cured?
Outcomes vary a lot when treating this disease. So, there’s no across-the-board answer.
But when we think about how a tumor is going to behave, we consider its:
- grade
- stage, and
- primary location.
When a tumor hasn’t spread yet, there’s a possibility of a cure, assuming it’s safe and appropriate to cut it out.
For some patients, though, surgery may carry more risks than the cancer itself. So, in those situations — especially if a tumor is not growing or spreading or causing troublesome symptoms — we may decide to just watch it carefully.
We can treat neuroendocrine tumors that have spread, but right now, we cannot cure metastatic neuroendocrine tumors.
Why are neuroendocrine tumors so challenging to treat?
Many patients with neuroendocrine tumors have hormonal symptoms that get progressively worse. So, they can experience symptoms for an average of five to seven years before finally receiving a neuroendocrine tumor diagnosis.
Because they have gone undiagnosed or misdiagnosed for so long, it can be very hard for some patients to rebuild a trusting relationship with their medical team. By the time they have an accurate diagnosis, they have often already moved from doctor to doctor seeking help, hearing over and over, “I don’t do this very often, so it’s not my strength,” or “What you have is rare and very weird.”
But whatever diagnosis you may have, it’s not rare or weird to us at MD Anderson. We care for hundreds of people with neuroendocrine tumors each year, so this will not be our first time treating them. And we are very happy to work together to find the best treatment plan for you.
Why should patients come to MD Anderson for neuroendocrine tumor treatment?
MD Anderson’s strength is two-fold.
First, we have specialists who care for patients with these tumors all day, every day. And we see plenty of rare and unusual cases even among neuroendocrine tumors. So our neuroendocrine tumor specialists have a lot of individual and collective experience deciding on which treatments to use and in what order.
Second is our clinical trial options. Most of the FDA-approved treatments for neuroendocrine tumors were tested at MD Anderson through clinical trials. We have been one of the largest contributors to their development, and we’ve played a substantial role in the studies of almost all of the drugs that have been approved in this field.
That means our patients had access to them long before most people in the general population. One example is everolimus, which James Yao, M.D., was studying as early as 2006 – six years before it was approved by the FDA.
We also played a leading role in establishing peptide receptor radionuclide therapy as a first-line option for patients with more aggressive neuroendocrine tumors through the NETTER-2 study. That means many of our patients contributed to that progress while also benefitting from the approach over the past 2 to 3 years.
Are there any clinical trials for patients with neuroendocrine tumors?
Yes. Many clinical trials are building on the success of peptide receptor radionuclide therapy right now. It’s not just one treatment; it’s more of an overarching concept of therapy.
The idea is that you use a particular protein on the front end to bind to cells and deliver a payload of radiation to your target at the back end. But you can change out the front or the back to get different combinations with different capabilities.
Lutathera, which uses Lutetium-177, is the only drug on the market that does this right now, but we have several clinical trials ongoing with it or other agents in the same family. One Phase III trial (NETTER-2) recently showed it could reduce the risk of disease progression by up to 72% when used as a front-line therapy for patients with more aggressive tumors. And we’re making progress toward having new agents deliver the radiation, such as actinium, another radioactive isotope.
Other clinical trials are building on targeted therapies and immunotherapy. Like many people, we are extremely interested in the promise of immunotherapy to one day help our patients live longer and better lives. The data we have right now suggests it’s not easy to use immunotherapy against neuroendocrine tumors, but we are looking at more combinations of agents and trying to figure out how to break the immune tolerance of these tumors.
Anything else you want newly diagnosed patients and their families to know about neuroendocrine tumors?
Where you go first for treatment matters. And the sequencing of that treatment matters. We believe that patients benefit most when they get the right treatment at the right time with doctors who specialize in treating these complex diseases.
We’re very proud that MD Anderson is one of the few places with such an experienced and dedicated team in this area.
Request an appointment at MD Anderson online or call 1-877-632-6789.
How surgery can treat neuroendocrine tumors in the gastrointestinal tract
From your growth to your digestion, your hormones regulate many basic functions within your body. These chemical messengers are produced and released into the blood by neuroendocrine cells. If the cells malfunction, they can develop into a neuroendocrine tumor. Because neuroendocrine cells can be found throughout the body, these tumors can, too. But they’re most commonly found in the gastrointestinal tract (GI) at the rectum, the small bowl and the pancreas.
If you’ve been diagnosed with a GI neuroendocrine tumor, you may be told you need surgery. We talked with Jess Maxwell, M.D., about surgery options for treating these tumors and what to expect.
How can surgery benefit a patient with a neuroendocrine tumor in the gastrointestinal tract?
20% of patients have what’s called a functional tumor. That means they experience symptoms linked to hormones. For example, a tumor may produce insulin and result in low blood sugar. Or, a tumor can produce glucagon, which can lead to diabetes or, if it produces gastrin, cause ulcers.
Removing even a portion of a functional tumor can improve quality of life by relieving some hormone-related symptoms. And in some cases where we can’t remove the entire tumor, surgery can extend a patient’s life.
Is surgery done before or after chemotherapy for patients with neuroendocrine tumors?
It depends. Most neuroendocrine tumors are slow-growing, so we have time to evaluate all treatment options. At MD Anderson, our multidisciplinary team works together to determine the best treatment plan, including which therapies are needed and when.
Because patients can live years after a neuroendocrine tumor diagnosis, we’re mindful about the order of treatments. One treatment’s effectiveness can make another more successful. And some treatment side effects can make other treatments more challenging. Keeping surgery as a treatment option for every patient is helpful, especially because chemotherapy isn’t effective for everyone. Don’t be surprised if surgery is our recommended first-line treatment.
What are the types of surgery for a pancreas neuroendocrine tumor?
There are three approaches, and the right one for you depends on your specific diagnosis, the location of the tumor and your overall health.
If the tumor is on the head of the pancreas, you’ll most likely receive a Whipple procedure. A surgeon removes the head of the pancreas, the distal bile duct, the gallbladder, regional lymph nodes, and the duodenum, which connects the small intestine to the stomach.
If the tumor is in the tail or body of the pancreas, we’ll remove that area of the organ with an approach called a distal pancreatectomy.
Other times, we’re able to remove only the tumor and a small amount of the surrounding healthy tissue through enucleation. This approach allows us to preserve the pancreas and limit long-term side effects. For example, losing a larger portion of your pancreas with a Whipple or distal pancreatectomy can cause or worsen diabetes, requiring you to take artificial insulin and possibly follow a low-sugar, low-carbohydrate diet.
All three procedures can be done either minimally invasively or in an open fashion. It’s dependent on if and where the cancer has spread, as well as if we see signs of the cancer cells invading the blood vessel.
How is a small bowel neuroendocrine tumor treated with surgery?
For a small bowel neuroendocrine tumor, we perform open surgery, which means we make a larger incision down your abdomen to allow for a full view of your organs. That’s because 30% to 45% of patients have tiny tumors throughout the small bowel that can’t be detected through the small incisions of a minimally invasive approach. We feel for the tumors through an exploration that only comes with an open surgery.
Unfortunately, we see patients who come to MD Anderson with a recurrence only a few years after their initial surgery elsewhere because something was missed with the minimally invasive approach. These tumors are rare, and many surgeons out in the community don’t have the experience with these complexities. That’s the benefit of coming to a cancer center like MD Anderson where we have surgeons who specialize in treating your specific type of cancer and see hundreds of cases a year and who work with medical oncologists and other experts who also specialize in treating your disease.
Is surgery an option if a neuroendocrine tumor has spread?
It’s important to understand that a neuroendocrine tumor in the pancreas isn’t the same as pancreatic cancer. Even though surgery isn’t usually an option for advanced pancreatic cancer, it can be beneficial for patients with advanced pancreas neuroendocrine tumors.
When they occur in the gastrointestinal tract, neuroendocrine tumors most commonly spread to the liver. Although the surgery becomes more complex, it can still be an option. We use a tissue-sparing approach to remove only the tumor and little surrounding tissue. This helps maintain the patient’s liver function for long as possible, which is important because you’re more likely to receive other treatments for the tumors in the liver. But with this tissue-sparing approach, you may need the same surgery in the future, if the cancer returns.
Does the surgery leave a scar?
You’ll have a scar on your stomach, but it will vary on which procedure you receive. Patients undergoing a minimally invasive approach will have five or six smaller incisions plus a slightly larger opening to allow the tumor to be removed. Patients undergoing an open surgery will have a longer vertical incision down the stomach. If the cancer has spread to the liver, we’ll make that vertical incision plus it’ll hook toward your right, in the shape of a J.
What is recovery like for patients who undergo surgery for a neuroendocrine tumor in the GI tract?
At MD Anderson, our patients typically recover in the hospital for few days to a week. How soon you get to leave the hospital depends on how quickly your bowel function comes back and how productive it is. There’s data that shows patients who walk after surgery can speed up that recovery. Our patients often receive prehabilitation to ease their recovery. Prior to going to the operating room, they receive guidance on nutrition and fitness so that when we ask them to take a walk a few hours after their surgery, they're prepared.
It’s hard work, but we need to all be on the same page and be ready to take this head on as a team. If we’re going to help you successfully overcome the cancer, you have to get yourself in that frame of mind rather than take it lying down – literally.
What’s your advice for someone newly diagnosed with a neuroendocrine tumor?
When deciding where to receive care for a neuroendocrine tumor, it’s important to know these tumors are rare. To ensure you get the best possible outcome, expertise matters. And the number of surgeries a doctor performs is just part of the picture. A specialized cancer center like MD Anderson will have the resources to support you throughout your cancer experience, from a pathologist specializing in your tumor type to cutting-edge clinical trials. Those resources allow us to personalize your care, so that you’re able to have the best possible results.
Request an appointment at MD Anderson online or by calling 1-877-632-6789.
