ON THIS PAGE: You will learn about the different types of treatments doctors use for people with a neuroendocrine tumor (NET) of the lung. Use the menu to see other pages.
This section explains the types of treatments, also known as therapies, that are the standard of care for a lung NET. “Standard of care” means the best treatments known. Information in this section is based on medical standards of care for lung NETs in the United States. Treatment options can vary from one place to another.
When making treatment plan decisions, you are also encouraged to discuss with your doctor whether clinical trials offer additional options to consider. A clinical trial is a research study that tests a new approach for management or new treatment. Doctors learn through clinical trials whether a new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of treatments, or new doses of standard drugs or other treatments. Clinical trials are an option for all stages of cancer. Your doctor can help you consider all your treatment options. Learn more about clinical trials in the About Clinical Trials and Latest Research sections of this guide.
How a lung NET is treated
In cancer care, different types of doctors who specialize in cancer, called oncologists, often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team and is especially important for people with a NET. Health care teams include other health care professionals, such as physician assistants, nurse practitioners, oncology nurses, social workers, pharmacists, counselors, dietitians, physical therapists, occupational therapists, and others. Learn more about the clinicians who provide cancer care.
Treatment options and recommendations depend on several factors, including the stage and grade of tumor, possible side effects, and the patient’s preferences and overall health. Learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. These types of conversations are called “shared decision-making.” Shared decision-making is when you and your doctors work together to choose treatments that fit the goals of your care. Shared decision-making is important for lung NETs because there are different treatment options and the treatment choice and order in which treatments are given is not always well defined. Learn more about making treatment decisions.
The common types of treatments used for a lung NET are described below. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care.
Surgery, also called resection, is the removal of the tumor and some surrounding healthy tissue during an operation. A surgical oncologist is a doctor who specializes in cancer surgery. The surgeon will consider where the NET is, its stage, and other factors in deciding upon a specific surgery, including how much tissue will be removed. The lungs have 5 lobes: 3 in the right lung and 2 in the left lung (see Medical Illustrations).
When it is possible, completely removing the entire tumor is the standard treatment. Most localized (early stage I to II and some stage III) lung NETs are successfully treated with surgery alone. The surgeon will usually remove some tissue surrounding the tumor, called a margin, in an effort to leave no traces of cancer in the body. Lymph nodes should be tested for cancer, and sometimes they will need to be removed.
Surgical options for a lung NET include:
Sleeve resection. The tumor is surgically removed, along with parts of the airway above and below the tumor. The airway is then reconnected. This procedure may be recommended for centrally located tumors.
Sublobar resection (wedge resection or segmentectomy). In this type of surgery, less than an entire lobe of the lung is removed. During a wedge resection, a small, wedge-shaped piece of the lung is removed. During a segmentectomy, 1 or more segments of a lobe are removed, but not an entire lobe. This surgery is used if the tumor is very small or located on the edge of a lung, away from the large airways. Clinical trials are studying these treatments.
Lobectomy. During this surgery, an entire lobe of a lung is removed. This surgery is the most common procedure for lung NETs.
Pneumonectomy. During this operation, an entire lung is removed. This surgery is often done if sleeve resection is not possible for a centrally located tumor. This type of surgery is more commonly performed on the left lung than the right lung because removing entire left lung has less impact on quality of life.
Lymph node dissection. Removal of the lymph nodes near the lungs is also common during surgery for a lung NET. Removing the lymph nodes is often required to be considered a complete surgical resection.
Liver resection. This operation removes cancer that has spread to the liver. It may not be recommended for all people with cancer that has spread to the liver. The goal of this surgery is not to eliminate the cancer, but it often helps relieve or reduce the symptoms of carcinoid syndrome or prolong the time until a next treatment is needed.
It is important to tell your surgical team if you have experienced symptoms of or have a diagnosis of carcinoid syndrome before your operation. This is because people who have carcinoid syndrome are at risk of experiencing a carcinoid crisis during surgery (see Symptoms and Signs). To avoid major complications from a carcinoid crisis, the anesthesiology team must be fully aware of this risk before surgery, so they can have treatment on hand to control carcinoid crisis if it occurs.
Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have. Learn more about the basics of cancer surgery.
Sometimes your doctor will recommend treatment after surgery that may include chemotherapy and/or radiation therapy (see below). Be sure to have a discussion with them about the risks and benefits of additional treatments after surgery.
If surgery is not possible, it is called an “inoperable” or "unresectable" tumor. In these situations, the doctor will recommend another type of treatment.
The treatment plan may include medications to paralyze or destroy tumor cells. Medication may be given to reach cancer cells throughout the body. When a drug is given this way, it is called systemic therapy. Medication may also be given locally, which is when the medication is applied directly to the tumor or kept in a single part of the body.
This treatment is generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.
Medications are often given through an injection, intravenous (IV) tube placed into a vein using a needle, or as a pill or capsule that is swallowed (orally). If you are given oral medications to take at home, be sure to ask your health care team about how to safely store and handle them.
The types of medications used for a lung NET include:
Peptide receptor radionuclide therapy (PRRT)
Each of these types of therapies are discussed below in more detail. A person may receive 1 type of medication at a time or a combination of medications given at the same time. Sometimes, they can also be given as part of a treatment plan that includes surgery and/or radiation therapy.
The medications used to treat NETs are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications.
It is also important to let your doctor know if you are taking any other prescription or over-the-counter medications or supplements. Herbs, supplements, and other drugs can interact with cancer medications, causing unwanted side effects or reduced effectiveness. Learn more about your prescriptions by using searchable drug databases.
Somatostatin is a hormone in the body that controls the release of several other hormones, such as insulin and glucagon. Somatostatin analogs are drugs that are similar to somatostatin and are used to control the symptoms created by the hormones released by a NET, also called carcinoid syndrome. They may also slow the growth of a NET, although they do not generally shrink tumors.
There are 2 somatostatin analogs used to treat NETs: octreotide and lanreotide. Neither of these medications has been approved by the U.S. Food and Drug Administration (FDA) for lung NETs, but when they are used, that use is based on how other NETs are treated, along with small studies conducted in people with lung NETs. Octreotide is available in 2 forms: short-acting is given under the skin (subcutaneously) and long-acting is given as an intramuscular (IM) injection. Short-acting is generally prescribed for people to control breakthrough symptoms of carcinoid syndrome. Lanreotide is given as a long-acting deep subcutaneous injection.
The most common side effects are pain at the injection site, headache, high blood sugar levels, the development of gallstones, and mild digestive system upset, such as bloating, nausea, and changes in bowel movements. Ask your health care team about how side effects can be managed.
Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells.
Not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. Although this testing may find specific abnormalities in a lung NET, at this time this information does not generally help with making treatment decisions for lung NETs. However, research studies continue to find out more about specific molecular targets and new treatments directed at them. Learn more about the basics of targeted treatments.
Everolimus (Afinitor) is currently the only targeted therapy approved by the FDA for the treatment of advanced lung NETs. It can help slow down the growth of these tumors in some people, but it does not usually shrink tumors. Side effects include mouth sores, rash, fluid retention or edema, lowering of blood counts, diarrhea, stomach pain, nausea, loss of appetite, fatigue, and less commonly, high blood sugar or cholesterol levels.
Other targeted therapies for lung NETs are being researched in clinical trials. They include drugs that interfere with new blood vessel formation or with specific survival pathways of cancer cells. Talk with your doctor about possible side effects for a specific medication and how they can be managed.
Your doctor may recommend running laboratory tests on a tumor sample to identify specific genes, proteins, and other factors unique to the tumor that may be targeted by certain medications.
Chemotherapy is the use of drugs to destroy tumor cells, usually by keeping the cells from growing, dividing, and making more cells. Chemotherapy for a lung NET is most often used when the tumor has spread to other organs, is growing quickly, or is causing severe symptoms. In a small proportion of patients, chemotherapy can result in tumor shrinkage.
A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or a combination of different drugs given at the same time. The most common chemotherapy regimens for a lung NET include:
Carboplatin (available as a generic drug) or cisplatin (available as a generic drug), plus etoposide (available as a generic drug)
Temozolomide (Temodar), with or without capecitabine (Xeloda)
FOLFOX, a combination of fluorouracil (5-FU) with leucovorin (folinic acid) and oxaliplatin (Eloxatin)
CAPOX, a combination of capecitabine and oxaliplatin
The side effects of chemotherapy depend on the individual, the type of drugs, and the dose used, but they can include fatigue, low blood counts, risk of infection, nausea and vomiting, hair loss, loss of appetite, numbness or tingling in fingers and toes (neuropathy), and diarrhea. Not all chemotherapies cause hair loss. These side effects usually go away after treatment is finished. In rare cases, some chemotherapy drugs increase a person’s risk of developing secondary cancers. Talk with the doctor about possible side effects of chemotherapy in your treatment plan.
Learn more about the basics of chemotherapy.
A treatment called 177Lu-dotatate (Lutathera) is approved by the FDA for treating advanced pancreas NETs and advanced gastrointestinal (GI) tract NETs that are bright and easily seen on a positron emission tomography (PET) scan. This is a form of radiation therapy (see below) and is called peptide receptor radionuclide therapy (PRRT).
PRRT, specifically 177Lu-dotatate (Lutathera), is being studied for the treatment of some lung NETs but is not approved by the FDA. It is a radioactive drug that works by binding to a cell’s somatostatin receptor, which may be present on certain tumors. After binding to the receptor, the drug enters the cell, allowing radiation to damage the tumor cells.
Common side effects of this drug include nausea and vomiting, fatigue, and decreased appetite. Talk with your doctor about ways these can be avoided or managed. There are also rare but serious risks of kidney problems and secondary blood cancers, such as myelodysplastic syndrome and leukemia. Other forms of PRRT are also being researched in clinical trials.
Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time. A doctor who specializes in giving radiation therapy to treat a tumor is called a radiation oncologist.
The most common type of radiation treatment for a lung NET is called external-beam radiation therapy, which is radiation given from a machine outside the body. It is most often used as part of palliative care to relieve symptoms, such as pain, caused by cancer that has spread to the bone or other areas of the body (see “Physical, emotional, and social effects of a tumor” below). Occasionally, radiation therapy is used to treat a tumor that cannot be removed with surgery but otherwise has not spread.
Patients receiving radiation therapy may experience fatigue during treatment, and the skin in the treated area may become red and dry. Radiation therapy to the chest or neck may cause a dry, sore throat, difficult or painful swallowing, or a dry cough. Some patients have shortness of breath during or after radiation therapy. A condition called pneumonitis, or inflammation of the lungs, can occur after radiation therapy to the lungs and may cause symptoms of shortness of breath or cough, which can often be treated with steroids. Most side effects go away after the treatment is finished.
Learn more about the basics of radiation therapy.
If cancer has spread to the liver, the treatments below may be recommended. These procedures are usually performed by an experienced interventional radiologist and may require an overnight hospital stay. Side effects may include pain around the liver, fever, and higher levels of liver enzymes as measured by blood tests for a few days or weeks after treatment.
Radiofrequency ablation (RFA). RFA destroys a tumor by heating it with an electric current. It is usually used for small liver metastases and does not work well on larger tumors.
Hepatic artery embolization. This procedure blocks the tumor’s blood supply by sealing off the blood vessels leading to the tumor. If embolization is done by itself, it is called bland embolization. When combined with chemotherapy, it is called chemoembolization. When it is combined with radiation, it is called radioembolization.
It is not yet clear if one form of embolization treatment is more effective than the other. These treatments are usually used for people with metastatic disease that mostly affects the liver, in particular those who have symptoms caused by the size of the tumor or by hormones produced by the tumor.
A lung NET and its treatment can cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative and supportive care. This often requires a team approach. It is an important part of your care that is included along with treatments intended to slow, stop, or eliminate the cancer.
Palliative and supportive care focuses on improving how you feel during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of the tumor, may receive this type of care. And it often works best when it is started right after your diagnosis. People who receive palliative and supportive care along with treatment for the tumor often have less severe symptoms, better quality of life, and report that they are more satisfied with treatment.
Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. You may also receive palliative treatments, such as chemotherapy, surgery, or radiation therapy, to improve symptoms.
For some people, such as those with high-risk heart disease or other specific medical conditions that increase the risk of problems from surgery, surgery cannot be used to successfully treat the lung NET. In these cases, less-risky procedures to relieve symptoms may be helpful. This may include treating symptoms of blocked airways by removing most of the tumor through a bronchoscope or vaporizing portions of the tumor with a laser. Bronchoscopic approaches may be used together with radiation therapy.
Before treatment begins, talk with your doctor about the goals of each treatment in the recommended treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative and supportive care options. Many patients also benefit from talking with a social worker and participating in support groups. Ask your doctor about these resources, too.
Medical care is often expensive, and navigating health insurance can be difficult. Ask your doctor or another member of your health care team about talking with a financial navigator or counselor who may be able to help with your financial concerns.
During treatment, your health care team may ask you to answer questions about your symptoms and side effects and to describe each problem. Be sure to tell the health care team if you are experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.
If the lung NET spreads to another distant part in the body from where it started, doctors call it metastatic cancer. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Clinical trials might also be an option. Learn more about getting a second opinion before starting treatment, so you are comfortable with your chosen treatment plan.
Your treatment plan may include a combination of the types of treatment described above. It is very uncommon to use surgery to treat metastatic disease. If it is used, surgery is used to control disease locally or relieve symptoms rather than eliminate the cancer at this stage (see above). Surgery to remove liver metastases may be performed, sometimes in people with carcinoid syndrome in an effort to reduce hormone levels or delay the time to when the next treatment is required. Local bronchoscopic procedures or radiation therapy may be used to relieve symptoms if a tumor blocks an airway. Radiation therapy may also be offered to help relieve symptoms, such as bone pain.
Participation in clinical trials is encouraged. Palliative and supportive care will also be important to help relieve symptoms and side effects. Systemic therapy with medication is an important part of the treatment for metastatic disease. However, observation may be an option for those with a very-slow-growing tumor. Observation means that doctors closely monitor the patient using physical examinations, imaging tests, and/or laboratory tests on a regular basis. Active systemic treatment with medication only begins if the person develops symptoms or tests indicate that the cancer is getting worse.
For many people, a diagnosis of metastatic cancer is very stressful and difficult. You and your family are encouraged to talk about how you feel with doctors, nurses, social workers, or other members of your health care team. It may also be helpful to talk with other patients, such as through a support group or other peer support program.
A remission is when the lung NET cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED.
A remission may be temporary or permanent. This uncertainty causes many people to worry that the NET will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the tumor returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if it does return. Learn more about coping with the fear of recurrence.
If the lung NET returns after the original treatment, it is called a recurrent lung NET. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).
If a recurrence happens, a new cycle of testing will begin to learn as much as possible about it. After this testing is done, you and your doctor will talk about the treatment options. Depending on the specific features of the recurrence, the treatment plan may include the treatments described above, such as surgery, medications, and radiation therapy, but they may be used in a different combination or given as separate lines of treatment. Your doctor may suggest clinical trials that are studying new ways to treat a recurrent lung NET. Whichever treatment plan you choose, palliative and supportive care will be important for relieving symptoms and side effects.
People with a recurrent tumor sometimes experience emotions such as disbelief or fear. You are encouraged to talk with your health care team about these feelings and ask about support services to help you cope. Learn more about dealing with cancer recurrence.
For some people with advanced, incurable lung NETs, there may be a point where there are no further treatment options and the cancer can no longer be controlled.
This diagnosis is stressful, and for some people, this situation is difficult to discuss for many people. However, it is important to have open and honest conversations with your health care team to express your feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help. Making sure a person is physically comfortable, free from pain, and emotionally supported is extremely important.
Planning for your future care and putting your wishes in writing is important, especially at this stage of disease. Then, your health care team and loved ones will know what you want, even if you are unable to make these decisions. Learn more about putting your health care wishes in writing.
People who have advanced, incurable cancer and who are expected to live less than 6 months may want to consider hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to talk with your doctor or a member of your palliative care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Learn more about advanced cancer care planning.
After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.
The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with a lung NET. Use the menu to choose a different section to read in this guide.