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For people with metastatic carcinoid tumors who have carcinoid syndrome (facial flushing, diarrhea, wheezing, rapid heart rate), several medicines can help control the symptoms and may help keep the tumor from growing for a time.
Octreotide
This drug is related to somatostatin, a natural hormone that seems to help slow the growth of neuroendocrine cells. It is very helpful in treating the symptoms of carcinoid syndrome. Sometimes octreotide can temporarily shrink carcinoid tumors, but it does not cure them. Side effects can include pain or burning at the injection site, stomach cramps, nausea, vomiting, headaches, dizziness, and fatigue.
The original version of octreotide (Sandostatin®) is given as an injection under the skin (subcutaneously) at least twice daily. Some people can learn to give this injection themselves at home. A newer, long-acting version of the drug (Sandostatin LAR®) is given as an injection into a muscle once a month by your doctor or nurse. When first starting treatment, most people are given injections every day. Once the doctor finds the correct dose, the longer-acting monthly injection may be used.
Lanreotide
Lanreotide (Somatuline®) is a drug similar to octreotide. It is given as an injection under the skin once a month. It may be given by your doctor or nurse, or you may learn how to give the injection at home. Side effects are similar to those of octreotide, although pain at the injection site is less common.
Interferons
These drugs are natural substances in the body that normally help activate the immune system. They also suppress the growth of some tumors. Interferon alfa is sometimes helpful in shrinking or slowing the growth of metastatic carcinoid tumors and improving symptoms of carcinoid syndrome. But its usefulness is limited by its flu-like side effects, which may be severe. It can also cause depression. Interferon alfa is given by injection, either daily or several times a week.
Targeted drugs
In recent years, anti-cancer drugs that work differently from standard chemotherapy drugs have been developed for some types of cancer. These drugs target specific parts of cancer cells. They are sometimes helpful even when chemotherapy is not, and they often have less severe side effects.
Two targeted therapy drugs, sunitinib (Sutent®) and everolimus (Afinitor®), have been shown to help treat neuroendocrine tumors that start in the pancreas. These drugs may also be helpful against carcinoid tumors, which are a type of neuroendocrine tumor. Studies are now in progress to try to prove this, but some doctors may already be using these drugs for carcinoid tumors.
Other medicines can be used to help control specific symptoms. It is important to describe your symptoms to your doctor so that they can be treated effectively.
Radiation therapy for lung carcinoid tumors
Radiation therapy is the use of high-energy radiation in the form of x-rays or radioactive particles to kill cancer cells. Unfortunately, radiation therapy usually has only a limited effect on lung carcinoid tumors.
Surgery is the main treatment for most carcinoid tumors, but radiation therapy may be an option for those who can't have surgery for some reason. It may also be given after surgery in some cases if there's a chance some of the tumor was not removed. Radiation therapy can also be used to help relieve symptoms such as pain if the cancer has spread to the bones or other areas.
External beam radiation therapy
External beam radiation therapy uses a machine that delivers a beam of radiation to a specific part of the body. This is the type of radiation used most often for lung carcinoid tumors.
Before your treatments start, the radiation team will determine the correct angles for aiming the radiation beams and the proper dose of radiation. Radiation therapy is much like getting an x-ray, but the radiation is more intense (stronger). The procedure itself is painless. Each treatment lasts only a few minutes, but the setup time – getting you into place for treatment – usually takes longer. Most often, radiation treatments are given 5 days a week for several weeks.
The main side effects of lung radiation therapy are fatigue (tiredness) and temporary sunburn-like skin changes. If high doses are given, radiation therapy can cause scar tissue to form in the lungs, which can lead to trouble breathing and an increased risk of infections.
Radioactive drugs
Drugs containing radioactive particles may be useful in treating some widespread carcinoid tumors. For this type of treatment, doctors use some of the same drugs used in radionuclide scans , such as MIBG and octreotide. These drugs attach to carcinoid tumor cells. By using more strongly radioactive particles than are used in the scans, doctors can deliver higher doses of radiation directly to the tumors. Some early results have been promising, but this type of treatment is not widely used at this time.
Treatment of lung carcinoid by type and extent of disease
The treatment of lung carcinoid tumors depends to a large extent on the type (typical versus atypical) and extent of the cancer. Other factors, such as a person's overall health and ability to withstand surgery, are also important.
Many doctors use the TNM staging system to formally describe the extent of these cancers. But for treatment purposes most doctors use a simpler system, dividing these tumors into 2 groups: those that can be treated with surgery (resectable cancers) and those that can’t be completely removed (unresectable cancers).
Resectable carcinoid tumors
Resectable carcinoid tumors haven’t spread far beyond where they started and can be completely removed. In the TNM staging system, this includes most stage I, II, and IIIA cancers.
These cancers are treated with surgery. The extent of the surgery depends on the type of carcinoid tumor and the size and location of the cancer. Atypical carcinoids may need more extensive surgery than typical carcinoids. Nearby lymph nodes are usually removed as well, especially if you have an atypical carcinoid.
Most patients with resectable lung carcinoid tumors are cured with surgery alone and don't need other treatments. Some experts recommend additional treatment for people with an atypical carcinoid that has spread to lymph nodes. This can be chemotherapy, radiation therapy, or both. But it’s not clear if the added therapy lowers the chance of the cancer coming back, or if it helps people live longer.
Unresectable carcinoid tumors
Unresectable carcinoid tumors include those that have grown too much or spread too far to be completely removed by surgery (including most stage IIIB and stage IV cancers), as well as tumors in people who are not healthy enough for surgery.
Treatment depends on where the cancer is and whether you have symptoms of the carcinoid syndrome. In general, this is a slow-growing cancer, and chemotherapy has not proven to be very successful. If you have only a small number of tumors that can be removed, surgery (on both the lung and at the site of metastasis) is likely to be your best option.
Lung carcinoid tumors usually spread to the liver first. If the carcinoid has spread only to your liver but can't be removed there with standard surgery, another option might be to have a liver transplant. This is a very involved operation that most doctors still consider experimental. It is done at only a few transplant centers.
If the carcinoid is in your liver and is causing symptoms, procedures such as ablation or hepatic artery embolization may be helpful. They may relieve symptoms or slow the growth of the cancer, but are very unlikely to result in a cure. These treatments are discussed in detail in the section on surgery.
If tumors in your liver are too large or numerous to be treated directly, or if the carcinoid has spread to other parts of your body, then drug therapy can be helpful. The drugs octreotide and lanreotide, which can slow the growth of the cancer and stop the secretion of the chemicals that cause the carcinoid syndrome, are often helpful. In some cases, they can sometimes actually shrink the cancers. Another drug, interferon alfa, can act the same way. Sometimes these 2 types of drugs are given together. Newer targeted drugs such as sunitinib (Sutent) and everolimus (Afinitor) may be helpful as well, although this still needs to be proven. Adding chemotherapy may also help reduce symptoms, but it seldom shrinks the tumor very much more.
For people with earlier stage cancers who can’t have surgery, most doctors recommend radiation therapy for typical carcinoids and chemotherapy and radiation therapy for atypical carcinoids.
External radiation therapy can also be used to relieve symptoms caused by tumors such as bone pain. For more widespread disease, radioactive drugs may be helpful.
Recurrent carcinoid tumors
When cancer comes back after treatment, it is called a recurrence. Recurrence can be local (in or near the same place it started) or distant (spread to organs such as the liver or bone).
Some carcinoid tumors come back, sometimes several years after the initial treatment. If this happens, further treatment options depend on where the cancer is and what treatments have already been used. Cancers that recur locally or in only 1 or 2 areas can sometimes be treated with further surgery. If surgery is not an option, radiation therapy, chemotherapy, or other drugs may be tried. Because recurrent cancers can often be hard to treat, clinical trials of new types of treatment may be a good option.
What happens after treatment for lung carcinoid tumors?
For many people with carcinoid tumors, treatment may remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about cancer coming back. (When cancer comes back after treatment, it is called recurrence.) This is a very common concern in people who have had cancer.
It may take a while before your fears lessen. But it may help to know that many cancer survivors have learned to live with this uncertainty and are living full lives.
For other people, the cancer may never go away completely. These people may get regular treatments with chemotherapy, radiation therapy, or other therapies to try to help keep the cancer in check. Learning to live with cancer that does not go away can be difficult and very stressful. It has its own type of uncertainty.
Follow-up care
When treatment ends, your doctors will still want to watch you closely. It is very important to go to all of your follow-up appointments. During these visits, your doctors will ask questions about any problems you may have and may do exams and lab tests or x-rays and scans to look for signs of cancer or treatment side effects.
Your doctor will most likely want to see you fairly often (every couple of months or so) at first. The time between visits may be extended if there are no problems. Lung carcinoid tumors are often cured by the initial treatment, but in a small portion of cases the cancer can come back (recur) many years later, which is why doctors often advise close follow-up for at least 10 years.
Almost any cancer treatment can have side effects. Some may last for a few weeks or months, but others can last the rest of your life. During your doctor visits, talk to your cancer care team about any changes or problems you notice and let them know about any questions or concerns you have.
It is important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think of their cancer coming back, this could happen.
If lung carcinoid tumor treatment stops working
If cancer keeps growing or comes back after one kind of treatment, it is possible that another treatment plan might still cure the cancer, or at least shrink the tumors enough to help you live longer and feel better. But when a person has tried many different treatments and the cancer has not gotten any better, the cancer tends to become resistant to all treatment. If this happens, it’s important to weigh the possible limited benefits of a new treatment against the possible downsides, including treatment side effects. Everyone has their own way of looking at this.
This is likely to be the hardest part of your battle with cancer – when you have been through many medical treatments and nothing's working anymore. Your doctor may offer you new options, but at some point you may need to consider that treatment is not likely to improve your health or change your outcome or survival.
If you want to continue to get treatment for as long as you can, you need to think about the odds of treatment having any benefit and how this compares to the possible risks and side effects. In many cases, your doctor can estimate how likely it is the cancer will respond to treatment you are considering. For instance, the doctor might say that more treatment might have about a 1 in 100 chance of working. Some people are still tempted to try this. But it is important to think about and understand your reasons for choosing this plan.
No matter what you decide to do, it is important that you feel as good as you can. Make sure you are asking for and getting treatment for any symptoms you might have, such as nausea or pain. This type of treatment is called palliative care.
Palliative care helps relieve symptoms, but it is not expected to cure the disease. It can be given along with cancer treatment, or can even be cancer treatment. The difference is its purpose – the main goal of palliative care is to improve the quality of your life, or help you feel as good as you can for as long as you can. Sometimes this means using drugs to help with symptoms like pain or nausea. Sometimes, though, the treatments used to control your symptoms are the same as those used to treat cancer. For instance, radiation might be used to help relieve bone pain caused by cancer that has spread to the bones. Or chemo might be used to help shrink a tumor and keep it from blocking the bowels. But this is not the same as treatment to try to cure the cancer.
At some point, you may benefit from hospice care. This is special care that treats the person rather than the disease; it focuses on quality rather than length of life. Most of the time, it is given at home. Your cancer may be causing problems that need to be managed, and hospice focuses on your comfort. You should know that while getting hospice care often means the end of treatments such as chemo and radiation, it doesn’t mean you can’t have treatment for the problems caused by your cancer or other health conditions. In hospice the focus of your care is on living life as fully as possible and feeling as well as you can at this difficult time.
Staying hopeful is important, too. Your hope for a cure may not be as bright, but there is still hope for good times with family and friends – times that are filled with happiness and meaning. Pausing at this time in your cancer treatment gives you a chance to refocus on the most important things in your life. Now is the time to do some things you’ve always wanted to do and to stop doing things you no longer want to do. Though the cancer may be beyond your control, there are still choices you can make.
What’s new in lung carcinoid tumor research and treatment?
Many medical centers across the nation are researching the causes and treatment of lung carcinoid tumors. This is a challenging disease to study because it is not common. But each year, scientists find out more about what causes the disease and how to improve treatment.
Genetics
Researchers have made great progress in understanding how certain changes in DNA can cause normal cells to become cancerous. DNA is the molecule that carries the instructions for nearly everything our cells do. We usually look like our parents because they are the source of our DNA. But DNA affects more than how we look.
Some genes (parts of our DNA) contain instructions for controlling when our cells grow and divide into new cells. Certain genes that cause cells to grow and divide are called oncogenes. Others that slow down cell division or cause cells to die at the right time are called tumor suppressor genes. Cancers can be caused by DNA changes that turn on oncogenes or turn off tumor suppressor genes. Researchers have characterized many of the DNA changes in lung carcinoids in the past few years. Continued research in understanding these changes will lead to new tests for earlier diagnosis and new drugs for more effective treatment.
Diagnosis
Because the outlook and treatment of lung carcinoids and other types of lung cancer are very different, accurate diagnosis is important. Researchers have made great progress in developing tests that can detect specific substances found in the cells of carcinoid tumors but not other lung cancers. Most of these tests treat tissue samples with special man-made antibodies in the lab. The antibodies are designed to recognize specific substances in certain types of tumors.
Treatment
Doctors are learning how to treat lung carcinoids more effectively. For example, newer surgical techniques allow doctors to remove parts of the lung through smaller incisions, which can result in shorter hospital stays and less pain for patients. And new radiation therapy techniques help doctors focus the radiation more precisely on tumors, lowering the amount of radiation that normal tissues get and reducing side effects.
Metastatic carcinoid tumors remain hard to treat. Most carcinoid tumors grow fairly slowly. Because standard chemotherapy drugs work by attacking quickly growing cells, they are not very effective against carcinoid tumors.
Newer drugs called targeted therapies may prove to be more effective against carcinoids. Targeted therapies attack the cancer cells’ inner workings – the programming that makes them different from normal, healthy cells. These drugs attack cancer cells, often while doing little damage to normal cells. Each type of targeted therapy works differently, but all alter the way a cancer cell grows, divides, repairs itself, or interacts with other cells in some way.
Two targeted therapy drugs, sunitinib (Sutent®) and everolimus (Afinitor®), have recently been shown to be helpful in treating neuroendocrine tumors that start in the pancreas. Studies of these drugs in carcinoid tumors (which are a type of neuroendocrine tumor) are in progress.
Targeted drugs called angiogenesis inhibitors affect the growth of new blood vessels (angiogenesis), which tumors need to grow larger. Some of these drugs are already used to treat other types of cancer and are now being studied for use against carcinoid tumors. Examples of these drugs include bevacizumab (Avastin®), pazopanib (Votrient®), and axitinib (Inlyta®).
Researchers are also looking to try to improve upon drugs related to somatostatin, which helps some people with carcinoid tumors. An example is pasireotide, which may prove to be more potent than current drugs such as octreotide. Another new treatment being studied is 90Y-edotreotide. This pairs a drug similar to octreotide with a radioactive atom. The drug binds to the carcinoid cells, delivering the radiation to those cells and lessening the effect on normal cells. In early studies, this treatment has helped patients with advanced carcinoid tumors that were no longer responding to other treatments.
These and other new drugs are now being studied in clinical trials.
