ON THIS PAGE: You will learn about the different types of treatments doctors use for people with appendix cancer. Use the menu to see other pages.
This section tells you the treatments, also known as therapies, that are the standard of care for appendix cancer. “Standard of care” means the best treatments known. Information in this section is based on medical standards of care for appendix cancer in the United States. Treatment options can vary from one place to another.
When making treatment plan decisions, you are 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 to 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 standard treatments, or new doses of standard drugs or other treatments. 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 appendix cancer 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. Cancer 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 type and stage of cancer, possible side effects, and the patient’s preferences and overall health. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care. Take time to 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 appendix cancer because there are different treatment options. Learn more about making treatment decisions.
The most common types of treatments for appendix cancer are described below. Find treatment information for neuroendocrine tumors of the appendix in another section of this website.
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Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. It is the most common treatment for appendix cancer. Most often, appendix cancer is low grade (see Stages and Grades) and, therefore, slow growing. Often it can be successfully treated with surgery alone. A surgical oncologist is a doctor who specializes in treating cancer using surgery.
Types of surgeries for appendix cancer include:
Appendectomy. An appendectomy is the surgical removal of the appendix. Often, an appendectomy is not the surgery performed to treat this kind of cancer. Instead, it is more typical that patients have an appendectomy for appendicitis and then when the tissue is sent to the pathologist, cancer is discovered. If the pathology analysis finds a neuroendocrine tumor and it is small, less than 1 or 2 centimeter (cm), then the appendectomy may be enough to remove all of the cancer. However, if the neuroendocrine tumor is larger or has aggressive features or if it is not a neuroendocrine tumor, such as an adenocarcinoma or a goblet cell carcinoma, then a second operation to remove more tissue will likely be needed.
Hemicolectomy. For a neuroendocrine tumor larger than 2 cm or appendix cancers that are not neuroendocrine, a hemicolectomy may be recommended. This is the removal of a portion of the colon next to the appendix. Removal of nearby blood vessels and lymph nodes is often done at the same time. A right hemicolectomy is surgery performed on the right side of the colon, which is where the appendix is normally attached. Even though a large amount of the large intestine is removed, the operation usually does not result in the need for a colostomy or stoma, which is an opening in the abdomen through which the bowel contents are emptied into a bag.
Debulking surgery. For later-stage appendix cancer, debulking (or cytoreduction) surgery may be considered depending on the type of appendix cancer and if there is only spread in the abdominal cavity. In this surgery, the doctor removes as much of the tumor “bulk” as possible, which can benefit the patient even though it will not remove every cancer cell from the body. Sometimes, debulking surgery will be followed with chemotherapy (see below) to destroy remaining cancer cells.
When the tumor produces mucus (also known as mucin in medical terms), much of the bulk of the abnormal tissue often is not cancer but is due to accumulation of the mucus. The mucus looks like jelly, and this condition may be referred to as “jelly belly” (also known as pseudomyxoma peritonei or PMP in medical terms). Removing the mucus from the abdomen can often relieve a patient’s bloating.
Removal of the peritoneum. Some surgeons recommend aggressive surgery to remove the peritoneum (the lining of the abdomen) in order to remove as much of the cancer as possible. This type of surgery is also called a peritonectomy. Not all surgeons agree that extensive surgery like peritonectomy is necessary in people with slow-growing, low-grade appendix cancer that has spread beyond the colon to involve other areas of the abdomen.
In patients with a very slow-growing tumor, peritonectomy can be effective in removing the majority of the cancer cells. This can benefit the patient by reducing the amount of cancer, even if it does not remove every cancer cell. However, it is a difficult operation that can have significant side effects. The doctor will consider many different factors, such as the patient’s age and overall health, before recommending this extensive surgery. Patients should talk with a specialist with expertise in this type of procedure beforehand.
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.
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Therapies using medication
The treatment plan may include medications to destroy cancer cells. Medication may be given through the bloodstream 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 cancer 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 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 appendix cancer include:
Each of these types of therapies is 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. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy. The type of medication depends on the type of appendix cancer. The drugs used for neuroendocrine tumors, for example, are not the same as those used for adenocarcinomas. Your doctor will need to know the type of appendix cancer to know which drugs may be effective.
The medications used to treat cancer 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.
Chemotherapy is the use of drugs to destroy cancer cells, usually by keeping the cancer cells from growing, dividing, and making more cells.
For appendix cancer that is not a neuroendocrine tumor, chemotherapy is most often used soon after surgery when cancer is found outside the appendix region. It may also be given after surgery for a non-neuroendocrine appendix cancer to prevent the cancer from coming back, depending on the stage of disease.
There are different types of chemotherapy, depending on how the drugs are delivered to the body:
Local/intraperitoneal chemotherapy. For local chemotherapy, the medication is delivered to 1 area or section of the body. More specifically, this is called intraperitoneal chemotherapy, which is chemotherapy that is given directly into the abdominal cavity. Typically, the surgeon will try to remove as much of the tumor as possible (debulking surgery, see above) and then insert a tube in the abdomen through which chemotherapy can be given after the operation. In some cases, the chemotherapy is warmed above body temperature to increase its ability to penetrate the tissue that may be lined with tumor cells; this is called hyperthermic (or heated) intraperitoneal chemotherapy or HIPEC. Once chemotherapy is completed, the tube is removed, generally without the need for another operation.
Systemic chemotherapy. This type of chemotherapy is given through the bloodstream to reach cancer cells throughout the body. This can be done using an intravenous (IV) tube, which is a tube placed into a vein using a needle or as a pill or capsule that is swallowed (orally). Some people may receive this type of chemotherapy in their doctor’s office or outpatient clinic; others may go to the hospital.
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.
For non-neuroendocrine appendix cancers, systemic chemotherapy is similar to that used for colorectal cancer. Chemotherapy may include fluorouracil (5-FU), leucovorin (folinic acid), capecitabine (Xeloda), irinotecan (Camptosar), and oxaliplatin (Eloxatin).
The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, mouth sores, diarrhea, nerve problems (neuropathy) in the hands and feet, and cold sensitivity. These side effects usually go away after treatment is finished.
Learn more about the basics of chemotherapy.
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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 and limits damage to healthy 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. This helps doctors better match each patient with the most effective treatment whenever possible. For example, if your tumor has a particular marker called mismatch repair deficiency or dMMR, then it will most likely respond well to specific treatments like immunotherapy. In addition, 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.
Targeted therapy for non-neuroendocrine appendix cancer is similar to that used for colorectal cancer and includes bevacizumab (Avastin), ziv-aflibercept (Zaltrap), ramucirumab (Cyramza), cetuximab (Erbitux), and panitumumab (Vectibix).
Talk with your doctor about possible side effects for a specific medication and how they can be managed.
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Immunotherapy uses the body’s natural defenses to fight cancer by improving your immune system’s ability to attack cancer cells.
Certain advanced appendix cancers may have a molecular feature called microsatellite instability (MSI-H). Doctors can check for MSI-H by using biomarker testing. If the cancer is MSI-H, then pembrolizumab (Keytruda) or nivolumab (Opdivo) plus ipilimumab (Yervoy) may be treatment options. These medications are immune checkpoint inhibitors that target receptors on tumor cells and prevent tumor cells from hiding from the immune system.
Different types of immunotherapy can cause different side effects. Common side effects include skin reactions, low thyroid hormone level, mild fatigue, flu-like symptoms, diarrhea, and weight changes. Talk with your doctor about possible side effects for the immunotherapy recommended for you. Learn more about the basics of immunotherapy.
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Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. A radiation therapy regimen, or schedule, usually consists of a specific number of treatments given over a set period of time.
Radiation therapy is rarely used in the treatment of appendix cancer. Sometimes it may be used to relieve symptoms when the cancer has spread to a particular area, such as the bone.
Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.
Learn more about the basics of radiation therapy.
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Physical, emotional, social, and financial effects of cancer
Cancer and its treatment 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. 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 cancer, may receive this type of care. And it often works best when it is started right after a cancer diagnosis. People who receive palliative and supportive care along with treatment for the cancer 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.
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.
Cancer 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.
Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative and supportive care in a separate section of this website.
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Metastatic appendix cancer
If cancer spreads to another 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 surgery, chemotherapy, targeted therapy, and radiation therapy. Palliative and supportive care will also be important to help relieve symptoms and side effects.
For many people, a diagnosis of metastatic cancer is very stressful and difficult. You and your family are encouraged to talk about how you are feeling 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.
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Remission and the chance of recurrence
A remission is when cancer 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 cancer will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the cancer returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.
If the cancer returns after the original treatment, it is called recurrent cancer. 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 treatment options. Often the treatment plan will include the treatments described above such as surgery, chemotherapy, targeted therapy, and radiation therapy, but they may be used in a different combination or given at a different pace. Your doctor may suggest clinical trials that are studying new ways to treat recurrent appendix cancer. Whichever treatment plan you choose, palliative and supportive care will be important for relieving symptoms and side effects.
People with recurrent cancer 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.
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If treatment does not work
Recovery from cancer is not always possible. If treatment is not successful, the disease may be called advanced, end-stage, or terminal cancer.
This diagnosis is stressful, and for some people, advanced cancer is difficult to discuss. 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 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.
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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 cancer. Use the menu to choose a different section to read in this guide.