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This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options.
In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team.
Listed below are descriptions of the most common treatment options for appendix cancer that is not a carcinoid tumor. (Find treatment information for carcinoid tumors of the appendix.) 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.
Surgery is the removal of the tumor and surrounding tissue during an operation. It is the most common treatment for appendix cancer. Most often, appendix cancer is low-grade 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. It is usually the only treatment needed for an appendix tumor smaller than 1.5 centimeters (cm).
In cases when appendix cancer is discovered unexpectedly after an appendectomy was performed for what was originally thought to have been appendicitis, a second operation to remove more tissue (using surgical techniques described below) is often recommended.
Hemicolectomy. For a tumor larger than 2 cm, 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. 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 (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 performed. In this surgery, the doctor removes as much of the tumor “bulk” as possible, which could benefit the patient even though it will not remove every cancer cell from the body. Sometimes, debulking surgery will be followed with chemotherapy to destroy any remaining cancer cells.
In cases when the tumor produces mucous, much of the bulk of the abnormal tissue often is not cancer but is due to accumulation of the mucous. The mucous looks like jelly, and this condition is often referred to as “jelly belly.” Removing the mucous from the abdomen can often relieve a patient’s symptoms of bloating.
Removal of the peritoneum. There is some controversy about the extent of surgery that is necessary in patients with slow-growing, low-grade cancer that has spread beyond the colon to involve other areas of the abdomen. Some surgeons recommend aggressive surgery that includes the removal of the peritoneum (the lining of the abdomen) to remove as much of the cancer as possible. This type of surgery is also called a peritonectomy.
In patients with a very slow-growing tumor, such surgery 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.
Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. For appendix cancer, chemotherapy is most often used soon after surgery when cancer is found outside of the appendix region. There are different types of chemotherapy, depending on how the drugs are delivered to the body:
Local/intraperitoneal chemotherapy. For local chemotherapy, the medication delivery is focused on one area or section of the body. This is the most common type of chemotherapy used in the treatment of appendix cancer; more specifically, it 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) and then insert a tube in the abdomen through which chemotherapy can be given after the operation. In some cases, the chemotherapy is warmed beyond 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. Once chemotherapy is completed, the tube is removed, generally without the need for another operation.
Systemic chemotherapy. This type of chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. This can be done using an intravenous (IV) tube (a tube inserted into a person’s vein). 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 (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time. Specific drugs given in systemic chemotherapy are similar to those for colorectal cancer and can include fluorouracil (5-FU, Adrucil), leucovorin (Wellcovorin), capecitabine (Xeloda), irinotecan (Camptosar), oxaliplatin (Eloxatin), bevacizumab (Avastin), and cetuximab (Erbitux).
The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away when treatment is finished.
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.
Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. A radiation therapy regimen (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. In certain cases, a form of radiation called P32 may be recommended. In this procedure, radioactive phosphorus is dissolved in a liquid and placed inside the body after surgery through a tube inserted in the abdomen (see above). P32 delivers strong radiation therapy to a specific area. Because the radioactivity disappears quickly (within a few hours), there is no need to remove the substance from the abdomen after treatment.
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.
Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.
Palliative care can help a person at any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.
Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem, so it is addressed as quickly as possible.
Recurrent appendix cancer
A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED.
A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the cancer does return.
If the cancer does return 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).
When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as surgery, chemotherapy, and radiation therapy) but may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.
People with recurrent cancer often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope.
Metastatic appendix cancer
If cancer has spread to another location in the body, it is called metastatic cancer.
Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan.
Your health care team may recommend a treatment plan that includes a combination of surgery, chemotherapy, and radiation therapy. Supportive care will also be important to help relieve symptoms and side effects.
For many patients, a diagnosis of metastatic cancer can be very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.
If treatment fails
Recovery from cancer is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.
This diagnosis is stressful, and this is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.
Palliative care given toward the end of a person’s life is called hospice care. You and your family are encouraged to think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families.
Coping with Side Effects
Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.
Common side effects from each treatment option for appendix cancer are described in detail within the Treatment section. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your overall health.
Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with appendix cancer.
In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies.
During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care.
After treatment for appendix cancer ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. People treated for appendix cancer will generally need to follow up with an oncologist, a surgeon, or an internal medicine specialist to monitor any symptoms of abdominal recurrence, such as pain, nausea, blood in the stool, severe bloating, and cramping. CT or MRI scans may be recommended as part of follow-up care.
People recovering from appendix cancer are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level.
Doctors are working to learn more about appendix cancer, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease.
However, research is hampered by the rare nature of the disease. Because appendix cancer is uncommon, appendix cancer-specific clinical trials may be challenging to find. Patients are encouraged to talk with their doctors about broader clinical trials that may be open to them, such as those studying gastrointestinal carcinoid tumors or colorectal cancer.
The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you.
Enhanced delivery of chemotherapy. Doctors are looking for different ways to deliver chemotherapy to the abdomen. One approach is called hyperthermic intraoperative peritoneal chemotherapy (HIPEC). This is similar to the hyperthermic peritoneal (local) chemotherapy described under Treatment, but the drugs are delivered directly to the open abdomen during debulking surgery, instead of after surgery.
Combination chemotherapy. Research is underway to determine the best combinations of different drugs that are the most effective for appendix cancer.
Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current appendix cancer treatments in order to improve patients’ comfort and quality of life.