Appendix cancer develops when healthy cells in your appendix mutate and grow out of control, forming a tumor. It’s usually found during surgery for appendicitis or when an imaging test is taken for an unrelated condition. Smaller tumors are less likely to spread, while larger tumors usually require aggressive treatment.
Appendix cancer — also called appendiceal cancer — is a rare disease. It occurs when the cells in your appendix mutate (change) and grow out of control.
Your appendix is part of your digestive system. It’s a small tube-like pouch located on the lower right side of your abdomen, near the junction of your large intestine and small intestine. The function of your appendix is unknown. Some experts believe it has something to do with immune function. Others consider it a vestigial organ (one that is no longer useful from an evolutionary standpoint).
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There are several benign and malignant tumors that can develop in the appendix, including carcinoid tumors, mucinous cystadenomas and appendiceal adenocarcinomas.
Approximately half of all appendix cancers are carcinoid tumors. They affect neuroendocrine cells, which receive signals from the nervous system and release hormones. In most cases, carcinoid tumors are slow-growing.
These noncancerous tumors form in the epithelium (lining) of the appendix. Mucinous cystadenomas won’t spread to other parts of your body as long as the appendix remains intact.
Adenocarcinoma cancers start in the glandular tissue that lines your organs. There are several categories of appendiceal adenocarcinoma, including:
Appendix cancer can occur at any age, but it’s more likely to develop in people over 50. It’s also more common in women than men.
This disease is very rare. In the United States, appendix cancer affects approximately 1 to 2 people out of every 1 million.
Sometimes people with appendix cancer don’t develop any symptoms. When warning signs occur, they can vary depending on the person. Appendix cancer symptoms may include:
Appendix cancer starts when the cells in your appendix mutate and grow out of control. But experts don’t know what causes that process to begin with.
There are, however, certain risk factors associated with appendix cancer. These include:
Experts don’t think there is a genetic or familial factor to appendix cancer. Research is ongoing in this area, but most health providers agree that appendix cancer is not hereditary.
People are often diagnosed with appendix cancer after an appendectomy (removal of the appendix) due to appendicitis. Sometimes imaging tests, such as X-rays or CT scans, reveal existing tumors.
If your healthcare provider suspects appendix cancer, then they may recommend more testing. These tests may include:
There are several approaches when it comes to treating appendix cancer. Your healthcare provider will take several things into account before planning your treatment, including the size and stage of the tumor, your overall health and your personal preferences. Appendix cancer treatments may include:
There are several potential side effects that can occur during cancer treatment. The side effects that you experience can vary depending on which treatment you receive, the stage of cancer and your body’s healing capacity. Some of the most common cancer treatment side effects include:
There is no known way to prevent appendix cancer.
How you respond to treatment for appendix cancer depends on several factors, including the type of appendiceal cancer you have, the location of the tumor and whether cancer has spread to other parts of your body. Your healthcare provider will discuss your options with you in detail.
Some types of appendix cancer are curable. The smaller the tumor, the more likely it is that treatment will be successful. Larger tumors are typically aggressive and may not respond as well to treatment.
The five-year survival rate for low-grade appendix cancer is 67% to 97%. That means that 67% to 97% of people diagnosed with the disease are still alive five years later. However, the five-year survival rate for aggressive tumors can be much lower.
Keep in mind that survival rates can’t tell you how you’ll respond to treatment or how long you’ll live. These numbers represent the experiences of people who’ve been diagnosed with appendix cancer in the past. Because appendix cancer is a rare disease, survival rates for this type of cancer may not be precise. If you have questions about survival rates, talk to your healthcare provider.
If you develop any appendix cancer symptoms, such as appendicitis, bloating or abdominal pain, call your healthcare provider right away.
If you’ve already been diagnosed with appendix cancer, you should call your provider if your symptoms change or worsen. They can find ways to ease your discomfort.
Learning all you can about your condition can help you make informed decisions about your health. Here are some questions to consider asking your healthcare provider:
A note from Cleveland Clinic
An appendix cancer diagnosis can feel scary and uncertain. Talk to your healthcare provider about how you’re feeling. They can answer all of your questions and find resources to help you through this difficult time. Because appendix cancer is a rare disease, you may feel alone and isolated. Many people join support groups so they can talk with others who are going through the same thing.
Last reviewed by a Cleveland Clinic medical professional on 11/30/2021.
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