Appendiceal Cancer

Cancer of the appendix occurs when normal appendix cells become abnormal and form a tumor. There are several types of tumors that can form in the appendix:

• Neuroendocrine tumors (or carcinoid tumors) account for more than half of appendiceal cancer cases.
• A mucinous cystadenocarcinoma begins in the mucus-filled sacs in the appendix wall and accounts for about 20% of all appendiceal cancer cases.
• 10% of appendix cancer tumors are colonic-type adenocarcinomas, which can spread to other areas of the body.
• A mucinous cystadenoma is a benign tumor and does not spread outside the appendix.
• A goblet cell carcinoma, also known as an adenoneuroendocrine tumor, can spread more aggressively than a neuroendocrine tumor.
• Signet-ring cell adenocarcinomas are fast-growing tumors that are rare and difficult to treat or remove.
• Paraganglioma tumors in the appendix are usually benign, with only 1 reported case of a malignant paraganglioma.

A cancerous appendix can rupture, which spreads cancer cells to the surfaces of other organs in the abdomen. Multiple tumors can develop if this occurs. The resulting peritoneal surface disease, which affects the tissue lining the abdomen, can prove deadly.

In the early stages of appendix cancer, most people don’t notice any symptoms. The symptoms of appendix cancer can easily be missed and are common to many different possible health conditions. For this reason, appendiceal cancer is sometimes not accurately diagnosed until the later stages of the disease. Appendix cancer can cause more symptoms if it has spread to other organs or areas of the body. In some cases, doctors may discover appendiceal cancer while treating a different healthcare concern or performing an unrelated procedure. For example, sometimes a routine colonoscopy can reveal appendiceal cancer.

The specific appendix cancer symptoms you may experience depend on the type of tumor and the effects the tumor causes, but can include:

• Abdominal pain
• Swollen abdomen
• Loss of appetite
• Feeling full without eating much food
• Nausea or vomiting
• Constipation or diarrhea
• Hernia (more common in men)

Appendicitis (inflammation of the appendix) may be the first sign of appendiceal cancer. If an appendix tumor blocks the appendix, bacteria can become trapped and cause appendicitis. It’s not uncommon to discover appendix cancer during surgery to remove the appendix.

There are no known risk factors for appendix cancer, and there are no known causes.

There is no standardized process or testing to diagnose or screen for cancer of the appendix. Appendix cancer is difficult to diagnose because its symptoms are not unusual. Doctors diagnose most cases of appendiceal cancer while treating a different medical condition, or even during surgery for appendicitis.

If your doctor suspects you may have appendix cancer, he or she will order further tests to help make an accurate diagnosis, such as imaging tests or a biopsy. Imaging tests can reveal the presence of tumors on your appendix, while a biopsy removes small samples of tissue to test for cancerous cells.

Treatment for appendiceal cancer depends on the type, size and stage of appendix tumor, as well as other factors. Your doctor may recommend surgery to remove the tumor. In some cases, your doctor may schedule chemotherapy before the surgery to shrink the tumor first.

Surgery for appendix cancer can remove the entire appendix, the tumor(s) or part of the colon near the appendix. Your doctor may debulk (partially remove) very large tumors, then use chemotherapy to shrink them further. Cancer of the appendix that has spread may require more aggressive surgery.

The University of Kansas Cancer Center offers combination therapy for low-grade (slow-growing) appendiceal cancer called cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC).

HIPEC starts with surgery to remove all visible tumors. Next, your doctor fills the abdominal cavity with heated chemotherapy medication. This approach causes fewer side effects than traditional chemotherapy and allows for a higher dosage. Because the chemotherapy bath is heated, the medicine can penetrate the tissue more deeply.

The University of Kansas Cancer Center has used CRS-HIPEC to successfully treat high-grade (fast-growing) cancers of the appendix, colon, ovaries and stomach, as well as abdominal mesothelioma.

Appendiceal cancer specialists at The University of Kansas Cancer Center are at the forefront of discovering new treatments that save lives.

These specialists have years of experience treating hundreds of patients. They meet weekly to discuss specific cases. Together, this team of experts will review your case and develop a treatment plan tailored to your individual needs.

Because we are an academic medical center, we offer some of the region's most advanced treatments, including clinical trials. We are constantly finding new treatments to lengthen survival and improve quality of life. We use leading-edge techniques and technologies to improve patient outcomes and reduce side effects. And we work side by side with pharmaceutical companies to help you get access to the medicines you need.

Our staff is also accustomed to working with community healthcare providers. In many cases, you can receive some of your treatment near your own home in coordination with the staff at The University of Kansas Cancer Center.