At UofL Health - Brown Cancer Center, you will have some of the area’s top experts to focus on finding the best treatment approach for appendix cancer for you. Our experts work together closely and communicate often to ensure you receive the most advanced care with the least impact on your body.
Surgery is often the main therapy for cancer of the appendix, and the skill of the surgeon is an important part of your successful treatment. Brown Cancer Center surgeons are among the most experienced in the region in the delicate procedure.
Our physicians see more appendix cancer cases than most oncologists. This gives us a level of expertise that offers a higher chance of successful treatment.
Appendix cancer is diagnosed in fewer than 1,000 Americans each year.
The appendix is part of the digestive system, and it is located close to where the large intestine and small intestine come together. Tumors in the appendix may be interpreted as malignant (cancer) or benign (not cancer). Given the rarity of this disease, all patients with an appendix tumor should have their pathology formally reviewed and seek the advice of doctors who specialize in treating appendix cancer.
Most cases of appendix cancer are found when a person has surgery for another condition. Almost half are found during surgery for acute appendicitis; others are discovered when an abdominal mass is seen during a CT scan for an unrelated condition.
The outcome for appendix cancer depends a great deal on the size of the tumor. When the tumor is smaller than two centimeters, the cancer is less likely to spread. However, when tumors are larger they generally require more aggressive treatment.
Appendix cancer types
Appendix cancer is classified by the type of cells within the tumor. The main types are:
Carcinoid tumors: About half of appendix cancers are carcinoid tumors. Carcinoid tumors are most often found in women in their 40s. Most carcinoid tumors are small, and they often can be treated successfully.
Non-carcinoid tumors: These tumors begin in the epithelial cells that line the inside of the appendix. Most epithelial cells produce mucin, a gelatinous material. These tumors have a tendency to spread, and the success of treatment depends on several factors.
Pseudomyxoma peritonei (PMP): Mucin within the abdomen has few tumor cells, but cells may spread outside the appendix into the abdomen.
Adenocarcinoid tumors, also known as goblet cell carcinomas, have characteristics similar to both carcinoid and adenocarcinoma tumors of the appendix. Most patients are diagnosed in their 50s.
Anything that increases your chance of getting appendix cancer is a risk factor. Risk factors include:
- Smoking tobacco
- Gender: Women are more likely to develop carcinoid tumors than men
- Certain health conditions, such as atrophic gastritis, pernicious anemia or Zollinger-Ellison syndrome, which affect the stomach’s ability to make acid
- Having a family history of multiple endocrine neoplasia type 1 (MEN1) syndrome, a disorder also called endocrine adenomatosis and Wermer syndrome
Not everyone with risk factors gets appendix cancer. However, if you have risk factors, you should discuss them with your doctor.
Appendix cancer usually does not cause symptoms until it is in an advanced stage and has spread to other parts of the body. When appendix cancer symptoms are present, they vary from person to person and may include:
- Acute appendicitis: Most cases of appendix cancer are discovered during surgery for appendicitis
- Increase in abdomen size/girth, bloating
- Vague abdominal discomfort in the lower right abdomen
- Pelvic discomfort
- New hernias
- Bowel obstruction
- Ovarian masses
- Acute or chronic abdominal pain
If appendix cancer spreads to the liver, you may develop carcinoid syndrome. Symptoms include:
- Flushing (redness or feeling of warmth in face and neck)
- Shortness of breath or wheezing
- Heart valve disease on the right side
- Pain or feeling for fullness in the abdomen
These symptoms do not always mean you have appendix cancer. However, it is important to discuss any symptoms with your doctor, since they may signal other health problems.
Since appendix cancer often does not have symptoms in the early stages, it frequently is not diagnosed until surgery for another condition, such as acute appendicitis, or during tests for another condition.
Sometimes appendix cancer is found as part of the routine procedure after abdominal surgery for another condition. If your doctor finds what might be appendix cancer during abdominal surgery, a biopsy will be performed.
If you have symptoms that may signal appendix cancer, your doctor will examine you and ask you questions about your health, your lifestyle -- including smoking and drinking habits -- and your family medical history.
One or more of the following tests may be used to find out if you have appendix cancer and if it has spread. These tests also may be used to find out if treatment is working.
- Blood and urine tests
- Imaging tests, which may include: CT or CAT (computed axial tomography) scans, MRI (magnetic resonance imaging) scans and PET (positron emission tomography) scans
Appendix cancer staging
If you are diagnosed with appendix cancer, your doctor will determine the stage of the disease.
Staging is a way of classifying cancer by how much disease is in the body and where it has spread when it is diagnosed. This helps the doctor plan the best way to treat the cancer.
Once the staging classification is determined, it stays the same even if treatment is successful or the cancer spreads.
Appendix cancer stages:
- Localized: Cancer is found in the appendix, colon, rectum, small intestine and/or stomach only
- Regional: Cancer has spread from the appendix, colon, rectum, stomach and/or small intestine to nearby tissues or lymph nodes
- Metastatic: Cancer has spread to other parts of the body
If you are diagnosed with appendix cancer, your doctor will discuss the best options to treat it. This depends on several factors, including:
- The type of tumor
- Where it is in the appendix
- If it has spread
- Your overall health
Your treatment for appendix cancer will be customized to your particular needs. One or more of the following therapies may be recommended to treat the cancer or help relieve symptoms.
This is the main treatment for appendix cancer. Chemotherapy may be used with surgery. The type of surgery depends on the type of appendix cancer.
Carcinoid tumors: Often, surgery is done to remove the appendix, right colon and surrounding lymph nodes.
Non-carcinoid tumors: If appendix cancer has spread within the abdomen, the most effective approach usually is:
- Cytoreductive (tumor debulking) surgery to remove the tumor and mucin in the abdomen. Parts of the intestine, gallbladder, ovaries, uterus and lining of the abdominal cavity may be removed.
- Hyperthermic intraperitoneal chemotherapy (HIPEC), also known as heated chemotherapy, is performed during tumor debulking surgery. The abdominal cavity is filled with a chemotherapy drug, which is heated to more than 104 degrees Fahrenheit. Your abdomen is rocked gently back and forth for 90 minutes to ensure the drugs go to all areas of the abdominal cavity.
Pseudomyxoma peritonei (PMP): Surgical removal of the tumor (also called cytoreduction), combined with HIPEC, is usually recommended.
Adenocarcinoid tumors: Treatment may include all the following:
- Removal of the right part of the colon
- Cytoreductive surgery with HIPEC
- Chemotherapy before surgery