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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). Since this is a rare cancer, all patients with an appendix tumor should have their pathology formally reviewed and seek the advice of doctors who specialize in treating appendix cancer.
The outcome for appendix cancer depends a great deal on the size of the tumor. When the tumor is smaller than 2 centimeters, the cancer is less likely to spread. However, when tumors are larger they generally require more aggressive treatment.
Types of appendix cancer
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.
Appendix Cancer Risk Factors
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 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
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
(source: National Cancer Institute)
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.
As one of the nation’s most active cancer centers, we see more patients with this complex type of cancer than most others. This gives us an extraordinary level of expertise and experience.
Our renowned surgeons are among the most recognized in the country and perform more appendix cancer procedures in a year than many cancer surgeons do in a lifetime.
Our Appendix Cancer Treatments
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.
Carcinoid tumors: Often, surgery is done to remove the appendix, right colon and surrounding lymph nodes. For more information, see carcinoid tumors.
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, which 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
Why come to MD Anderson for appendix cancer care?
When you are treated in MD Anderson’s Gastrointestinal Center for appendix cancer, some of the nation’s top experts focus on finding the best treatment for you. They work together closely and communicate often to be sure you receive the most advanced personalized care with the least impact on your body.
Appendiceal Cancer Expertise
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. MD Anderson’s surgeons are among the most experienced in the nation in the delicate procedure.
As one of the nation’s most active cancer centers, MD Anderson sees more appendix cancer cases than most oncologists. This gives us an exceptional level of expertise that often gives you higher chances for successful treatment.
Innovation and Support
Because we are one of the nation’s leading research centers, we’re able to offer clinical trials of experimental treatments of groundbreaking drugs and delivery methods for every type and stage of appendix cancer.
Somehow, some way, you'll get through this. And no matter the outcome, you'll be grateful for the gift of time.
BY Ivanna Kern
I recently celebrated my sixth cancerversary with no evidence of appendix cancer. Since my appendix cancer diagnosis, I’ve learned the importance of appreciating each day and celebrating the little things in life.
Here are some of the ways my family and friends helped me find joy during treatment and ways I continue to celebrate life post-treatment.
Celebrating milestones during appendix cancer treatment
My family and friends helped me get through my treatment by finding ways to cheer me up and celebrate different milestones in my appendix cancer journey.
- I invited my best friends to "chemo parties.” We celebrated when I was halfway through with chemo, done with chemo and when I found out there was no evidence of disease. We had a blast spending the evening laughing, enjoying each other's company and being silly. I still remember those parties in such vivid detail and how they energized me.
- During chemo treatment, a friend gave me a hand-knitted, purple, soft prayer shawl made by the "Knit Wits," a church knitting group ministry that provides prayer shawls to people experiencing health crises. I was very touched to know that strangers were thinking about me. When a friend was diagnosed with cancer a couple of years later, I passed the prayer shawl on to her along with the story of how it arrived in my hands. We both shed some tears.
- My friends let me know they were thinking of me by giving unusual little morale lifters. I loved the smooth glazed stone to keep in my pocket that said "Be Strong" and the large silver fortune cookie to remind me of good luck.
- My husband and I celebrated our 15th wedding anniversary toward the end of my chemotherapy treatments. Several of our friends were married that same year, so someone threw a party for all of us and asked that we all wear our wedding attire. And we did! It was such a fun, memorable evening.
Embracing life after appendix cancer
As I’ve learned, it’s important to celebrate life all year round, not just on cancerversaries or National Cancer Survivors Day in June.
Take time to splurge and give yourself a whole day of luxury with a massage, facial, pedicure or manicure. Enjoy a good healthy meal as a reminder that you are still on the planet!
Here are some of my favorite ways to celebrate the little things in life:
- Enjoy an early morning cup of coffee with my husband
- Walk with my dog on the hike ‘n bike trail
- Watch my two daughters grow and rock their worlds
- Be present for my husband and kids no matter how challenging the day
- Appreciate the love and friendship I share with my extended family
- Support my friends on their various journeys, cancer and otherwise
- Celebrate yet another wedding anniversary
- Camp in the Sierra Nevadas and make s’mores with my kiddos
- Hash out life’s big and little issues with great friends on a regular basis
- Seek out my spirituality
- Remember to laugh more and not take myself too seriously