Appendix cancer: What you should know

Each year, fewer than 1,000 Americans are diagnosed with appendix cancer, also called appendiceal cancer.

The appendix is located in your digestive system, near where your large intestine and small intestine meet. 

Unfortunately, because appendix cancer is so rare, there's still a lot we don't know about its causes and prevention. Appendix cancer can also be hard to detect. A routine preventive colonoscopy to detect colon cancer may incidentally detect appendiceal cancer, but not always.

We recently spoke with Cathy Eng, M.D., associate professor of Gastrointestinal Medical Oncology, to learn more about appendix cancer symptoms, diagnosis and treatment options.

Here's what she had to say.

What are some common appendix cancer symptoms?
Common symptoms include: weight loss, bloating/abdominal distention, early satiety, fatigue, nausea, abdominal pain and change in bowel habits.

These symptoms are similar to those of a lot of other gastrointestinal conditions. Do these symptoms result infrequent misdiagnoses? 
Yes, appendix cancer is often mistaken for appendicitis. The tendency to misdiagnose mainly stems from the very low prevalence of the disease. Community physicians rarely consider appendix cancer when a patient experiencing such symptoms, and thus appendiceal cancer tends to be diagnosed at a later stage than many other cancers. 

The difficulty in diagnosis also stems from the difficulty of measuring this disease with standard imaging techniques such as CT scans.

What advice do you have for patients who think they've been misdiagnosed?
If you're diagnosed with appendicitis and your symptoms don't go away after a few weeks, get a second opinion.

How is appendix cancer typically diagnosed?
The patient typically has emergent symptoms of abdominal pain and fever. Since the exact symptoms of appendix cancer are similar to other conditions, a patient will typically have to wait until surgery to confirm the disease diagnosis.

Surgery is a common therapy for appendix cancer. Why is that the case?  And, when is surgery coupled with other therapies such as chemotherapy and/or radiation?
Surgery has demonstrated durable survival benefits for those patients who are able to endure the long surgical procedure. Ideally, surgery involves bathing the abdomen in heated chemotherapy after all of the tumor has been removed in a process called cytoreductive surgery (CRS) and HIPEC (heatedintraperitoneal chemotherapy). 

For patients who are not considered optimal surgical candidates, our data suggests there is some benefit to providing them with systemic chemotherapy in an attempt to control their tumor as much as possible. 

What makes a patient a good candidate for appendix cancer surgery?
Surgical cytoreduction, which I talked about above, is a very long and involved operation, usually lasting 8-14 hours. Only those patients who are thought to be able to both tolerate the surgery well, and benefit most from it by having most or all of their tumor removed, are recommended for surgery.  

Patients with many co-morbidities (multiple diseases ordisorders) which would make them risky surgical candidates, or those whose tumor is simply too bulky or poorly located to be fully removed, are instead referred for systemic chemotherapy, which seems to provide them with some benefit in terms of reducing their symptoms and even survival.

Is MD Anderson pursuing any clinical trials for patients with appendix cancer?
We are currently working on submitting a trial for approval that will utilize a chemotherapy backbone we have demonstrated to be effective; it will also include a novel biologic agent.

What advice do you have for patients who have recently been diagnosed with appendix cancer?
As with all cancers, staying positive is immensely important.  Surgery is an effective treatment, and we've also had promising results in managing this disease using modern combination chemotherapy, when surgery is not an option. 

Appendiceal cancer may be rare, but you are not alone. The most important thing is to get evaluated by a multidisciplinary team like we have at MD Anderson. That way, your surgeons, pathologists, and medical oncologists can work together to optimize treatment for this rare and complex cancer.