Researchers are seeing a significant rise in appendix cancer over the last decade, and not just in people over age 50. Gen X and Millennials are now at three to four times the risk when compared to a generation ago. This marks a clear shift in demographics.

Appendix cancer is rare. About 3,000 to 5,000 cases are diagnosed each year in the United States. However, the increase among younger adults (in their 20s, 30s and 40s) experiencing this diagnosis mirrors a larger trend of cancers being diagnosed at younger ages, like colorectal cancer. 

Little is known about why this is happening. With no known risk factors and no guidance on prevention, more research is needed to identify the factors that may be causing this trend.

Are there symptoms? 

Not every abdominal symptom means you have appendix cancer, and symptoms are often not specific. Appendix cancer symptoms can be similar to those of appendicitis, pelvic issues or inflammatory disease. For this reason, it’s important to discuss any unusual, ongoing, worsening or recurring symptoms with your doctor.

Symptoms may include:

• Pain in the lower right side of the abdomen, nausea, vomiting and fever.     
• Bloating with growing abdomen.  
• Feeling full right away when eating.
• Unexplained weight loss.
• A lump in the abdomen.

Although there is no clear genetic link, be aware of your family history. Multiple family members with cancer may increase your risk as well.

If I have appendicitis, is antibiotic treatment enough?

Recently, some cases of uncomplicated appendicitis have been treated with antibiotics instead of surgery. However, because the symptoms of appendicitis and appendix cancer can look alike, antibiotic treatment alone may increase the risk of missing an underlying appendix cancer.

“Given the rise of these cancers in younger adults, we need to be more thoughtful about not just treating acute appendicitis, but also thinking about the cause in the younger age group,” says surgical oncologist Jason Foster, MD.

To reduce the chance of missing cancer:

• Follow-up imaging may be needed to be sure the appendix looks normal.
• A delayed appendectomy may be considered.
• If your appendix is removed, ask that the pathology review specifically checks for a tumor. 

How is appendix cancer diagnosed?

Many appendix cancers are found incidentally during a CT scan for appendicitis or other abdominal pain.

Appendix cancer is usually found in one of two ways:

• The appendix is removed for appendicitis, and cancer is found on a pathology review.
• Unidentified abdominal pain that leads to a scan.

CT or MRI scans alone cannot tell the whole story and can only identify that something looks suspicious. If a scan shows something of concern, additional steps will be needed to rule out cancer. 

Steps may include follow-up testing:

• A thin-cut CT scan of the abdomen with IV and oral contrast.
• An MRI if a lesion is seen and further investigation is needed.
• A diagnostic laparoscopy or appendectomy if the appendix looks abnormal, but without acute appendicitis. 

Appendix removal is the only definitive way to diagnose appendix cancer. 

After an appendectomy, a review of the pathology report is essential to confirm that no cancer was missed.

“Get a copy of the pathology report if you’ve had appendicitis (for all ages) and you’ve had your appendix removed,” adds Dr. Foster. “If you are informed there is a benign or malignant tumor, be sure you get a second opinion with a provider who specializes in appendix cancer so that you can be sure the lab tests needed to look for cancer were performed.”

Who should be involved in your treatment?

Since there are several types of appendix tumors, each behaving differently, a specialized team is required for optimal care. Your cancer team will discuss what treatment options are best for you.

Your treatment plan should involve:

• A gastrointestinal surgeon with expertise in surgical oncology or colorectal surgery.
• A medical oncologist who specializes in gastrointestinal cancers if the disease has spread. 

Remission is possible for many patients, especially when detected early and has not spread beyond the appendix. Most important for patients diagnosed with appendix-only disease, ongoing monitoring is essential, even after initial treatment, due to the risk of recurrence.

“If you have a diagnosis and it hasn't spread, there is a concern that you could develop metastatic appendix cancer in your lifetime, particularly in the first five years,” says Dr. Foster. “Make sure you are seeing a provider who is an expert in monitoring your risk of the tumor recurring.” 

All cancers of the appendix should be evaluated at a medical facility that offers surgical cytoreduction (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC).

How does appendix cancer spread?

Like lung, breast, and colon cancer, some types of (high-grade) appendix cancer can spread to draining lymph nodes around the appendix and right colon. For these high-grade cancers, a second surgery to remove the right colon is recommended.

However, unlike most cancers, which primarily spread through the bloodstream to sites such as liver, lung or brain, appendix cancer can also spread due to micro-perforation by the tumor through the wall of appendix and then onto the surfaces of other abdominal organs and the peritoneal lining (peritoneal metastasis). This is the most common pattern of tumor recurrence for appendix cancer and is the cause of cancer mortality for appendix cancer patients.

Early diagnosis is key to preventing spread. Plus, early detection of peritoneal disease will lower tumor burden. 

“Some of the patients who come to us with appendix cancer did not have obvious appendicitis symptoms but instead presented later with symptoms of increasing abdominal girth, early satiety, and weight loss. Unfortunately, these patients often have tumor that has spread throughout the abdomen and they did not realize they had appendix cancer to begin with,” says Dr. Foster. “Be sure you are getting the right treatment beyond an appendectomy for appendix cancer.”

The Nebraska Medicine specialists are among the most experienced in the region for the diagnosis and treatment of appendicitis and appendix and abdominal cancers.