What is appendiceal cancer? Actor Adan Canto dead from rare disease

Adan Canto, who starred in “Designated Survivor” and “The Cleaning Lady,” died Monday following a private battle with appendiceal cancer.

Also called appendix cancer, appendiceal cancer is a very rare condition that has become more common in recent years.

One study found that the incidence of malignant appendiceal tumors increased by 232% in the US and 292% in Canada between 2000 and 2016.

About one in three appendix cancer patients are diagnosed before they turn 51, according to a recent report. Canto died this week at the age of 42.

Because appendix cancer is so rare — only a few thousand Americans are diagnosed each year — the research is not as abundant compared to other cancers.

Here’s what we know about this disease.

What is appendix cancer?

Most humans — but not everyone — have an appendix. Some people are even born with a double appendix, which is extremely unusual.

The function of the appendix — a small pouch located on the lower right part of the belly — has long been the subject of debate.

Some researchers say it exists to protect good gut bacteria, playing a key role in immune function, while others say it’s not necessary at all.

In fact, some 300,000 appendectomies are performed each year in the US to remove an inflamed, swollen or infected appendix. Patients go on to have normal lives without it.

Most appendiceal tumors are discovered during an appendectomy.

What are the symptoms?

Appendix cancer is often confused with colon cancer, the second leading cause of cancer-related deaths worldwide, which can lead to misdiagnosis.

Patients may experience acute pain similar to appendicitis or chronic abdominal pain, abdominal swelling, pelvic discomfort, changes in bowel habits, weight loss, nausea, anemia or infertility.

If a person notices those symptoms and is not undergoing an appendectomy, a doctor will use imaging tests to examine the appendix. A biopsy may be needed to remove a small piece of tissue to look for cancerous cells.

“Unless it is caught early, by the time you know that you have appendix cancer, you’ve got tumor all over your abdomen,” Dr. John Paul Shen said in a 2021 post on the University of Texas MD Anderson Cancer Center site. “The tumor that’s in the appendix is no longer the problem.”

Vanderbilt University researchers are studying genetic links to appendiceal cancer to learn more about what causes it.

What are the treatment options?

Surgery and heated chemotherapy administered directly into the abdomen may treat early-stage appendix cancer.

University of California San Diego researchers said there is a five-year survival rate of 67% to 97% for low-grade tumors detected early.

Treatment is more complicated for high-grade tumors.

“High-grade tumors grow too fast. If you can’t take all the tumor out, the tumor that’s left is going to keep growing,” Shen explained.

The doctor said his team is trying to find an easier way to screen for appendix cancer, which could improve treatment options.

“One of the reasons that it’s hard to make a drug for appendix cancers is that we don’t have good ways to test it,” he said. “Pretty much any time someone goes to surgery, we’re capturing their tissue so we can study it and see what genes are mutated. We’re using that knowledge to help design drugs.”