From Symptoms to Survival: What to Know About Appendix Cancer

For too long, the appendix has been overlooked, but not by cancer surgeon Kiran Turaga, MD, division chief of surgical oncology at Yale School of Medicine.

“The appendix is an organ that for a very long time we thought was vestigial. We thought it didn’t have any function and it was just an evolutionary remnant in human beings,” says Dr. Turaga, who came to Smilow Cancer Hospital and Yale Cancer Center a few years ago from the University of Chicago. “But recently, several studies have identified the important role of the appendix in maintaining normal bacteria, as well as the important role it has in the immune system.”

The appendix has been in the spotlight this year after a study in the Annals of Internal Medicine reported a dramatic increase in diagnoses of cancer of the appendix, with cases tripling among people between the ages of 49 and 54.

An expert in the treatment of gastrointestinal cancers, Dr. Turaga has also felt cancer’s impact personally, having lost his father and grandfather to cancer.

“Every day, I come to work with the resolve to make things better for my patients and their families,” says Dr. Turaga, a physician–scientist who also is assistant medical director of the Clinical Trials Office. “The courage that cancer patients and their caregivers demonstrate every day reminds me that we need to hurry up and find a way to cure cancer.”

About one-third of all patients with appendix cancer initially present with appendicitis. A tumor or a polyp can block the narrow channel of the appendix, causing it to become inflamed and leading to appendicitis.

Others may experience symptoms such as fullness, bloating, or difficulty eating. Many people, though, have no symptoms at all. The cancer is sometimes discovered incidentally on a scan for another reason and the presence of fluid, nodules, lumps, or thickening raises red flags. Particularly in younger patients, symptoms may be present for a long time before being diagnosed, and these symptoms may be initially dismissed or misdiagnosed, for example, as irritable bowel syndrome.

Research is ongoing to understand the causes and risk factors for appendix cancer, but three main factors have emerged. About 10% of people with appendix cancer have hereditary mutations, a new finding supported by multiple studies.

Another factor is autoimmune diseases, such as lupus, rheumatoid arthritis, and ulcerative colitis. About one-third of appendix cancer patients have an autoimmune condition, compared with only about 10% of patients with breast or colon cancer.

Finally, different types of bacteria in the appendix appear to be linked with appendix cancers.

Most patients are diagnosed in their 40s or 50s. Here at Yale Medicine, the average age is about 52. Once considered extremely rare—roughly one in a million—appendix cancer is now closer to one in 100,000. While still uncommon compared with breast, lung, or prostate cancers, the numbers are rising.

Yes. For some specific types of appendix cancer, patients can be cured or can expect to live 20 to 30 years with a relatively normal life. But with more aggressive types of appendix cancer, survival may only be two to three years despite extensive treatment.

Outcomes depend heavily on early diagnosis and a thorough pathological assessment, so the care team can identify what genetic mutations are driving the cancer. Treatments can involve surgery, intra-abdominal heated chemotherapy (HIPEC), or intravenous chemotherapy.

Appendix cancers usually spread within the peritoneum—which is the lining of the abdominal cavity—rather than to distant organs such as the lungs or liver.

Appendix cancers range from slow-growing, highly treatable forms that allow for long survival, to very aggressive and malignant types. While not definitively clear, some studies suggest slight differences in predominance between sexes: women tend to have a slightly higher prevalence of mucinous subtypes, while men more often present with goblet cell types.

Mucinous adenocarcinomas are cancers that produce large amounts of mucus. They often require surgery and chemotherapy and have a tendency to recur.

Goblet cell carcinoids combine features of mucus-producing and neuroendocrine cells. They are rarer, more aggressive, and usually treated with surgery and chemotherapy. Overall, goblet cell tumors tend to have a worse prognosis.

Both types of appendix cancer are complex and need specialized treatment plans.

For people who do not have appendicitis, the cancer may be found when they have symptoms such as bloating, fullness, or difficulty eating, leading to a CT scan. The scan may reveal fluid, nodules, lumps, or thickening, that prompt further testing. Occasionally, the cancer is discovered completely by accident during scans performed for an unrelated condition.

No, but Yale Medicine researchers are actively working in this area and have developed a blood test to identify these tumors. Further their research in circulating DNA has advanced the field for treatment of these diseases.

Treatment for appendix cancer often involves a team approach, integrating the expertise of cancer surgeons, medical oncologists, radiation oncologists, geneticists, pathologists, nurses, and social workers. Surgery remains the mainstay and can be curative for many patients. When the cancer spreads to the peritoneal lining, surgeons can remove the visible disease and deliver heated chemotherapy directly into the abdomen.

During the first part of my career, we barely saw any new treatment for cancer. But in just the last five years, the pace of progress—thanks to research—has been remarkable.

Immunotherapy, in particular, is changing the landscape, and I’m hopeful we’ll see the same breakthroughs for appendix cancer.

This Q&A was adapted from Connecticut Public Radio’s “Yale Cancer Answers,” a weekly program that shares information on the latest cancer breakthroughs and treatments through conversations with experts from Yale Cancer Center.