Appendix cancer, while rare, still requires expert treatment

Kiran Turaga, MD, MPH, in front of window overlooking Chicago skyline from Center for Care and Discovery

Most people don’t think about their appendix until it gives them trouble. And most people probably don’t know it is possible to get cancer of the appendix.

Appendix cancer is rare, but the multidisciplinary team medical and surgical oncologists at the University of Chicago Medicine expertly treats the disease guided by the latest research and advanced techniques.

To learn more about appendix cancer, we spoke with Kiran K. Turaga, MD, MPH, a surgical oncologist who treats a wide range of abdominal and peritoneal diseases, including cancer of the colon, stomach, mesothelioma as well as appendix cancer.

What is appendix cancer and how common is it?

Turaga: The appendix can develop many different problems. Previously thought to be a vestigial (or non-functional) organ, the appendix is now known as a source of healthy bacteria and vital immune cells necessary for normal functioning of the body. One of the most common conditions people are familiar with is appendicitis, in which the appendix becomes painfully inflamed. Appendicitis typically requires surgical removal known as an appendectomy. Appendicitis is caused by a bacterial infection, so while it is dangerous, it is not cancer.

Appendix cancer, sometimes referred to as appendiceal cancer, is rare – it is estimated that fewer than 1,500 people in the United States are affected by appendix cancer each year. Unfortunately, because of its rarity, cases of appendix cancer are typically clustered into one group. The treatment and prognosis, however, vary greatly depending on the type of cancer and how much it has spread. Furthermore, cancer of the appendix actually manifests as several conditions, not just one disease. Young adults, people in their late teens and early twenties, can get appendix cancer, but the disease is rare in children.

What are the different types of appendix cancer?

Turaga: There are several different types of appendix cancers. The cells that make up the appendix produce mucus (which is a sticky, protein-rich material), but sometimes the cells become overactive and make too much mucus which results in a condition called low-grade appendiceal mucinous neoplasm. It can be effectively treated with surgery. If these tumors rupture, they fill the abdomen with mucus fluid. This condition is called low-grade carcinoma peritonei and is denoted by a syndrome called Pseudomyxoma peritonei, which requires expert management at a peritoneal disease center. Pseudomyxoma peritonei is treatable with surgery to remove all the mucus and tissues laden with mucus, followed by heated chemotherapy (called hyperthermic intraperitoneal chemoperfusion, or HIPEC) to the abdominal linings to prevent more mucus from being produced.

There are also appendix cancers that behave more like other cancers, such as colon cancer. These are called adenocarcinomas, and they tend to be more aggressive. They can spread to lymph nodes and travel to other parts of the body, a process known as metastasis. Effective treatment of these conditions requires a careful, multidisciplinary evaluation at a high-volume center that has expertise in taking care of this condition.

Tumors of the appendix called carcinoids are neuroendocrine tumors that are often found, incidentally, during an appendectomy. Most carcinoids are treated with an appendectomy, but occasionally more radical surgery is necessary. Goblet cell carcinoids, a hybrid of a carcinoid and an adenocarcinoma, are a special type of appendix cancer. This tumor has a high propensity to metastasize and requires multidisciplinary care.

What are the signs and symptoms of appendix cancer? How is appendix cancer diagnosed?


Turaga:
Many times people don’t even know they have appendix cancer. Typically, the cancer is found incidentally, during surgery or diagnostic imaging, such as a CT scan. One-third of patients can present with appendicitis, so many times appendicitis can be a sign of a manifestation of appendix cancer. Sometimes patients with appendix cancer will present with a feeling of fullness in their belly because of the volume of fluid inside. 

What are the treatment options for appendix cancer?


Turaga:
The treatment of appendix cancer is nuanced. It should be planned and executed by surgeons experienced in the management of this type of cancer. Since it is such a rare condition, there are only a few people in the country who routinely treat appendix cancer. The University of Chicago Medicine is one of the few high-volume centers for patients with appendix cancer. 

Treatment depends on whether the disease is localized or it has spread to the lining. If it’s localized, many times it’s treated with surgery to remove either the appendix or the appendix plus part of the colon. If it has spread, treatment involves cytoreductive surgery and HIPEC, which involves heated chemotherapy inside the abdomen.

What is the prognosis for appendix cancer?

Turaga: The good news is that a lot of people can be treated effectively, even cured, and can live a long, full life. How patients respond to treatment depends on three factors:

  • The exact nature of the cancer
  • The amount of cancer
  • The expertise of the treating team

Every patient is different. We provide each one with a specific plan. This includes the details about their cancer and an explanation of how the therapy works, helping patients better understand the prognosis and treatment of the disease.

Why is the University of Chicago Medicine considered an expert in this area?

Turaga: The University of Chicago Medicine is focused on providing exemplary personalized care for patients with appendix cancer, by crafting the best possible treatments. Our pathologists and radiologists are world renowned for their understanding and contributions to appendiceal disease. Our medical and surgical oncologists provide patients with care that is at the forefront of medicine, helping balance decisions regarding work, family and life with the treatment of this cancer. Our multi-disciplinary team includes a dedicated dietician, physical therapist, supportive oncologists, care coordinators and clinical specialists who tailor treatment plans to fit our patients’ best interests. 

Our healthcare team has actively contributed to the management of appendix cancer nationally and internationally and is renowned for it. We have helped advance the field for years and continue to do so, with passion.

What are the new advances happening in research?

Turaga: Our work with scientists and clinicians at the University of Chicago, one of the world’s leading research universities, is combined with our own efforts to unravel the complexities of the appendix. We study how the microbiome affects the pancreas and how genetic flaws may lead to this disease. We explore preventive strategies as well. We collaborate closely to help solve our common challenge — to cure cancer.

Kiran K. Turaga, MD, MPH

Kiran Turaga, MD, MPH, is a surgical oncologist with a special interest in the treatment of advanced malignancies, including metastatic cancers. He is an expert in regional perfusion including hyperthermic intraperitoneal chemotherapy (HIPEC), a technique that delivers high doses of heated chemotherapy directly to abdominal organs.

Learn more about Dr. Turaga

Hyperthermic intraperitoneal chemoperfusion (HIPEC)

Hyperthermic (or Heated) Intraperitoneal Chemoperfusion (HIPEC) is a surgical procedure that’s giving new hope to patients with abdominal cancers. Immediately after removing visible tumors through what’s called cytoreductive surgery, our surgeons pump a powerful dose of heated chemotherapy inside a patient’s abdomen.

Learn more about HIPEC