Appendix Cancer: Why You Need Expert Answers

• Only a few centers specialize in this rare cancer

  Appendix cancers are rare. Fewer than 1,500 people are diagnosed with them each year. But for those who do receive this diagnosis, getting the right treatment depends on knowing what type of appendix tumor they have.  

  To understand why, it helps to know a little about this disease.  

  A difficult cancer to detect

  Appendix cancer starts in the appendix, a small organ located near the junction of the large and small intestines. Because appendix cancer often does not cause symptoms at first, it is hard to diagnose in its early stages. Often, it’s revealed during an imaging scan for an unrelated condition. In many cases, appendix cancer is diagnosed after a person has their appendix removed for what was thought to be appendicitis.  

  “These cancers can hide and commonly be mistaken for appendicitis,” says Fox Chase Cancer Center surgical oncologist Stephanie H. Greco, MD, FACS.  

  Some appendix tumors produce mucin, a jellylike substance. The mucin from these tumors can spread cancer to the abdomen, leading to a condition called pseudomyxoma peritonei (PMP), or “jelly belly.” Mucin can accumulate in a person’s abdomen for many years before it’s discovered.  

  “Eventually, the abdomen becomes distended,” Dr. Greco says.

  It isn’t just one disease  

  Appendix tumors are grouped according to the cells where the cancer starts, and there are many different types. These include:  

  •  Epithelial appendiceal tumors, which start in the appendix lining. Some of these tumors can produce the mucin that leads to PMP.  
  • Neuroendocrine tumors, which grow from cells involved in digestion and colon movement. They are also called carcinoid tumors.  

  Other appendix cancer subtypes include goblet cell carcinoids, colonic-type adenocarcinomas, and signet ring cell carcinomas.  

  In addition to determining the type of an appendix tumor, pathologists also determine the grade—how abnormal the cells look when viewed under a microscope. This helps predict how slowly or quickly a cancer is likely to grow. Appendix tumors can range from less aggressive to more aggressive, depending on their grade.  

  All of this information can affect a patient’s treatment options.  

  “Knowing the type of appendix cancer is very important,” Dr. Greco says.  

  It can also be a challenge. Because appendix cancer is rare, many doctors don’t diagnose or treat it often—if ever.  

  Appendix cancer treatments are tailored

  The main treatment for appendix cancer is surgery, sometimes with chemotherapy before, during, or after surgery. In addition to the type of appendix cancer, its size and location—whether it is only in the appendix or has spread elsewhere—are also important factors when deciding how best to treat it.

  Surgical options include:

  • Appendectomy. Certain low-grade tumors confined to the appendix can often be treated successfully with surgery alone, Dr. Greco notes. Options may include appendectomy (removal of the appendix) or right hemicolectomy (removal of the right side of the colon along with the appendix).  
  • Cytoreductive surgery and HIPEC (hyperthermic intraperitoneal chemotherapy). This treatment, which is only offered at specialized centers like Fox Chase, can be an option for appendix cancers that have spread throughout abdomen and/or have produced mucin. In a two-step procedure, surgeons remove the cancer, mucin, and parts of the complete organs.  

  Next, the abdominal cavity is bathed with heated chemotherapy drugs. Heating increases the chemotherapy’s toxicity, which makes it more effective at killing any remaining cancer cells, Dr. Greco explains.

  Consider a second opinion

  If you are diagnosed with appendix cancer, a second opinion from an experienced specialist can be key to getting the right treatment. But because appendix cancer is so rare, only a few hospitals around the country have experience treating it.  

  Fox Chase is one of the handful of U.S. cancer centers that specializes in treating appendix cancer, offering a full array of treatment options, including cytoreductive surgery and HIPEC.

  To schedule an appointment with an appendix cancer specialist at Fox Chase, call 888-369-2427 or request an appointment online.