Actor’s Death Highlights Rare Cases of Appendix Cancer

Actor Adan Canto, known for his roles in “X-Men: Days of Future Past,” “Designated Survivor” and “The Cleaning Lady,” has died of appendiceal cancer, his publicist announced. He was 42.

Appendiceal cancer is a relatively rare condition that causes rapidly dividing cells to form malignant tumors within the appendix. According to the National Cancer Institute, the disease affects about 1 to 2 people per 1 million per year.

“It’s generally a quite rare malignancy,” said Dr. Iman Imanirad, a medical oncologist in the Gastrointestinal Oncology Program at Moffitt Cancer Center. “Incidence rates are still low, but it’s been growing significantly over the last decade.”

A study published in JAMA Network Open looked at cancer incidences in people younger than 50 from 2010-19. Researchers found that gastrointestinal cancers had the fastest growing rate over that time period. Among those gastrointestinal cancers, the fastest-growing incidence rates were in the appendix.

While there’s no concrete cause, Imanirad believes the rise in appendiceal and other gastrointestinal cancers is likely due to a combination of diet, lifestyle and improved diagnostics.

Dr. Iman Imanirad, medical oncologist

There are currently no screening guidelines for appendiceal cancers. The disease typically doesn’t produce symptoms until it reaches an advanced stage. Because of this, many cases are detected in imaging scans or during abdominal surgery performed for an unrelated reason.

“Typically during these scans, doctors will notice some thickening or swelling in the appendix,” Imanirad said. “This is usually how the diagnosis begins.” Oftentimes, the cancer can present as acute signs and symptoms of appendicitis, and it is only confirmed on pathology evaluation.

The types of appendix cancer are generally classified based on the type of cell in which the condition originated. The specific types of appendiceal cancer and some of their characteristics include:

• Carcinoid tumor – These tumors usually develop in the hormone-producing cells at the tip of the appendix. Approximately half of all appendix tumors are carcinoid tumors.
• Mucinous adenocarcinomas – These tumors develop in the epithelial cells that line the appendix. The cells produce a jellylike substance called mucin and could be fast or slow growing.
• Colonic-type adenocarcinoma – Under the microscope they resemble the cancers originating from the nearby colon.
• Signet-ring cell adenocarcinoma – This rare and aggressive type of adenocarcinoma usually forms in the stomach or colon, but it can also develop in the appendix and cause appendicitis.
• Goblet cell carcinomas/adenocarcinoids – Goblet cell carcinomas have features of both adenocarcinomas and carcinoid tumors. They are more aggressive than carcinoid tumors.

While symptoms of appendiceal cancer will depend on the type, the most common are abdominal distension, bloating and changes in bowel habits like constipation or bowel obstruction. If the disease spreads, it tends to metastasize to the peritoneum, a lining in the cavity of the abdomen. 

Dr. Sean Dineen, surgical oncologist

Dr. Sean Dineen, an associate member of the Department of Gastrointestinal Oncology, heads up Moffitt’s Peritoneal Disease Team that has become a national leader. According to Dineen, treatment options will usually be a combination of chemotherapy and surgery.

“We would approach a low-grade appendix cancer differently than a gastric cancer depending on the aggressiveness of the cancer,” Dineen said. “The more aggressive the cancer, the more likely we are to combine systemic therapy with a regular type of IV chemotherapy. For the less aggressive cancers, surgery alone may be the approach.”

Moffitt’s Peritoneal Disease Team uses cytoreductive surgery and heated intraperitoneal chemotherapy. Surgeons investigate all the surfaces of the abdomen and can remove the lining of the abdominal cavity and any organs that have tumor on the surface.

Following surgery, a heated chemotherapy solution is put into the abdomen. After about 90 minutes, the solution is rinsed out and incisions are closed. The single chemotherapy treatment helps to treat and sterilize microscopic disease.