Appendiceal cancer is in the spotlight after 42-year-old actor Adan Canto dies. Here's what you need to know about this rare cancer.

By Lisa Marie Basile | Fact-checked by Davi Sherman
Published January 17, 2024

Key Takeaways

  • The death of 42-year-old actor Adan Canto has brought appendiceal cancer under the spotlight. Appendiceal cancer refers to several types of tumors affecting the appendix. 

  • This form of cancer is relatively rare, but it’s on the rise and is being diagnosed more frequently in younger patients. 

  • Treatment ranges from appendectomy to chemotherapy to removal of regional tissue, although it depends on the type of tumor.

Actor Adan Canto, 42, of “Designated Survivor,” died on January 8, 2024, of appendiceal cancer, bringing his rare form of cancer—into discussion.[]

Appendiceal cancer is cancer of the appendix, even though it’s a hollow organ whose function is up for debate—with some recent theories postulating that it offers immunoprotective functions or stores helpful bacteria—says Wael Harb, MD, a board-certified hematologist and medical oncologist at MemorialCare Cancer Institute at Orange Coast and Saddleback Medical Centers in Orange County, CA.[]

However, “it can be confusing to just say ‘appendiceal cancer,’ as there are a few types,” Dr.Harb adds. 

Appendiceal cancer subtypes include epithelial (mucinous, non-mucinous adenocarcinoma, and signet ring cell tumors) and non-epithelial ( neuroendocrine tumors, lymphomas, and sarcomas). The majority of appendiceal tumors are neuroendocrine, while adenocarcinomas make up about 20%.[] 

“Some tumors are very slow-growing, and some are aggressive,” Dr. Harb says. A third of tumors have metastasized once discovered.[] 

According to Angela T. Alistar, MD, Medical Director of GI Medical Oncology at Atlantic Health System and the Breakthrough Treatment Center at Morristown Medical Center, “While [appendiceal cancer] shares some similarities with bowel cancers, it’s important to note that it’s a distinct entity with different characteristics and treatment approaches.”

“Appendiceal cancer is relatively rare,” Dr. Harb explains, “occurring in less than one in 100,000 people annually.” That said, appendiceal cancer has been on the rise over the past few decades. 

Recent research published in Cancer shows that between 2000 and 2016, there was an overall increase in the incidence of malignant appendiceal tumors—both malignant adenocarcinomas and neuroendocrine appendiceal tumors—) of 232% in the United States and 292% in Canada. This increase occurred in patients of all ages, sexes, and stages of disease.[]

While appendiceal cancer most commonly affects people in their 50s and 60s, Dr. Harb says, younger groups are being diagnosed, especially with appendiceal neuroendocrine malignant tumors.[]

There is also an increased association of appendiceal cancer with both colonic neoplasia and chronic ulcerative colitis.[] 

Diagnosing and treating appendiceal cancer

Diagnosing appendiceal cancer can be a challenge, Dr. Harb says. There are no reliable blood or urine tests for diagnosing appendix cancer. While appendiceal cancer is often found coincidentally by surgeons performing an appendectomy, Dr. Harb says, “if a tumor has metastasized and is more aggressive, a patient might present with swelling or pain in the abdomen.” 

Dr. Alistar adds that, in general, patients might present with “vague early symptoms…including abdominal pain (especially in the lower right abdomen), bowel obstruction, nausea and vomiting, weight loss, and bloating.” 

“The treatment for appendiceal cancer depends heavily on the stage of the disease,” Dr. Alistar says. For early stage diagnosis, surgery to remove the appendix and the surrounding tissue is the primary treatment option.  

For advanced cancer, Dr. Harb also says that treatment depends on tumor type. He notes that the use of hyperthermic intraperitoneal chemotherapy (HIPEC) treatment is common in patients with advanced appendiceal cancer, such as the mucinous subtype. Dr. Harb also says that surgeons may remove part of the colon in addition to performing an appendectomy. There are several surgery options.[][] 

“It’s important to remember that these [advanced] tumors may be more resistant to chemo,” Dr. Alistar adds. “Compared to colon and small bowel cancers, appendiceal cancer tumors may have increased resistance to chemotherapy [and] mucin production, and [a] higher burden of local complications.”

Dr. Alistar says that physicians should know that, historically, treatment options for appendiceal cancer are not very effective. However, proper treatment requires a multidisciplinary team, she says. “Encouraging patient enrollment in clinical trials is crucial for finding new and more effective treatment options,” Dr. Alistar adds. 

Dr. Harb says that patients who are concerned about developing cancer—especially after reading news headlines—should be encouraged to maintain their regular routine exams. 

“A lot of people skip these appointments, but this is where you discover things,” he says. “Share information with your physician to see if something should be pursued or not.”

Additionally, patients with genetic predispositions to cancer—those with multiple cancers or cancers at a young age in their families—should seek genetic testing, Dr. Harb says. 

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