Mysterious rise in appendiceal cancer in young people: The disease that killed Designated Survivor star Adan Canto at just 42 has risen 200% since 2000 among under 50s

Doctors are warning of a mysterious rise in appendiceal cancer cases among young adults – after actor Adan Canto died from the disease at the age of 42.

The cancer is very rare, with fewer than 1,000 cases diagnosed in the US each year – equivalent to one to three per million people.

But research shows that the disease is becoming increasingly common, with disease rates among the over-50s increasing by 200 percent over the past twenty years.

Appendix cancer is easily dismissed in its early stages, with symptoms – including bloating and abdominal pain – attributed to other causes, increasing the risk of the cancer not being diagnosed until later stages when it is more difficult to treat.

Doctors are unsure of the cause of the increase but link it to an unexplained increase also seen in bowel cancer – with more cases being recorded among young people.

Read on for everything you need to know about appendiceal cancer:

What is appendiceal cancer?

Appendiceal cancer, or appendix cancer, is a form of cancer that grows from cells in the appendix – a small finger-shaped pouch at the end of the colon.

Doctors are unsure of the function of the appendix. Some experts say it may act as a storehouse for good bacteria, while others say it is an evolutionary holdover from a time when human ancestors ate more plants.

But the organ, like other organs in the body, can develop cancer.

There are two types of appendices cancer: epithelial appendices cancer and neuroendocrine appendices cancer.

Epithelial appendiceal cancer grows from cells that form the lining of the appendix. This leads to a buildup of mucin, a jelly-like substance that protects the lining of the stomach, intestines and appendix.

When mucin builds up too much, it leads to rupture of the appendix.

Neuroendocrine appendiceal cancer – the most common form of the disease – grows from enterochromaffin cells, which are involved in digestion and movement in the intestines.

What are the symptoms?

One challenge with appendiceal cancer is that the symptoms are vague and can be mistaken for more common conditions.

According to the National Cancer Institute (NCI), most appendiceal cancers do not initially cause any symptoms.

But as tumors grow, the cancer can cause appendicitis – or inflammation of the appendix.

There is also a risk of it causing ascites – or fluid in the abdomen – as well as bloating, abdominal pain, an enlarged waist, changes in bowel habits and infertility.

Some patients may also experience nausea, vomiting and a feeling of fullness shortly after eating.

The lack of symptoms in the early stages means that the disease is often only discovered by accident at this point – when someone has their appendix removed or a scan for another condition.

How common is appendiceal cancer?

Appendiceal cancer is very rare, affecting only one or two per million Americans per year — equivalent to 1,000 people, according to the NCI.

The cancer is most common among people between the ages of 50 and 55, but rates have risen exponentially in recent years, especially among younger adults.

A 2020 study published in the journal Gastroenterology found that while the number of people undergoing appendectomy – removal of the appendix – has remained stable over the years, the number of appendiceal cancer cases has increased.

The researchers found that between 2000 and 2016, the number of appendiceal cancer cases increased by 232 percent.

The team noted that the strongest increase occurred in patients younger than 50 years of age.

They said this could be due to it being mistaken for more common conditions such as appendicitis, and bowel cancer, which is also on the rise among the over-50s.

Doctors are largely unaware of what is causing the increase in the number of young people suffering from cancers such as colon cancer, although some experts blame the rise in diabetes and obesity, yeast infections and the overuse of antibiotics.

Lead researcher Dr Andreana Holowatyj from Vanderbilt University Medical Center said: ‘The potential misclassification of appendiceal cancer as colorectal cancer poses a barrier to discovering disease-specific risk factors and tumor biomarkers, which would have implications for risk assessment, screening, surveillance and treatment. ‘

What causes appendiceal cancer?

Doctors are largely unaware of what causes appendiceal cancer.

According to the Moffitt Cancer Center in Florida, smoking is a risk factor for appendiceal cancer.

Additionally, the center cited a family history of multiple endocrine neoplasia type 1 (MEN1) syndrome as a potential risk factor. MEN1 causes tumors to form along the endocrine glands, which produce hormones.

Appendiceal disease is not known to run in families, the NCI says.

Other causes include obesity, which is linked to a range of health problems, and lifestyle factors such as not exercising often.

But doctors are still looking for an explanation for what causes the cancer in healthier individuals.

What is the prognosis for appendiceal cancer?

Research shows that between 67 and 97 percent of patients diagnosed with neuroendocrine tumors – the most common form – live more than five years after their diagnosis.

But for patients whose cancer is caught at a later stage and has spread to other parts of the body, the rate is lower.

Experts say the rarity of the cancer makes it difficult to calculate five-year survival.