What Stuart Scott's Story Can Teach Us About a Rare Cancer

What he said about the word "beat"

Stuart Scott anchored ESPN's SportsCenter. He changed how sports were talked about on television. The Guardian reported that he died on Sunday, January 4, 2015, at the age of 49. It described "a long battle with cancer."

The line people still quote came from his Jimmy V Award speech at the 2014 ESPYs. "When you die, it does not mean that you lose to cancer," he said. "You beat cancer by how you live, why you live, and in the manner in which you live."

Reporting at the time did not consistently name which cancer he had. This page will not fill that in. Instead it explains cancers of the appendix. They are an uncommon group, and they show why rare cancers are harder to find, harder to name, and harder to treat.

Why "rare" changes the medicine

A rare cancer is not just an unusual one. Rarity has practical consequences.

Fewer patients means fewer randomized trials. Guidance then leans on case series and expert consensus. Pathologists also see fewer specimens. A second opinion from a specialist center really does change diagnoses. And symptoms overlap with common conditions, so the path to an answer is longer.

The appendix illustrates all three.

The appendix, and what grows in it

The appendix is a narrow pouch a few inches long. It hangs off the first part of the large intestine, low on the right side of the belly.

Tumors there fall into several groups. Neuroendocrine tumors, once called carcinoids, start in hormone-making cells. Mucinous tumors make thick jelly-like mucus. Adenocarcinomas look like colon cancer. Goblet cell tumors sit in between.

The National Cancer Institute (NCI) puts the worldwide rate of gut neuroendocrine tumors at roughly 2 per 100,000 people. Average age at diagnosis is 61.4 years. Appendix tumors are only a slice of that.

Most are found by accident

Here is the striking part. These tumors are usually found when the appendix is taken out for something else. That is most often suspected appendicitis. The surgeon operates for an inflamed appendix. Days later, the pathologist finds a tumor in the specimen.

NCI notes that these patients tend to be much younger than patients with other appendix tumors. It also adds a caution. The age and sex patterns may reflect who gets an appendectomy in the first place, rather than who gets the disease.

Size drives the operation

This is a rare instance where a single measurement dictates surgery, and the thresholds are worth knowing.

NCI reports that about 90% of these tumors measure under 1 cm. Most also sit away from the base of the appendix. Those, it states, "can be consistently cured by appendectomy." Nothing more is needed.

Tumors larger than 2 cm are different. NCI states they require a right-sided hemicolectomy. That means taking out the right part of the colon, plus the nearby lymph nodes. The risk of spread is too high to do less.

Between 1 and 2 cm, the answer is genuinely unsettled. NCI says a hemicolectomy may fit if the tumor has grown into the mesoappendix. The same is true if tumor was left at the cut edges, or if lymph nodes are involved. Other features can tip the decision. They include a high Ki-67 index, meaning many dividing cells, a high mitotic count, or growth into blood vessels.

Say a family member has had an appendix tumor removed. Two questions matter most. How large was it, and were the margins clear?

Goblet cell tumors are their own problem

NCI describes goblet cell carcinoid, also called adenocarcinoid, as a rare variant. It mixes hormone-making and gland-forming features.

It behaves differently from ordinary appendiceal neuroendocrine tumors. It usually appears around age 50, often with a widely inflamed appendix. NCI describes it as aggressive. It often spreads to the peritoneum, the lining of the abdominal cavity, and to the ovaries. Under the microscope it can look like a mucinous adenocarcinoma.

Carcinoid syndrome, and why the liver matters

Some neuroendocrine tumors release vasoactive amines. Those are chemicals that act on blood vessels and airways. NCI reports that carcinoid syndrome hits fewer than 20% of patients with these tumors. It causes flushing, belly pain, diarrhea, wheezing, and carcinoid heart disease.

There is a neat reason for who gets it. Blood from the gut passes through the liver first. The liver breaks these chemicals down well. So the syndrome rarely appears until the tumor reaches the liver.

When to seek care

Appendix tumors have no symptom list of their own. That is part of the problem. See a clinician for:

• Pain in the lower right abdomen that comes and goes over weeks
• A gradually swelling abdomen, or clothes becoming tight around the waist
• A change in bowel habit lasting more than three weeks
• Unplanned weight loss
• Episodes of facial flushing with diarrhea and wheezing, especially together

Go to an emergency department for severe belly pain with fever and vomiting, especially low on the right. That is appendicitis until proven otherwise. It is a surgical emergency.

Outcomes

NCI states that survival is excellent when the tumor stays local. That means confined to its original area and nearby nodes. With distant spread, 10-year survival falls to about 30%.

Across gut neuroendocrine tumors as a whole, NCI reports five-year survival of 39% to 60% once spread has occurred. It adds a caveat. Some patients follow a slow course lasting years. Others progress fast. The presence of spread alone does not tell you which.

That caveat applies to every number here. These figures summarize groups studied in the past. They do not tell any one person what will happen.

Sources

• https://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq
• https://www.theguardian.com/sport/blog/2015/jan/05/nfl-wild-card-weekend-did-the-officials-or-the-cowboys-kill-the-lions