Why pancreatic cancer is so deadly



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The cause of his death was not immediately announced. Trebek revealed in March 2019 that he was diagnosed with stage 4 pancreatic cancer, triggering a surge of support.

About 95% of people with pancreatic cancer die from it, experts say. It’s so deadly because during the early stages, when the tumor is most treatable, there are usually no symptoms. It tends to be discovered at an advanced stage when abdominal pain or jaundice may result. Currently, there is no general screening tool.

As people get older, the risk of developing pancreatic cancer increases. Most of the patients are over 45 and almost 90% are over 55. The average age at diagnosis is 71 years.

Men have a slightly higher chance of developing pancreatic cancer than women, which may in part be the result of increased smoking in men. In the past, when men smoked more often than women, the gender gap was greater. Currently, the lifetime risk of developing it is about 1 in 63 for men and 1 in 65 for women.

There is also a noted association with race: African Americans are more likely to develop pancreatic cancer than whites. Doctors don’t know why, but speculate that higher rates of men who smoke and have diabetes, and overweight women, may contribute to this association.

What are the types of pancreatic cancer?

The pancreas is an oblong organ that lies deep in the abdomen and is an integral part of the digestive and endocrine system. It secretes hormones to regulate the body and digestive enzymes to break down food.

There are two types of pancreatic cancer: exocrine tumors and endocrine tumors.

Exocrine tumors are the majority of pancreatic cancers, and the most common form is called adenocarcinoma, which begins in glandular cells, usually in the ducts of the pancreas. These tumors tend to be more aggressive than neuroendocrine tumors, like the ones experienced by Apple Inc. co-founder Steve Jobs, but if caught early enough, they can be effectively treated with surgery.

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Pancreatic neuroendocrine tumors make up only 1% of all pancreatic cancers. They can be benign or malignant, but the distinction is often not clear and sometimes not apparent until the cancer has spread beyond the pancreas.

The five-year survival rate for neuroendocrine tumors can range from 50% to 80%, compared to less than 5% for adenocarcinoma.

More advanced tumors have a higher risk of recurrence and can spread to the liver, said Dr. Steven Libutti, pancreatic cancer expert and director of the Montefiore-Einstein Center for Cancer Treatment in the Bronx.

Treatment options

According to the National Cancer Institute, pancreatic cancer is usually only controllable with surgical removal and only if detected before it spreads. Palliative care can improve a patient’s quality of life if the disease has spread.

Two drugs approved in 2011 may help patients with pancreatic neuroendocrine tumors. They are believed to suppress blood supply and tumor cell metabolism. This is good progress since the previous year the standard of care was chemotherapy, said Dr. Michaela Banck, a medical oncologist in New York City who treats patients with neuroendocrine tumors.

Everolimus, marketed by Novartis as Afinitor, has received approval from the United States Food and Drug Administration to treat pancreatic neuroendocrine tumors and prevent transplant rejection. The potential side effects, however, are serious: lung or respiratory problems, infections and kidney failure, which can lead to death.

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Sunitinib malate, marketed by Pfizer as Sutent, is prescribed for the treatment of neuroendocrine tumors of the pancreas, as well as kidney cancer and GIST, a rare cancer of the bowel, esophagus, or liver. ‘stomach. As with everolimus, there are risks to be aware of: it can cause liver problems and death.

Jobs underwent surgery to remove his tumor in 2004 and died in 2011. His seven-year survival after treatment is consistent with the average survival for these types of tumors, Libutti said.

If pancreatic cancers are caught early, it can increase the chances of survival, but it also depends on the aggressiveness of particular tumors in a patient. If the surgery leaves behind aggressive microscopic tumor cells, they can cause the cancer to come back.

Jobs also underwent a liver transplant in Tennessee in 2009, which is a “cutting edge trick” for the spread of neuroendocrine tumors, said Dr. Maged Rizk, director of the Chronic Abdominal Pain Center at the Cleveland Clinic, specializing in gastro. -enterology and hepatology.

Do transplants help?

Because it’s so rare, there isn’t much evidence to support transplant as a cure; the procedure could prolong life, but immunosuppressive drugs may allow any remaining cancer to grow faster, doctors say. And a European study found that the majority of patients who had a liver transplant for this type of tumor had a recurrence of the disease.

But many pancreatic cancers are detected at later stages because when the tumor is small, it often does not produce symptoms. As they grow, adenocarcinomas can block the liver ducts and cause severe back pain. Neuroendocrine tumors sometimes produce insulin, so a patient’s first symptoms may be low glucose levels. But most tumors don’t produce hormones, Libutti said.

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There are two rare genetic syndromes – multiple endocrine neoplasia type 1 (MEN1) and von Hippel-Lindau syndrome (VHL) – which increase the risk of pancreatic neuroendocrine tumors. Other than that, however, it is not clear whether having a family member with pancreatic cancer increases an individual’s risk.

Pancreatic cancer has hit the family of former President Jimmy Carter hard. He lost his father and all his siblings, his brother Billy and his sisters Ruth Carter Stapleton and Gloria Carter Spann.

The future of treatment

Researchers are working to better understand how pancreatic tumors grow and spread, Libutti said. According to the American Cancer Society, there is also a lot of research aimed at finding better treatments, targeted therapies, immune therapy, improved surgery and radiation therapy.

“There are a number of agents that are being looked at in clinical trials that focus on pathways that can allow pancreatic cancer to escape normal processes,” Libutti said.

Another line of research focuses on the discovery of pancreatic cancer biomarkers in order to be able to develop a simple blood or urine test. Unlike screenings for other conditions such as colon, breast, and prostate cancers, there is no routine way to see if a patient has a pancreatic tumor.

The future of medicine to help people with pancreatic cancer will lie in genetics, Banck said. This would involve matching a person’s particular type of tumor using genomic information with treatment.

“What will make a real difference in the future is the revolution of the genomic age,” she said.

Jacqueline Howard of CNN contributed to this report.

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