The news was released Thursday that Aretha Franklin, known as the “Queen of Soul,” died of a rare form of pancreatic cancer.

Eric Liu, MD, a neuroendocrine tumor surgeon with Sarah Cannon at Presbyterian/St. Luke’s Medical Center addressed key questions about this diagnosis below.

What type of pancreatic cancer did Aretha Franklin have?

When discussing pancreatic cancer, people usually talk about adenocarcinoma of the pancreas, which is the more common form of pancreas cancer. The survival time for this type of cancer is 1-3 years in people who have surgery, chemotherapy and radiation treatment.

However, another kind of pancreatic cancer that is not as common is pancreatic neuroendocrine cancer, some call it “PNET”. This is the form of cancer that Aretha Franklin and Steve Jobs had.

What are the main functions of the pancreas?

The pancreas has two major functions – one for digestion, the other for hormones. It is a banana-shaped organ tucked in the upper, back part of the abdomen. Its primary function is to secrete enzymes to digest food. About 1 percent of the pancreas’ job is to secrete hormones through these tiny cells – neuroendocrine cells – that control things like blood sugar. For example, when individuals are diagnosed with diabetes, that’s a disease of the pancreas.

What causes pancreatic neuroendocrine cancer?

We don’t know what causes neuroendocrine pancreatic cancer in most patients.  There are rare genetic syndromes where patients are at increased risk for pancreatic neuroendocrine cancers.

What’s the difference between pancreatic ductal carcinoma cancer and neuroendocrine pancreatic cancer?

Neuroendocrine pancreatic cancer tends to grow much slower than adenocarcinoma of the pancreas – the type of cancer Patrick Swayze died of in 2009. The survival rates for patients with pancreatic neuroendocrine cancer is much longer than for patients with pancreatic adenocarcinoma.  The most common organ affected by neuroendocrine cancer is the liver. However, it can spread to other areas of the body including lymph nodes and bones, for example.

How many people are affected by neuroendocrine pancreatic cancer?

Neuroendocrine pancreatic cancer is found in about 6 percent of pancreatic cases. There are roughly 177,000 cases of overall (pancreas and other organs) neuroendocrine cancer cases, and an estimated 7,000 new cases of total neuroendocrine cancers per year.  This is compared to pancreatic adenocarcinoma, where there are around 55,000 cases diagnosed in the United States per year.

How is it diagnosed?

It’s difficult to diagnose. It’s typically diagnosed by a CT scan that can show tumors in places like the pancreas or liver.

Who’s most at-risk?

Unfortunately, it’s affecting a growing number of people, but there is no particular population. It’s usually diagnosed later in life, typically between the ages of 50 and 60. The diagnosis is almost equally distributed between genders, however, slightly higher in men.

What are the symptoms?

There can be a wide-range of symptoms including:

  • Diarrhea
  • Changes in blood glucose
  • Ulcers
  • Cough
  • Joint pain
  • Wheezing
  • Flushing (blushing)

It can also be asymptomatic. For example, a person may go to the hospital with a fractured rib, and get a CT scan that will show tumors in the pancreas or other organs.

How is it treated?

There are a variety of different therapies used to treat neuroendocrine pancreatic cancer including:

  • Surgery, removing the tumors
  • Hormone treatments by medication
  • Chemotherapy
  • Liquid radiation

There are also ways to treat specific organs like the liver, called liver embolization.

What is the survival rate of this particular cancer?

We cannot say for sure the life expectancy, as it can vary. Most of the time it is a slow-growing cancer, but it can be aggressive. Sometimes people can live for 20-30 years with this disease, and some people live for one to two years. It depends on the aggressive nature of the disease.

How can I help lower my risk?

There is no way to reduce the risk of neuroendocrine pancreatic cancer, but maintaining a healthy lifestyle is important. This includes exercising, refraining from smoking and eating a balanced diet.

According to the American Cancer Society (ACS), the risk of getting the more common pancreatic cancer (adenocarcinoma) is about twice as high among smokers compared to those who have never smoked. About 20 percent to 30 percent of pancreatic cancers are thought to be caused by cigarette smoking. Cigar and pipe smoking also increase risk, as does the use of smokeless tobacco products.

Additionally, obesity is also a risk factor for pancreatic cancer (adenocarcinoma).  The ACS estimates that very obese people are about 20 percent more likely to develop this form of cancer.

If you have questions about pancreatic cancer, you can talk to a nurse available 24/7 through askSARAH. Visit askSARAHnow.com.

Dr. Eric Liu is the co-director of the Neuroendocrine Institute at HealthONE’s Presbyterian/St. Luke’s Medical Center in Denver, Colorado. Presbyterian/St. Luke’s Medical Center is an affiliate of HCA Healthcare.