The Queen of Soul, The Amazing Aretha Franklin, Was Taken By Neuroendocrine Tumors: Here’s What You Should Know About These Tumors On World NET Cancer Day.

Although these cancers are rare as is denoted by the NET cancer community adopting the zebra as a mascot it’s important that people are aware of this group of cancers. Especially since they are often hard to diagnosis.

Now, let’s take a look at the 18-time GRAMMY award winning singer and songwriter’s battle against this cancer to lear more about the disease.

Aretha Franklin’s Cancer Battle

Aretha Franklin died on August 16, 2018, at age 76 from “advanced pancreatic cancer of the neuroendocrine type,” as confirmed by one of Franklin's doctors. The “Respect” singer was diagnosed with this rare type of pancreatic cancer in 2010, and her doctors told The Associated Press that she went through treatment with grace and determination.

"I think she had her priorities very clear in her mind… She would ask me how long this treatment would go for, what would be her restrictions," one of her doctors said. "As far as I can see, she was able to live that dream, or her plan."

That doctor also described Franklin as an “extremely kind” person who “didn’t let cancer cripple her.”

"Aretha as a person who was fighting cancer, she was very curious, she was very calm. She was hopeful, she was an optimist,” this doctor said. “She did not have that feeling that cancer was the main center of her life

“She lived her life as simply and beautifully and as full as possible every day. For us, it was such an inspiring journey of several years with her.”

Stay Positive, It Matters

For treatment, she underwent both targeted drug therapy and chemotherapy. But eventually, her brave battle against the disease came to an end.

"She was full speed she wasn't even complaining," another one of her doctors said. "That was different than what I expected.

“She never showed signs that she was close to thinking that she may give up… until the end, close to the end.”

Understanding Pancreatic Cancer

Pancreatic cancer is an aggressive disease that is difficult to detect because symptoms including jaundice and weight loss typically present at a later stage in the cancer's development. In a previous interview with SurvivorNet, Dr. Anirban Maitra, the co-leader of the Pancreatic Cancer Moon Shot at MD Anderson Cancer Center, explains what he typically sees when patients develop this disease.

RELATED: If You're A First Degree Relative of Someone With Pancreatic Cancer, Screening and Surveillance Could Save Your Life; Here's Why

"Because the pancreas is inside the abdomen often doesn't have symptoms that would tell you that something is wrong with your pancreas," he says. "By the time individuals walk into the clinic with symptoms like jaundice, weight loss, back pain or diabetes, it's often very late in the stage of the disease."

Detecting Pancreatic Cancer Early Is Crucial

Parents, siblings and children of someone with pancreatic cancer are considered high risk for developing the disease because they are first-degree relatives of the individual. PGVs (pathogenic germline variants) are changes in reproductive cells (sperm or egg) that become part of the DNA in the cells of the offspring. Germline variants are passed from parents to their children, and are associated with increased risks of several cancer types, including pancreatic, ovarian and breast cancers. Germline mutations in ATM, BRCA1, BRCA2, CKDN2A, PALB2, PRSS1, STK11 and TP53 are associated with increased risk of pancreatic cancer.

Jessica Everett, a genetic counselor at NYU Langone's Perlmutter Cancer Center, encourages people in this category to look into possible screening options.

"If you're concerned about pancreatic cancer in your family, start by talking to a genetic counselor to learn more about your risk and what options you have," Everett said.

In addition, note that up to ten percent of pancreatic cancer cases are caused by inherited genetic syndromes. So, if two or more members of your family have had pancreatic cancer, or if you have pancreatic cysts, it's worth asking your doctor to check for pancreatic cancer since you're at high risk.

What Are Neuroendocrine Tumors?

As we’ve mentioned before, Aretha’s Franklin’s cancer (pancreatic neuroendocrine cancer) is a very rare form of pancreatic cancer. In fact, the American Cancer Society estimates that pancreatic neuroendocrine tumors only account for about 7 percent of all cancers that occur in the pancreas.

Neuroendocrine tumors (NETs), in general, are a group of uncommon tumors that start in specialized cells in your neuroendocrine system, according to the Cleveland Clinic. These cells combine the traits of nerve cells and hormone-producing endocrine cells. They link your endocrine system which manages your hormones and your nervous system.

To help break down what neuroendocrine tumors are, SurvivorNet spoke with Dr. Renuka Iyer of Roswell Park Comprehensive Cancer Center. Dr. Iyer specializes in neuroendocrine cancer and is the co-director of the Liver and Pancreas Tumor Center.

"These [NETs] are brain and hormone-related cancers,” Dr. Iyer said. “That's one thing that's different about this disease, is the fact that it can produce hormones. Some [NETs] produce hormones like insulin."

Dr. Iyer shared some of the basics about NETs:

• Neuroendocrine tumors are rare.
• NETs are most commonly found in the GI tract.
• Because of their hormone production, they can be found anywhere.
• They're not as easy to diagnosis as other diseases, so that can be a challenge. In fact, Dr. Iyer says, "The average patient sees 7-12 doctors before their NETs are properly diagnosed. Most people think they're gallstones or related to the appendix."

Incidences of NETs have been on the rise. But experts believe this is due to an increased awareness of the tumors and improvements in diagnosis. Imaging tests such as CT and MRI scans have led to a greater discovery of the tumors, along with the improvement of the ability to distinguish NETs from other cancers in labs. Dr. Iyer specifically mentioned how the NetSpot scan came out within the last 12 years to detect these cancers and created a better method for finding these tumors.

"With better ways to find them and measure them, it is helping to move the field along," Dr. Iyer said.

Treatment for NETs primarily include surgery, chemotherapy, and radiation, according to Dr. Iyer. He said surgery was the nuumber one preference for treatment, when possible.

"Even if it has spread if it is at stage four it can be surgically removed," he said.

But drugs can also be a good option. And Dr. Iyer says that drugs used to block the hormone signals and slow the cancer spread are effective.

"We used targeted agents, targeted radiation,” he said. “Radiologists have options to offer where they can burn these tumors or put other particles in the liver to try to attack [the tumor]." (The most common type of NETs are found in the liver.)

Contributing: Anne McCarthy

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