The Queen of Soul’s death is raising questions — and awareness — about a rare form of cancer.

On August 16, the worldlost a legendwhen Aretha Franklin, the one and only “Queen of Soul,” passed away from advanced pancreatic neuroendocrine cancer at the age of 76.

With memorial services and tributes beginning Tuesday, August 28 in Detroit, Michigan, leading up to her funeral on Friday, August 31, many will be reflecting on Franklin’s legacy and her impact on pop culture.

However, in the days since her death, people have been pondering a common question: What is pancreatic neuroendocrine cancer?

如果听起来很熟悉,那就是因为你可能会听到它之前 - Apple Pioneer Steve乔布斯也从这种情况下死亡。

尽管胰腺neuroend吸引注意ocrine cancer from these famous cases, a significant amount of misinformation exists about what it is and what you should know about it.

About 55,440 people will be diagnosed with and about 44,330 will die from a form of pancreatic cancer this year, according to the American Cancer Society . But it remains the least discussed and most underfunded compared with other types of cancer.

As its name suggests, this type of cancer affects the pancreas, a crucial organ that consists of two functioning parts — the exocrine and endocrine pancreas.

The exocrine pancreas generates enzymes needed to help you digest food, filtering it out through ducts that flow out into your small intestine.

The endocrine pancreas contains cells that release important hormones into your body’s bloodstream, like insulin, for instance, said Dr. Steven K. Libutti, FACS, the director of the Rutgers Cancer Institute of New Jersey.

Reports often use the term “pancreatic cancer” to describe all cancers of the pancreas at large. Libutti stressed that people should be aware of the difference between the more common pancreatic adenocarcinoma, which impacts the exocrine pancreas, and pancreatic neuroendocrine cancer, which is what Franklin had.

“The two couldn’t be more different. They come from completely different cell types and are treated very differently,” Libutti said. “Aretha Franklin had a very different kind of cancer than what’s commonly referred to as ‘pancreatic cancer,’ which is really pancreatic adenocarcinoma.”

He added, “Fortunately, pancreatic neuroendocrine tumors are relatively uncommon when you look at cancers that originate in the pancreas in general. They make up approximately five to six percent of pancreatic cancers.”

Libutti said that neuroendocrine tumors tend to grow more slowly and originate from the small islet cells where insulin is generated.

Pancreatic adenocarcinoma makes up about 95 percent of cancers in the exocrine pancreas. It usually occurs in the ducts of the pancreas but can also occur in the cells in pancreatic enzymes, according to the American Cancer Society .

“Neuroendocrine tumors can be very slow going, well behaved, low-grade tumors but they can go all the way up the spectrum to high-grade poorly differentiated tumors,” said Dr. Mary Mulcahy, professor of hematology and oncology at Northwestern Feinberg School of Medicine in Chicago.

Mulcahy told Healthline that she’s often struck by how the two types of cancer are often described interchangeably. She said it’s important that people know these are “two very different tumor types.”

One of the biggest challenges of treating cancers that impact the pancreas is that not a lot about them is understood. A person with any kind of pancreatic cancer might have pain in their abdomen, weight loss, loss of appetite, pain in their stomach, or show signs of jaundice or skin yellowing, said Dr. Davendra Sohal, MPH, who practices hematology and medical oncology at Cleveland Clinic.

“There really isn’t a whole lot known about risk factors for this kind of cancer,” Sohal told Healthline. “Some risk factors could be smoking, perhaps alcohol use, perhaps obesity. Beyond that, we aren’t very sure. Family history is a strong risk factor. That’s pretty much all we know at this time.”

In terms of treatment, Libutti added that no matter the type of pancreatic cancer, the best treatment is detecting a tumor early enough to perform surgery. He said that some people with hypoglycemia who use insulin need to watch out for functional pancreatic neuroendocrine tumors that might produce too much insulin.

In general, most of the tumors people will develop are not functional, meaning they don’t produce active hormones.

When tumors have spread from the pancreas to other parts of the body — commonly the liver — and when surgery isn’t an option, Libutti explained people are treated with a variety of approaches.

“我们有口服和可注射剂,可用于在他们传播时对这些肿瘤进行治疗,我们还有几项关于治疗和在我们所处理的成像侧的新技术,”他说。

Mulcahy added that there are chemotherapy options out there and currently some drug and clinical trials being carried out to devise the best treatments.

She said there are a number of molecular-based therapies that can be effective at stopping the growth of pancreatic neuroendocrine tumors, but these don’t necessarily get rid of the cancer entirely.

She also pointed out there’s a new radioactive hormone therapy recently approved in the United States. This therapy goes directly to the tumor and releases radiation to it, but it hasn’t been used often yet.

Since pancreatic cancers have received less funding for research than some of their counterparts in the medical world, they can be especially difficult to treat.

“Research done on the pancreas has been kind of a slog and a slow process,” Mulcahy said.

According to the Pancreatic Cancer Action Network, pancreatic cancer is poised to be the second leading cause of cancer-related deaths in the United States by 2020.

With those numbers, Sohal urged people to encourage anyone around them with pancreatic cancer to participate in research studies to raise awareness of and insights into the disease.

他强调,还没有足够的人来了解各种形式的胰腺癌。

When public figures like Jobs and Franklin are revealed to have a rare condition like pancreatic neuroendocrine cancer, it invariably raises attention and interest.

Additionally, Mulcahy pointed out one unintended positive of the coverage surrounding Franklin is awareness around the importance of hospice care.

“The fact that she enrolled in hospice care — which is underutilized — was significant. A lot of people shy away from hospice. They don’t like the word. This is even more so in the African American community; the service is underutilized. In fact, a greater percentage of the African American community tends to die in the hospital,” she said. “I think [a greater] awareness [of] hospice care [is one thing] that came from this unfortunate story that could be really helpful.”

Libutti added, “The diagnosis of a high-profile person like Aretha Franklin drives attention to these rare tumors that are rising in incidence. It puts a spotlight on the tumor type and generates public understanding and research dollars.”

Libutti mentioned it’s also important people touched by news of Franklin’s passing keep their own health in perspective. He stressed that these tumors are rare and, if caught early, don’t have to result in death.

“It’s not by any means a death sentence to get that diagnosis,” he said.