Editor’s Note (Aug. 17, 2018): On Thursday, the family of Aretha Franklin confirmed that the 18-time Grammy Award-winning singer and songwriter best known for hits like “Respect,” “(You Make Me Feel Like) A Natural Woman” and “Spanish Harlem,” died of pancreatic neuroendocrine cancer. She was 76 years old.
Pancreatic neuroendocrine tumors (pancreatic NETs or PNETs) account for about 6 percent of all pancreatic tumors.
Learn more about the disease that claimed this timeless icon’s life:
- CBS: What Makes Pancreatic Cancer So Deadly?
- Axios: The Cancer that Killed Aretha Franklin
- Forbes: The Agonizingly Slow Progress Against the Cancer That Killed Aretha Franklin
- Health.com: Aretha Franklin Died of Advanced Neuroendocrine Pancreatic Cancer. What Is That?
- USA Today: Soul Legend Aretha Franklin Had Pancreatic Cancer, One of the Most Deadly Forms
Here are some important things to know about PNETs:
1) PNETs originate from the endocrine (hormone-producing) cells of the pancreas. The pancreas has two main functions: exocrine functions involve the production of enzymes to break down food, and endocrine functions involve the production and regulation of hormones that maintain proper sugar levels in the blood.
2) About 6 percent of pancreatic cancer diagnoses are PNETs. The two main types of pancreatic cancer are adenocarcinoma (exocrine tumors) and neuroendocrine (also referred to as islet cell tumors). About 6 percent of the 55,440 pancreatic cancer diagnoses expected in 2018 will be PNETs. PNETs are generally slower growing and less aggressive than adenocarcinoma.
3) PNET symptoms vary depending on the type of tumor. PNETs can be classified as functional (hormone-producing) or nonfunctional (don’t produce hormones). Functional PNETs, which are less common, can cause symptoms that depend on the type of hormone that the tumor cells produce. For example, gastrinomas overproduce gastrin, which leads to acid reflux, burning abdominal pain and other gastrointestinal symptoms. More commonly, PNETs are nonfunctional, and these tumors lead to vague symptoms like pain or jaundice if the tumor is large.
4) PNETs are treated differently than adenocarcinoma. There are some treatment strategies that could apply to both PNETs and adenocarcinoma, such as surgery, radiation therapy or chemotherapy, although different chemotherapy agents are typically used. Targeted therapies, such as Sutent® and Afinitor®, have been approved for the treatment of PNETs. Lutathera, approved in January 2018, represents the next generation of hormonal therapies that can be effective for PNET patients whose tumors express somatostatin receptors. Patients with PNETs are also encouraged to consider clinical trials.
The Pancreatic Cancer Action Network (PanCAN) maintains a comprehensive database containing up-to-date information about all pancreatic cancer clinical trials taking place in the United States – including trials for PNET patients. Every treatment available today was approved through a clinical trial. PanCAN strongly recommends clinical trials at diagnosis and during every treatment decision.
5) Patient Central is here to help. PanCAN’s Patient Central Associates can provide PNET patients and their caregivers with accurate, up-to-date information about the diagnosis and treatment of neuroendocrine tumors of the pancreas. Resources available to PNET patients include personalized clinical trials searches and access to PanCAN’s Know Your Tumor® precision medicine service for molecular profiling to determine if a particular treatment aligns with the patient’s tumor’s biological characteristics. In addition, PanCAN’s physician database can provide a list of PNET specialists.
Want to learn more about PNETs? Check out our recent educational webinar discussing treatment approaches and highlighting research developments for PNETs. And please contact Patient Central with questions about any type of pancreatic cancer – we are here to help.