On August 16th, 2018, Aretha Franklin, the “Queen of Soul,” passed away at age 76. Aretha will be immortalized for generations because of the success of her classic soul ballads like “Respect.” Her immense skill, talent, and persona left a lasting impact on American music and culture.
Pancreatic Neuroendocrine Tumors
Aretha’s cause of death has been widely reported at pancreatic cancer. However, there is more to this information than meets the eye. Some news sources, such as USA Today, cited that Aretha did not have your run of the mill pancreatic cancer tumor; she had a less common type, called a pancreatic neuroendocrine tumor (PanNET). In this article, we will deep dive into this rare cancer and highlight the differences between it and more frequently encountered types of pancreatic cancer.
Not Your Average Pancreatic Cancer
In about 85 percent of pancreatic cancer cases, the type of tumor encountered is known as pancreatic adenocarcinoma, but a smaller number of patients (about two percent) have PanNETs. These types originate within different cells of the pancreas. Adenocarcinomas originate in the area of the pancreas that is responsible for the production of digestive enzymes, but PanNETs appear in cells of the endocrine or nervous system within the organ. PanNETs are generally considered less dangerous than adenocarcinomas, but, as Aretha’s case clearly demonstrates, that does not mean that they can’t be deadly. The majority of PanNETs are benign and do not cause symptoms.
Who is at Risk?
The risk factors for PanNETs are distinct from adenocarcinoma and there appears to be a genetic basis for the appearance of these tumors in some cases. PanNET tumors tend to have different mutations in comparison to adenocarcinoma. They are also connected to several different genetic disorders and syndromes, such as Wermer’s syndrome, von Hippel-Lindau disease, tuberous sclerosis, and neurofibromatosis type 1. An important risk factor for PanNETs are mutations of the MEN1 gene.
Signs and Symptoms
Pancreatic cancer is notorious for not causing symptoms until it has spread and reached an advanced stage, but some malignant PanNETs can be detected earlier because they cause certain hormones to be released in abnormal amounts. These are considered “functional” PanNETs, which are classified by which hormone they release; each one can cause specific symptoms. Nonfunctional tumors are more difficult to detect and as a result are often diagnosed later, leading to worse outcomes.
When PanNETs are symptomatic, they have often reached a large size. They have distinct symptoms from pancreatic adenocarcinoma, although there is a a degree of overlap. These symptoms include jaundice, diarrhea, indigestion, and pain or a feeling of pressure in the back or abdomen. Functional PanNETs cause distinct symptoms that vary on the type.
Treatment approaches for PanNETs are distinct from those used for adenocarcinoma. Surgery for PanNETs is a viable option for localized tumors or those with limited metastasis. Outcomes from surgery can vary greatly, however. Octreotide may be used to control the excessive hormone production. Some tumors also respond well to chemo, but is many cases it is reserved as a last resort. There are some targeted therapies available for treating PanNETs, such as everolimus and sunitinib. These are effective in relieving symptoms and slowing down the progression of the cancer. Five year survival rate for PanNETs is 65 percent. To learn more about PanNETs, click here.