Pancreatic cancer has been in the national spotlight lately with "Jeopardy" host Alex Trebek revealing his diagnosis. For the majority of all pancreatic cancer cases, the cancer begins in the ducts of the pancreas. This is known as pancreatic ductal adenocarcinoma and is the deadliest form of the disease. The other 5%-10% fall under the category of pancreatic neuroendocrine tumors (pNETs). While rare form, pNETS are still potentially deadly. Two prominent people who succumbed to pNETs include Aretha Franklin and Steve Jobs.
To learn more about pNETs and how they differ from other types of pancreatic cancer, we spoke with Xavier Keutgen, MD, the director of University of Chicago Neuroendocrine Tumor Program and one of the few surgeons in the country with advanced expertise in extensive removal of neuroendocrine tumors. Keutgen is also a lead investigator on several clinical trials focusing on new diagnostic and therapeutic methods for endocrine and neuroendocrine tumors.
What is the best way to understand what a neuroendocrine tumor (NET) is? Why is it called "neuro?"
Neuroendocrine tumors are tumors that develop from neuroendocrine cells. These are cells that can be found in almost every organ in the body yet, despite the word "neuro," they have nothing to do with the brain. Their name stems from the cells' connection to the autonomic nervous system (the nervous system that you cannot control). Neuroendocrine tumors can develop in any organ that has neuroendocrine cells. These cells produce hormones or proteins that can be secreted in our bloodstream. For example, in the pancreas, you can find these cells in pancreatic islets, which secrete hormones like insulin that regulate our blood sugar. Although pancreatic neuroendocrine tumors develop from these islet cells that normally secrete hormones, about 75% of the tumors are nonfunctional and do not produce hormones.
How common are NET tumors? What is the most common NET tumor you see at UChicago Medicine?
Currently, 170,000 people live with a NET tumor in the United States. Each year, 400-700 people in the Chicagoland area are diagnosed with a neuroendocrine tumor. It's not as rare as most people think and is even getting more common. That's most likely due to a combination of being able to better diagnose these tumors and just a general increase in occurrence. I think that, overall, the most common NETs we see in a clinical setting at UChicago Medicine are NETs of the lung, small bowel and pancreas.
What is the difference between pancreatic ductal adenocarcinomas and pancreatic neuroendocrine tumors (pNET)?
The most common mistake is that "pancreatic cancer" is confused with "pancreatic neuroendocrine tumors." Usually pancreatic cancer is defined as adenocarcinoma of the pancreas (derived from pancreatic ductal cells), which has a bad prognosis. Only 5% to 10% of pancreatic tumors are pNETs. They behave differently, have a much better prognosis and are treated completely differently. Unlike the more common type of pancreatic cancer, 50% of patients that have metastasized pNET (or cancer that has spread to other parts of the body) live five years or longer after diagnosis.
What is the treatment for pNETs?
The most common treatment is surgery. If the tumor is still localized and has not spread, we remove tumors greater than 2 centimeters. We recently published a study examining the correlation between tumor size and surgery and what size is best to perform surgery for. If the tumor secretes hormones (~10-25% of patients), the tumor is resected regardless of size. If the tumor has spread, surgery is still common. Here at UChicago Medicine, we are very good at and specialized in this type of surgery. Related Stories
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