Seven years after Apple co-founder Steve Jobs passed, the Neuroendocrine cancer community still gets “no respect.” As fans pay their respects to the Queen of Soul Aretha Franklin this week, the need for Neuroendocrine (NET) cancer awareness is heightened as her physician, Dr. Philip A. Philip, confirms her correct diagnosis–pancreatic neuroendocrine tumors, commonly referred to as pNETs. Many media outlets only reported “pancreatic cancer” and gave statistics that do not apply for pNETs. “Pancreatic cancer of the Neuroendocrine type,” as reported by Franklin’s publicist, is a completely different disease than the typical and more common pancreatic cancer (adenocarcinoma). Franklin’s physician, Dr. Philip shared, “The only thing they have in common is they both arise from the pancreas.” When the media does not get it right in the case of a beloved well known public icon, it can have a serious impact on the diagnosed and undiagnosed patient.
Neuroendocrine tumors occur in the endocrine cells of the pancreas and not the exocrine cells. This means the diagnosis and prognosis of the disease is completely different than that of the more common pancreatic cancer (adenocarcinoma). Dr. Philip explains, “Unlike the common variety pancreatic cancer (adenocarcinoma), patients with pNETs on average survive significantly longer and have an increasing number of treatment options.”
Why does the terminology matter? The incidence of neuroendocrine cancer is rising. The disease is considered rare, but based on the National Cancer Institute’s SEER data, over 171,000 Americans are living with the disease, and each year over 20,000 more will be affected. The average time to diagnose runs five to six years due to a lack of awareness about the disease in the medical community. Patients can also be under-treated or over-treated if their physician does not know the proper tests for the cancer, or does not understand the difference from other more familiar cancers.
Cindy Lovelace, co-founder of The Healing NET Foundation and a NET patient survivor exclaims, “It’s hard to get across what a big deal this is in the NET community. Even today there is anger and frustration when media refers to Steve Jobs as having pancreatic cancer or liver cancer when he really had pNETs. This is the classic misunderstanding about this disease we continue to be up against.” In the case of Lovelace, who was diagnosed with a pancreatic neuroendocrine tumor resected in 2011, the disease is now managed by a combination of periodic surgery to remove metastatic neuroendocrine tumors in the liver and medication designed to slow down the growth of the tumors.
Neuroendocrine tumors can also appear in any body part with endocrine or hormone producing cells, such as the lungs, small intestine, rectum, pancreas, stomach, appendix, and a number of other sites. Often those cancers are mistakenly referred to as a cancer of that organ, which leads to further misdiagnosis and inaccurate treatments.
According to Healing NET’s co-founder, Eric Liu, MD, a Neuroendocrine surgeon and specialist in Denver, “Awareness about this disease needs to occur at all levels from physicians, to nurses, to the general public. The number of people with neuroendocrine cancer is on the rise and they can only get the best care when there is a better understanding that it is a different type of cancer. When well treated, people can live quite a long time. But in some cases, it is very aggressive. Good care starts with more education”