Post by Lauren Erb, Director of Research
Originally published as a blog post on October 13, 2011 (updated to reflect organization’s new name)
Visionary innovator Steve Jobs, co-founder and former CEO of Apple Inc., passed away on October 5, 2011. We join the many who are saddened by the loss and my thoughts and prayers go out to Steve’s loved ones.
As a passionate advocate for patients with carcinoid cancer and pancreatic neuroendocrine cancer, I am concerned over all of the misinformation that’s been propagated about Steve Jobs’ cancer.
Steve Jobs was not diagnosed with pancreatic cancer in 2004. He was diagnosed with pancreatic neuroendocrine cancer. These cancers behave differently and the reports in the media describing his cancer as “pancreatic cancer” do a disservice to everyone working to build awareness and raise funds for research in pursuit of cures for pancreatic neuroendocrine cancer.
My goal with today’s blog is to clarify the differences between these two very distinct forms of cancers. To the extent you feel I’ve succeeded, please forward my blog, post it to your Facebook page, and help spread the word.
About the Pancreas
The Pancreas is an organ that can be thought of as having two components – the exocrine pancreas and the endocrine pancreas.
The exocrine pancreas makes pancreatic enzymes and transports these enzymes in ducts that empty into the small intestine. These enzymes help your body digest the foods you eat. 95% of the cells in the pancreas serve an exocrine function.
The endocrine pancreas consists of endocrine cells that are arranged in “islets” and release hormones like insulin into the bloodstream.
What is Cancer?
Cells are the building blocks of life. All cells have highly specific functions, but not all cells have the same function. When cells that have similar functions are grouped together they form a tissue. Tissues, when grouped together to perform a specific function, are called organs (like the pancreas). All cells of the human body use the same genetic language, or building blocks, known as DNA. Cell growth and replication is highly controlled and is encoded in each cell’s DNA. However if there are enough mutations (changes) within a cell’s DNA, a cell can grow and replicate uncontrollably.
A tumor is a mass formed by an abnormal growth of cells within the body. A tumor can be non-cancerous (benign) or cancerous (malignant). A tumor is considered cancerous when it has uncontrolled proliferation (abnormal growth) and can invade and destroy surrounding tissue. Malignant tumors also have the ability to metastasize (spread to other organs of the body).
Pancreatic Cancer and Pancreatic Neuroendocrine Cancer are two distinct cancers with distinct behaviors that originate in different types of cells in the pancreas.
What is Pancreatic Cancer?
Pancreatic adenocarcinoma is a cancer of the exocrine pancreas and is often referred to as “pancreatic cancer” or “pancreatic adenocarcinoma.”
What is Pancreatic Neuroendocrine Cancer?
Pancreatic neuroendocrine cancer is a cancer that originates in the endocrine cells of the pancreas. Tumors that originate in the endocrine cells are referred to as “pancreatic neuroendocrine tumors” or “pancreatic islet cell tumors.”
The NET Research Foundation is dedicated to discovering cures for patients with neuroendocrine cancers – specifically, carcinoid cancer and pancreatic neuroendocrine cancer and so I would like to share some more information about pancreatic neuroendocrine cancer.
Different Types of Pancreatic Neuroendocrine Cancer
A “functioning” pancreatic neuroendocrine tumor secretes biologically active hormones causing a characteristic clinical syndrome. “Non-functioning” pancreatic neuroendocrine tumors do not cause a characteristic clinical syndrome.
Functioning pancreatic neuroendocrine tumors can hypersecrete (over produce) substances such as gastrin, insulin, glucagon, vasoactive intestinal peptide (VIP), and somatostatin, resulting in a characteristic clinical syndrome (Tomasseti, Migliori, Lalli, Campana, Tomassetti, Corinaldesi, 2001).
Pancreatic neuroendocrine tumors are at times associated with low blood sugar (due to secretion of insulin), diabetes (due to secretion of glucagon), or ulcer disease (due to secretion of gastrin). In other cases, neuroendocrine tumors may not secrete any hormones (Oberg, Reubi, Kwekkenboom, & Krenning, 2010).
Functioning pancreatic neuroendocrine tumors are often named based on the substance they hypersecrete: gastrinomas secrete gastrin, insulinomas secrete insulin, glucagonomas secrete glucagon, etc.
Pancreatic neuroendocrine tumors can be difficult to diagnosis with the average time between tumor development and diagnosis being 5 to 10 years (Vinik, Feliberti, Perry & Nakave, 2008; Vinik et al., 2009). Survival rates for individuals with pancreatic neuroendocrine tumors vary and depend upon tumor type, the location of the tumors, the size of the tumors, the extent and growth rate of liver and bone metastases, proliferative indices, presence of clinical syndromes and many other factors (Metz & Jensen, 2008).
Currently, surgery is the only option that offers hope for a cure (Ramage, Ahmed, Ardill, Bax, Breen, Caplin, Corrie, Davar, Davies, Lewington, Meyer, Newell-Price, Poston, Reed, Rockall, Steward, Thakker, Toubanakis, Valle, Verbeke, Grossman, and UK and Ireland Neuroendocrine Tumor Society, 2012; Metz & Jensen, 2008).
Pancreatic neuroendocrine tumors can be associated with genetic syndromes such as Multiple Endocrine Neoplasia Type 1 (MEN1), Von Hippel-Lindau Disease (VHL), Tuberous Sclerosis Complex and Neurofibromatosis Type 1 (NF1) (Metz & Jensen, 2008). MEN1 is the most significant genetic syndrome – over 80% of patients with MEN1 develop pancreatic neuroendocrine tumors, over 40% of patients develop gastrinomas, and smaller percentages develop other types of pancreatic neuroendocrine tumors (Metz & Jensen, 2008; Gibril & Jensen, 2004; Brandi et al., 2002).
Pancreatic Neuroendocrine Tumor Treatment
Pancreatic neuroendocrine tumors can be very difficult to treat. These tumors can be benign to highly malignant, indolent (slow growing) to very aggressive in development, and range from asymptomatic to causing debilitating syndromes. As a result, a multi-disciplinary team consisting of specialist physicians in NETs (gastroenterologists, oncologists, and endocrinologists), surgeons, radiologists, nuclear medicine specialists, histopathlogists, and clinical nurse specialists is often recommended (Ramage, Ahmed, Ardill, Bax, Breen, Caplin, Corrie, Davar, Davies, Lewington, Meyer, Newell-Price, Poston, Reed, Rockall, Steward, Thakker, Toubanakis, Valle, Verbeke, Grossman, and UK and Ireland Neuroendocrine Tumor Society, 2012).
Pancreatic Neuroendocrine Tumor treatment must be tailored to each patient’s tumor burden and symptoms. Treatments may be focused on inhibiting tumor growth or symptom relief. Often, this means that any given treatment plan may consist of a combination and/or series of several treatments.
If you, or someone you know, has pancreatic neuroendocrine cancer, be sure to discuss your treatment options thoroughly with your physician(s). Ultimately, all treatment decisions should be made by the patient. Click here to visit the NET Research Foundation’s Doctor Database for help finding a physician. Click here for more information about current treatments for patients with pancreatic neuroendocrine tumors.
New Treatments for Pancreatic Neuroendocrine Tumors
Earlier this year, Everolimus and Sunitinib were the first therapies approved by the FDA for treatment of patients with neuroendocrine tumors in over thirty years. I was honored to have had the honor of representing the voice of patients with neuroendocrine tumors at FDA hearings in consideration of their approval.
How is Pancreatic Neuroendocrine Cancer Different from Pancreatic Cancer?
Pancreatic neuroendocrine cancer and pancreatic cancer are two distinct types of cancer and it is important to understand the differences. These two types of cancer have different clinical courses, different diagnostic procedures, different treatment patterns, and different molecular underpinnings.
Through a grant provided by the Caring for Carcinoid Foundation, researchers at the Johns Hopkins Sidney Kimmel Comprehensive Cancer Center discovered several key mutations in pancreatic neuroendocrine tumors. A team led by Dr. Nickolas Papadopoulos uncovered the set of genetic alterations present among patients with non-functioning pancreatic neuroendocrine tumors. In addition, they concluded that pancreatic neuroendocrine tumors are distinct at the DNA-level from pancreatic cancer on the basis of having distinct genetic mutations.
The authors state, “This suggests that mutations in PANNETS (pancreatic neuroendocrine tumors) and PDAC (Pancreatic Cancer) arise through different mechanisms, perhaps because of exposure to different environmental carcinogens or through the action of different DNA-repair pathways.” (Jiao et al, 2011).
This project confirms what clinicians have known for a long time – that these cancers are distinct and that accurate diagnosis and identification are critical to optimizing patient care.
Please help spread the word about the differences between pancreatic neuroendocrine cancer and pancreatic cancer and the critical need for accurate distinctions to be drawn.
Brandi, M., Gagel, R., Angeli, A., Bilezikian, J., Beck-Peccoz, P., Bordi, C., Conte-Devolx, B., Falchetti, A., Gheri, R., Libroia, A., Lips, C., Lombardi, G., Mannelli, M., Pacicni, F., Ponder, B., Raue, F., Skogseid, B., Tamburrano, G., Thakker, R., Thompson, N., Tomassetti, P., Tonelli, F., Wells, S., and Marx, J. (2002). Guidelines for diagnosis and therapy of MEN type 1 and type 2. The Journal of Clinical Endocrinology and Metabolism, 86(12): 5658-5671. Retrieved from: http://www.ncbi.nlm.nih.gov/pubmed/11739416
Gibril, F. and Jensen, R. (2004). Zollinger-Ellison syndrome revisited: diagnosis, biologic markers, associated with inherited disorders, and acid hypersecretion. Current Gastroenterology Reports, 6(6), 454-463. Retrieved from: http://www.ncbi.nlm.nih.gov/pubmed/15527675.
Jiao, Y., Shi, C., Edil, B., de Wilde, R., Klimstra, D., Maitra, A., Schulick, R., Tang, L., Wolfgang, C., Choti, M., Velculescu, V., Diaz, L., Vogelstein, B., Kinzler, K., Hruban, R., and Papadopoulos, N. (2011). AXX/ATRX, MEN1, and mTOR Pathway Genes Are Frequently Altered in Pancreatic Neuroendocrine Tumors. Science, 331(6021), 1199-1203. Retrieved from: http://www.sciencemag.org/content/331/6021/1199.
Metz, D. and Jensen, R. (2008). Gastrointestinal neuroendocrine tumors, pancreatic endocrine tumors. Gastroenterology, 135(5), 1469-1492. Retrieved from: http://www.ncbi.nlm.nih.gov/pubmed/18703061.
Oberg, K., Reubi, J., Kwekkeboom, D., and Krenning, E. (2010). Reviews in Basic and Clinical Gastroenterology and Hepatology. Gastroenterology. 139(3):742-53, 753.e1. Retrieved from: http://www.ncbi.nlm.nih.gov/pubmed/20637207.
Ramage, J., Ahmed, A., Ardill, J., Bax, N., Breen, D.J, Caplin, M.E., Corrie, P., Davar, J., Davies, A.H., Lewington, V., Meyer, T., Newell-Price, J., Poston, G., Reed, N., Rockall, A., Steward, W., Thakker, R.V., Toubanakis, C., Valle, J., Verbeke, C., and Grossman, A.B., and UK and Ireland Neuroendocrine Tumor Society (2012) . Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut, 61(1):6-32. Retrieved from: http://www.ncbi.nlm.nih.gov/pubmed/22052063.
Tomasseti, P., Migliori, M., Lalli, S., Campana, D., Tomassetti, V., Corinaldesi, R. (2001). Epidemiology, clinical features and diagnosis of gastroenteropancreatic endocrine tumours. Annals of Oncology, 12(2), S95-S99. Retrieved from: http://www.ncbi.nlm.nih.gov/pubmed/11762360.