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Lauren and Nick.

Dave, Suzi, and Lauren at 2011 Focus on Neuroendocrine Tumors Conference in Philadelphia, PA

Dave, Suzi, Lauren - Philadelphia, PA

Pancreatic Neuroendocrine Cancer vs. Pancreatic Cancer

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 Caring for Carcinoid 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.  Please click here to visit the Caring for Carcinoid Foundation’s Doctor Database or call 617 948 2514 for help finding a physician.  For more information about current treatments for patients with pancreatic neuroendocrine tumors please visit CFCF’s website.

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 though 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.

 

References:

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. Science331(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.

I think you have taken a great initiative to make clear to the people, the difference between Pancreatic Cancer and Pancreatic Neuroendocrine Cancer. I have also read the news on Steve Jobs being diagnosed for Pancreatic Cancer. Thanks for sharing this stuff.
Thanks for writing this up Lauren. I was appalled by the lack of accuracy across the media. It was plain lazy and sloppy journalism and did no one any favors. I think it's incumbent on all of us to try to clear this up. This article really helped.
We join the many who are saddened by the loss and my thoughts and prayers go out to Steve’s loved ones. Your content is brilliant in my opinion. I think this is engaging and eye-opening material.
I am really like it very much for the interesting info in this blog that to this website is providing the wonderful info in this blog that to utilize the great technology in this blog. You have a very inspiring way of exploring and sharing your thoughts. It is very uncommon nowadays, lots of sites and blogs having copy pasted or rewritten info. But here, no doubt, info is original and very well structured. Keep it up.
Thank you for this post! I recently starting dating someone and his mother was diagnosed with Pancreatic Neuroendocrine Cancer about 13 months ago and this article has made me understand alot about what she is going through. Thank you again for making it known to the public that this is not an easy topic to digest.
I'm so excited to see your post and it's really good.It helps me a lot and thanks for your share.As the population ages throughout the region, it is important to know not only the average life expectancy, but also the predicted life expectancy in good health.Yes, i stand by you.I completely agree with the above comment, the internet is with a doubt growing into the most important medium of communication across the globe and its due to sites like this that ideas are spreading so quickly. dvdmallonline.com
Hey, thanks for writing this up. I've been looking at several blogs to find something like this. Do you by any chance have a contact email that you could share? I would like to contact you directly. Google promoter
Yes, I heard the sad news that Steve Jobs, co-founder and former CEO of Apple Inc., passed away. I got actually of what's your points and I learned from it. It is really a two different distinct form of cancer. Thanks for imparting us your knowledge. - buy youtube cheap views
Interesting topic, great blog. Nice job! Check this out Press Release Service.
I was diagnosed December 21, 2012 and had surgery January 16, 2013. This part of the journey started after at least 2 years of going for treatment of same pain. The last part of the journey to diagnosis started with waking up 2:00 AM with stabbing left side pain that lasted in excess of 24 hours, couldn't eat or drink not able to throw up or go the bathroom. Drove myself to the ER and they said what I was told the year prior, diverticulitis I said nope wrong answer because I had good colonoscopy year prior, so they did a CAT scan with showed a very inflamed pancreas. I was antibiotics to get rid of the infection. After many scans I finally went to Memorial Sloan Kettering. There they looked at all my scans and they did one of their own. I then had a endoscopy with EUS (a needle that goes through your stomach and take takes pictures of the tumor and biopsies the tumor). In total I am guessing it took about 2 years to be diagnosed with Pancreatic Neuroendocrine Cancer. My surgery involved removing the pancreatic tail where the 2 cm tumor was located (pathology stage 2 because of metastasis), removing my spleen which pathology showed benign, and removing 5 lymph nodes which 3 showed metastasis. My surgery was robotic with only five 2 to 3 inch incisions across my abdomen. I insisted on scans sooner than the 6 month recommendation, so 4 months post surgery I have had CAT w/contrast, MRI w/contrast, and coming up this week on an Octreoscan (nuclear contrast) to find where all the neuroendocrine tumors are located and how many. I do understand this will not show the size of each tumor just location. I am hoping and it is my understanding this will help the doctors prescribe and come up with a treatment plan to slow the growth of the tumors. They can currently see neuroendocrine tumors in my liver. The surgeon said eventually they will have to put in a catheter to increase blood flow to the liver. Wow I did pretty good with that explanation. It is still all too confusing to explain to others so I too refer to Steve Jobs which is an injustice for those of us who can survive for a much longer with the new treatments. I feel that when I say same as Steve people think I am dying. I tell them I am going to fool everyone and live to 90! Probably not realistic since my parents died young 69 and 67 with their respective diseases lung cancer for Dad and Parkinson's for Mom. I knew I was up for a fight in early 50's because that's when my Mom got sick. Menopause also confused getting diagnosed and caused havoc with my health. I am being treated in NYC at Memorial Sloan Kettering. Apologies this is long but I hope it helps someone get diagnosed even sooner than I did. And if you are wondering if I have a medical background, nope, but I have a cousin who happens to be an over 30 year board certified endocrinologist and she is supervising my case. Only wished I shared more with her sooner.
I was diagnosed with a neuroendocrine tumor which the doctors called a gastronoma. The tumor was on the head of my pancreas and I had a whipple procedure in November 2012. They did not say it was cancerous but that it could show up somewhere else for instance the liver. I originally went to the doctor and was told I had food poisoning but after finding blood in my stool they sent me to another doctor and he told me he thought I had a gastronoma tumor. He then sent me for a ostreoscan which the results were sent to an endocrinologist in Dallas. I went to him and he said that he didn't think I had anything but heart burn. He said but to be on the safe side lets do a blood test. He tested my blood for gastrin and chromogranin A levels. He immediately called me and told me that the chromogranin A level was 3270 and should not be over 95. He said you definitely have a tumor. I then went back to Dallas UT Southwestern for a endoscopy with ultrasound to see where the tumor was. The doctor told me immediately after the test that I had a tumor on my pancreas about 2 cm. I set up the surgery for the whipple procedure. They took the head of my pancreas, part of my stomach and small intestine. I was told that I would have to have the blood test every six months to see what my levels are. I am due to have another one in March but in the last few weeks I have had different colored stools and that has me worried that maybe it has moved to my liver. It seems like it is going to be something for the rest of my life.
Marie, My wife had a 4cm neuroendocrine tumor removed from her pancreas 4 years ago along with a 2cm in her liver and one lymph node. Octreocans & MRI just showed several small 1cm have recurred in her liver and one lymph. Weighing options now and would love to compare notes. 9729780697
I was told I had pancreatic cancer after being admitted to hospital with a kidney stone. It was a 4 centimeter mass on my pancreas and was told I had about 4 months to live. After surviving beyond this time my oncologist did blood tests and it was discovered that I actually had carcinoid tumour of the pancreas with secondaries in the liver. I was lucky to be put in a clinical trial where for some years was having a sandostatin injection every 4 weeks. For the last few years I have been having a somatuline auto gel injection four weekly. This happened in 2002 and I am doing really well. I have a scan every 12 months and the mass has grown very slowly so far without complications where it is now about 6 centimeters. The reason why I was unable to have surgery was because the mass is on the head of the pancreas and had grown over the arteries.
my dad was diagnosed with neuro endocrine carcinoma just this year. he already had his first chemotherapy last february. we are wondering, what are the causes and favtors for this kind of illness. it would be a great help for us if you could provide with the answer, since we have diffuclty dealing with his insufance policy. thanks in advance
Hi I read your blog. I was diagnosed in aug. Last year with neuro endrecan tumors without finding a primary site. I have gone thru 5 tace treatments with chemo beading. If you would like to talk I would love to hear from you. I live I san diego.... but, can email or call I hope everything is going well!
To read that Apple has over 100 Billion $ in cash and letting it sit is very disturbing. I have dealth with NETS since diagnosis in 1998. Major surgery by Dr. Jeff Norton(a genius)at UCSF saved my life. Was free of Nets for 11years. Running into a major stressful Situation(neighbor from Hell)1n 2009 Tumors reappeared in liver. Been taking sandstatin(octreotide) shots once a month since then. Tumors size has remained constant. My attitude about this whole thing is just stay ahead of it. It would seem that about 1 Billion dollars would really be nice to go to research of Nets. Wonder if apple and Steve Jobs widow are thinking along those lines?
Great site, I had a whipple @ ucsf in 7/2010, & in 1/2013 my NET had metastisized to the liver with 10-12 small tumors, biopsy confirmed NET, I am interested in finding out the side effects, ect on this octreotide, by your blog, it seems to be working,
Great article! Knowing Steve Jobs had Neuroendocrine Pancreatic Cancer frustrates me because he could have brought attention to this rare form of cancer. My dad has Neuroendocrine Carcinoid Cancer, but the research and treatment is limited. With Jobs fame and power it would have been really great for him to bring awareness and research for others that have this disease.
Hi, This blog is awesome as it is filled with various useful medical information. I love to visit your blog regularly and love to read it's posts. This post was very much informative and helpful for me as I am a medical student. Thanks for this important article.... Sustainable consumption
Thank you i understand it better now, 2weeks ago my 36 year old husband was told he has pancreatic neuroendocrine cancer, they told us he has a baseball size tumor in his stomach and seen spots on his liver and in his pancreas,i'am a little frustrated because know one is telling us nothing.We have 4 sons, 1 daughter and 2 grand children, i just wanna know if hes gonna be ok what is the survival rate?
Thank you so much for this well written, very informative article. I was diagnosed with this condition in 2008 and have dealt with the misinterpretation of the disease. This article was very clear in the explantation of the condition. Your posting is so very appreciated.
I have recently been diagnosed with neuroendocrine tumor in the pancreas. Many people don't understand it at all here -, I just tell people that I have the same as Steve Jobs - Thank you for a very informtive article Sue
I too was diagnosed in 2009.And so many people dont understand the difference. everyone keeps asking me is it pancreatic cancer? I have been telling everyone i have the same disease as steve jobs. thank you for posting this.
Thank you so much for such a well written article. I, too, was upset by the fact that they never referred to Steve Jobs' cancer as a NETS. So few people know about NETS...need to get the word out there.
I have read a lot about pancreas cancer since a close person to me died because of it but I have never hear of anything called NETS before... will Google it...
Did you find anything about NETS. I checked and I could not find anything..
Although mine is not a pancreatic neuroendocrine cancer, I too followed with geat dismay the story of Steve Jobs. It seemed like such a lost opportunity to "spread the word". Then of course, I was hoping a large charitable donation would land on the doorstep of this wonderful foundation. That also did not happen. But you have inspired me with your work of educating, research and support. Thank you.
Lauren -- Thank you for this post. The misinformation continues to fly across the internet, and I'm finding it increasingly frustrating.
Thanks for writing this up Lauren. I was appalled by the lack of accuracy across the media. It was plain lazy and sloppy journalism and did no one any favors. I think it's incumbent on all of us to try to clear this up. This article really helped.
Thank you Lauren.I am a widow.I lost my husband of almost 15 yrs back in Nov of 08.stage 4 low-grade Neuroendocrine carcinoma.

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