I have joined the inspire.com pNET group where patients can interact with each other online.
Hi there and I’m sorry to hear about your brother’s diagnosis. I took a look at your page, and it looks like you’ve gathered a lot of good resources. I found the series of videos on YouTube from NetRF and LACNETS to be really helpful.
#1 piece of advice: find a specialist. I see you have the link for carcinoid.org’s find a doctor. Folks on here will give you their thoughts on who they have found helpful. Some of us don’t live near a specialist, but we have our local oncologist consult with a distant specialist, meeting in person only as needed. Because this disease often needs attack using different approaches, having a specialist with access to a treatment team (surgery, interventional radiology, oncology, nutrition, etc.) is very important
Sorry about your brother, he is fortunate to have you as his advocate.
Do you know where this is on his pancreas? If it’s the tail, body or head, or multiple spots? Is he a surgical candidate? Do you know what tests or imaging he has had to diagnose his condition.
Starting with the somatostatin injections, Sandostatin or Lanreotide, is a good first step. Recommend he try to get a Gallium 68 or a CU 64 scan, which is a head to mid-thigh scan to see if there is activity in the liver or lymph nodes. That would help you determine if he needs systematic (full body) treatment or local treatment, like liver and/or just pancreas.
You might want to get on Ronny Allen’s site, he has a web page and on Facebook. He is a GI NET in England, but there are many overseas patients on these sites, so probably several from South Africa who could maybe recommend a specialist. Finally, is there any way he could travel to NY for treatment if needed? There are NET experts at Mt. Sinai and Sloan Kettering. If he could travel in Europe, there are experts in quite a few places such as Germany and Holland.
Finally, there is a lot of recent NET information on line, virtual conferences. NCAN just had one yesterday, and Univ of Penn had one last month.
- Great website that you are building with some very useful information. This website is very helpful as well and has given me so much insight. You are in the right place. Dr. Eric Liu is very good. Best of luck to you and your brother in this journey.
If it is wide spread, you really need to get him to a specialist. There are many different approaches to treatment. I go with the surgical club. my husband had a whipple in 2003. To me the best long term survival comes from a combo of surgery and drugs. Get on Ronny Allans site https://ronnyallan.net/
Also Dr liu https://www.rockymountaincancercenters.com/physicians/eric-liu/
He will do surgery when others will not. MSK is very conservative now. And i am not a fan of Mt Sinai although experience seems to vary by person.
I think there must be some experts in South AFrica
PRRT is a possible treatment for your brother. Good luck!
I have actually decided against surgery for now because there is no reliable data to show that this intense whipple surgery (which will have to include a portal vein replacement in my case ugh) that I am a candidate for will change the outcome of the disease once it has already spread.
Just seems like such an extensive surgery to go through if it is not curative at this point (in my opinion after gathering an extensive amount of information on my diagnosis). Currently on lanreotide and stable for now. My symptoms include pain (manageable on most days), some acid reflux and a few other issues here and there. At this point, the symptoms that I have are not significantly affecting my functioning (I know that this can change at anytime) but are definitely not a walk in the park and some days are better than others.
May have to consider CAPTEM in the future. I have seen multiple specialists and oncologists/surgeons for opinions. Their opinions have varied. Quality of life is important to me as a young patient. The surgery is just not something that I am striving for at this time. Best of health to everyone on here and keep fighting the good fight.
- In circumstances like that, many patients are put on CAPTEM to shrink what is there. Perhaps surgery will be available down the line!
Call Dr Eric Liu’s office. He sees patients from all over the world. He is the best.
+1 (303) 388-4876 Ask for Natalia, she is his scheduler. He can also work with his local oncologist I believe. My husband I love his entire office. Also check out “The Healing Net”. He began that to help patients
Much of the information in these brochures overlaps
- INCA https://incalliance.org/wp-content/uploads/2020/12/Neuroendocrine-Cancer-of-the-Pancreas_EN.pdf
- UK NPF https://www.neuroendocrinecancer.org.uk/wp-content/uploads/2020/03/NET-Patient-Foundation-Handbook.pdf
- Novartis https://www.novartis.com/sites/www.novartis.com/files/net-patient-guide.pdf
- NCCN https://www.nccn.org/patients/guidelines/content/PDF/neuroendocrine-patient.pdf
- HealingNet https://static1.squarespace.com/static/52853805e4b08b5554d6cece/t/5fe0f9e9cb12b74da7615bac/1608579562712/NET-WEBPatientNav.pdf
- HN Primer https://static1.squarespace.com/static/52853805e4b08b5554d6cece/t/5d54620f7227700001bd6182/1565811217238/HNET-Primer2019%28revisedweb2%29.pdf
- Guide for Nurses: https://www.carcinoid.org/wp-content/uploads/2015/10/NET-Nurse-Guide-_online-version_-24MAY12.pdf
- Canada https://cnets.ca/wp-content/uploads/2020/11/CNET-patient-booklet-20-07-17-E-Interactive.pdf
- Australia https://engonetuf.blob.core.windows.net/assets/uploads/files/UF_Nets_PatientBooklet.pdf
- RSA https://cansa.org.za/files/2019/02/Fact-Sheet-Pancreatic-Neuroendocrine-Tumours-NCR-2014-web-Feb-2019.pdf
- summary: https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq
This is the patient version. There is a link to the health professional version
- wikipedia: https://en.wikipedia.org/wiki/Pancreatic_neuroendocrine_tumor
- Podcast https://netrf.org/podcast/episode-10-pancreatic-nets/
- Book: https://www.interscienceinstitute.com/docs/Neuroendocrine-Tumors-4th-Edition.pdf (2009)
- Presentation slides: https://www.esmo.org/content/download/8584/174877/file/ESMO-Epidemiology-classification-and-clinical-presentation-of-NETs-A-European_Perspective.pdf
- Article on advances since Steve Jobs https://www.survivornet.com/articles/steve-jobs-died-nearly-10-years-ago-from-pancreatic-neuroendocrine-tumors-pnets-treatment-advances-since-have-been-significant/
Checklist for the Newly Diagnosed
- You will need a specialist in NETs to provide a second opinion and to assist your local oncologist in your treatment because, unless you are an exception, the local doctors will not know about current treatments or advances in our disease. The main quality you need from your local oncologist is a willingness to follow your lead.
- After you visit with the specialist for your second opinion, encourage your local oncologist to call him/her and discuss your condition and treatment. Some people have been known to get more than one second opinion. You will know when you have enough information to be the team leader in your treatment and make decisions that will work for you.
- Know that you can choose another doctor if he/she is not willing to listen to your concerns or information, or to provide you with the correct treatment, proper support or access to experts.
- Find and attend support group meetings or NET patient conferences. For a list of national support groups check with NETRF.org or Carcinoid.org
- Once you have been correctly and completely diagnosed, read all you can about your particular NET disease, whether carcinoid, pancreatic or other Nets. Be your own best, well-informed advocate. Keep to major NET sites for the most current information. In addition to our site you may want to visit sites hosted by NETRF.org, Carcinoid.org, TheHealingNet.org, and NET Cancer Awareness Network.
Even if you stick to the major NET websites, which we highly recommend, it is important to note that a lot of the statistics will be out-of-date, so don’t be frightened by the statistics you see. Most of the statistics you see are based on old data as clinical trials take several years to conclude and evaluate. Look at the dates of the reference material and newly published papers. New drugs, treatments and procedures have been developed over the years and are being developed now, which have very positive bearing on our longevity.
- Immediately start a notebook/medical record summary to bring with you when visiting your doctors. Let your doctor know that you are going to be very proactive in your treatment. and that your treatment will be a team effort right from the start. Include a list of tests and procedures, and obtain copies of all tests and reports and keep them available for second opinion consultations. This includes all scans reports and images. Many of these records are contained on your electronic medical record that your provider maintains. For Pet/CT, MRI and Octreoscans you can request a disk be provided to you with your images at the time of your scan.
- If you have carcinoid or carcinoid syndrome, it is advisable to wear a medical alert bracelet or necklace tag indicating that you should not receive epinephrine, which could precipitate a carcinoid crisis. Octreotide (Sandostatin) will control this crisis. *** One exception is the administration of epinephrine in the case of an allergic anaphylactic reaction (i.e. a bee sting), so it cannot be avoided in this case, just make sure that octreotide (Sandostatin) is also available.
- Don’t sit around and isolate yourself. For most of us, there is power in not being alone, in knowing others who have been on this road for many years and in sharing our stories and expertise.
- Ok. Now. Stop. Live your life. Know that you know where to get the information you need when you need it. Get busy living.
- Net vitals form for specialist https://static1.squarespace.com/static/596bb220cd39c3a76bf18f05/t/5bd08a0a9140b7dc42c4dc7a/1540393484032/NETVITALS-final-print-outlined+-10.23.18.pdf
7 TIPS FOR THE NEWLY-DIAGNOSED PATIENT
(https://www.lacnets.org/newlydiagnosed for more on each tip.)
- GET ORGANIZED
- EDUCATE YOURSELF ON THE DISEASE: LEARN TO SPEAK “NET.”
- FIND SUPPORT
- FIND A NET SPECIALIST
- DECIDE WHO WILL BE YOUR QUARTERBACK
- ATTEND A NET PATIENT EDUCATION CONFERENCE
DIAGNOSED WITH NEUROENDOCRINE CANCER? – 10 QUESTIONS TO ASK YOUR DOCTOR (AND WHERE TO FIND A NET SPECIALIST WORLDWIDE)
- Where is my primary tumour and what type of NEN is it?
This is a fundamental question and it’s likely many will already have some inkling about location and perhaps a type. The difference between NENs and other types of cancer is the primary can be found wherever there are Neuroendocrine cells rather than a specific part of the anatomy in terms of naming the type of cancer, i.e. a NEN of the pancreas is not Pancreatic Cancer.
The type of NEN is key as it may drive a lot of other stuff including treatment. Location and type of NEN are not always aligned and there’s also the factor of whether a tumour is functional or non-functional (see Q4 below).
For some the primary will not yet be found (i.e. cancer of unknown primary or CUP). There may also be multiple primaries. Specialists in Neuroendocrine Cancer are best placed to find unknown primaries – they know stuff.
- What is the grade and differentiation of my tumour(s)?
Another fundamental question as this defines the aggressiveness of the disease and is absolutely key in determining overall treatment plans. Treatment plans for poorly differentiated can be very different from well differentiated. The differentiation if Grade 3 (or High Grade is a very important question. Read more here – Grading and here – Benign or Malignant
- What is the stage of my disease?
Fundamental to understanding the nature of your disease. Stage confirms the extent of your disease, i.e. how far has it spread. Again this will drive treatment plans and long-term outlooks. Scans are really important in determining the Stage of your cancer – check out my scans post here. Read more here on Staging
- Do I have an associated Hormonal Syndrome?
Many NET patients will have been experiencing symptoms prior to diagnosis, perhaps for some time. It’s possible these symptoms form part of what is known as a ‘Syndrome’ and there are several associated with NETs. Syndromes are mostly caused by the effects of over-secretion of hormones from the tumours, a hallmark of Neuroendocrine disease. Carcinoid Syndrome is the most common but there are many more depending on the primary location. NECs are not normally hormonal in behaviour but read more here – NET Syndromes.
- What is my treatment plan, and what are the factors that will influence my eventual treatment? When will I start treatment
This is a very complex area and will depend on many factors. Thus why your specialist may not have the answers to hand. Decisions on treatment are normally made by some form of Multi-disciplinary Team (MDT). Many people diagnosed with cancer expect to be whisked away to an operating theatre or chemotherapy treatment. However, for many this is not what actually happens. Depending on what testing has been done up to the actual diagnosis, it’s possible that even more testing needs to be done. Additionally, for those with an accompanying syndrome, this will most likely need to be brought until control before certain treatments can be administered; and even then, there may be checks to make sure the treatment will be suitable. Sometimes it’s a case of ‘Hurry up and wait’. My first treatment was 6 weeks after diagnosis and that was designed to control my syndrome ready for surgery which was undertaken 14 weeks after diagnosis. It’s also possible you will be placed on a ‘watch and wait’ regime, at least to begin with. Surgical decisions can be based on many factors – read more here.
- Can you comment on the potential for my type of NEN to be related to any familial or genetic aspects of cancer?
A small percentage of NENs are hereditary/genetic in nature. This is mostly associated with those who have Multiple Endocrine Neoplasms (MEN) syndromes and a few other less common types of NET including Pheochomocytoma / Paraganglioma(Pheo/Para) and Medullary Thyroid Carcinoma (MTC) (the familial version of MTC is often referred to as FMTC). However, please note this does not mean that all those diagnosed with pancreatic, parathyroid, pituitary, Pheo/Para and MTC tumours, will have any hereditary or genetic conditions, many will simply be sporadic tumors.
- Will you be able to get rid of all my disease and what are the chances of recurrence or growth?
This is a really difficult question for any specialist, even a Neuroendocrine expert. All published articles on NENs will say they are a heterogeneous collection of diseases (i.e. consisting of dissimilar entities) which makes this question (and others) difficult. I have read articles written by the world’s foremost NET experts and they all have the word ‘curative’ mentioned in various places, normally associated with surgery. So I guess in particular scenarios with certain NETs, and if the disease is caught early enough, that possibility exists. However, for many, the disease could be incurable, particularly where there are distant metastases. But, the disease has many treatment options for most types and for many it’s possible to live as if it were a chronic condition. I call it ‘incurable but treatable’. Read more here – Incurable vs Terminal
- What Surveillance will I be placed under?
Again, this is very individual in NENs and is mainly dependent on type of NEN, grade and stage and how the patients reacts to treatment. This may not be known until you have undergone your initial treatment. For example, surveillance scans can be any period from 3 months to 3 years depending on tumour type(location) and stage/grade. Marker testing tends to average around 6 monthly but could be more or less frequently depending on what’s going on. Read more here – click here
- Will I receive support and specialist advice after my treatment?
Let’s not be afraid of the word ‘Palliative’, it does not always mean ‘end of life’ care. Another example is nutrition. Many people with NENs, the condition in combination with the side effects of treatment may necessitate an alteration of diet and this is a very individual area. I would also emphasise that dietitians not well versed in NENs might not offer the optimum advice. Read more – My Nutrition Series.
- How will treatment affect my daily life?
This is a question that many people miss but it’s becoming more important as we all live longer with cancer Again, this may not be possible to answer immediately but perhaps this question could be reserved once you know which treatment(s) you will be receiving. All treatment comes with side effects and can last for some time or even present with late effects after some years. The ‘consequences’ of cancer treatment need to be factored in earlier so that the necessary knowledge and support can be put in place. See also Unmet Needs for NET Patients
Your doctor might tell you the number stage of the NET. This system divides pancreatic NETs into 4 main groups, depending on the size of the cancer and whether it has spread.
- Stage 1: This is the earliest stage. It means the tumour is less than 2 cm and contained within the pancreas.
- Stage 2 is divided into 2A and 2B.
- Stage 2A means the tumour is contained within the pancreas and is between 2 cm and 4 cm. In the TNM staging, this is the same as T2, N0, M0.
- Stage 2B means the tumour is bigger than 4 cm. It is contained within the pancreas or has grown into the small bowel (duodenum) or bile duct.
- Stage 3 is divided into 3A and 3B.
- Stage 3A means the tumour has grown outside the pancreas into the blood vessels, stomach or bowel. But it hasn’t grown into the lymph nodes.
- Stage 3B means the tumour has grown outside the pancreas and into the lymph nodes. It hasn’t spread to other areas of the body.
- Stage 4 means the tumour has spread to other parts of the body such as the liver.
The Ki-67 or mitotic index are ways of describing how many cells are dividing. A specialist doctor (pathologist) counts the number of NET cells that have started to divide into 2 new cells (mitoses) under a microscope. And a special stain measures the Ki-67 value.
Your doctor might tell you the number of cells that are dividing (number of mitoses), or you may see this on your pathology report. This helps your doctor decide which treatment you need.
- Ki-67 index of 2% or lower: A Ki-67 index of 2% or lower means that fewer than 2 in every 100 cells (2%) are dividing. This is a grade 1 NET (NET G1).
- Ki-67 index between 3% and 20%: This means that between 3 and 20 cells in every 100 cells (3% and 20%) are dividing. This is a grade 2 NET (NET G2).
- Ki-67 index higher than 20%: A Ki-67 index of more than 20% means that more than 2 in every 10 cells (20%) are dividing. This is a grade 3 neuroendocrine carcinoma (NEC G3). They often grow and spread quickly.
What are the most recent advancements in NET research?
Somatostatin receptor imaging, which uses single photon-emitting or positron-emitting radiopharmaceuticals to detect the somatostatin receptors on the surface of neuroendocrine cells, has been used for nearly 20 years for both diagnosis and staging of NETs. However, the last four years has seen the approval of newer agents with increased sensitivity,4 including 68Ga-DOTATATE. These give more accurate information and help us to plan therapies more confidently for our patients.
The past ten years have also given us more treatment options including targeted therapies, such as everolimus and sunitinib, and peptide receptor radionuclide therapy4 such as 177Lu-DOTATATE. These agents can be used in patients with metastatic gastrointestinal or lung NETs, and many have shown significant improvements in progression-free survival.5
Equally, we’ve seen treatment advances in disease management. New drugs such as telotristat can treat carcinoid syndrome, which commonly affects NET patients. And the ‘centre of excellence’ model is improving the way NETs are managed at the country level. In summary, diagnostic and therapeutic advances are increasing overall survival for the majority of NET patients.
Daily, every other week or monthly injections of somatostatin analogs are available to control some of the unpleasant symptoms caused by carcinoid tumors.
Somatostatin analogs (e.g. lanreotide, octreotide) are synthetic versions of somatostatin, a naturally occurring hormone produced in the brain and digestive tract that inhibits the release of several other hormones and chemicals from our internal organs.
The octreotide formulation can be given by infusion, self injections daily, or in a monthly injection which is a slow release formula. The lanreotide formula can be given every other week and in a monthly slow release formula. The monthly injections are mostly administered by healthcare professionals, but it is possible to self inject the lanreotide monthly injection.
Injection of these analogs can stop the overproduction of hormones that cause symptoms such as flushing, wheezing and diarrhea. Under clinical supervision, these treatments may also be used in NET patients with no clinical syndrome. Long-acting octreotide can also control tumor growth.
Everolimus (which is marketed as Afinitor) is used to treat well-differentiated and moderately-differentiated pancreatic NETs that can’t be removed by surgery, have spread and are growing. It’s also used to treat non-functional gastrointestinal and lung NETs. It works by blocking a particular protein, called mTOR, that causes cancer cells to grow. Everolimus may also stop cancer cells from developing blood vessels. Without a blood supply, cells are starved of oxygen and nutrients and so can’t grow. In the UK, Everolimus is licensed for use in advanced non-functioning neuroendocrine tumours that originate from the stomach, bowels, lung or pancreas. That is NETs that are progressing and are inoperable and do not overproduce specific hormones or other related natural substances.
Everolimus comes as a capsule. You take the capsules once a day, at the same time each day, with a glass of water. It can be taken with or without food. Avoid grapefruit or grapefruit juice during treatment as this can interfere with the way the drug is used by the body. You’ll have regular blood tests before you start taking Everolimus and while you’re taking it. Treatment with Everolimus continues for as long as it appears to be controlling your NET, and is well tolerated (that is no or minimal side effects). Everolimus may not be given if you have certain health conditions – such as diabetes or respiratory disease i.e. asthma – or are on certain drugs.
Please ensure that your care team is aware of all of your medical history and medications.
Source: NET Patient Foundation Handbook
However, a July 2004 CT scan revealed that his tumor had grown and likely spread. Jobs finally took medical leave and underwent surgery at Stanford University Medical Center, as physicians performed a modified "Whipple procedure" that removed part of his pancreas.
After his tumor was removed, Jobs became one of the first twenty people in the world to have his cancer tumor genetically sequenced, which at the time cost more than $100,000. The sequencing and analysis, performed by teams at Stanford, Johns Hopkins, and the Broad Institute of MIT and Harvard, ultimately would allow Jobs to receive molecular targeted therapy—essentially, enabling physicians to craft specific drug regimens that directly targeted defective cells—that proved more effective than traditional chemotherapy in fighting off his cancer's effects.
While the targeted therapies had proved promising—one physician even told Jobs that there were signs his cancer, and others like it, would eventually be treated as a manageable chronic disease—they had ceased to be effective.
- INCA https://incalliance.org/
- Netrf https://netrf.org/for-patients/newly-diagnosed/assistance-support/
- NaNEts https://nanets.net/resources/patient-resources
- Enets(europe) https://www.enets.org/courses.6.html#/introduction
- NCAN https://www.netcancerawareness.org/
- Healing Net: https://www.thehealingnet.org/
- Cancer.net https://www.cancer.net/cancer-types/neuroendocrine-tumor/view-all
- Cnets(canada) https://cnets.ca/
- UK https://www.neuroendocrinecancer.org.uk/
- Australia https://neuroendocrine.org.au/
- More: https://www.lacnets.org/resources
- netrf https://netrf.org/events/patient-and-caregiver-conferences/
- upenn https://www.med.upenn.edu/nets2021/
- NETPC https://www.carcinoid.org/event/2021-national-neuroendocrine-tumor-patient-conference-atlanta/
- ajmc https://www.ajmc.com/conference/nanets
- USA: https://netrf.org/for-patients/neuroendocrine-tumor-doctor-database/page/3/?multidisciplinary-net-team-sites=multidisciplinary-net-team-sites&specialty=all&clin_state=all&radius=5&zip&practice_type=all#038;specialty=all&clin_state=all&radius=5&zip&practice_type=all
- Europe: https://www.enets.org/coe_map.html
- carcinoid.org https://www.carcinoid.org/for-patients/treatment/find-a-doctor/
- David Paul Kelsen, MD, Sloan Kettering
- David Metz, MD, UPenn (WITS alumnus)
- Dermott O'Toole, UK
- Diane Reidy-Lagunes, MD, Sloan Kettering, Potentially relevant trial
- Edward M. Wolin, MD, an internationally-renowned authority on neuroendocrine tumors, is Director of the Center for Carcinoid and Neuroendocrine Tumors and Professor of Medicine, Hematology and Medical Oncology (Mount Sinai). https://youtu.be/BB3xSQQTHqg
- Electron Kebebew, MD, FACS, Stanford
- Dr. Emily Bergsland is a specialist in gastrointestinal oncology at the UCSF Helen Diller Family Comprehensive Cancer Center. Her expertise includes treating colorectal and other gastrointestinal cancers, and neuroendocrine tumors.
- Dr Eric Liu of Denver https://youtu.be/dKGhyrPOrm0
- Jennifer Chan MD, MPH, is clinical director of the Neuroendocrine and Carcinoid Tumor Program at Dana-Farber and an assistant professor of Medicine at Harvard Medical School.
- Jin He, M.D., Ph.D., is an associate professor of surgery at the Johns Hopkins University School of Medicine. He is a surgical oncologist specializing in tumors from the Hepato-Pancreato-Biliary organs.
- Leonard Saltz, Sloan Kettering
- Dr. Matthew H. Kulke, a physician at Harvard-affiliated Dana-Farber Cancer Institute in Boston who specializes in pancreatic neuroendocrine tumors
- Nitya Raj, MD, Sloan Kettering
- NYP https://doctors.nyp.org/?name_or=Neuroendocrine+Tumor
- Thomas E. Clancy, MD, FACS, is a surgical oncologist specializing in pancreaticobiliary and liver surgery at Brigham and Women's Hospital and Dana-Farber Cancer Institute, and is co-director of DF/BWCC's Pancreas and Biliary Tumor Center. He obtained his medical degree from Harvard Medical School, followed by general surgical residency and surgical oncology fellowships at Brigham and Women's Hospital. Clancy's clinical focus is the treatment of gastrointestinal malignancies, specifically pancreatic, biliary, and liver tumors. In addition, Clancy has a specific clinical and research interest in neuroendocrine tumors.
Online 2nd Opinions
- Europe: https://www.indigomedconnect.net/neuroendocrine-tumors/
- dana-farber https://www.dana-farber.org/appointments-and-second-opinions/online-second-opinion-program/
- RSA https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5905263/
- These are just the trials that specifically mention Grade 2:
- Broader list of NET trials:
- Please note that I am not a health professional. I have put this together as part of the research for a family member.
- Email me