Living with Cancer – Worrier or Warrior?


WORRIER OR WARRIOR (2)

If you only believe what you see on social media, you would probably classify cancer patients into two main groups, warriors or worriers.  I guess people have, or adopt, these traits from their cancer experience but I suspect many people are simply ‘wired’ that way.  I also believe there are many people who have a bit of both, perhaps slanting to mostly warrior or worrier, I mean who doesn’t worry about a single thing?  However, the extent of worrying can often have a negative effect on quality of life. You’re not going to stop worrying by simply reading this article but if you read no further, at least check out the lead graphic, it might help putting things into perspective.

Warriors

I used to do that for a living so I guess I’m an automatic fit, right? Believe it or not, professional ‘warriors’ also worry about stuff but they don’t like showing it (one of the reasons there are serious PTSD issues happening currently).

Despite the protests at the use of ‘war’ metaphors, many cancer patients do see themselves as ‘warriors’.  People probably label me as one but …… spoiler alert, behind my positive exuding exterior, there’s a man worried about a problem which might decrease quality of life and also a man worried about potential surgery most likely needed to correct the issue. We all worry but it’s the way we handle stress that is important.  Not talking about it is never a good option.

poker face
Poker Face or Cancer Card

I also think ‘Warriors’ can sometimes be complacent and for a cancer patient, this can be detrimental to their health.  In a previous life, nothing was going to stop me getting on with life, nothing was going to stop me going to work, nothing was going to stop me completing my mission.  One day, some of my co-workers complained about my constant sneezing, coughing and sniffing. I told them the only way I was leaving the building would be on a stretcher having lost consciousness or in a body bag.   I said it whilst I continued typing up a report which was my mission for that day.  I said it without any hint of joviality in order they knew that I was deadly serious.  Little did I know that my ‘poker face’ was just about to become seriously deadly.

We’ve all read the blogs and memes on Facebook…. ‘kicking cancer’s ass‘, etc etc. There’s even twitter handles, Facebook sites, blog names all with the word ‘warrior’ in the title. Nothing wrong with that, nothing wrong with having a positive attitude. The only thing potentially wrong with that, is becoming too complacent about the danger of cancer. Cancer will kill you whether you are a ‘warrior’ or a ‘worrier’.

Worriers

I come into contact with a lot of Neuroendocrine Cancer patients, either from a thread in my group, my blog site or my Facebook site(s).  I get a substantial amount of private messages from patients including on twitter. Many of them are from people who are ‘worried’ – these can often be the toughest conversations I have.

People have many different reasons for worrying ,and there can be totally different factors involved at diagnosis than those surfacing during and after treatment. We worry about a diagnosis and what it actually means, we worry about the treatment we are given, and then we worry about the cancer coming back, this is all very natural. Some people have taken a considerable amount of time to get a diagnosis and their stress levels are already high.  Some are young with a long life ahead of them, that is really tough. Some have young children and are worried about their future as well as their own, that is also really tough. There are many other scenarios adding further layers of complexity to an individual’s situation.

I have no magic answer for someone who is constantly worried except to say that they must do something about it as it’s not a good place to be.  I once dabbled in this area by writing an article with 8 tips for conquering fear, this was specifically aimed at my own cancer type but I guess it could apply to many others.

Many cancers are not as ‘black and white’ as a lot of people think, i.e. you are diagnosed, it is treated, you are observed, you are cured.  Neuroendocrine Cancer is quite definitely a ‘grey’ cancer, i.e. many people take some time to be diagnosed, many are diagnosed at the incurable stage, the treatment regimes differ depending on the primary, stage, grade and many other factors, you are observed for much longer periods than the average cancer patient, perhaps for life, and for many, there is no cure.

For those with incurable conditions, the stress levels can be higher and for longer, potentially leading to other illnesses. The psychological impact of long-term cancer is a developing but as yet unmet need in the Neuroendocrine world and I suspect elsewhere.

Don’t forget the carers and caregivers.  I think a lot of carers/caregivers can also be under some stress and worry, I certainly see this a lot in the Neuroendocrine community.

Summary

I think there’s a fine line between being a ‘warrior’ or a ‘worrier’ and I believe many people flit between the two quite easily.  I think many people might actually be in the middle ground, having accepted their diagnosis, decided it was just another chronic illness and try to live life as best they can.

Cancer can be a very individual disease, not just the different types and sub-types, but also stages, grades, pre-existing medical issues, age, gender. To add to the mix, I think it’s also true to say that how people handle the stress, how people cope is another dimension to be considered.

For those who are predominately ‘warriors’, please don’t forget to look after yourself.  For those who are predominantly ‘worriers’, the lead graphic might help put things into perspective.

Take care all

 

Thanks for reading

Ronny

I’m also active on Facebook.  Like my page for even more news.  I’m also building up this site here: Ronny Allan

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Round up of NANETS 2017 – Let’s talk about NETs #NANETS2017

NANETS (North American Neuroendocrine Tumor Society) is one of the biggest NET conferences, bringing together NET Specialists from around the world to discuss state-of-the-art treatment modalities, new therapies, and ongoing controversies in the field of Neuroendocrine Neoplasms (Tumors and Carcinomas). This is fairly complex stuff but much of it will be familiar to many. I’ve filtered out several outputs from the conference which I think are both relevant and topical to patients. The list is below allowing you to easily peruse and read further via linkages if you need to read more.  Remember, some of these are extracts so do not contain all the details of the research or study – although some of the linkages will take you to in-depth information if that’s your bag. Where applicable, I’ve also linked to some of my blog posts to add context and detail in patient speak. The list comprises articles which were published in medical news media and for which I received alerts.  It does not comprise the entire schedule of NANETS 2017. I may add more to the list if other relevant and interesting articles are published downstream.

Please note:
Some of the output from the conference is in ‘study form’ and has not yet been published as peer-reviewed data (important notice to readers).

NANETS to Bring All Specialties in the NETs Community Together for 10th Annual Symposium

Interview with Michael Soulen MD.  Nice introduction.

https://goo.gl/tMT6KS
Location of Neuroendocrine Tumors in the Small Bowel Does Not Affect Survival

 

https://goo.gl/zf9k9j
Diagnosing and Treating NET-Related Diarrhea

 

Incorporated into my Diarrhea article – https://goo.gl/PwsXmX
Emerging Therapies, Biologic Discoveries, and Improved QoL on Horizon for NETs

 

https://goo.gl/p4cCyd
Retrospective Database Analysis Studies Somatostatin Analog Usage in NETs

 

https://goo.gl/KWM4p7
Regional Lymph Node Involvement and Outcomes in Appendiceal Neuroendocrine Tumors: A SEER Database Analysis. https://goo.gl/vfF4DA
Personalizing Therapy With PRRT and Improving Imaging With SSTR-PET Brings Novel Options to NETs Landscape

(new term SSTR-PET generically meaning any PET scan using somatostatin receptors), e.g. Ga68 etc.

https://goo.gl/s8sked
PFS and OS After Salvage Peptide Receptor Radionuclide Therapy (PRRT) with 177-Lu[Dota⁰,Tyr³] octreotate in Patients with GastroEnteroPancreatic or Bronchial NeuroEndocrine Tumours (GEP-NETs) – The Rotterdam Cohort https://goo.gl/yZ56YZ
Molecular Classification of Neuroendocrine Tumors: Clinical Experience with the 92-gene Assay in >24,000 Cases https://goo.gl/aqgfRf
Neuroendocrine Tumors: A Patient Survey

“Regarding their biggest challenges, patients reported fatigue as their biggest challenge followed by diarrhea, sleep disturbances, and pain.”

https://goo.gl/qEeNRM
Phase III Trial Needed to Confirm Clinical Benefit of Cabozantinib in NETs

 

Incorporated into my Cabozantinib article – https://goo.gl/mR2yFT
QOL Improvements in NETTER-1 Phase III Trial in Patients with Progressive Midgut Neuroendocrine Tumors. (I think this is well-known but no harm in repeating it!) https://goo.gl/UmKsFi

 

The full link to all poster abstracts for NANETS 2017 can be found here

Thanks for reading

Ronny

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!

Things not to say to someone with cancer

things-not-to-say
Graphics courtesy of https://emilymcdowell.com/

This topic comes up regularly on patient forums, twitter, Facebook….. in fact everywhere!  Personally, I don’t tend to get too excited about it, although there can be extremes.  Most people (not all) are just stumped to know exactly what to say.  Even as a person with cancer, I sometimes feel awkward when faced with someone I just found out has a serious illness. It’s really difficult to know what to say, knowing how they might react and it’s particularly difficult if you don’t really know the person, for example on social media, you could be talking to someone who you have never met, you don’t know anything about them; and they may not even speak English as a first language (those who have tried google/twitter or Facebook translator, will know it’s not perfect!).

I’ve dabbled in this arena before with my blogs “I look well but you should see my insides” and “You must be doing OK, you’ve not had chemotherapy”.

This video was produced by BBC and shared by my friends in Macmillan Cancer Supportand it’s getting a huge amount of comments and opinions.  Someone suggested it should have been entitled “Things to say to someone with cancer” and there’s something in that I guess – although I suspect it’s just as difficult!

Personally, I think there’s no right or wrong answer. However I know a lot of you guys will enjoy the video

 

 

Thanks for reading

You may also enjoy these similarly related articles:

Shame on you! – click here

I look well but you should see my insides – click here

Things are not always how they seem – click here

Not every illness is visible – click here

Not the stereotypical picture of sick – click here

An Ode to Invisible Illness – click here

Poker Face or Cancer Card – click here

 

Thanks for reading

Ronny

I’m also active on Facebook. Like my page for even more news. I’m also building up this site here: Ronny Allan

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!

patients included

Please Share this post

The 5 E’s (of Carcinoid Syndrome)

Guidance and Risk Management
Guidance and Risk Management

Since my diagnosis, I seem to have been in a perpetual learning phase!  What not to do, what not to eat, what not to read!  However, a couple of years ago, I came across a list of ‘E’ words (5 of them) which is a handy reminder for Carcinoid Syndrome patients, particularly those whose symptoms are not under control.  When I say “carcinoid syndrome” in this article, I only mean the syndrome that is caused by what was once called “Carcinoid Tumors”, i.e. mainly serotonin secreting types but include tumours which are well differentiated found in the small intestine, appendiceal, rectal, lung, and one or two other less common places.

There are many variations of this list but this is my take!  I suspect some of this also applies to other types of NETs and other NET Syndromes.

On analysis of this list, it struck me that I was aware of the issues and their potential effects and I’m certain there is science to substantiate the content. These E’s are apparently the most common ‘triggers’ for Carcinoid Syndrome.  Clearly, they are not going to have the same effect on every patient e.g. I have the occasional drink of ‘Ethanol’ and I always enjoy it, I go for long exhausting walks and I always feel great after.  I had dental treatment without any precautions before I was aware of the risks …….. nothing happened!  Before I was treated, stressful meetings at work would make me flush though!  As for eating – well that’s another couple of blog’s worth!   (see the Diarrhea Jigsaw and Nutrition Blog 4 – Food for Thought)

The 5 Es are, however, very important, as a severe attack of Carcinoid Syndrome symptoms could be debilitating and life-threatening and I’m fairly certain the list was compiled with this in mind.  Some people are more affected by Carcinoid Syndrome and this is not necessarily related to the extent or aggressiveness of their disease.  Some people just react differently.  An extremely severe attack of Carcinoid Syndrome can also be known as a ‘Carcinoid Crisis’ which is very dangerous on the operating table due to the effects of anaesthetics  – thus why many NET patients may be infused with somatostatin analogues (usually Octreotide) prior to and during surgery or other medical procedures.  There’s a lot of excitement generated around the term ‘Carcinoid Crisis’ but it is generally uncommon.

I’m not saying the 5Es should be ignored but NET Cancer is complex and most things need to be read in the correct context. What works for some may not work for others. There can also be confusion surrounding the source of symptoms, i.e. are they syndrome or something else?  This is why I believe NET patients need to answer some key questions when considering the risks associated with the 5 E’s:

  • Are you currently syndromic?   If you are, then the 5 ‘E’ list is probably very good advice but interpreting the advice in the correct context remains important.
  • Are your syndrome related biochemistry results normal (e.g. 5HIAA)? Normal readings (in range) tend to mean the syndrome is under control and many people who were diagnosed with a syndrome may actually be non-syndromic following treatment.
  • Have you had treatment or are having treatment likely to produce side effects which might be confused with Carcinoid syndrome? For example, surgery can be the long term cause of diarrhea and other issues. Despite the role of somatostatin analogues, these could also be the root cause of certain reactions.
  • Do you have any other illnesses?  If yes, do these other illnesses produce effects similar to carcinoid syndrome? e.g. asthma, diabetes, rosacea, thyroid disorders, vitamin & mineral deficiencies, malabsorption, gut bacterial imbalance.  Sorting out the symptoms can be a jigsaw with a missing piece sometimes.

The vagaries of this disease will no doubt throw up some exceptions and additions. There will be patients who have no syndrome but have elevated biochemistry and vice versa!  Additionally, there will be patients who have had surgery and/or are being treated with somatostatin analogues but will still be syndromic in varying degrees of severity.

The so-called ‘5 Es’ are as follows:

Epinephrine: This was a new piece of information for me and I only discovered this as a potential problem when I started monitoring some of the USA Facebook forums.  This does not appear to be that well-known in UK. Epinephrine (commonly known as adrenaline) is often used in dentistry mixed with a local anaesthetic. I won’t risk this, so I’ve instructed my Dentist to place a note on my record asking for epinephrine not be used (and clearly I’ll remind them each visit!). According to NET guru Dr Woltering, plain novocaine, carbocaine or plain marcaine are preferred.  You should also check that your anaesthetist for any procedure you may be undergoing is aware of your carcinoid syndrome. However, the danger is not just with dentistry work.  Any anaesthesia is risky.  Check out my post ‘carcinoid crisis’.

For those who have standby ‘Epi Pens’, I did read the following statement on the Carcinoid Cancer Foundation website:  “ …….. 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“.  This advice is also extremely relevant to Pheochromocytoma and Paraganglioma patients who may be a high risk of intraoperative hypertensive crisis.

Eating: This is very individual.  Certain foods or large meals can be difficult, particularly if you have had any gastrointestinal surgeries. I keep a personal diary trying to identify things that upset my system. I try to find some balance between what I know is good for me and also what I know I enjoy. For example, I found that very large meals do not agree with my ‘new plumbing’. If I eat a lot of sweets, I’ll also suffer …..so I just eat a little – check out my  blog post Chocolate – The NET Effect.

Personally speaking, I’m fairly certain the vast majority of my issues are related to my treatment (past and present) rather than being provoked by Carcinoid Syndrome, i.e. if I rush to the toilet after a meal, it’s not syndrome, it’s a reaction of my compromised digestive system. So with this in mind, I try to reduce those things but additionally strike a balance between quality of life and excessive and rigid adherence to some of the guidance out there (see below) – as I said above, interpretation and context is important. My compromised system cannot deal with big meals so I ‘graze’ most of the day and then eat a small to medium-sized meal in the evening. I’ve been doing this for 3 years and reduced my visits by 300% without any special or expensive medication.

In my blog Nutrition Blog 4 – Food for Thought, I’ve linked to authoritative sources on potential diet triggers.  I’m not suggesting you cut out all of the foods on these lists (you won’t last long!). Some can indulge in those foods and some cannot. For example, chocolate and caffeine (tea/coffee) are on the lists but I eat/drink those frequently (in moderation) and have no problem. It’s a case of testing things out.  I like to describe my eating as ‘The Risk Management of my Quality of Life’. By the way, no-one is suggesting that a NET patient with carcinoid syndrome (and don’t forget this is only one syndrome of many with NETs) should stop eating foods high in the offending amines or are precursors to serotonin (e.g. tryptophan).  They do not make tumours grow (a myth) but just make sure you adhere to the dietary restrictions for any 5HIAA test.

Emotions:  Stressful situations can cause symptoms to flare up. While it is difficult to avoid all stress (work, home, commuting, etc), it is helpful if you can manage or reduce it. Like eating, this is a very individual area. From personal experience, I know stress can exacerbate carcinoid syndrome. Before I started my treatment, I was regularly flushing in meetings at work (….. think boxing matches!). After my treatment, stress was definitely a factor causing increased bowel motility.  I’ve removed a lot of stress from my life and it helps. You may need to be ruthless in managing this aspect of your illness.

Exercise:  Exercise is extremely important for overall health and well-being and I know quite a lot of NET Cancer patients who exercise regularly without issues. It can, however, trigger carcinoid syndrome if you overdo it – it is, however, like eating, a very individual thing. I take the view that ‘zero’ exercise might potentially be an even higher risk. Even a walk around the garden or gardening is exercise. When I was at work, I would walk to see people rather than phone them. Sometimes I walk to town rather than drive, it all adds up! I have evidence from my own exercising regime proving in my case that exercise can reduce the knock-on effects of some of the other E’s (emotions and eating) and/or the side effects of treatment – check out my blog entitled Exercise is Medicine.  Those who are syndromic and/or have other conditions to manage are probably best to take medical advice on how much exercise they need to do.

Ethanol (alcohol, liquor): Many NET patients have difficulty tolerating wine, beer and spirits (hard liquor). I was never a big drinker so for me it was easy to go almost teetotal. I do have the occasional beer but very infrequently and normally on holiday – I personally don’t get any issues with the odd beer but again this is trial and error.  I really enjoy my beer when I celebrate my Cancerversaries. Also check out my blog Alcohol – the NET Effect

Summary

I’m sure there could be a 5 A’s to 5 Z’s list of things to avoid but as I said above, this needs to be balanced with what the actual risks for you are and if you’re like me, quality of life. If you read most Facebook closed group or forums, you will always find at least one person is affected by something which affects no-one else. Please note this article is just my own appreciation of these issues and I emphasise once again that everyone has different experiences. I do, however, think it’s important to consider any secondary illnesses, effects of surgery and biochemistry results (or indeed a combination of one or more of these factors). Everything in life involves some kind of risk management and if you are totally risk averse, then you are unlikely to have much of a life (or a diet!).

It’s not easy but my daily diary helps me assess trends and work out what things upset me more than others – I can then reduce or eliminate. You need to tailor your own advice perhaps with the help of a doctor and/or dietician versed in NET Cancer.  I also have some related posts on the subject of vitamin and mineral deficiencies, malabsorption and probiotics – check them out as the problems associated with these subjects could potentially look like a worsening of carcinoid syndrome and lead to unnecessary worry and unnecessary treatment.

For most, Carcinoid Syndrome can normally be controlled by the use of debulking surgery and/or somatostatin analogues (Octreotide/Lanreotide).  However, there is a new drug called ‘Teloristat Ethyl’ (XERMELO) which looks like it may provide supplementary treatment for patients whose carcinoid syndrome diarrhea is not adequately controlled by somatostatin analogues. It’s an expensive drug and comes with side effects so you need to be sure it’s your syndrome causing the problem before you commit to a prescription.

Thanks for reading

Ronny

I’m also active on Facebook.  Like my page for even more news.  I’m also building up this site here: Ronny Allan

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!

patients included
This is a Patients Included site

 

Cancer? it’s what other people get

Other people get Cancer but not me
Other people get Cancer but not me

I talk often about my diagnosis but not about an ‘incident’ which occurred almost immediately prior to being formally told.

I was well into the ‘diagnostic phase’, having had all sorts of tests including a liver biopsy.  I vividly remember thinking these tests were a ‘nuisance’, I was far too busy and I didn’t even feel ill.  In hindsight, I was fortunate to have had such a thorough bunch of physicians who diagnosed me with metastatic Neuroendocrine Cancer in about 6 weeks ‘flash to bang’.  I intentionally use a phrase associated with ‘quick’ because in the world of Neuroendocrine Cancer, 6 weeks is ‘warp speed’.

So why was I admitted to hospital during the diagnostic phase? Because I was stupid.  In fact I was double-stupid. Firstly, despite having had to undergo a liver biopsy and a referral to an Oncologist, I was in a dismissive frame of mind and was blanking out any thought that I actually had cancer.  I didn’t have time for it, I was far too busy. I’m in control!  Secondly, despite being told to take it easy after the liver biopsy, I ignored that advice because I was far too busy getting on with a normal life. After all, this is just another test hurdle and I’ll get the all clear. Other people get Cancer but not me.

On the weekend following the liver biopsy, the family came round, so I decided to do normal things like lifting one of my grandsons up (as one does) and I prepared the BBQ which involved lifting a 13.5kg cannister of gas from the garage onto the patio.  Why not? I didn’t have anything wrong with me and I didn’t even feel ill.

However, as that Saturday afternoon progressed so did the pain; and to the point that I knew I had to seek help. To cut a long story short, I was eventually admitted to hospital for what was to be diagnosed as a bleed on my liver at the biopsy site.  Oh how the mighty fall.

On the positive side, I got another bunch of tests including scans as confirmation (….a second opinion from a different hospital).  However, it was the wake-up call I needed to take it seriously. I was discharged on the Monday in time for my very first Oncology appointment with my wife Chris in attendance.  For the first time, we were officially told I had Cancer – it was much more than just a ‘scare’. For me, the denial was over, indicating that I was never actually in control of what was happening to me.

Finally some food for thought …… In hindsight, I made the serious mistake of not talking to anyone about my denial and I suspect that led to me acting stupidly.

It really is OK to talk about Cancer

p.s. I’m now slightly mellower about Cancer 🙂 You might say I’m back in control?

Neuroendocrine Cancer Syndromes – Early Signs of a Late Diagnosis

Early signs of a late diagnosis (2)One of the curious things about Neuroendocrine Cancer (NETs going forward) is that it can very often exhibit one or more vague symptoms collectively known as a ‘syndrome’.  Syndrome is an apt word to describe these complications as the most general meaning in medical terms is a group of symptoms that together are characteristic of a specific disorder or disease”.  Having a syndrome can often be the difference between having a ‘functional’ condition or a non-functional’ condition – see more below.

This frequently makes Neuroendocrine Cancer very difficult to diagnose quickly.  It’s a very devious disease.

It’s not all about Carcinoid Syndrome!

Most people think of Carcinoid Syndrome when they discuss NETs. Anyone suggesting that all NET patients get carcinoid syndrome or that all symptoms of NETs are caused by carcinoid syndrome, is WAY off the mark. Firstly, not everyone will have a ‘syndrome’ in addition to their tumours – the percentage is actually well below 50%. Secondly, there are in actual fact, several associated syndromes depending on the anatomical location and type of NET. As an example of one syndrome, statistics vary from source to source but it is estimated that around a 30-45% of all ‘midgut’ patients will present with metastatic disease and around a third of those (∼10-15% of all midgut) will exhibit Carcinoid Syndrome indicating their tumours are ‘functional’ (secreting excess hormones, particularly serotonin).  It follows that Carcinoid Syndrome itself is not that common and it could be the same with other types of NET (even though it can appear more prevalent on forums).

Diagnostic Challenges in NETs (this graphic only covers so-called Carcinoid Syndrome).  Inner segments are the key symptoms, outer segments are some of the potential misdiagnosis/delayed diagnosis. Graphic courtesy of Modlin IM, Kidd M, Latich I, et al. Current status of gastrointestinal carcinoids. Gastroenterology 2005; 128: 1717-1751

Functional / Non-Functional

These tumours and associated syndromes are treatable for most but the difficult part can be arriving at a diagnosis. Moreover, without a syndrome, some of these tumours can be silently growing and as they grow slowly, the ‘silence’ can go on for some years. Even with a syndrome, the root cause can remain disguised as the symptoms are similar to many day-to-day illnesses, again the reason for the title of this blog. Curiously, the lack of a syndrome can sometimes lead to an even later presentation and the consequences that arise (i.e. no signs to aid a diagnosis). In fact a large proportion of Pancreatic NETs are non-functional at diagnosis. There can be the odd exception but in general terms, NETs are either functional (with a syndrome) or non-functional (no syndrome). It’s also possible that patients can move from one state to another.

It’s useful to know about the range of tumor markers and hormone markers – read more here

Syndrome and Tumors – ‘Chicken or Egg’ ?

I’m always confused when someone says they have been diagnosed with a Syndrome rather than a NET type.  You normally need a tumor to produce the symptoms of a syndrome.

The exception might be hereditary syndromes e.g. MEN.  MEN syndromes are genetic conditions. This means that the cancer risk and other features of MEN can be passed from generation to generation in a family. A mutation (alteration) in the various MEN genes gives a person an increased risk of developing endocrine/neuroendocrine tumors and other symptoms of MEN. It’s also possible that the tumors will be discovered first.  It’s complex!

Major NET Syndromes  

(information mainly taken from the ISI Book on NETs with a cross-reference from ENETS and UKINETS Guidelines)

The ISI Book on Neuroendocrine Tumors 2016 (Woltering et al) confirms there are a number of syndromes associated directly and indirectly with NETs and are described as individual syndromes according to their secretory hormones and peptides. The reference publication expands on this list to aid diagnoses by including common presentations, associated tumour types and locations and the offending secreting hormones. You can see why Neuroendocrine Cancer is a diagnostic challenge!

Carcinoid – a syndrome connected with (mainly) serotonin secreting tumours in certain locations (mainly small intestine, lung, stomach, appendix, rectum). The key symptoms include diarrhoea, flushing of the skin (particularly the face), stomach cramping, heart problems such as palpitations, and wheezing. The syndrome is actually caused by the release of a number of hormones, in particular Serotonin, Bradykinin, Tachykinin (Substance P), Histamine, and Prostaglandins.

(there’s also a very rare instance of pancreatic based tumours producing carcinoid syndrome effects – according to ENETs less than 1% of all tumours associated with carcinoid syndrome)

Whipple’s Triad – Whipple’s Triad is the classic description of insulinoma which includes symptoms of hypoglycemia with a low blood glucose concentration relieved by the ingestion of glucose. These tumours can be located anywhere within the pancreas in the cells that make insulin. Insulin is a hormone that controls the amount of  glucose (sugar) in the blood. It moves glucose into the cells, where it can be used by the body for energy. Insulinomas are usually slow-growing tumors that rarely spread. Some of these tumours will be associated with MEN1 syndrome.

Zollinger-Ellinson SyndromeA tumour that forms in cells that make gastrin and can be known as a Gastrinoma. Gastrin is a hormone that causes the stomach to release an acid that helps digest food. Both gastrin and stomach acid are increased by gastrinomas.  This is a condition in which one or more tumours form in the pancreas, the upper part of the duodenum or the stomach (these organs are very close and tightly packed together). These tumours secrete large amounts of the hormone gastrin, which causes your stomach to produce too much acid. The excess acid can lead to peptic ulcers, in addition to diarrhea and other symptoms.  Associated with Gastrinoma (pNET) and Gastric NETs.  Some of these tumours may be associated with MEN1 syndrome.

Werner-Morrison SyndromeVasoactive Intestinal Peptide (VIP) is secreted thus the pNET term – VIPoma –  Sometimes the syndrome is referred as WDHA – Watery Diarrhea, Hypokalemia (potassium deficiency), and Achlorhydria (absence of hydrochloric acid in gastric secretions).  Sometimes known as Pancreatic Cholera. Some of these tumours may be associated with MEN1 syndrome

Glucagonoma.  A tumour that forms in cells that make make glucagon. Glucagon is a hormone that increases the amount of glucose in the blood. It causes the liver to break down glycogen. Too much glucagon causes hyperglycemia (high blood sugar) rendering most patients diabetic. A glucagonoma usually forms in the tail of the pancreas.  Some of these tumours may be associated with MEN1 syndrome.  See also Sweet’s Syndrome below.  Sometimes known as the 4D syndrome – Dermatological, Diabetes, DVT, Depression.

Somatostatinoma is a very rare type of NET, with an incidence of one in 40 million persons. These tumours produce excess somatostatin arise from the delta cells in the pancreas, although these cells can also be present in duodenal/jejunum tissue where around 44% of these tumours occur. Somatostatin is a naturally occurring peptide that inhibits the function of almost all gut hormones (author’s note – this fact should give you an appreciation of how somatostatin analogues tackle associated syndromes whilst giving you certain side effects as a result!)

Pancreatic Polypeptide (PP)PPoma A complicated one and not too much information (even in the ISI book or ENETS Guidelines). However, it’s the third most common type of islet cell tumour (i.e. pNET).  The function of pancreatic polypeptide is not completely understood. Patients present with weight loss, jaundice, and abdominal pain. The diagnosis is confirmed by pancreatic polypeptide levels > 300 pg/ml. Some of these tumours may be associated with MEN1 syndrome.

Hedinger Syndrome – the technical name for Carcinoid Heart Disease and an ideal replacement term now that Carcinoid is being phased out.

Cushing’s – also known as hypercortisolism.  A collection of symptoms caused by very high levels of a hormone called cortisol in the body.   In Cushing’s disease, oversecretion of pituitary ACTH induces bilateral adrenal hyperplasia. This results in excess production of cortisol, adrenal androgens, and 11-deoxycorticosterone. Cushing’s disease, a subset of Cushing’s syndrome, is due to a pituitary corticotroph adenoma and results in a partial resistance to the suppression of ACTH by cortisol so that secretion is unrestrained. In contrast, causes of Cushing’s syndrome may include the following:

•   Adrenal adenoma or carcinoma arise spontaneously. ACTH levels are undetectable.

•   Non-pituitary (ectopic) tumours produce ACTH. They most frequently originate in the thorax and are highly aggressive small cell carcinomas of the lung or slow- growing bronchial or thymic carcinoid tumours. Some produce corticotropin- releasing hormone (CRH) instead, which stimulates pituitary ACTH secretion and can therefore mimic a pituitary tumour.

•   Other causes include NETs of the gastric, pancreatic, and intestinal organs; Pheochromocytomas, and MCT.

The hallmark of Cushing’s syndrome is that ACTH levels are partially resistant to suppression with dexamethasone, even at very high doses. Some MEN patients with pituitary tumours may have Cushing’s Syndrome. AdrenoCorticoTropic Hormone (ACTH) releasing tumours are somerimes known as ACTHoma.

Sweet’s – Dermatitis/rash associated with Glucagonomas.  Not to be confused with Pellagra (B3 deficiency)

Neuroendocrine / Endocrine tumors can be seen in several inherited familial syndromes, including but not limited to:

  • Multiple Endocrine Neoplasia type 1 (MEN1)
  • Multiple Endocrine Neoplasia type 2 (MEN2)
  • Multiple Endocrine Neoplasia type 4 (MEN4)
  • SDHx mutations – Hereditary Pheochromocytoma/Paraganglioma Syndromes.
  • Pituitary.
  • Von Hippel-Lindau (VHL) Disease
  • Neurofibromatosis Type 1 (also known as Recklinghausen’s Disease). Not covered further.
  • Tuberous Sclerosis (not covered further)
  • Carney Complex

see Genetics and Neuroendocrine Tumors

MEN1 – Mainly involved the 3 Ps, Pituitary, Pancreas and Parathyroid.  The pituitary tumours are primarily Prolactinomas, the pancreatic tumours are mainly PPomas, Gastrinomas and Insulinoma.  Many also have association with Zollinger-Ellinson  syndrome (ZES).  Sometimes known as Wermer Syndrome.  Associated with the MEN1 gene.

MEN2A – associated with the RET gene, can result in Medullary Thyroid Carcinoma, Pheochromocytoma, and overactive parathyroid glands characterised by a high calcium level.

MEN2B. An inherited disorder characterised by the certain development of Medullary Thyroid Carcinoma, plus the possible development of pheochromocytomas and characteristic tumours (mucosal neuromas) of the lips, tongue and bowels. Parathyroid disease is extremely rare in MEN2B.  Also connected with the RET gene.

MEN4.  A relatively new MEN variant and related to the CDKN1B gene.  Similar to MEN1 but normally only 2 of the 3 Ps, parathyroid and pituitary; and potentially other places.

SDHx mutations/Hereditary pheochromocytoma/paraganglioma syndromes

  • Succinate dehydrogenase (SDH) is an enzyme which is important for the metabolic function of mitochondria. Patients with mutations of these genes have increased risk of pheochromocytomas, paragangliomas, stomach tumors and kidney tumors.
  • SDHx mutations (SDHA, SDHB, SDHC, and SDHD) can present as Pheochromocytomas/Paragangliomas and other non-NET conditions.  If this interests you see site http://www.SDHcancer.org

Von Hippel-Lindau (VHL) – not an exclusively NET syndrome. VHL is a rare disorder caused by a faulty gene. It is named after the two doctors who first described the disease, and affects about one in 35,000 people. Tumours develop in one or more parts of the body. Many of these tumours involve the abnormal growth of blood vessels in parts of the body which are particularly rich in blood vessels. Areas most frequently affected are the eyes, the back of the brain (cerebellum), the spinal cord, the kidneys, the adrenal glands and the pancreas. People are affected differently, even within the same family. The only VHL tumour which tends to run in families affects the adrenal glands (Pheochromocytoma). Different VHL features tend to develop at different ages. The eye angiomas often develop in childhood. Others, including tumours found in the cerebellum, spinal cord or adrenal glands (Haemangioblastomas and Pheochromocytomas) can develop from late childhood onwards. The kidney tumours are usually the last things that develop, from the mid-twenties onwards.  Most VHL related tumours are benign.

Summary

As for my own experience of syndromes, I did once show symptoms of the most common NET syndrome (currently known as Carcinoid syndrome) where the key symptoms include diarrhoea, flushing of the skin (particularly the face), stomach cramping, heart problems such as palpitations, and wheezing.  You can see why those symptoms are frequently and easily confused with other conditions. If you have a similar diagnosis, you may benefit from looking at something known as The 5 E’s which is a useful list of things to be wary of.

I did have issues for a year or two in 2010 leading up to diagnosis and until my treatment was underway.  I was experiencing flushing and infrequent bouts of diarrhea but I totally ignored it (hear me talk about this). However, it ended up being instrumental in my diagnosis albeit some good luck was involved in getting to that point.  My twist of fate which involved a low hemoglobin score led me to a scan and ‘bingo’.  I had a ‘gastrointestinal blip’ some 18 months previously but that proved colonoscopy negative.  Despite my distant and metastatic tumour disposition and seemingly late diagnosis, I’m current non-syndromic due to “early” intervention and good treatment.  However, my ongoing treatment continues to play its part.

For many, the vague and routine symptoms generated by a syndrome contribute to the fact that NET Cancer is frequently misdiagnosed with some people suffering from the side effects for many years before a correct diagnosis is made.

There are many other less known syndromes that appear to be directly or indirectly connected with Neuroendocrine Tumours and I may update this post if I discover they are more prevalent than I think.  Please let me know if you’ve been told you have a NET related syndrome not listed.

Neuroendocrine Cancer – shh! Can you hear it? 

Thanks for reading

Ronny

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