“I’m only as good as my last scan”. I received this comment last week in response to one of my posts and I thought it was a very pragmatic thing for someone to say.
A NET patient under surveillance has regular tests at determined intervals but the one that is most likely to spot disease progression, stability or regression is a scan. Markers such as (say) Chromogranin A (CgA) or 5HIAA are clearly useful but in an ongoing surveillance scenario, they alone would not be used as a firm declaration of progression, stability or regression. Every picture tells a story and a scan is normally the confirmation required whether it’s a CT, MRI or PET (etc).
Scans are also important at the diagnostic phase and I’m sure like myself, many people had their first ever scan at this point. You can have many checks, investigations and tests but for most, the scan is normally the main test that is going to confirm the presence of tumours. This then leads to further checks to confirm the staging and grading (i.e. a biopsy) and then hopefully, a proper diagnosis.
I don’t mind scans, they are probably the test that is going to alert my team to anything odd going on. Thus why I don’t mind doing them – in fact, they are a piece of cake!
OPINION. When I was being officially told I had an advanced and incurable cancer, I did what most people seem to do on films/TV ….. I asked “how long do I have“. The Oncologist said ” … perhaps just months“. That must have been quite a shock because for a few moments after that, I heard nothing – my brain was clearly still trying to process those words – I wasn’t even feeling unwell! The really important bit I missed was him go on to say “…but with the right treatment, you should be able to live for a lot longer”. Fortunately, my wife Chris heard it all and I was refocused. “OK Doc – let’s go” I said. Always take someone with you to take notes at important meetings with Oncologists!
I continue to see quite a few posts and articles about death and dying and I noticed some patients were using the word ‘terminal‘ to describe Neuroendocrine Cancer, despite in some cases, having been diagnosed some years ago, despite in most cases in reference to well differentiated diagnoses. This label is not just confined to use within Facebook forums, I’ve also seen this on wider social media including twitter, blogs and newspaper items. For some, this appears to be the prognosis given to them by their doctors. I find this surprising. However, I’m much less surprised to see many comments on forums from people who had been told the worst by their doctors but were still alive and kicking WAY beyond those worst case prognostic statements, including the higher grade cases.
Definitions are important so what does ‘terminal cancer’ actually mean?
I’m conscious there are legal ramifications with the definitions (wills, life insurance, disability etc) and that these may differ on an international/federal basis. I therefore intentionally confined my searching to a couple of ‘big hitter’ and ‘authoritative’ sites:
Cancer Research UK defines terminal as “When cancer is described as terminal it means that it cannot be cured and is likely to cause death within a limited period of time. The amount of time is difficult to predict but it could be weeks to several months”.
The American Cancer Society defines terminal as “an irreversible condition (it cannot be cured) that in the near future will result in death or a state of permanent unconsciousness from which you are unlikely to recover. In most states, a terminal illness is legally defined as one in which the patient will die shortly whether or not medical treatment is given.”
Can terminal as defined above be applied to Neuroendocrine Cancer?
I’m sure it can, e.g. with very advanced and very aggressive disease and for any grade when taking into account the condition of the patient and other factors (secondary illnesses/comorbidities, refusal of treatment etc). Clearly, that is a terrible situation. I’m also conscious that some people do eventually die because of this disease or its consequences and that is also terrible.
How long is a piece of string?
I think with most Neuroendocrine Cancer patients, “how long do I have” can be a tough question to answer. Thinking back to my own situation, although it was an obvious question to ask my Oncologist, I can see it might have caught him unawares. I suspect he was erring on the side of caution as I don’t believe he had formulated my treatment plan ….. i.e. my case had not yet been looked at by a Multi-Disciplinary Team (MDT), a bit like a ‘Tumor Board’. I had already been confirmed Grade 2 (via liver biopsy) and my CT scans were indicating widespread disease. I was yet to have an Octreotide scan and the conventional biochemical markers (CgA and 5HIAA). I suspect, faced with my question, he went for the worst case, based on the statistics he had access to at the time. What I now know is that, in the year of my diagnosis, the median survival was 33 months in patients with advanced Grade 1/Grade 2 NETs with distant metastasis. These statistics are certainly better today but my Oncologist was probably on the right track. However, at no time did he use the word ‘terminal’.
The Cancer story is changing
What I also found during my research is that as more and more people in the UK are now living with cancer (all cancer) rather than dying from it, there is a new class of patients emerging – Macmillan UK call this “treatable but not curable” and I believe this is very relevant to Neuroendocrine Cancer. I touched on this in an awareness blog entitled “Living with Neuroendocrine Cancer – it takes guts“. You will find some data in this blog about a major increase in the amount of people with cancer who eventually die of something else (…… basically it has doubled). For many, Cancer is no longer a death sentence. I do accept that it can be difficult to live with certain cancers and this is also covered in my “it takes guts” blog linked above.
Survivorship and Hope
You can find numerous examples of long-term survivors of advanced Neuroendocrine Tumours on the ‘airwaves’, many with a relatively good quality of life (QoL). I don’t normally pay much attention to prognostic data, I take my lead from the huge number of patients living a long time with Neuroendocrine Cancer. However, I was particularly interested to read a set of USA statistics from NOLA (Boudreaux, Woltering et al) which said “Our survival of stage IV midgut NET patients that we performed surgical debulking on was published in the Journal of the American College of Surgeons in 2014. It showed our 5, 10 and 20-year survival rates were 87%, 77% & 41% respectively. It’s also worth noting the comparison with the 2004 SEER database analysis which listed the 5 & 10 year SEER survival at 54% and 30% respectively”. Clearly, the NOLA figures are guidelines (and only for midgut) but they do seem to reflect my previous statement about seeking out positives rather than dwelling on the negatives. The SEER 2012 figures are much better than the 2004 versions stating “Survival for all NETs has improved over time, especially for distant-stage gastrointestinal NETs and pancreatic NETs in particular, reflecting improvement in therapies.
Exciting times ahead
On the subject of therapy improvement, there has been a plethora of new treatments coming online and more entering and progressing through the approvals pipeline. Check out my article entitled Exciting Times Ahead. Also listen to a NET Expert along the same lines. PRRT is making a real difference.
Summary
Following my diagnosis in 2010, I went on to receive really good treatment and it continues to this day with Lanreotide backed up by a rigorous surveillance regime (and this is backed up by my own advocacy!). However, I have totally accepted the fact that I have metastatic Neuroendocrine Cancer and that it cannot be cured. By the way, I intentionally used ‘metastatic‘ rather than Stage IV. Mention of Stage IV can set off alarm bells and send the wrong message to the recipient. I don’t believe Stage IV has the same ‘red flag’ meaning for well-differentiated NETs as it does with more aggressive cancers of the same stage. Given what I know now, I would certainly challenge any doctor who told me I had a ‘terminal disease’ and at the same time told me I had a slow-growing well differentiated Neuroendocrine Cancer.
I now live with this disease (….and it’sconsequences) and do not feel like I’m dying of it. Moreover, I most certainly do not see myself as a ‘terminal’ cancer patient, particularly as I’ve now been living with it since 2010.
Whilst we’re on this subject, please note Palliative Care is not just end of life / hospice care. That’s another misunderstanding bordering on mythical status. Read more here.
One of the most discussed and sometimes confusing subjects on forums is the staging and grading of Neuroendocrine Neoplasms (NENs). Mixing them up is a common error and so it’s important to understand the difference despite the apparent complexity. If I was to make a list of questions for my specialist/Oncologist at diagnosis, it would include “What is the stage, grade and differentiation of my cancer”. To enable me to synchronise with the documented guidance, I’m going to use the following WHO 2017 approved terms in this post:
Neuroendocrine Neoplasm (NEN) – all types of Neuroendocrine tumour of whatever grade (please note Neoplasm is another word for tumour)
Neuroendocrine Tumour (NET) – all well-differentiated tumours (an explanation of differentiation will be provided below)
Neuroendocrine Carcinoma (NEC) – all poorly differentiated tumours
Stage vs Grade
In the most basic of terms, stage is the spread or extent of cancer and grade is the aggressiveness of cancer. They are totally different things and an understanding of both is important as they are critical to predict outcome (to a certain extent) and guide therapy. There is no correlation between the two, you can have the lowest grade with the highest stage (actually very common with NETs).
As patients, we deal with many medical specialists during diagnosis and subsequent treatment. However, we rarely meet the pathologist who plays a critical role in the outcome. Precise diagnosis is what drives patient decisions and treatment. If the pathology is wrong, everything that follows could be incorrect as well. It’s a very important area.
Grading (aggressiveness)
Grading and Ki67
Antigen KI-67 is a nuclear protein that is associated with and may be necessary for cellular proliferation. Ki-67 is therefore an excellent marker to determine the growth fraction of a given cell population. The fraction of Ki-67-positive tumour cells (the Ki-67 labeling index) is often correlated with the clinical course of cancer.
Pathologists normally need to count about a thousand cells in order to determine the percentage of cells that are Ki67 positive – thus why you see Ki67 expressed as a percentage. 0% is the lowest, 100% is the highest. Often, they add greater or less than signs depending on the sample involved, i.e. >5% or <5%. There are other measurement systems in place, mainly Mitotic Count.
The ranges for Neuroendocrine Neoplasms (NENs) are divided in to 3 Grades depending what range the Ki67 number lands in. With NENs, the differentiation (well or poorly) is almost as important at grade 3 (high grade). Grades 1 and 2 are by default well differentiated NETs).
Why is differentiation important?
To fully understand grading, you also need to understand the concept of ‘differentiation’. In the most basic of terms, ‘differentiation’ refers to the extent to which the cancerous cells resemble their non-cancerous counterparts. This is an important point for NETs because many low-grade tumour cells can look very similar to normal cells. The differentiation of a NET has an impact on both prognostics and treatment regimes.
NENs fall into one of three grades based on their differentiation and their proliferative rate. The proliferative rate is measured mainly using two methods known as Miotic Count and Ki-67 index, the latter seems to be more frequently used (but see below for Lung NETs). The Ki-67 index can usually be determined, even in cases of small biopsies but Mitotic rate counting requires a moderate amount of tumour tissue (at least 50 HPFs or 10 mm) and may not be feasible for small biopsies. The Miotic Count method may be preferred or used in addition to Ki-67 for certain Lung NET scenarios as it is said to be more helpful in distinguishing atypical from typical NET (what some might ‘old fashionably’ and incorrectly refer to as Lung Carcinoid tumours), and for small and large cell lung Neuroendocrine Carcinomas (NEC).
Some of you may have heard the term ‘moderately differentiated’ which tended to align with an intermediate grade or Grade 2. However, please note that the term moderately differentiated as a classification was phased out in 2010 by WHO reducing from 3 differentiation levels to 2. Grade 2 is also defined as well differentiated but based on different proliferative rate (see table). High grade was normally referred to as Neuroendocrine Carcinoma indicating it is a faster growing and more aggressive cancer. However, see below for an important change to high grade classification.
Grading – Key WHO 2017 Changes
WHO Classifications of Cancer are published in something known in medical world as “The Blue Book”. For NETs, the 2017 version comprises only the “WHO Classification of Tumours of Endocrine Organs”. Technically this would preclude the digestive system and lung NETs but the leading NET pathologists have submitted a recommendation to normalise all NET Blue Books along the same classification scheme. Worth also noting that the latest ENETS Guidelines are already using the new grading terms. Many sites remain out of date so be careful where you look.
Misc Grading Issues
The proliferative rate can be diverse in NENs, so sampling issues can limit the accuracy of grading. More substantial samples of tumour are therefore preferable for grading thus why the Ki-67 index is preferred for biopsies where large amounts of tissue may not be available. The distinction of low-grade from intermediate grade can be challenging when using small samples. A couple of interesting observations about NET grading which I spotted during my research and ‘forum watching’. You can have multiple primary tumours and these might have different Ki-67 scores. Additionally, on larger tumours, Ki-67 scores can be different on different parts of the tumour. And something I know from my own experience, secondary tumours can have different Ki-67 scores than primary – even a different grade. In my own case, my liver secondary tumours were graded higher than my primary which according to my surgeon is in keeping with a clone of the disease having become more aggressive over time. Royal Free Hospital NET Centre indicates a person’s grade should be taken from the highest biopsy grade taken. This is a fairly complex area but a recent study published by the US National Institute of Health and many anecdotal comments made by NET specialists indicates that is a fairly common scenario.
Summary of Key Changes
The 2017 World Health Organisation (WHO) classification sub-divided Grade 3 into two new entities: a well differentiated high-grade NET and a poorly differentiated high-grade NEC. There may also be a cut-off point in proliferative rate (i.e. Ki-67) between NET and NEC in relation to potential treatment strategies (55% is mentioned for pNETs but I’m currently investigating). Physicians don’t really have much data to support specific guidelines for treatment so all cases will be personalised.
The Grade 1 to 2 Ki-67 cut-off is changed from 2 to < 3 for clarification purposes. There was some discussion as to whether it should be <5 but this was not accepted.
Well differentiated High Grade NETs are now recognised. These are known as a NET rather than a NEC. Both Grade 3 (NET) and Grade 3 (NEC) have the same biopsy marker cut-offs as per the leading slide but it is thought that a threshold reading of 55% could have some influence on the treatment regime. For example, a well differentiated tumour with a Ki67 of less than 55% might benefit from the same treatment given to Grade 1 or 2 patients, whereas a well differentiated tumour with a Ki67 of more than 55% might benefit from the same treatment given to poorly differentiated NEC. Only a guideline and I suspect this is like many things in NENs, very individual, relies on many factors, so your specialist will drive this accordingly. You may see these 2 grades listed as Grade 3a for NET and Grade 3b for NEC.
Previously, Pheochromocytoma did not have an official grading regime, i.e. they were just benign or malignant. Now they are using the same grading system as above. I’m assuming this is the same for Paraganglioma and I will confirm in due course. This is an excellent change and a continuation from the WHO 2010 classification where there was great emphasis away from a benign/malignant classification to formal grade levels on the basis that all NETs have malignant potential.
It also introduced a change to the naming of mixed cell tumours from Mixed AdenoNeuroendocrine Carcinoma (MANEC) to Mixed Neuroendocrine Non-Neuroendocrine Neoplasms (MiNEN). A full explanation of MiNEN will follow but I would suggest the use of the term ‘Neoplasm’ has been chosen rather than ‘Carcinoma’ is because these neoplasms can be well or poorly differentiated.
It’s not possible at this time to acquire copies of the official output but I will keep this blog live.
The source material for the 2017 version of this article.
From leading Pathologist Dr Anthony Gill – Remember this is based on Endocrine Organs only but it will eventually apply to all. I am awaiting access to free documentation to update this article further – only ones I can currently find are not free!
Staging (spread)
Staging is the extent or spread of disease. Most types of cancer have 4 stages, numbered from 1 to 4 indicating a rising spread as the number is bigger. Often doctors write the stage down in Roman numerals, perhaps this is to stop any confusion between standard numbers used for Grades? So you may see stages written as I, II, III and IV. In addition to this standard method, there is also an agreed model known as TNM (Primary Tumour, Regional Node, Distant Metastasis) which is essentially a more detailed staging definition when combined with the Stage 1-4 model. Please note with TNM models, there could be different stage descriptions depending on the location of the primary tumour and similarly different TNM models for different tumour locations.
WHO 2017 changes
WHO 2017 has recommended enhancements to the TNM system mainly the use of additional suffixes indicating the extent of lymph node involvement. Details to follow when I can free access.
The following example shows the stage descriptions for a NET of the small intestine (the others are similar but worded accordingly for that part of the anatomy):
Stage I tumour is less than 1 cm in size and has not spread to the lymph nodes or other parts of the body.
Stage II tumour is greater than 1 cm in size and has started to spread beyond the original location, but has not spread to the lymph nodes or other parts of the body.
Stage III is any size tumour that has spread to nearby areas of the body and also to at least one lymph node.
Stage IV is any size tumour that has spread to one or more lymph nodes and has also spread to other, more distant areas of the body (such as the liver).
It’s also worth pointing out that Stage IV does not necessarily mean a cancer is more dangerous than other cancers of lesser stages. This is an important point for NETs where Stage 4 can be matched up with a low-grade tumour i.e. Stage 4 for lower grade NETs is very often not the ‘red flag’ it is for other more aggressive cancers. For example, doctors may surgically remove a Stage IV NET, while surgery might not help a patient with a cancer of a higher grade at such a late stage.
Notes:
Sometimes doctors use the letters to further divide the number categories – for example, stage 2A or stage 3B. This is normally to clarify or provide more detail of the primary tumour size/invasion in conjunction with the TNM model.
You may also see something called Stage 0 which is for ‘Carcinoma in situ’. It means that there is a group of abnormal cells in an area of the body. However, the number of abnormal cells is too small to form a tumour and may, therefore, be currently classed as benign. The World Health Organisation (WHO) system does not appear to recognise Stage 0 for NETs.
The most generic model for TNM staging is below but please note this could be adjusted for particular types of NET.
Primary Tumor (T) TX: Primary tumor cannot be evaluated T0: No evidence of primary tumour Tis: in situ (abnormal cells are present but have not spread to neighbouring tissue; although not cancer, in situ may become cancer and is sometimes called preinvasive cancer)
T1, T2, T3 and T4 is a measure of the size of, and/or invasion/penetration by, the primary tumour and the wording varies between different NET sites. e.g. for a small intestinal NET:
T1 tumour invades mucosa or submucosa and size <=1 cm
T2 tumour invades muscularis propria or size >1 cm
T3 tumour invades subserosa
T4 tumour invades the visceral peritoneum (serosa)/other organs
Distant Metastasis (M) MX: Distant metastasis cannot be evaluated M0: No distant metastasis M1: Distant metastasis is present
You may occasionally see TNM staging be prefixed by lower case letters. The most commonly used prefix is ‘p’ simply meaning the grading has been confirmed by pathology. e.g. pT4 N1 M1
Specialists can combine the Stage to create a TNM – for example:
This slide will be updated when I get access to WHO 2017 or updated AJCC pubication.
Summary
A complex area and I hope I have condensed it sufficiently for you to understand enough for your purposes. Despite looking very scientific, it is not an exact science. There are many variables as there always are with Neuroendocrine disease. NENs can be very challenging for a pathologist even an experienced one who may not have encountered NENs before. However, it is an extremely important part of initial diagnosis and also when needed during surveillance. It is a vital tool used by Multidisciplinary Teams (MDT) in treatment plans and for prognostic purposes. If you need to learn further, I recommend this document:
If you are interested in this subject and have one hour to spare, there is a great video here from LACNETS worth watching.
There’s a constant debate regarding the validity of the term ‘Carcinoid‘. I’ve posted about this a few times and as far as I know, the debate has been raging for some years.
You may have noticed that ‘Carcinoid’ is often used as a standalone word and tends not to be suffixed with the word ‘Cancer’ or ‘Tumour’ – unlike Bowel Cancer, Breast Cancer, Prostrate Cancer, Lung Cancer, Brain Tumour, etc. Nobody goes around saying “Breast” or “Bowel” do they? But they happily say “Carcinoid”. Unfortunately, the term ‘Carcinoid’ has become entrenched in both pathology and clinical literature over the past 100 years. The main problem with the word Carcinoid is that it means different things to different people. Some use the term almost exclusively to designate serotonin-producing tumours that arise from the enterochromaffin cells that can result in carcinoid syndrome i.e. most commonly in the appendix, small intestine, stomach, lung, rectum and uncommonly in other places. Some use it to (incorrectly) refer to all Neuroendocrine Tumours. The most worrying connotation of the use of the word ‘Carcinoid’ is the belief that they all have benign clinical and biological behaviour. That is dangerous thinking and has the potential to kill people. Fortunately, NET specialists are starting to move away from using the word – check out the quote below:
Siegfried Oberndorfer
The Origins
The following history of ‘Carcinoid’ is well documented: Siegfried Oberndorfer (1876-1944) became the first to adequately characterise the nature of Carcinoid tumours and refer to them as “benign carcinomas.” During his tenure at the Pathological Institute of the University of Munich, Oberndorfer noted in 1907 that the lesions were distinct clinical entities and named them “karzinoide” (“carcinoma-like“), emphasizing in particular their benign features. However, In 1929 he amended his classification to include the possibility that these small tumours could be malignant and also metastasise. (Author’s note – a name change would have been handy at this point).
100 years later
NANETS, UKINETS and ENETS seem to defer to the WHO classification nomenclature and it is here another term is introduced – Neuroendocrine Neoplasms (NENs). NANETs state that “all of the entities under discussion are neoplastic, and neoplasm is therefore a more accurate term than tumor, which means only a mass“. These organisations tend to use the term Neoplasm as a catch-all for all Neuroendocrine disease and then the term ‘tumor’ and ‘carcinoma’ applies to well and poorly differentiated respectively. It’s worth noting that since 2010, the WHO classification is based on the concept that all NETs have malignant potential, and has therefore abandoned the division into benign and malignant NETs and tumours of uncertain malignant potential. Neuroendocrine Carcinoma is malignant by defintion. All of this has been reinforced in the 2017 publication. The term ‘Carcinoid’ is conspicuously missing from these texts.
To put it simply – the term ‘carcinoid’ is no longer credible
Due to its historical meaning, Carcinoid does not adequately convey the potential for malignant behaviour that accompanies many of these neoplasms as described above. The term Carcinoid decodes to ‘Carcinoma like’. Contextually “Carcinoid Cancer” decodes to “carcinoma like cancer” which is, of course, totally misleading and its use simply perpetuates the claim by some that it is ‘not a proper cancer’. If we only needed one reason to ditch the word ‘Carcinoid’, this would be it.
I mentioned confusion above and this has led to a hybrid effect of naming the condition. For example, there is a tendency by some (including medical establishments and patient organisations) to use the term ‘Carcinoid’ and ‘Neuroendocrine Tumors’ interchangeably which is patently incorrect. Neither is it helpful that many patients and organisations continue to refer to this disease as “Carcinoid Neuroendocrine Tumor”, “Neuroendocrine Carcinoid Tumor”, “Neuroendocrine Carcinoid Cancer”, “Carcinoid/Neuroendocrine”, “CNET”; and many other variations along these lines. Many seemingly credible organisations will say “Carcinoid and Neuroendocrine Tumors” not realising it’s a contradiction in terms. Continued use of the term in any phrase or standalone context is not doing our case for recognition any good – it’s bad enough that some seem to cling to outdated and invalid diagnostic clichés and icons from the 1980s. All of it needs to go.
I know I’m not alone in this thinking given the decrease of its use in the NET world, including NET Specialists (see lead graphic) and NET Specialist organisations (some have changed their names). There’s an interesting article written by a NET specialist where the term ‘carcinoid’ is described as “unfortunate”, “misleading”, “outmoded”, “archaic”, “confusing” and “misnomer”. Exactly! In the recent SEER NET study, a NET specialist reaffirmed this thinking by stating that “the belief these tumors did not metastasize, did not reach any great size, and appeared harmless, has since been proven false”. Continued use of the term ‘Carcinoid’ has the potential to regress this thinking. We must not let this happen.
So what terms should we be using?
People and organisations will be out of date with modern Neuroendocrine Neoplasms nomenclature and some will still want to continue with their own nomenclature (….. and because of the confusion, some will fall into both categories not realising they’re out of date). Here’s a classic example of the problem we face – the American Cancer Society(ACS) does not even list Neuroendocrine Tumor as a cancer type. Instead you can find “Gastrointestinal Carcinoid Tumors” and “Lung Carcinoid Tumor”. You’ll find Pancreatic NETs inside Pancreatic Cancer. Americans should harangue the ACS to get this right. I could go on with many similar observations on seemingly respectable sites. I intentionally used a US example as this country appears to be way behind in the changes to NET nomenclature, pretty surprising as they tend to be at the forefront of many other aspects in the world of NETs.
Personally, I think the acceptance of a common worldwide nomenclature should come from the World Health Organisation (WHO) classification for Neuroendocrine Neoplasms. They are divided into a number of chapters including ‘Endocrine Organs’, Digestive System, Lung Tumours….. and no doubt some others. Frustrating, but medical people tend to look at things in anatomical terms. Nonetheless, the agreed classification nomenclature for the whole group of Neuroendocrine Neoplasms can be found with some research and access to clinical publications. The correct nomenclature should then be flowed down in regional groupings, e.g. ENETS representing Europe, NANETS representing North America, etc. As I understand it, ENETS and UKINETS are already essentially aligned with WHO and NANETS appears to be. From these organisations, the use of the correct terminology should then rub off on patients, patient advocate organisations and general cancer sites. However, the biggest challenge will be with hospitals/medical centres, cancer registries and insurance companies whose medical record processing is run using reference data (think drop down selections and database structures). Easier said than done but ‘change’ always has to start somewhere. Technically it has started (albeit late) as the big NET medical organisations are already starting to reduce the use of outmoded words such as ‘carcinoid’.
I once argued that the term ‘carcinoid’ needed to be retained as it represented a histopathological grouping of a particular type of NET comprising mostly appendiceal, stomach (gastric), rectal, small intestine and lung NETs. However, reading through the ENETS 2016 guidance in conjunction with the most up to date WHO classification publications, I’ve changed my mind after noticing they no longer use the word ‘Carcinoid’ in relation to a tumor type. Rather, they use the latest WHO terms above and then use the anatomy to distinguish the different types of NET (like we already do for Pancreatic NET or pNET).
Perhaps patients can lead the way here ………
Rather than say:
‘Carcinoid’ or ‘Carcinoid Tumor’….. why not say Neuroendocrine Tumor or NET (adding your primary location if required – see below);
‘Carcinoid Cancer; ….. why not say Neuroendocrine Cancer;
‘Lung Carcinoid’ ….. why not say Lung NET (adding typical or atypical if required);
‘Small intestine Carcinoid’, why not say Small Intestine NET (or ‘SiNET which is becoming popular); p.s. I’m not a fan of ‘small bowel’ due to the potential for confusion with the widely used term ‘bowel cancer’);
‘Gastric Carcinoid’, why not say Gastric NET (adding your type if required);
‘Rectal Carcinoid’, why not say Rectal NET;
‘Appendiceal Carcinoid’, why not say Appendiceal NET;
…. and so on. And you can add your stage and grade/differentiation for a richer picture.
You can listen to a very well known NET Specialist say something similar in this videohere.
Worth noting that even ENETS and NANETS cannot agree on tumor type terminology – the latter uses Small Bowel NETs (SBNETs) whereas ENETS uses Small Intestine NENs (SiNENs). I did say it’s easier said than done.
As I said above, the term ‘Carcinoid’ has become entrenched in both pathology and clinical literature over the past 100 years so it will still appear in many texts and need to be searchable online to support medical and advocacy business. However, these are technical issues and I don’t therefore believe people need to use the terms to make them searchable online. I tag all my posts with ‘Carcinoid’ even if I don’t mention the word in my text. I have started only using the term for context when it is required and am currently reviewing all of my posts to ensure that is still the case.
Hang on…what about Carcinoid Syndrome
When someone wants to know which syndrome you have, you can’t just state (say) “small intestine syndrome” or “midgut syndrome”. ‘NET Syndrome’ doesn’t work either as there are several NET syndromes. This has led to the situation where people try to drop the word ‘carcinoid’ and just say “the syndrome” which is even more confusing! I accept this one is a difficult challenge but I don’t believe it’s insurmountable, just needs some willpower and agreement. I could come up with other terms in about 5 minutes.
What about Carcinoid Heart Disease
Personally I don’t see why this cannot be renamed to ‘Neuroendocrine Heart Disease’ or its technical name – ‘Hedinger syndrome’.
What about Carcinoid Crisis
World renowned NET specialists already make statements that these issues can apply to all types of NET; and it’s well-known that a similar crisis situation already applies to other types e.g. Pheochromocytomas.I cannot see why something along the lines of ‘Neuroendocrine Crisis’ or ‘NET Crisis’ would not be acceptable.
Summary
We as patients are unlikely to be able to force changes on the medical and insurance communities but we can be a ‘force for change’ by setting the example of using a correct and more apt terminology to describe our disease.
Thanks for listening
Ronny
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There was a 60 minute silence last night as another episode of Game of Thrones was aired. Not a Facebook post or tweet in sight. This has to be ‘up there’ in a list of the best TV series ever? Don’t know about you but I’m sometimes confused about who is who and how they are related and/or connected! (see useful chart at the bottom of this post)
Chris and I love the introduction bit. She likes the music, I like the geography. There are some obvious correlations there, e.g. ‘The Wall’ is meant to relate to Hadrian’s Wall with those horrible barbarian Scots to the north 🙂 Thank God Hadrian’s Wall and the climate in particular, isn’t as bad as portrayed on GOT!
I did contemplate using ‘trousers’ as the theme of today’s blog following some interesting banter on my Facebook page. The debate centred on the type of causal clothing one should wear if one is of a certain age. Most of the debaters were ex-military which made for some interesting input followed by even more interesting output! Anyway…. having recently reduced from a 32″ to a 30″ waist, I made the trip down town today to buy some new jeans and came back with nothing, the ones I really liked seemed to either start at 32″ waist or did not include a short leg option.
However, Chris and I did manage to pick up two suitcase bargains in the Debenhams sale. For the last 6 holidays we have been enviously watching all those flash people with their ‘push along’ suitcases whilst we huff and puff behind them with our 1980s luggage. ‘Roll on’ Gatwick next month!
I’ve skipped a couple of days worth of A to Z terms so need to catch up. Read on as I know from feedback that quite a lot of you guys enjoy my ‘layperson’ explanations of complex medical stuff! Two important ‘techy’ bits here to cover the ‘M’ and ‘N’ components of my A to Z.
Metastatic Cancer
In a nutshell, Metastatic cancer is a cancer that has spread from the part of the body where it started (the primary site) to other parts of the body. When cancer cells break away from a tumour, they can travel to other areas of the body through the bloodstream or the lymph system. The spread of a cancer is often described in terms of a ‘stage’. This shouldn’t be confused with ‘grade’ which is normally how aggressive a cancer is – I talked about this in my blog of 11 May. Staging and Grading for Neuroendocrine Cancer can be complex and there doesn’t appear to be a standard that all NET centres adhere to. There is a complex grading system proposed for Neuroendocrine Cancer known as TNM (Tumour, Node, Metastasis) but mostly it’s still referenced by the simple Stage 0 to IV (0 to 4) model. For some types of Neuroendocrine Cancer there appears to be an even simpler model of ‘Local’, ‘Regional’ and ‘Metastatic’. My stage is ‘Metastatic’, Stage IV or TNM – T4N2M1 depending on which one is being used.
Neuorendocrine
Appears to be pronounced ‘….crin’ which was a revelation for me after 12 months! I had never heard of this word until it had been imprinted all over my internal workings! It’s amazing how much of the human body we take for granted. The neuroendocrine system is made up of the nervous system and the endocrine system. The systems work together to keep your body functioning regularly. Neuroendocrine describes certain cells that release hormones into the blood in response to stimulation of the nervous system; i.e.
Your brain sends signals to your nervous system that control various body functions
Some messages are sent to the glands of the endocrine system. These include the pancreas, hypothalamus, thyroid and parathyroid, pituitary gland, adrenal glands, ovaries, and testes.
These glands release hormones that control important body functions, such as growth, metabolism, reproduction, and response to stress or injury.
Sometimes neuroendocrine cells can become cancerous and in certain types (mainly Carcinoid), it can result in the neuroendocrine system secreting excess hormones which will create dangerous problems for sufferers. This is one of the reasons I now avoid alcohol, very large meals, certain types of food and stress…….. so if you’re out for a meal with me and you notice the bill is exorbitant, don’t let me see it 🙂
I went on to produce a blog explaining the word ‘Neuroendocrine’ to laypersons – you can read this here: CLICK
Don’t forget to check out the GOT chart below!
Thanks for reading
Ronny Allan
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