LUTATHERA (PRRT) – NETTER 2 Clinical Trial for Grade 2/Grade 3 Neuroendocrine Neoplasms


netter 2

Brief Summary

The NETTER-1 trials led to the approval of Lu177 (or Lutathera), more commonly known in the community as Peptide Receptor Radio Therapy (PRRT).  This led to an explosion of availability across the world but many gaps in service remain.

Many PRRT spin off trials are in the pipeline looking at different types of PRRT, mainly using slightly different radionuclides and techniques.  However, NETTER-2 builds on the success of the approved version formally known as Lutathera.

The aim of NETTER-2 is to determine if Lutathera in combination with long-acting octreotide prolongs PFS in GEP-NET patients with high proliferation rate tumors (G2 and G3), when given as a first line treatment compared to treatment with high dose (60 mg) long-acting octreotide. Somatostatin analog (SSA) naïve patients are eligible, as well as patients previously treated with SSAs in the absence of progression. This is a phase 3 trial that will be hoping for 222 participants at multiple locations.

This is an exciting trial because there are already data produced indicating that PRRT can be used on high grade tumours providing they have sufficient and efficient somatostatin receptors and a Ki67 of less than 55%.  2019 Updated data for Grade 3 Neuroendocrine Neoplasms: “Compared to studies evaluating the efficacy of chemotherapy for NEN patients with a Ki-67 index less than or equal to 55 percent, PRRT has a longer overall survival rate–22 months versus 14 months,” the researchers pointed out. “These results suggest that PRRT, rather than chemotherapy, may be a superior first-line therapeutic option in selected patients with a high level of SSTR expression and a Ki-67 index of less than or equal to 55%.”  Read more here.

This article will be fleshed out in due course. No locations listed yet.  Not recruiting yet.  Criteria listed – important section if this trial interests you. Clinical Trials document here.

In the meantime, read more about Lutathera (PRRT) by clicking here.

This looks like a very exciting development.

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!

Ronny Allan is an award winning patient leader and advocate for Neuroendocrine Cancer.


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Neuroendocrine Cancer: No one gets it until they get it


Over the years of my advocating, I’ve tried to explain Neuroendocrine Cancer to many people outside the community.  Some ‘get it’ but many don’t.  Most understand ‘Cancer’, they have real difficultly understanding ‘Neuroendocrine‘.  Despite how hard I try, I can see that some of them just don’t get it!

One of the challenges of explaining Neuroendocrine Cancer is the sheer complexity and spectrum of types. It’s a heterogeneous grouping of cancers ranging from some quite indolent versions through to very aggressive versions similar to many dangerous adenocarcinomas.  Unlike many of the more understood cancers, Neuroendocrine Cancer can literally appear anywhere in the body, adding to an already complex description, in addition to creating a disadvantage of awareness opportunities because of the use of incorrect cancer types, clearly many doctors and media organisations don’t ‘get it’ either!

Add in the symptoms caused by Neuroendocrine Tumours and their associated ‘Syndromes‘ and ‘Hormones‘, the external audience is now falling asleep or lost interest. Trying to explain why these diseases cannot be diagnosed earlier is also very complex.  “How can it be so difficult” many of them ask.

If you have managed to keep their interest and get onto the subject of living with the disease, it gets even more mind-blowing.  Non-stop surveillance, lifetime surveillance, permanent side effects of treatment. “No way” many of them remark.  The problem is that many people have a really simple outlook on cancer; something goes wrong, you get diagnosed, you get treated, you either die or live.  Simple isn’t it?

One group that normally ‘gets it’ is those who have currently got it, i.e. Neuroendocrine Cancer patients and their close families and supporters.    They may not ‘get it’ before someone is diagnosed and they may still not ‘get it’ once someone is diagnosed, but they eventually will ‘get it’. I have many people who ‘get it’ in my private group and on my main campaign sites.

Despite the difficulties, I’ll continue talking to those who have not yet ‘got it’ hoping to make them understand the disease.  I also intend to continue to help with the undiagnosed (some of these guys probably do ‘get it’ but just not yet formally ‘got it’).  I also want to help those at and beyond diagnosis who despite having it, don’t yet quite ‘get it’.

No one gets it until they get it. It shouldn’t be that way. 

Thanks for reading

Ronny

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Clinical Trial SPARTALIZUMAB – Immunotherapy for Neuroendocrine Neoplasms (PDR001)

THUMBNAIL_CarTcell.jpg

PDR001 (anti-PD-1) is an investigational immunotherapy being developed by Novartis to treat both solid tumors and lymphomas (cancers of the blood).  It is currently being trialled on many cancers including Neuroendocrine.  It’s brand name is SPARTLIZUMAB.

How PDR001 works

PDR001 is a type of immunotherapy, meaning that it acts by activating the body’s own immune system to recognize and fight cancer cells. Normally, an immune system cell called T-cells recognizes and kills infected or abnormal cells, including those that are cancerous. To prevent T-cells from accidentally damaging healthy and essential tissues, however several immune system checkpoints exist to inhibit, or block, them from going about this work. One example is the programmed cell death 1 (PD-1) pathway. Healthy cells produce and display a protein called programmed cell death ligand-1 or ligand-2 (PD-L1 or PD-L2) on their surface. These proteins bind to and activate a receptor called PD-1 that is produced by T-cells. When activated, PD-1 sends a message to the T-cells that prevents them from attacking that particular cell. Cancer cells can hijack this system by producing PD-L1 or PD-L2, effectively hiding from T-cells and evade destruction.
PDR001 is an antibody, a protein designed to interact with and block a specific target. It acts by binding to PD-1, blocking it from interacting with both PD-L1 and PD-L2. This binding blocks the PD-1-mediated inactivation of the T-cells, so that they are able to recognize and target cancer cells. This should result in a reduction in tumor growth and size.

PDR001 in clinical trials

PDR001 has been investigated in multiple completed and ongoing clinical trials, both alone and in combination with a wide range of other agents.

Novartis presented results from an ongoing first-in-human Phase 1/2 clinical trial (NCT02404441) of PDR001 at the American Society of Clinical Oncology (ASCO) meeting in 2016. Preliminary trial results suggested that the drug is well-tolerated and safe, with a similar profile to other anti-PD-1 drugs currently being developed. The trial is still recruiting patients with various types of advanced cancer at 43 sites across North America, Europe, and Asia; more information is available by clicking on its identification number.

Novartis then initiated several dozen other Phase 1, 2 and 3 trials, all registered on clinicaltrials.gov, to continue investigating the safety and anti-tumor activity of PDR001 in a wide range of cancer types, and in combination with other investigational and approved therapies. For example, a Phase 3 trial (NCT02967692) is comparing the safety and efficacy of PDR001 to a placebo, in combination with Tafinlar (dabrafenib) and Mekinist (trametinib), as a treatment for advanced melanoma.

What about Neuroendocrine?

A phase 2, multi-center study assessed the efficacy and safety of PDR001 in patients with non-functional well and poorly-differentiated Neuroendocrine Neoplasms.  According to the clinical trial document, the types of NENs covered are:

  • Well-differentiated Non-functional NET of Thoracic Origin
  • Well-differentiated Non-functional NET of Gastrointestinal Origin
  • Well-differentiated Non-functional NET of Pancreatic Origin
  • Poorly-differentiated Gastroenteropancreatic Neuroendocrine Carcinoma

The clinical trial indicates the trial is active but not recruiting but it would look like they have all the patients needed and are currently analysing the trial data so far awaiting the next phase perhaps.  In fact I have discovered two pieces of evidence from the trial sponsors:

pdr001 results conclusion
Annals of Oncology (2018) 29 (suppl_8): viii467-viii478. 10.1093/annonc/mdy293

In another analysis of the results:  “Patients with well-differentiated advanced NETs were eligible if they had progressed on prior therapy, including everolimus, while the GEP-NEC patients were eligible if they had progressed on one line of chemotherapy. All patients in the trial received spartalizumab via a 30-minute infusion once every 4 weeks until disease progression or unacceptable toxicity.

In the full well-differentiated cohort, there were 7 partial responses (7%), and 55% had stable disease, while 31% had progressive disease. The confirmed objective response rate was 7%, and the disease control rate was 63%. In the GEP-NEC cohort, the objective response rate was 5%, and the disease control rate was 19%.

The thoracic NETs patients fared best with spartalizumab, with limited responses seen in the pancreatic and GI NETs groups; responses seemed to be associated with PD-L1 expression. In the thoracic NETs cohort, two of five PD-L1–positive patients had a partial response. PD-L1 positivity was more common in the GEP-NEC cohort; among 14 PD-L1–positive patients in that group, the partial response rate was 43%.

The most common adverse events regardless of cause included abdominal and back pain, anemia, dyspnea, and hypertension.

Kjell Öberg, MD, PhD, of Uppsala University in Sweden, discussed the study for ESMO. “We have hope,” he said. “We see that maybe there are some tumor types that might respond to immunotherapy.” In general, NETs are considered an “immunological desert.” There is usually very low infiltration of immune cells in these tumors, and there are a low number of genetic mutation events.”

You can also listen to two very well known NET experts (Simron Singh and Jonathan Strosberg) talk about this trial and the drug ……. “the highest response rate was seen in atypical lung neuroendocrine tumors. It was approximately 20%, but in most cases was not durable”.  See the remainder of the discussion by clicking here.

Also watch Dr Lowell Anthony talking about this drug by clicking here.

You can read more about immunotherapy trials for Neuroendocrine Neoplasms by clicking here. This article includes some advice in interpreting the ‘hype’ that can surround immunotherapy which is still a developing approach to treating cancer.

Thanks for reading

Ronny

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Neuroendocrine Cancer – is normally slow growing BUT …..



I have a lot of be thankful for – I’m still here for starters!


BUT

……… here’s a list of 10 things I’m NOT thankful to Neuroendocrine Cancer for!

Thanks for growing inside me for years before making your vague announcement

Sorry too late, I’m metastatic and around 50% of patients will be at diagnosis (so I’m not alone!). It’s very SNEAKY!

No thanks for making a right mess inside my body!

I mean, I look really good, I look really well, but you should see my INSIDES

No thanks for generating fibrosis throughout my mesentery and retroperitoneum!

I really didn’t know what to make of this issue at diagnosis, although I did know the aorta was pretty important!  Fortunately I had a surgeon who had operated on many NET patients and has seen this issue before.  After my first surgery, he described it as a “dense fibrotic retroperitoneal reaction encircling his aorta and cava (inferior vena cava (IVC))”. My surgeon was known for difficult and extreme surgery, so as part of the removal of my primary, he also spent 3 hours dissecting out the retroperitoneal fibrosis surrounding these important blood vessels and managed 270 degree clearance. The remnant still shows on CT scans. Some of the removed tissue was tested and found to be benign, showing only florid inflammation and fibrosis (thankfully).  That said, the abstract papers above has led me to believe that my retroperitoneal fibrosis is clinically significant.  In fact I have spent the last 3 months worrying about some of it growing into reach of important vessels and only just been given the all clear (for now). 

fibrosis an unsolved mystery

No thanks for screwing up some of my hormones

There are many hormones involved with Neuroendocrine Cancer which is unique in that different types can result in elevated levels of different hormones, often more than one is involved.  Serotonin has caused fibrosis in my retroperitoneal area and is currently threatening important vessels. I don’t really need that right now!


No thanks for the ongoing symptoms and side effects

I was showing symptoms of a Neuroendocrine Cancer syndrome known as Carcinoid Syndrome (currently) such as flushing and diarrhea and fatigue was probably there too, but these were thought to be something else or ignored (by me).  I don’t suffer too much nowadays other than side effects of the disease or the treatment I’ve had or receiving.  However, I know from speaking to many patients the effects of the various syndromes associated with Neuroendocrine Cancer can be pretty debilitating and oppressive to quality of life.

These syndromes can be so strange and so weird, they can be very difficult for patients, nurses and doctors to treat. They can be a real ‘witch’s brew’.

Surveillance and treatment for life SUCKS!

But I need constant surveillance, it’ll keep me alive.

No thanks for the weight loss

As if I needed it

No thanks for the hypothyroidism

Another pill for life. I have a left-sided thyroid lesion and my treatment also messes with my hormone levels.

No thanks for increasing my diabetes risk

No thanks for pushing me into pre-diabetes. My blood sugar is spiking, most likely due to treatment.

No thanks for making me retire early

I loved my job but not if it was going to kill me. I made my own decision  based on how I could survive in a financial sense. Made easier as I was only 8 years from retirement but I guess I’m one of the lucky ones despite the fact I took a big hit on the income going into my bank account.

The truth is that many people still need to work whilst struggling with side effects of the cancer and its treatment. Getting some form of financial assistance from the government is not a done deal.

Neuroendocrine Cancer is a very expensive disease to treat.

This is fast becoming a big issue regardless of country and regardless of healthcare system in place. However, in privately funded healthcare, it can be exacerbated by the level of insurance cover. Read more about financial toxicity for cancer patients which is a growing problem worldwide.

……….. and no thanks to anyone who says it’s a “good cancer


Thanks for reading

Ronny

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Pancreatic Cancer vs Neuroendocrine Tumors of the Pancreas

pancreatic vs neuroendocrine

I campaign hard for Neuroendocrine Cancer awareness including continually pointing out that a Neuroendocrine Cancer with a pancreatic primary is NOT Pancreatic Cancer as is often quoted in the press.  The two main reasons I take up these campaigns are as follows:

1.  They are totally different cancers despite an anatomical relationship.  Although they share some similar presentation, they have different signs, different treatments and vastly different prognostic outcomes.  What that means is that anyone who is looking for useful information on either needs to be very careful on interpretation, they could end up with very bad advice and in some situations, become more concerned than they should be (particularly with the prognostics).  See more below. 

2.  These two different cancer types have different awareness organisations, patient support groups and patient leaders/advocates. In most cases, vastly different awareness messages. Both of these organisations and advocates need all the help they can get, they need all the resources and funding they can get. 

Both Pancreatic Cancer and Neuroendocrine Cancer are diseases that need maximum publicity, both types of cancer have their own unique situations, thus why the awareness messages can be so vastly different.  It’s really important, therefore, that publicity surrounding famous patients be attributed to the correct cancer type in order that the advocate organisations and supporters can gain maximum benefit to forward their causes.  Unfortunately, thanks to doctors and media, this very often doesn’t work out in favour of Neuroendocrine Cancer due to the Human Anatomy of Neuroendocrine Cancer (this problem actually goes beyond the pancreas).

Where the press and doctors regularly get it wrong

Two famous people in particular, one in 2011 and the other this year, are regularly reported in the press as having died of Pancreatic Cancer.

Steve Jobs.  One of the most famous technical innovators of his time and creator of the most valuable company in the world. He had a Neuroendocrine Cancer with a pancreatic primary.  Read his story here.

steve-jobs-55-to-2011

Aretha Franklin. One of the most famous soul singers of her time.  She had a Neuroendocrine Cancer with a pancreatic primary. Read her story here.

rip-Aretha-Franklin-1

To summarise, Neuroendocrine Cancer is not a “type” of another cancer.

What are the differences? 

For me, one of the two main differences are the cell type. When people talk about Pancreatic Cancer, they really mean something known as “Pancreatic Adenocarcinoma”.  It starts in the exocrine cells, which produce enzymes to support digestion.  Neuroendocrine Tumors start in the endocrine cells which produce hormones.

For me, the other big difference is prognostics.  Unfortunately, it is statistically proven that most people with Pancreatic Adenocarcinoma will die, whereas most people with Neuroendocrine Tumors with a pancreatic primary will live.

For a more detailed comparison, see this excellent article from NET Research Foundation.

iStock-536892277-768x891
diagram courtesy of NET Research Foundation

Pancreatic Cancer – Why I support their campaigns

Personally speaking, as a healthcare advocate online, I do support many cancer awareness campaigns, I think this is important to get similar help coming the other way (this frequently works for me).  However, I very much suspect, other than Neuroendocrine Cancer, my biggest support area online is for Pancreatic Cancer and other “less survivable” cancers.  I’m drawn by their excellent campaigns where they focus on key messages of prognostics for what is essentially a silent disease (in many ways the same issue with Neuroendocrine Cancer) and they make these more compelling by focusing on people rather than gimmicks. The prognostics can be upsetting reading as they are quite shocking figures which have not changed much in the past 40 years, a key sign that more must be done for this awful disease.   I frequently share this symptom graphic below because it might save a life and I ask that you do too.

pan can symptoms

Often though, the patients with a Neuroendocrine Cancer (pancreatic primary) are drawn to getting support from Pancreatic Cancer organisations. I suspect this is a combination of their own perceptions, their doctor’s language in describing their cancer type, and even something as simple as it was the first place they found help and support and they stick with that organisation.   You may also be interested in my article “I wish I had another cancer” – click here.

Thanks for reading

Ronny

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Don’t worry, it’s benign!


OPINION

One of the most controversial aspects of Neuroendocrine Tumours (NETs) is the ‘benign vs malignant’ question. It’s been widely debated and it frequently patrols the various patient forums and other social media platforms. It raises emotions and it triggers many responses ….. at least from those willing to engage in the conversation. At best, this issue can cause confusion, at worst, it might contradict what new patients have been told by their physicians (….or not been told). This post will not cover Neuroendocrine Carcinoma which by definition is malignant.

Any definition of the word ‘tumour’ will confirm it can either be benign or malignant. However, and while I’m sure there are benign NETs, the key statement to explain any slow growing or indolent NET is that they all have malignant potential – thus why surveillance and follow up is really important. This is the key factor in the changes found in the 2010 Digestive System World Health Organisation (WHO) classification system from the previous ‘flaky’ version. This reinforcement of the malignant potential of all NETs was duplicated in the recent 2017 Endocrine System equivalent, which is now proposed as a classification scheme for all NETs (see below).

“Carcinoid”

Of course we are not helped by the continued use of the term Carcinoid which decodes to ‘Cancer Like’ – that is potentially regressing the work of those specialists who are trying to undo the last 100 years of complacency in the medical world (and not really the type of awareness we need). The word is gradually being erased from NET nomenclature and the recent 2018 proposal by the International Agency for Research on Cancer (IARC) and WHO NET expert consensus panel to ditch it from the remaining versions of out of date WHO classifications (e.g. Pulmonary/Lung, Pituitary, Head & Neck, Genito-urinary, Adrenal and Paraganglia, Skin), is the final nail in the coffin for Carcinoid. RIP Carcinoid. This also supports our awareness issues with the media reporting the wrong cancer types based on anatomy of the primary tumour. Dear Doctors, Patient Advocates and Patients ….. please stop using the word!

I have lost count of the stories from Neuroendocrine Cancer patients who have been told their tumour was benign but then returned with incurable and metastatic cancer sometime downstream. Clearly there are doctors who do not understand NETs and/or are not aware of the changes in WHO classification schemes since 2010. Sure, some will prove to be ‘benign’ in nature and may not cause many issues but any Ki-67 below 3% is a formal grade of Neuroendocrine Neoplasm. I accept that it’s currently difficult to work out which cases will turn more aggressive and when, thus why surveillance and follow up are really important and also why patients should be seeing doctors who understand NETs. Worth also noting that many slow growing and indolent tumors can still often produce troublesome NET syndromes.

I’ve even heard one patient story where it was claimed a doctor called a metastatic NET case benign! Any definition of ‘benign’ on any respectable cancer site, will include the statement that they do not spread to other parts of the body. The NET Patient world is full of slow growing Grade 1 Stage 4 patients – by definition, they’re all malignant.

Read more detail in these articles as these issues are inextricably linked.

‘Benign vs Malignant’.
‘Carcinoid vs Neuroendocrine’
‘The Invisible NET Patient Population’
‘Staging and Grading’

I’m sure there are scenarios in all cancers where tumours can be benign and will never harm the person but if a Doctor says you have a Neuroendocrine Tumour and not to worry because it’s benign, ask questions.  Start with “how do you know it will never turn malignant” and “what will be done going forward to check”. 

Thanks for listening

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!


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Carcinoid vs Neuroendocrine

OPINION

CARCINOID misnomer etc

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:
carcinoid falling out of favor

Siegfried Oberndorfer
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.

carcinoid is inadequate oberg quote 2016

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.

carcinoid npf quote

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.

referring to carcinoid

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 video here.

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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