Don’t believe the hype – Neuroendocrine Cancer Myths debunked


Don't believe the hype - 10 myths

OPINION.

There’s a lot of inaccurate and out of date information out there.  Some of it is propaganda but most is a combination of misunderstanding and patient forum myth spreading …….

Myth 1:  All Neuroendocrine Tumours are benign

Not trueBy any scientific definition, the word ‘tumour’ means ‘an abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumours may be benign (not cancerous), or malignant (cancerous)’.  Sure, some NETs will be benign.  However, The World Health Organisation (WHO) 2010 classification for digestive system 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.  This has been reinforced in the 2017 update to include clarification for other endocrine organ types of NET including Pheochromocytoma. Read more here.  The word ‘Carcinoid’ is inextricably linked with this issue – read here why we need to stop using the term to help fight the benign myth.

Kunz His belief these tumors did not metastisize

Myth 2:  Neuroendocrine Tumours is a terminal condition

Not true.  By any definition of the word terminal in a medical diagnostic context, most NET patients have a good prognostic outlook, even those with metastatic and incurable variants of the disease. Read more here.

being_there_front
Graphic courtesy of Ellie McDowell

Myth 3: Carcinoid is another word for Neuroendocrine Tumours 

Not true.  Carcinoid is a very old term and was phased out years ago.  Carcinoid is not mentioned in the latest WHO Classification schemes for Neuroendocrine Neoplasms (a term covering Neuroendocrine Tumours and Neuroendocrine Carcinoma). Unfortunately, the problem is exacerbated by organisations and individuals who still use the word.  Also, those who use the following terms:

  • “Carcinoid Neuroendocrine”,
  • “Neuroendocrine Carcinoid”,
  • “Carcinoid and Neuroendocrine”,
  • “Neuroendocrine and Carcinoid”,
  • “Carcinoid NETs” or “CNET”

These are all contextually incorrect and misleading terms (not to mention the bad grammar). ENETS, NANETS and NCCN publications are gradually phasing the word out except in relation to Carcinoid Syndrome (and even then there could be easy solutions for this). Read more here and here.

carcinoid vs neuroendocrine

Myth 4:  All NET patients get ‘carcinoid syndrome’

Not true.  Firstly, many NET cancers are non-functional; and secondly, carcinoid syndrome is only one of a number of “NET Syndromes” associated with the various types of NET. However, the issue is further confused by those who use the word ‘Carcinoid‘ to incorrectly refer to all NETs and use Carcinoid Syndrome to refer to all NET Syndromes.  Read more here.

Early signs of a late diagnosis (2)

Myth 5:  Neuroendocrine Neolasms are rare

Not true.  As a collective grouping of cancers, this is no longer accurate. Read more here.  Also check out my post about the “Invisible NET Patient Population“.

Yao not rare

Myth 6:  Steve Jobs had Pancreatic Cancer

Not true.  Steve Jobs had a Neuroendocrine Tumour of the Pancreas.  Ditto for a few other famous names. Read more here.

steve jobs 2010
The last few years have reminded me that life is fragile

Myth 7:  I’m not getting chemotherapy, I must be doing OK?

Not true.  For some cancers or some sub-types of cancers, although it remains an option, chemotherapy is not particularly effective, e.g. some types of Neuroendocrine Cancer (NETs). In general, well differentiated NETs do not normally show a high degree of sensitivity to chemotherapy, although some primary locations fare better than others. However, many of the treatments for NET Cancer are somewhat harsh, have long-term consequences, and have no visible effects. NET patients are often said to “look well” but that doesn’t mean they are not struggling behind the scenes or under the surface.  Read more here.  P.S. Afinitor (Everolimus), Sutent (Sunitinib) are not chemo – Read more here.

chemotherapy-hand-and-arm

Myth 8:  All diarrhea is caused by carcinoid syndrome

Not true.  It could be one of the other syndromes or tumor types or a side effect of your treatment.  Check out this post.

NETCancer Diarrhea Jigsaw

Myth 9:  Neuroendocrine Tumours is a ‘good cancer’

Not true.  Simply, no cancer is good.  Some are statistically worse than others in prognostic terms, that’s true…… but living with NETs is very often not a walk in the park. However, no one cancer is better to get than any other – they’re all bad.  Read more here.

Good-Bad

Myth 10:  Every NET Patient was misdiagnosed for years

Not true.  Many NET Patients are correctly diagnosed early on in their investigation and in a reasonable time.  This myth is perpetuated because of two things: firstly, on forums, the ratio of long-term misdiagnosis is high creating a false perception; and secondly, the method of capturing patient surveys is not extensive enough – again creating a false perception.  In fact, the latest and largest database analysis from US indicates earlier diagnosis is improving, with more and more NETs being picked up at an early stage. Read more here.

if your doctors dont suspect something

Myth 11:  Somatostatin Analogues are a type of Chemotherapy

Not true.  Somatostatin Analogues (e.g. Octreotide and Lanreotide) are not chemotherapy, they are hormone inhibiting drugs.  They are more biotherapy. As the drugs latch onto somatostatin receptors, they are more targeted than systemic. For the record, Everolimus (Afinitor) and Sunitinib (Sutent) are not chemotherapy either. Read more here.

chemo-or-not-chemo

Myth 12:  Stuart Scott (ESPN) and Audrey Hepburn had Neuroendocrine Cancer. 

Not true. This is a common misunderstanding within the community.  They both had Pseudomyxoma Peritonei (PMP).  Read more about PMP here.

 

 

 

Myth 13:  I’ve been diagnosed with Neuroendocrine Tumours – my life is over

Not true.  Many patients live a very long time and lead fairly normal lives with the right treatment and support. It’s difficult but I try not to use ‘I can’t’ too much. Read more here.

I CAN

Myth 14:  There are only a handful of Neuroendocrine specialists in the world

Not true.  There are many specialists in many countries. Get links to specialists by clicking here.

10 questions to ask your doctor

Myth 15:  The Ga68 PET scan is replacing the CT and MRI scan in routine surveillance for all NET Patients

Not true.  It is actually replacing the Octreotide Scan for particular purposes,  or will eventually.  Read more by clicking here.

PET-CT-Scanner

Myth 16:  All NET Patients are Zebras

Not true.  They are in fact human beings and we should treat them as such. Please don’t call me a zebra and please don’t use the term on my social media sites, I refuse to perpetuate this outdated dogma.

hoofbeats

Myth 17: Multiple Endocrine Neoplasia (MEN) is a type of Neuroendocrine Tumour

Not true. Multiple Endocrine Neoplasia are syndromes and inherited disorders.  You can have MEN and not have any tumours.  However, these disorders can put people at more risk of developing Neuroendocrine or Endocrine Tumours. Read more here

genetics

Myth 18: Palliative Care means end of life or hospice care  

Not true. Palliative care is specialized medical care that focuses on providing patients relief from pain and other symptoms of a serious illness. A multidisciplinary care team aims to improve quality of life for people who have serious or life-threatening illnesses, no matter the diagnosis or stage of disease. Read more here

The P word

Myth 19: Serotonin is found in foods

Not true. Serotonin is manufactured in the body. Read more here

brain-neurotransmitter-serotonin

Myth 20: NETs cannot be cured

Not true. If caught early enough, some NETs can be treated with curative intent (totally resected) with little or no further follow up.  It says this in ENETS and NANETS publications which are authored by our top specialists. If we can’t believe them, who can we believe? Read more here.

cure quote

Myth 21: Pancreatic Enzyme Replacement Therapy (Creon etc) is only for pancreatic patients

Not true. It’s for any patient who is exhibiting exocrine pancreatic insufficiency. Read more here.

PERT

More to follow no doubt

For general cancer myths and the dangers of fake health news, please see my ARTICLE 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!

patients included

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‘Chinese Dumplings’ and Neuroendocrine Cancer

Chinese Dumpings
Chinese dumplings

One of my daily alerts brought up this very interesting article published in the Journal of Gastrointestinal Oncology last month (June 2015).  I personally found it fascinating. Moreover, it gave me some hope that specialists are out there looking for novel treatments to help with the difficult fight against Neuroendocrine Cancer.

This is an article about something generally described as “Intra-operative Chemotherapy”, i.e. the administration of chemo during surgery.  This isn’t any old article – this is written by someone who is very well-known in Neuroendocrine Cancer circles – Dr. Yi-Zarn Wang.  

The general idea behind this isn’t exactly new as there’s also a procedure known as HIPEC (Hyperthermic Intraperitoneal Chemotherapy) or “chemo bath”.  This is mostly used intra-operatively for people with advanced appendiceal cancers such as Pseudomyxoma Peritonei (PMP). It normally follows extreme surgery – you can read more about this in a blog I wrote at the beginning of the year entitled “The Mother of all Surgeries”.

However, this is both different and significant because it is targeted at midgut neuroendocrine tumour (NET) patients who are often diagnosed at an advanced stage with extensive mesenteric lymph node and liver metastasis. Despite extensive surgery which needs to be both aggressive and delicate, there can sometimes be small specks left behind which will not show up on any type scan, particularly in the mesentery area.  It is possible these specks could eventually grow big enough to cause fresh metastasis or syndrome recurrence/worsening and then need further invasive treatment.

The treatment aims to eliminate potential tumour residuals in mesenteric lymph node dissection beds using a safe and local application of chemotherapy agent 5-fluorouracil (5-FU). The 5-FU is delivered via ‘intraoperative application’ of 5-FU saturated gelfoam strips secured into the mesenteric defect following the extensive lymphadenectomy. The term ‘Chinese dumpings’ is used to describe the 5-FU saturated gelfoam strips once they are in place in the treatment site.  I understand from other research that they can also be used in liver surgery (anecdotal from a forum site).

The report concluded that those who were treated with the intra-operative 5-FU received less follow-up surgery than those who were not (the control group). However, it added that further studies were required to evaluate its effect on long-term survival.

So…. this form of intra-operative treatment is very interesting. Incidentally there is already a form of intra-operative treatment using radiotherapy (IORT) which is a similar concept but essentially still in its infancy. However, the first IORT machine of its kind in the UK was deployed in Jun 2016.  I blogged about this here.

You can read the report in full here:

Adjuvant intraoperative post-dissectional tumor bed chemotherapy—A novel approach in treating midgut neuroendocrine tumors

p.s. If you get time, the introduction section of this article is a very powerful explanation of the problems and challenges faced by surgeons when presented with extensive abdominal neuroendocrine disease.

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The Mother of all Surgeries

surgery
Surgery

My plan for this week’s blog was to continue with a surgery theme using the story of a lady who had what was described as the “Mother of all Surgeries” after being late diagnosed with a very rare and advanced type of appendiceal cancer. With NETs, surgery is a topical subject as not everyone will be able to have it and some might not even need it. Check out my blog to cut or not to cut.

I suggested in a previous blog that Surgery is a gift that keeps on giving and that is probably true for many cancer survivors. However, I then added that NETs were one of a small number of tumours for which surgical debulking can provide some survival advantage for those with metastatic and incurable disease. In my own case, I’ve had my primary removed as part of a small intestine resection and a right hemicolectomy ‘en bloc’ – plus a bunch of mesenteric and retroperitoneal ‘stuff’. Other metastases were removed at a later date including a 66% liver resection (which included an opportunistic removal of gall bladder for treatment side effect control), 9 lymph nodes removed from my left axillary area (armpit), 5 lymph nodes removed left clavicle area and two hotspots are still under investigation.  I’m now under constant surveillance but I’m doing OK …… and I intend to outlive my Oncologist 🙂

However, my experience would appear to be fairly straightforward when you consider the types of surgery some Neuroendocrine Patients with a Pancreatic primary (pNET) have to undergo. These include the ‘Whipples’, ‘Distal Pancreatectomy or ‘Total Pancreatectomy’.  These are all long and complex operations which remove numerous organs or parts of organs – and no doubt there are variants of these for unusual scenarios.

Although there are long term side effects from my surgery, I’m gradually adjusting my lifestyle to cope (a work in progress, even after 8 years!).  This is mainly through (not too drastic) diet adjustments and nutritional level surveillance.  My other weapon is to learn as much as I can about my disease in order that I can learn how best to live with the side effects of both the cancer and the treatment. Despite the extent of my surgery (to date) and my cancer’s stage, I still consider myself fortunate to have been diagnosed earlier than the average for this disease (….sheer luck) and then had access to what some might describe as aggressive surgery shortly after.  I suspect 7 years of ‘Lanreotide’ is also playing a big part.

My experience has given me the incentive to read a lot and occasionally I come across articles about cancer patients who have been treated surgically for very unusual cancers – even less common than NET Cancer.  One which recently caught my eye was a lady who had been diagnosed with Pseudomyxoma Peritonei (PMP).  This is a very rare condition (1 in every 1,000,000) characterised by the presence of mucin-producing tumours (and the mucin) in the abdominal cavity. There are a number of possible origins of PMP, but the most common source is appendiceal (appendix) cancer.  If you’re interested in learning more, further information can be found here.

When I was drafting this blog a couple of years ago, I had been carefully watching the story unfold about the death of respected ESPN anchor Stuart Scott from an “appendiceal cancer”.

RIP Stuart Scott
RIP Stuart Scott

The media presented a confusing picture about the exact type of cancer Stuart had and some of you will have seen the debate on social media. It’s amazing how quickly and ‘assumption’ can circulate to thousands of people in a very short space of time.  I read conflicting mainsteam media articles and social media posts; some referred to the disease as ‘gastrointestinal carcinoid’ and others insisted that it was unrelated to NETs. As the dust settled, it appears his appendiceal cancer was not a Neuroendocrine Tumor.

On the same subject, many NET patients claim Audrey Hepburn was a NET Patient but this is not accurate, she had PMP.

Back to the Mother of all Surgeries story ………

I was totally amazed by the extent of the surgery the lady had to undergo and wondered how she was going to cope with the side effects.  She was clearly an extreme case and many patients are caught early on and treated accordingly. They removed her appendix, large bowel, most of her small bowel, gall bladder, spleen, womb, ovaries, fallopian tubes and cervix as well as most of her stomach lining and navel.  Her surgery was described as the “Mother of all Surgeries” and included a 90 minute ‘chemo bath’ (formally known as Hyperthermic Intraperitoneal Chemotherapy (HIPEC)). Interestingly she was originally diagnosed with IBS, a similar scenario to many NET patients. I was also struck by the similarities of some of the symptoms with those found in NET Cancer.

The UK is very well equipped to deal with PMP and HIPEC with two centres in Basingstoke and Manchester renowned worldwide as treatment centres for this rare condition.

You can read more here about this amazing story where the patient had 9 organs removed.

And another here –  “9 organs removed”

And another here – “tumour weighing one stone”

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

Disclaimer

My Diagnosis and Treatment History

Most Popular Posts

Sign up for my twitter newsletter

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

patients included