Home » 2016 » December

Monthly Archives: December 2016

Blog Stats

  • 271,199 hits

Enter your email address to follow this blog and receive notifications of new posts by email.

Join 8,693 other followers

Recent Posts

Lanreotide vs Octreotide


somatuline-depot-injection-vs-sandostatin

long acting Lanreotide (left) – long acting Octreotide (right)

Somatostatin Analogues are the ‘workhorse’ treatments for those living with NETs, particularly where syndromes are involved. Although it can sometimes seem like they are only associated with serotonin releasing tumours (i.e. what might be described as Carcinoid), these types of drugs can be used to help with other NET types including Pancreatic NETs, or pNETs for short. For example, the drugs can be used for treating insulinoma, gastrinoma, glucagonoma and VIPoma (all types of pNETs). They are most effective if the NETs express somatostatin receptors.

Somatostatin is actually a naturally occurring hormone produced by the hypothalamus and some other tissues such as the pancreas and the gastrointestinal tract. However, it can only handle the normal release of hormones.  When NET syndromes occur, the naturally occurring somatostatin is unable to cope. The word ‘analogue’ in the simplest of terms, means ‘manufactured’ and a somatostatin analogue is made to be able to cope with the excess secretion (in most cases).

Although there is hidden complexity, the concept of the drug is fairly simple.  It can inhibit insulin, glucagon, serotonin, VIP, it can slow down bowel motility and increase absorption of fluid from the gut. It also has an inhibitory effect on growth hormone release from the pituitary gland (thus why it’s also used to treat a condition called Acromegaly). You can see why it’s a good treatment for those with NET syndromes, i.e. who suffer from the excess secretions of hormones from their NETs.  Clearly there can be side effects as it also inhibits digestive enzymes which can contribute to, or exacerbate, gastro-intestinal malabsorption.

Please note somatostatin analogues are not chemo.  There are two major types in use:

  • Octreotide – or its brand name Sandostatin.  It is suffixed by LAR for the ‘long acting release’ version.
  • Lanreotide – brand name Somatuline (suffixed by ‘Depot’ in North America, ‘Autogel’ elsewhere)

A frequently asked question on forums is “what is the difference between Octreotide and Lanreotide?”

  • They are made by two different companies.  Novartis manufactures Octreotide and Ipsen manufactures Lanreotide.  Octreotide has been around for much longer.
  • The long-acting versions are made and absorbed very differently.  Octreotide has a complex polymer and must be injected in the muscle to absorb properly.  Lanreotide instead uses has a novel nanotube structure and is water based (click here to see a video of how this works). It is injected deep-subcutaneously and is therefore easier to absorb and is not greatly impacted if accidentally injected into muscle.
  • Their delivery systems are mainly via injections but are fundamentally different as you can see from the blog graphic which shows the differences between the long acting release versions.  Octreotide long acting requires a pre-mix, whilst Lanreotide comes pre-filled.
  • The long-acting versions are 60, 90 and 120 mg for Lanreotide and 10, 20 and 30 mg for Octreotide.
  • Octreotide also has a daily version which is administered subcutaneously.
  • Octreotide has something called a ‘rescue shot’ which is essentially a top up to tackle breakthrough symptoms.  It is a subcutaneous injection.
  • You can also ‘pump’ Octreotide using a switched on/off continuous infusion subcutaneously.
  • To the best of my knowledge, there is no daily injection, rescue shot or ‘pump’ for Lanreotide.
  • Whilst both have anti-tumour effects, there are slight differences in US FDA approval: Octreotide (Sandostatin) is approved for symptom control whereas Lanreotide (Somatuline) is approved for tumour control.

Here are some interesting videos showing and explaining their administration:

Administering a Somatuline Depot (Lanreotide) injection:

Administering a Sandostatin LAR (Octreotide) injection:

This link also provides guidance on the “new formulation” Octreotide.  Click here.

My own experience only includes daily injections of Octreotide (Sep-Nov 2010) and Lanreotide (Dec 2010 onwards).  I’ve also had continuous infusion of Octreotide in preparation for surgical or invasive procedures over the period 2010-2012 (i.e. crisis prevention).  You can read about my Lanreotide experience by clicking here.  If you are interested in what might be coming downstream, please see my blog entitled ‘Somatostatin Analogues and Delivery Systems in the Pipeline’.

Thanks for listening

Ronny

Hey Guys, I’m also active on Facebook which comprises the bulk of my award-winning community.  Like my page for even more news.

Disclaimer

My Diagnosis and Treatment History

Most Popular Posts

Keep your light burning


candle_candle_light_4013

I recently met a colleague who I hadn’t seen for 30 years. He was more than just a colleague, he was once my ‘Commanding Officer’. He had been made aware of my illness but after asking how I was, he was content with my short explanation “I’m not dead yet“. The great thing about soldiery is that you can pick up where you left off 30 years ago as if it was only yesterday and ‘bravado’ is not only allowed, it’s expected! A week later, I received a very nice Christmas card with a message which included “…… the old light is still burning brightly“.  It was a metaphor but something I needed to hear.

Neuroendocrine Cancer can damage or take our body parts, cause us pain and discomfort, disrupt our lives through constant treatment and surveillance, giving us much uncertainty and anxiety in the process. It will most definitely try to kill us. Despite that, we must keep our lights burning as bright as we can.  The flame of hope never goes out.

Ronny

https://www.facebook.com/NETCancerBlog/

 

 

 

Neuroendocrine Tumours: a spotlight on Pheochromocytomas and Paragangliomas


spotlight-on-pheoI spend a lot of time talking about the most common forms of Neuroendocrine Tumours (NETs), but what about the less well-known types?  As part of my commitment to all types of NETs, I’d like to shine a light on two less common tumour types known as Pheochromocytomas and Paragangliomas – incidence rate approximately 8 per million per year. They are normally grouped together and the definitions below will confirm why.

So, let’s get definitions out of the way:

Pheochromocytomas (Pheo for short)

Pheochromocytomas are tumours of the adrenal gland that produce excess adrenaline. They arise from the central portion of the adrenal gland, which is called the adrenal medulla (the remainder of the gland is known as the cortex which performs a different role and can be associated with a different tumour type). The adrenal medulla is responsible for the normal production of adrenaline, which our body requires to help maintain blood pressure and to help cope with stressful situations.  The adrenal glands are situated on top of the kidneys (i.e. there are two).  Adrenaline is also called ‘epinephrine’ which is curiously one of the 5 E’s of Carcinoid Syndrome.

Paragangliomas

Paragangliomas are tumours that grow in cells of the ‘peripheral’ nervous system (i.e. the nerves outside the brain and spinal cord). Like Pheochromocytomas, they can release excess adrenaline.  There can be confusion between the two types of tumour as Paragangliomas are often described as extra-adrenal Pheochromocytomas (i.e. a Pheo external to the adrenal gland).

Going forward, I’m going to talk about both using the single term of ‘Pheochromocytoma’ in the context of an adrenaline secreting tumour but may refer to Paraganglioma where there might be a difference other than anatomical location.

“The 10% Tumour”

Pheochromocytomas are often referred to as the “ten percent tumour” because they do many things about ten percent of the time. The following is a fairly exhaustive list of these characteristics:

10 percent of all Pheochromocytomas are:

  • Malignant (i.e. 90% are benign).  However, a recent document issued by the World Health Organisation (WHO) stated that “Paragangliomas remain tumors of undetermined biologic potential and should not be termed benign”.
  • Bilateral (i.e. found in both adrenal glands: 90% arise in just one of the two adrenal glands)
  • Extra-Adrenal (found within nervous tissue outside of the adrenal glands … i.e. 10% are Paragangliomas)
  • In Children (90% are in adults)
  • Familial (10% will have a family member with the same type of tumour) – (however ……my research indicates there are wide-ranging figures published of up to 50% involvement with Multiple Endocrine Neoplasia (MEN),
  • Recur (10% or slightly less, will come back 5 to 10 years later)
  • Present with a stroke (10% of these tumours are found after the patient has a stroke)

Symptoms

The classic symptoms of Pheochromocytomas are those attributable to excess adrenaline production. Often these patients will have recurring episodes of sweating, headache, and a feeling of high anxiety.

  • Headaches (severe)
  • Excess sweating (generalized)
  • Racing heart (tachycardia and palpitations)
  • Anxiety and nervousness
  • Hypertension
  • Nervous shaking (tremors)
  • Pain in the lower chest or upper abdomen
  • Nausea (with or without vomiting)
  • Weight loss
  • Heat intolerance

Diagnosing Pheochromocytomas

According to the ISI Book on NETs (Woltering, Vinik, O’Dorisio, et al), Pheochromocytomas present with a classic triad of symptoms and signs:  headache, palpitations and sweating.  This symptom complex has a high specificity and sensitivity (>90%) for the diagnosis of Pheochromocytomas.  The figure is much lower in individual symptom presentations (palpitations 50%, sweating 30%, headaches 20%). In addition to correctly diagnosing from these symptoms, Pheochromocytomas may also be found incidentally during a surgical procedure even after a diagnosis of an ‘adrenal incidentaloma’

Markers.  Like serotonin secreting tumours, adrenal secreting tumours convert the offending hormone into something which comes out in urine. In fact, this is measured by 24 hour urine test very similar to 5HIAA (with its own diet and drug restrictions).  It’s known as 24-hour urinary catacholamines and metanephrines. This test is designed to measure production of the different types of adrenaline compounds that the adrenal glands make. Since the body gets rid of these hormones in the urine, we simply collect a patient’s urine for 24 hours to determine if they are over-produced.  Like 5HIAA, there is also a plasma (blood draw) version of the test.  According to the ISI Book on NETs, there is also an additional test called ‘Vanillylmandelic Acid (VMA).  This reference also indicates the most sensitive test is plasma free total metanephrines.

Scans.  Other than the usual range of scanners, ultrasound, CT/MRI, all of which may be used to find evidence of something, the other scan of note is called MIBG.  This is a nuclear scan similar in concept to the Octreotide Scan given to many NET patients (in fact some Pheo patients my get an Octreotide scan if they have somatostatin receptors).  The key differences with MIBG is the liquid radioactive material mix which is called iodine-123-meta-iodobenzylguanidine or 131-meta-iodobenzylguanidine  (this is where the acronym MIBG originates).  Together with the markers above, the results will drive treatment.  Depending on availability, the latest PET scans may also be available potentially offering greater detail and accuracy i.e. 18F-FDOPA, 18F-FDG and Ga68.

This statement and diagram was provided by Dr Mark Lewis who is an Oncologist and MEN patient.  “The algorithm for working up a hyperadrenergic state is attached (and was developed by Dr. Young at Mayo Clinic). It outlines the most reliable testing for a pheo or Paraganglioma”

work-up-for-diagnosing-pheo

Additional Factors and Considerations

  1. This is an awareness post so I’m not covering treatment options in any detail except to say that surgery if often used to remove as much tumour as possible.   Somatostatin Analogues may also be used in certain scenarios in addition to other hormone suppression or symptom controlling drugs.
  2. The adrenal cortex mentioned above is actually the site for Adrenocortical Carcinoma (ACC) – this is a totally different cancer (think Pancreatic Cancer and Neuroendocrine Tumour of the Pancreas).
  3. Pheochromocytomas are probably difficult to diagnose (you only have to look at the symptoms to see that).  The differential diagnoses (i.e. potential misdiagnoses) are: hyperthyroidism, hypoglycaemia, mastocytosis, carcinoid syndrome, menopause, heart failure, arrhythmias, migraine, epilepsy, porphyria lead poisoning, panic attacks and fictitious disorders such as the use of cocaine and benzedrine.
  4. Many Pheochromocytoma patients will also be affected by Multiple Endocrine Neoplasia (MEN), in particular MEN2 (there are some wide-ranging percentage figures online for this aspect).  There can also be an association with Von Hippel-Lindau (VHL) syndrome and less commonly with Neurofibromatosis type 1.
  5. Given the nature of the hormones involved with Pheochromocytomas, there is a risk of intraoperative hypertensive crises. This is similar in some ways to Carcinoid Crisis but needs careful consideration by those involved in any invasive procedure.

Summary

Pheochromocytomas are very complex involving many of the challenges found in the more abundant and common types of NETs.  This is an extremely basic overview offered as an awareness message about the lesser known types of NETs.  I refer you to my disclaimer.  If you wish to learn more about Pheochromocytomas and Paragangliomas, check out the links below.

Thanks for listening

Ronny

Hey Guys, I’m also active on Facebook which comprises the bulk of my award-winning community.  Like my page for even more news.

Disclaimer

My Diagnosis and Treatment History

Most Popular Posts

Research References used in this post:

ISI – Neuroendocrine Tumors 2016

http://www.amensupport.org/

http://www.pheosupportfoundation.org/

http://www.pheochromocytoma.org/

http://www.pheoparatroopers.org/

https://pheoparaproject.org/

http://endocrinediseases.org/

https://www.endocrineweb.com/

Various authoritative Neuroendocrine and Endocrine Sites.

Also ……why not take a look at Pheo vs Fabulous, a blogger who is a malignant Pheo patient – https://pheovsfabulous.wordpress.com

 

Drum Roll – Ronny Allan wins WEGO Best in Show ‘Community’ 2016


wego-community-win

community_titled_transparent_2013-10-22

Very happy to win the WEGO 2016 Best in Show Community which is some ways is a recognition for my blog based on the fact is at the core of what I do and in many ways, the other apps are (currently) just ‘fronts’ for this output.  Whether you read my blog direct from WordPress, Facebook, Twitter, Pinterest or any other platform you find it, you are all members of this award-winning community!

My WEGO Profile is here – look out for the updates!  I’ll expand this blog once the dust settles as this award opens up new avenues for the Neuroendocrine Cancer (NETs) and I’ll be involved in new and exciting activities.

Many thanks for everything you’ve done!

Ronny

Thanks for listening

Ronny

Hey Guys, I’m also active on Facebook which comprises the bulk of my award-winning community.  Like my page for even more news.

Disclaimer

My Diagnosis and Treatment History

Most Popular Posts

 

 

Neuroendocrine Tumours – Let’s give Carcinoid Crisis a red card!


at-risk-syndrome0001

an example card from NET Patient Foundation

 

at risk syndrome back0002

Example dosage shown on the rear of the NET Patient Foundation card

 

The word ‘crisis’ has a wide range of meanings and it’s well used in the media to catch the reader’s attention. Lately, the terms ‘political crisis’, financial ‘crisis’ and ‘constitutional crisis’ appear almost daily in media headlines. In a previous life, the term ‘crisis management’ was used daily in the work I was undertaking as I went from problem to problem, dampening or putting out fires (….. that’s a metaphor!).  Thinking back, my adrenaline (epinephrine), norepinephrine, and cortisol must have been very busy! 

However, in the world of Neuroendocrine Tumours (NETs), ‘crisis’ has a very significant meaning and its very mention will make ears prick up.  The word ‘crisis’ is normally spoken or written using the term ‘Carcinoid Crisis’ given this is the type of NET with which it has been mostly associated. However, I’ve studied and researched and it would appear that some form of ‘crisis’ might apply to other types of NETs. Perhaps this is another knock-on effect caused by the historical use of the word ‘Carcinoid’ to incorrectly refer to all NETs. In terms of ‘crisis’, maybe there should be a more generic NETs wide term?  More on that later.

What is ‘Carcinoid Crisis’?

In the simplest of terms, it is a dangerous change in blood pressure, heart rate, and breathing (technical term – cardiopulmonary hemodynamic instability).  On an operating table under anaesthetics or an invasive procedure such as liver embolization, this can actually be life threatening.  Incidentally, this happens with many other types of conditions and it is the cause of the ‘cardiopulmonary hemodynamic instability’ that is different with NET patients. For some, it could be a life or death situation

Why does it happen to some NET Patients?

NETs can release a variety of ‘vasoactive peptides’ (hormones) in excess (e.g. serotonin, catecholamines, histamine).  Under normal circumstances, these would just present as routine syndromes which may need to be controlled in most cases with somatostatin analogue treatment (Octreotide/Lanreotide).

Excess amounts of these vasoactive substances can cause both hypertension and hypotension (high and low blood pressure respectively). In extreme cases this can lead to what is known as a (carcinoid) crisis situation.  It is said by one very well-known NET expert to “not to be something which happens randomly to all patients, it is usually linked to a medical procedure of some sort when you are having anaesthesia”.  Dr Eric Liu also said “Luckily it is relatively uncommon”.

How is the risk managed?

If you research this plus perhaps from your own experience, you will know there are different ideas and ‘protocols’.  However, they all mostly involve some pre-procedure infusion of a somatostatin analogue (normally Octreotide) – although I’d love to hear from anyone who has had Lanreotide as an alternative.  Some doctors or hospitals are known to have their own ‘protocols’ and I’ve uploaded the one from the ISI NET book page 215 (Wang, Boudreaux, O’Dorisio, Vinik, Woltering, et al). Click here.  Please note this is an example rather than a recommendation as this is something the NOLA team have developed for their own centre.  In all the big procedures I’ve had done in my local NET Centre, I have always been admitted the day before to receive what they describe as an ‘Octreotide Soak’.

Patients are always asking about the risk and requirements for smaller procedures such as an Endoscopy.  There does not seem to common guidance on this but Dr Woltering who is always forthcoming with advice suggests 200 micrograms of Octreotide before the procedure commences.

Dental visits involving anaesthetics can also be an issue and you can see Dr Woltering’s advice in my blog about the 5 Es of Carcinoid Syndrome.  Additionally there is advice for users of ‘Epi Pens’. You also need to derisk those situations.

Carcinoid Syndrome vs Carcinoid Crisis

I have seen some discussion about the difference between a severe attack of carcinoid syndrome and carcinoid crisis and it’s a really difficult area.  Looking at Dr Liu’s definition, he said it was ‘usually’ linked to a procedure based scenario so I guess it could happen in a non-surgical scenario in extreme cases.  Most people are effectively managed on monthly injections of Octreotide/Lanreotide but some people still need ‘rescue shots’ (top ups) where they are experiencing breakthrough symptoms.  When I was symptomatic (syndromic), I would regularly flush in stressful situations but that was definitely syndrome rather than crisis. Check out my video explaining how I felt.  It’s worth reading something called the 5 E’s of Carcinoid Syndrome, probably useful to other types of NETs as I’m sure there is some overlap.

What about other types of NETs

The ISI Book Link above (here for convenience), does state “regardless of tumor type, all NETs should be pre-treated with Octreotide for protection against crisis“.  I know that NET patients other than those with ‘Carcinoid Tumours’ are also treated with somatostatin analogues, as they too can be subject to the effects of excess secretion of certain vasoactive peptides. I recently read an article about a person with a Pheochromocytoma (a less common NET that comes from the chromaffin cells of the adrenal medulla and secretes catecholamines).  The person had what was described as an ‘Intraoperative Hypertensive Crisis’ that appeared to be caused by her tumour type rather than the sort of incident that might occur in a standard surgery.  Hypertension (high blood pressure) can be a symptom of Pheochromocytoma so you can see the problem with surgery and other procedures. An interesting issue with this type of NET is that after surgery, the patient is at risk for hypotension (low blood pressure) from venous dilation caused by the sudden withdrawal of catecholamines.

Summary

I highly suspect there are many examples from the NET world beyond the ‘carcinoid’ subtype of NETs and I’ve already given you one above.  I’ll update this blog as I discover other examples.  In the meantime, make sure you ask your medical team about ‘crisis protection’ if you are to undergo any surgical or invasive medical procedure.

Do we need to rename the term ‘Carcinoid Crisis’?  Probably …… let’s give it a red card!

Thanks for listening

Ronny

Hey Guys, I’m also active on Facebook.  Like my page for even more news.

Disclaimer

My Diagnosis and Treatment History

Most Popular Posts

community_titled_transparent_2013-10-22

NETwork with Ronny © – Newsletter November 2016


 

network-with-ronny

Please share me!

 

Hi,

welcome to my first newsletter, a pilot for a monthly summary of NET news, views and ICYMI (in case you missed it!).

What a month November has been – we had NET Cancer Day build up and I’ve been working hard to put on a good show for the 2016 WEGO Health Activist Awards (results expected around 6/7 Dec) whilst at the same time maintain my other campaigning activity across a wide range of social media platforms.  Due to increased activity, I recorded the second highest monthly viewing figures ever – over 13,000 hits on my blog site in one month (and even more on Facebook).  Not bad for a little backstreet disease – but my intention is to take it to the high street (main street).  Stick with me because I really need your help and support and anyone else you know who can assist.

Blogging

I seem to have produced an above average amount of blogs this month. Due to the vagaries of Facebook inner workings, some of these may not have even shown on your timeline.  So, ICYMI …….here’s a summary with links:

Other News in Nov

New Audiences for NET Cancer.  From Day 1, I said it was my aim to find new audiences for NETS rather than just share stuff within our own community.  Two new openings in Nov to report:

  • Cancer Knowledge Network.  An article published in what is said to be North America’s most widely read cancer education resource.  Read it by clicking here.
  • Mentioned by Macmillan Cancer, one of the world’s biggest Cancer support organisations.  See here.  You could increase the impact of this opening by clicking on the black star here to increase my likes number.
  • And ….. there has been masses of exposure during the build up to the 2016 WEGO Health Activist Awards where I made the final in two categories:  Blog and Community.

Lutathera (PRRT) Delay. Not the best news from USA where it was announced the approval of Lutathera (PRRT) would be delayed beyond the projected date of 28 Dec 16. A delay of several months seems to be forming on the airwaves but the expanded access program (EAP) will be extended until this issue has been resolved.  CCF has great info on EAP – click here

Living with NETs Website. A new website sponsored by Ipsen (Lanreotide manufacturers) was launched on NET Cancer Day.  This is a special site for me as I was involved with other patients in advising and featuring on the site.  Take a look by clicking here.  Check out my patient video here.

More treatment options for Lung NETs?  Lung NETs lack many of the treatments available for other types of NET but a new trial of Lanreotide could help.  Read here.

Figures

For interest the 10 Ten Facebook followers by Country.

capture

 

Thanks for your great support in November.  Looking forward to serving you in December!

Ronny

Hey Guys, I’m also active on Facebook.  Like my page for even more news.

Disclaimer

My Diagnosis and Treatment History

Most Popular Posts

%d bloggers like this: