Translate
Subscribe to Blog via Email
Ronny Allan
This is an overview, if you require detail, try my more comprehensive post by clicking here or on the picture
Overview
We all know that Neuroendocrine Tumours (NETs) and their syndromes are complex but there is even more complexity to be found in a group of related disorders known as Multiple Endocrine Neoplasia (MEN). I recommend all NET patients should try to understand the basics of MEN and vice versa, particularly as both conditions seem to come with a plethora of endocrine-related effects.
MEN patients will normally have a tumour in at least two endocrine glands – thus the terms ‘Multiple’ and ‘Endocrine’ (tumours can also develop in other organs and tissues). Neoplasia is just another name for tumour and these can be non-cancerous (benign) or cancerous (malignant) with the potential to metastasize.
MEN syndromes can comprise varying combinations of tumours and many will be aware of the tumour risks from family knowledge. So putting the heredity aspects to one side, it’s potentially an extremely challenging surveillance and subsequent diagnostic scenario if (and when) these risks are realised. To add to the complexity, some of the associated tumours can be sporadic (non-hereditary) classic Neuroendocrine Tumours in various locations.
MEN Types
MEN is actually an umbrella term for a number of types (syndromes) of the disease – MEN1, MEN2a and 2b (2b was formerly MEN3). There’s a new kid on the block called MEN4 which is extremely rare.
In the most basic of terms regarding the relationship with tumours:
MEN1 seems to be centred on tumours of the parathyroid glands, the pituitary gland, and the pancreas (the 3 P’s). There can be other issues present including various skin issues and manifestations of foregut NETs, typically bronchial or thymic, and sometimes gastric.
MEN2a mainly focuses on medullary thyroid carcinoma, pheochromocytoma, parathyroid hyperplasia or adenomas (causing hyperparathyroidism), and occasionally cutaneous lichen amyloidosis or Hirschsprung disease (HSCR).
MEN2b is related to medullary thyroid carcinoma (MTC), pheochromocytoma, multiple mucosal neuromas and intestinal ganglioneuromas, and often a marfanoid habitus and other skeletal abnormalities.
MEN4 – A relatively new MEN variant and related to the CDKN1B gene, similar to MEN1 but normally only 2 of the 3 Ps, parathyroid and pituitary.
What is particularly distinctive with MEN is that they are inherited disorders. That means that they can be passed down in families, with each child of an affected parent having a 1 in 2 or 50% risk of inheritance. Consequently, genetic screening/testing is normally undertaken in established MEN families and those at risk of MEN.
Associated Types
You may also have heard of other NETs with a familial aspect, in particular Pheochromocytomas (adrenal gland tumours) and Paragangliomas (outside the adrenal gland), Not all are inherited, and I mention them because of the connection with MEN2a and MEN2b. Read more here – A spotlight on Pheochromocytoma and Paraganglioma
There are other less common connections with NET, mainly Von Hippel-Lindau syndrome (VHL), Neurofibromatosis type 1, Tuberous Sclerosis complex, and various Succinate dehydrogenase (SDHx) syndromes including Carney -Stratakis.
Further information
- AMEN Support
- A great video from Dr Mark Lewis who is an Oncologist and MEN patient. This is great as he speaks as both! Click here to watch
- For MEN patients, there is an excellent support group forum here – Join the AMEN Support patient support group.
I’m grateful to my friend and MEN patient Linda Hageman for supporting my blog activities and also for allowing me to join the AMEN support group to learn more. On this site, you will find Dr Mark Lewis, an Oncologist and MEN patient who supports Linda (who is a Nurse) with a ‘Ask the Doctor’ section on their website.
Further technical help on Pheochromocytomas (Pheo) and Paragangliomas (Para) can be found at the Pheo Para Alliance
Top 10 Posts & Pages in the last 48 hours (auto updates) (Click the titles to read them)
Thanks for reading.
Personal Facebook. Like this page please.
Blog Facebook. Like this page please.
Awareness Facebook Like this page please.
Sign up for my newsletters – Click Here
My Diagnosis and Treatment History
Check out my online presentations
Check out my WEGO Health Awards
Check out my Glossary of Terms – click here
Please Share this post for Neuroendocrine Cancer awareness and to help another patient
The trouble with the NET (Part 2) – Alternative Therapies – what’s the harm?
But it works, I read it on the internet! “But it works… I read it on the internet!” You may remember my article entitled The
‘Chinese Dumplings’ and Neuroendocrine Cancer
Update of one of my posts from 2015 which was interesting in that year. This is not really about a treatment which is available everywhere
“An odd disposition of tumours” – less common metastases in Small Intestine and Pancreatic NETs
Every day is a learning day in NET! When I was diagnosed in 2010, my Oncologist sent me for a specialist scan that would help
Chemotherapy for Neuroendocrine Cancer
Edited and checked April 2024One of the unusual aspects of Neuroendocrine Cancer is that chemotherapy is not normally considered as a ‘standard of care’ or
Carcinoid vs Neuroendocrine
OPINION – 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,
Things are not always how they seem
Graphic courtesy of SoBadAss.me (Sam Cleasby) In 2014, Chris and I walked 84 miles along Hadrian’s Wall on the English/Scottish border. It was a fantastic
Neuroendocrine Cancer Online Forums: frighteningly good or good at frightening?
OPINION When I was diagnosed, I was happy with my own research and kept away from forums on the advice of a fellow patient who
Colonoscopy Comedy
Last year I wrote a series of articles on the ‘coping’ side of cancer, one of which was about still being able to have a
Surgery – the gift that keeps on giving
I first wrote this article in December 2014. My thoughts went back to my first surgery in 2010 when I was discharged 4 weeks before
A cup of tea
I would also mention those who contributed to my “Tea Fund” which resides on PayPal. You don’t need a PayPal account as you can select a card but don’t forget to select the number of units first (i.e. 1 = £4, 2 = £8, 3 = £12, and so on), plus further on, tick a button to NOT create a PayPal account if you don’t need one. Clearly, if you have a PayPal account, the process is much simpler
Through your generosity, I am able to keep my sites running and provide various services for you. I have some ideas for 2023 but they are not detailed enough to make announcements yet.
This screenshot is from every single post on my website and depending on which machine you are using, it will either be top right of the post or at the bottom (my posts are often long, so scroll down!)
Related
Discover more from Ronny Allan - Living with Neuroendocrine Cancer
Subscribe to get the latest posts to your email.
Thank you so much for the focus on MEN syndromes. Every bit of awareness counts!! My family truly appreciates your dedication to NETs and your frank approach to serious and scary issues. I personally appreciate your humorous look at the touchy subjects.
what a lovely comment, thank you. I would like to use the section in my marketing ….. “My family truly appreciates your dedication to NETs and your frank approach to serious and scary issues. I personally appreciate your humorous look at the touchy subjects.” If that is OK with you? I can just say it came from Kelly or Kelly D or anonymise it, totally up to you.
Can you link to me please Saron?
I read a Pheo patients blog. It’s a nasty disease. Not a lot of treatment options for those either! SUCKS.