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
Subscribe to Blog via Email
Top 10 Posts & Pages in the last 48 hours (auto updates) (Click the titles to read them)
Thanks for reading.
I’m also active on Facebook. Like this page.
I’m also active on this Facebook page. Follow this page.
Also like this awareness page on Facebook.
Sign up for my newsletters – Click Here
My Diagnosis and Treatment History
Check out my online presentations
Check out my WEGO Health Awards
Like my new awareness page – click here or on the photo. (Like rather than follow please!)
Check out my Glossary of Terms – click here
Please Share this post for Neuroendocrine Cancer awareness and to help another patient

Summary of May 2023 on RonnyAllan.NET
Summary May produced the best statistics in 2023, mainly due to your support for my blog post on Maria Menounos whose announcement headlined as “Pancreatic

Getting back in the saddle
Those who follow my Facebook page Ronny Allan may remember my cycling faux pas in April causing me to bruise or fracture a rib (here

Clinical Trial: Phase 1/2a Study of 23ME-00610 in Patients With Advanced Solid Malignancies (incl Neuroendocrine Neoplasms)
Who are 23andMe? I personally had not heard of 23andMe but many people in North America might have. When you first look at what they

A spotlight on Chromogranin A
What is Chromogranin A? Chromogranin A (CgA) is an acidic protein released along with catecholamines from chromaffin cells and nerve terminals. This statement alone might

Neuroendocrine Cancer: Catch them early, not late!
Diagnosing Neuroendocrine Neoplasms (NENs). It’s no secret that Neuroendocrine Neoplasms (NENs) can be difficult to diagnose, particularly well differentiated slow growing types (NETs) which can

A spotlight on Rectal Neuroendocrine Neoplasms
What are Rectal NENs Rectal Neuroendocrine Neoplasms (NENs) (rNENs) account for approximately 1-2% of all primary cancers in the rectum. The other main cancer types

Clinical Trial: Novel Somatostatin Receptor Subtype 2 Antagonist Labelled With Terbium-161 (161Tb-DOTA-LM3) (Beta plus)
A new clinical trial post. What is Terbium-161 (161Tb-DOTA-LM3) (Beta plus). Terbium-161 is a radioactive substance. DOTA-LM3 is a novel somatostatin antagonist targeted using somatostatin

Repeat after me: Maria Menounos has Neuroendocrine Cancer
Like many people from outside USA, I don’t really know anything about Maria Menounos. For those in the same boat, let me confirm she is

Summary of April 2023 on RonnyAllan.NET
Summary of RonnyAllan.NET website activity in April 2023 The top 10 most read posts in April are included below as the main product of this summary
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.