Ronny Allan – Living with Neuroendocrine Cancer

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).  Until I read up for this post, I hadn’t fully realised how closely related the two conditions are.  I would now recommend that 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.

Overview

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. So putting the heredity aspects to one side, it’s potentially an extremely challenging diagnostic scenario.  To add to the complexity, some of the associated tumours can be sporadic (non hereditary) classic Neuroendocrine Tumours in various locations.

MEN is actually an umbrella term for a number of types (syndromes) of the disease – MEN1, MEN2 (2a and 2b (2b was formerly MEN3)) and Familial Medullary Thyroid Carcinoma (FMTC). There is a new kid on the block called MEN4 which is extremely rare.  In the most basic of terms regarding tumours, MEN1 seems to be centred on tumours of the parathyroid glands, the pituitary gland, and the pancreas (the 3 P’s) with MEN2 mainly focussed on the thyroid.  MEN4 is similar to MEN1 but caused by mutations in a different gene.

What is particularly distinctive with MEN is that they are inherited disorders (familial).  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.

You may also have heard of other rare NETs with a familial aspect, in particular Pheochromocytomas (adrenal gland tumours) and Paragangliomas (outside the adrenal gland, less common and not all are inherited).  These tend to be grouped under MEN for support and advocacy.

Further information:

• AMEN Support where there is a useful Primer Leaflet
• A great video from Dr Mark Lewis who is an Oncologist and MEN patient.  This is great as he speak as both!  Click here to watch
• More technical information can be found on the NIH site here including the genetic aspects.
• For 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.  This is one of the friendliest and well run support groups I’ve seen.  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.

There are other organisations including one specifically for Pheochromocytomas and I’m grateful to Jennifer Shepard for featuring my nutrition blog series.

Complex area.

Thanks for reading

Ronny Allan

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