NET Syndromes – chicken or egg?

We’ve all heard the age-old question about the chicken and the egg?  Scientists claimed to have ‘cracked’ the riddle of whether the chicken or the egg came first. The answer, they say, is the chicken. Researchers found that the formation of egg shells relies on a protein found only in a chicken’s ovaries. Therefore, an egg can exist only if it has been inside a chicken. There you have it!

On a similar subject, I’m often confused when someone says they have been diagnosed with ‘Carcinoid Syndrome’ and not ‘Carcinoid Tumours’ or ‘Neuroendocrine Tumours’.  So which comes first?  I guess it’s the way you look at it. In terms of presentation, the syndrome might look like it comes first, particularly in cases of metastatic/advanced disease or other complex scenarios.  Alternatively, a tumour may be found in an asymptomatic patient, often incidentally.  However, on the basis that the widely accepted definition of Neuroendocrine Tumours would indicate that a syndrome is secondary to tumour growth, then the tumour must be the chicken.

I sometimes wonder what patients are told by their physicians….. or perhaps by their insurance companies (more on the latter below). That said, I did see some anecdotal evidence about one person who was diagnosed with Carcinoid Syndrome despite the lack of any evidence of tumours or their markers. This might just be a case of providing a clinical diagnosis in order to justify somatostatin analogue treatment but it does seem unusual given that scientifically speaking, Carcinoid Syndrome can only be caused by a Carcinoid Tumour? Perhaps this is more widespread?

I have a little bit of experience with this confusion and it still annoys me today.  Shortly after my diagnosis, I had to fill out an online form for my health insurance.  The drop down menu did not have an entry for Neuroendocrine ‘anything’ but I spotted Carcinoid only to find it was actually Carcinoid Syndrome.  By this stage I had passed the first level of NET knowledge and was therefore suspicious of the insurance company list.  I called them and they said it was a recognised condition and I should not worry.  Whilst that might have been correct, I did tell them it was not a cancer per se but an accompanying syndrome; and was concerned about my eligibility for cancer cover. However, they persisted and assured me it would be fine on that selection.  On the basis it was really the only option I could select, I selected and submitted.  I did get my cover sorted.  However, it is now clear to me that their database was totally out of date.  A similar thing happened when I was prescribed Octreotide and then Lanreotide, the only ‘treatment type’ they could find on their database was ‘chemotherapy‘ – again their system was out of date.  I’m told by someone in the know, that individual insurance companies are not responsible for this list, they all get it from a central place  – I’d love to pay that central place a visit!

I quickly thought about all the other NET Syndromes for their ‘chicken and egg’ status! Pancreatic NET (pNET) Syndromes must all be ‘chicken’ given the tumour definition and the secretion of the offending hormones e.g. Insulin, Gastrin, Glucagon, Pancreatic Polypeptide (PP), Vasoactive Intestinal Peptide (VIP) and Somatostatin, etc. Even the more complex and obscure types including (but not limited to) Multiple Endocrine Neoplasia (MEN), Cushing’s and Zollinger-Ellinson Syndrome (ZES) are all related to a variety of neoplasia, tumours, hyperplasia and adenomas.  The NETs are still the chicken!

The NETs must be chicken but often the egg appears to show up first.  Go figure!

Thanks for listening