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Ronny Allan
It’s known that Neuroendocrine Cancer is quite different in many ways from other cancers, notwithstanding the misnomer term carcinoid which is thankfully being slowly moved out of terminology. As a few examples:
- It’s a wide spectrum heterogeneous cancer group with indolent isolated small tumours at one end all the way across to extremely aggressive metastatic cases at the other end.
- It’s a cancer type that can be syndromically functional or non-functional to add to diagnostic and management challenges.
- It’s a cancer that can appear almost anywhere in the human body.
One of it’s less well-known traits is the ability to produce multiple primary tumours. Most people might be thinking of Multiple Endocrine Neoplasia at this point (a syndrome that predisposes the patient to multiple primary tumours in different organs). However, I also mean multi-focal (multicentric), i.e. more than one primary in the same organ. And that is the focus of this post in regards Small Intestine NETs where this is a common occurrence (one Swedish study put that at 40%).
This new study from Sweden has appeared twice in the last month, one giving a highly technical summary of the genome mapping to a small group of tumours. The other gives a higher-level summary.
The conclusion from this small study is astonishing in that it would appear multifocal primary NETs in the small intestine are capable of producing their own metastases. I personally don’t believe that changes anything in terms of therapy, but it does reinforce the need for a full check during surgery, something known in surgical terms as “running the bowel” which can be done for different reasons depending on the scenario but in cases of small intestine NETs is important as many of these cases can have multifocal tumours (multiple primaries).
See the abstract below:
Let’s start with the overview as that is what most of you will be looking for. There’s also some technical stuff in this overview but genome mapping is a highly technical subject not understood by many.
Concrete clinical question
“When we remove neuroendocrine tumors in the small intestine, we often find many tumors in the same place, which is unusual. No other cancer really looks like this,” says Erik Elias, an endocrine surgeon at Sahlgrenska University Hospital and a post-doctoral researcher at the University of Gothenburg. “The cancer is also unusual because known driver mutations are largely absent.”
Surprising answer
“The result was both unambiguous and surprising. The intestinal tumors had developed independently. Yet the metastases were clearly related and could be traced back to a specific bowel tumor.”
Citing information:
Elias, E., Ardalan, A., Lindberg, M. et al. Independent somatic evolution underlies clustered neuroendocrine tumors in the human small intestine. Nat Commun 12, 6367 (2021). https://doi.org/10.1038/s41467-021-26581-5
Click this link to read full article:
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I recall asking how many tumours were found in the Small Bowel. The surgeon replied there were too many to count but we removed the affected length plus a clear % at each end. The tumours had already metastasised to the liver, lymph node and the mesentery. What was fascinating was the primaries being G1 and the metastases at G2.
it would have been an interesting correlation, but I did note that there was even one patient where they spotted the same signature in different metastases from the same primary tumour!
Admittedly much of this was way over my head. I will say how grateful I was for an astute surgeon. I presented with a pretty typical NET on imaging, but when they got in there they also found a second tumor in the Ileum and the 3 multifocal mesenteric tumor deposits that were .03 cm, .08 cm, and 1 cm. The correlation between the two nets (Jejunum and Ileum which were completely separate) and the tumor deposits is confusing to me.