Originally published 10th April 2020

“An unusual disposition of tumours”

A fairly common disposition of Neuroendocrine Neoplasms is a primary with associated local/regional secondary’s (e.g. lymph nodes), and often with liver metastases for late diagnoses. Apart from the primary tumour invading nearby tissue/organs, the most common spread is the lymph nodes, these can take you from a localised Stage 1 to loco-regional Stages 2 and 3. Often the term distant spread infers metastatic disease (stage 4) to the liver, but I had some lymph nodes ‘misbehaving’ much further away than that. After my first nuclear scan (In-111 Octreoscan) during my diagnostic workup in Jul-Aug 2010, two areas lit up – left axillary nodes (armpit) and left supraclavicular fossa (SCF) nodes (clavicle). However, my MDT remained focussed on my primary and liver metastasis as this was where the bulk of my issues was. These nodal issues were to be looked at later.

A reminder – how cancer spreads

In addition to the primary growing into other areas, cancer can form metastases through the bloodstream and the lymphatic system. This is a system of thin tubes (vessels) and lymph nodes that run throughout the body in the same way blood vessels do. The lymph system is an important part of our immune system as it plays a role in fighting bacteria and other infections; and destroying old or abnormal cells, such as cancer cells. The lymphatic system also contains organs, some of which feature regularly in NETs. If cancer cells go into the small lymph vessels close to the primary tumour they can be carried into nearby lymph glands where they stick around. In the lymph glands they may be destroyed (that is actually one of the jobs of the lymph glands) but some may survive and grow to form tumours in one or more lymph nodes.

Certain things are known about the behaviour of Neuroendocrine Neoplasms (NENs) (a term for Neuroendocrine Tumours and Neuroendocrine Carcinoma) and specialists will be analysing many factors when working out the type of NEN and how it might behave. This is useful in cases of unknown primaries as it can give them clues to the possible location(s). Read more about these issues in my article “Needle in a Haystack“

My experience with lymph node surgery

In my own straightforward (ish) case, I had a small intestine NET with the usual metastatic distribution of local and regional lymph spread and liver mets, but I also has these distant nodes as described above – my surgeon described my ‘distant’ lymph node issues as an ‘unusual disposition‘. I searched for others in the same situation and found only a handful of people with the same disposition. My research (including more recently) also found that these distant lymph node issues were more related to people with Paragangliomas, Lung NETs and tumours of the breast. I also discovered that sometimes when lymph nodes light up on nuclear scans, this is physiologic uptake and not really an issue, despite the worry it can bring.

As a metastatic Small Intestine NET case, I had the usual bulky chains of lymph node metastases in or around the mesentery that frequently appear with an abdominal primary. Most of these were removed during my primary resection. I still have some suspicious lymph nodes in the peri-aortic region, potentially responsible for my retroperitoneal fibrosis, another strange issue I live with.

In early 2012, 15 months after removal of primary and 10 months after liver resection, one of the axillary lymph nodes became palpable (signs of growth) and this coincided with a small spike in Chromogranin A. A total of 9 lymph nodes were removed very shortly after this surveillance, 5 of which tested positive for NETs (Ki-67 <5%). As part of the same operation, 5 SCF left clavicle nodes were removed but tested negative. I had some lymphatic fluid build-up in my armpit which became swollen and painful. I had it drained by fine needle aspiration (FNA) around 3 times before it settled down. I now live with some very mild lymphedema in my left hand which looks slightly puffier than my right. I also have to avoid the left arm for blood draws.

On a subsequent Octreoscan, the armpit was clear, but the clavicle area still lit up. However, there is no pathological enlargement or pain – so this is just monitored. The SCF nodes also light up on Ga68 PET which also showed additional glow in the left sub-pectoral nodes. This was subsequently checked on ultrasound, and nothing is pathologically enlarged. By the way, the surgeon carrying out this work wasn’t a NET surgeon, but he was known for working alongside my Oncologist who was part of the NET MDT. He was in fact well known for breast cancer surgery and these distant lymph node areas are common in breast cancer. So, he was perfect for the job. Sometimes you just need an anatomical specialist working under the direction of the NET MDT.

Life as a metastatic Neuroendocrine Cancer patient is interesting and thorough staging and grading at diagnosis plus efficient surveillance is absolutely critical.

In addition to linked posts in blue above, you may enjoy my posts:

My Primary Surgery Part 1

My Primary Surgery Part 2

My Liver surgery

Nodes, Lesions, Nodules and False Alarms