Frequently Asked Questions:

with my current understanding of the answers (aka: subject to change)

Q: What Makes NeuroEndocrine Tumors different from Proper Cancer? 


NETs are only recently understood to be a cancer. A tumor in the endocrine system was thought that even though they do weird things with their hormones, they could at least be treated. The hormones cause symptoms, but those symptoms are generally manageable without major life-interruptions. Such are Grades 1 and 2. Grade 3 has been called Carcinoid Syndrome, which means ‘cancer-like’ syndrome.

Because that’s where the understanding started, that is also why the terminology went the way that it did, but that is misleading. It is not cancer-like – it is a instead a different type of cancer. And it responds to treatment differently, as well.

Q: What do the Grades Mean?


Proper Cancer is measured in Stages, with Stage 1 being located only at the origination-point, and Stage 4 having spread far and wide from that point of origin. As NETs are a cancer that work through the endocrine system, it is systemic from the start. The Grades indicate its rate of proliferation.

Grade 1 has a Ki67 number of 1-10. The way forward with Grade 1, is to watch and wait. It doesn’t always get worse.

Grade 2 has a Ki67 number of 11-20. The way forward with Grade 2, is to take a Sandostatin shot once per month. It usually manages the symptoms, while keeping the growth rate at bay.

Grade 3 has a Ki67 number of 20+. The way forward with this, is changing quickly, which is the reason Shelli wanted to be in a clinical trial – the standard of care here is chemotherapy first, followed by radiation.

Shelli is hovering around 40. Which isn’t 90.

Q: What is Ki-67?


Okay, nobody is asking this question - I thought I would include it because I reference it above.  Ki-67 is a type of protein that is an indicator of the growth rate of the tumors.

Q:  Why don't they Remove the Original Tumor that is Causing the Problem?


The liver is where the cancer primarily is, but it’s not liver cancer. The original NeuroEndocrine Tumor, for Shelli, is in the intestine. Even still, all the other tumors, no matter where they are located, are also NETs, and they also are pushing out those same cancer-causing hormones. Removing the original one is irrelevant; it's a hive without a queen.

But even if the original tumor did operate like a queen bee, in that it was directing the others, that tumor can’t be removed without Shelli having to heal – and that is extensive surgery from which to recover. If it is removed, the cancer in the liver is still working against her – she can’t have both radiation (or chemo) and be recovering from such extensive surgery. So, removing the original tumor isn’t an option.

Q: Why don't they do a Liver Transplant?


A liver transplant isn’t an option because, although that is where the cancer primarily is, it won’t take long for one of the other tumors outside of the liver to invade it all over again. The cancer isn’t solely in her liver. Plus, all the medicine that she would need to be taking following a transplant would diminish her body’s ability to fight. Plus, liver transplants are crazy difficult. Plus, her liver is fully functional.

Q: As there is no Cure, How Long do People Live with Grade 3 NET's?


It's a slow-moving cancer, comparatively.  There are people who have lived 10-years and longer.

Q:  What about the Tumors?


There was a primary tumor that started it all; pushing out cancerous hormones. (Maybe not technically cancerous hormones, but cancer-causing hormones?) Even though it is the source of all the other cancerous tumors, treating the whole system, all of the tumors, is necessary, because now they all are working together.

The tumors can be either well-differentiated or poorly-differentiated. Up until recently, the distinction was not made by the doctors or the researchers – they simply didn’t note it (or maybe they didn't have the tools to tell the difference). They have since learned that there are two types, and they function vastly differently.

Well-differentiated tumors put out more hormones, which increase the proliferation rate of the cancer – but more importantly, how quickly the cancer develops a tolerance to the treatment. Undifferentiated tumors function less well, in both the damage they cause as well as in the way they develop tolerance to the various treatments.

Shelli has well-differentiated tumors, in the range of, “innumerable”.

Q: What is this Clinical Trial Looking to Answer:


The question this trial is trying to answer is: Can a lower beta-emitter stay attached to the cancerous tumors longer, causing damage to the cancer without it developing as quick of a tolerance?

One of the things that sets NETs apart, is that once they recognize an attack, they adapt quickly. If we hit it hard and fast, it takes over all that much faster.  But if we don’t try to stop it, though, it has free reign to do what it wants. We are trying to fly under its radar.

What's the Difference between Dotatate and dotatoc?


Dotatate is radiation that has been approved as a second-line of treatment, following chemotherapy (well, third if you count the Sandostatin shot as treatment). Dotatate targets the protein on the NeuroEndocrine Tumors, radiating only them while leaving the rest of the body alone. It does not kill or remove the cancer – the best case scenario is that it stops the growth of the cancer for a time-period. Eventually, the cancer develops a tolerance to that type of radiation, and any further infusions are without benefit. Four infusions, with each one being eight-week’s apart, is the protocol.

Dotatoc is not yet approved as treatment, but it is still a radiation that targets the protein on the NETs. Being a lower beta-emitter, the hope is that the cancer doesn’t notice a threat, and therefore the radiation can stay on the NETs longer, providing greater benefit. Dotatoc allows for up to six infusions.

Q: As this Cancer is Known, but the Treatment is Not, What Happens Next?


The next step for Shelli has not yet been determined. But we have been told that there are 4-5 other known approaches that we have available to us now, from the Dotatate radiation to various forms of chemo.  And who knows what might develop along the way, as there are other NET studies going on now in Europe that are trying to answer other questions.

Where Can I Find More Information on NET's?


There are now some trustworthy places online!  In the order that I was most recently on these pages, here is #1, and here is #2, and then the third.  I think we have grown in our capacity to take in information, at this point.

Even with the ignorance that Google spreads, we are happy to ask the NET Department in Mayo all the questions, and they have been more than able to explain the updates and research development that has been occurring. Hopefully, I answered some of those questions here. As you have more, feel free to ask Shelli or me. If we can’t answer directly, we’ll ask the experts and get back to you.