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Evo Terra (Host): Welcome to UK healthCast with the University of Kentucky Healthcare. I'm your host Evo Terra. Joining us today are Medical Oncologists, Dr. Aman Chauhan and Dr. Caryn Sorge. We'll cover caring for pediatric neuroendocrine tumors in this panel discussion. Perhaps we should start at the beginning with an overview of NETs or neuro endocrine tumors. Can you tell us more?

Aman Chauhan, MD, MBBS (Guest): Yes. So, NETs or neuroendocrine tumors previously also called as carcinoid tumors are rare cancers and can afflict anybody, pediatrics to adults, although primarily seen in adult patient population and also can affect any part of the body. Especially in adults nueroendoctine tumors can affect stomach, small intestines, colon, lungs. I've even seen neuroendocrine tumors in gonads, breasts, you name it. Neuroendocrine tumors are somewhat unique because they are abnormal growth on these specialized cells called neuroendocrine cells. When not growing abnormally, these cells have a function to play. For example normal neuroendocrine cells are lying in our GI tract and help us in digestion and peristalsis. But when they start screwing up, normally that's when the problem occurs. So, yes very unique set of cancers and very rare in adults. These are seen in about five or six cases per a hundred thousand general population.

Host: What are some of the symptoms of NETs?

Dr. Chauhan: That's a great question. Neuroendocrine tumors can be classified based on where they originate and that kind of dictates how they will present. So, if a neuroendocrine tumor starts in lung the symptoms might be shortness of breath, chronic cough. Some time reasoning. In fact, many of the lung neuroendocrine tumors can often be misdiagnosed as asthmatic for a long period of time.

And then that leads to delay in diagnosis. Similarly, if the neuroendocrine tumor originates in small bowel, the presenting symptoms could be bowel obstruction or belly pain. But it is not surprising that many times these neuroendocrine tumors are asymptomatic. These tumors are very slow growing, especially if they are low grade.

So, because of their indolent nature, sometimes these tumors can have no symptoms whatsoever, until that is a critical mass of the tumor from the tumor burden. And then the patient can have symptoms that are reflective of the mass effect of that particular tumor burden. For example if the tumors are impinging on liver, that can cause early satiety and pain in that area of the abdomen. If the tumors are affecting bones, it could be a bone pain. So, that can certainly dictate the way the patients present. One very unique aspect of these cancers is that some, a minority subset of neuroendocrine tumors can produce certain way, so active peptides or let's call them hormones innovative, simple terms.

So, these subset of neuroendocrine tumors, which produce hormones are called functional neuroendocrine tumors and based on the hormone they produce, the patient can manifest different symptoms. One of the commonest hormones they produce is called serotonin and these patients can present with flushing, diarrhea, wheezing, joint pains, skin rashes, et cetera. If the neuroendocrine tumors makes insulin, that's a hormone that we have heard of, so excessive insulin can cause low blood sugars. So, there are host of other hormones that these tumors can produce. Fortunately, the functional neuroendocrine tumors is a minority subset, maybe 15 to 20% of all NETs are functional. So, these are different ways that NETs patients can present with.

Host: And as mentioned Dr. Sorge, these apparently present differently in children than in adults, I take it.

Caryn Sorge, MD (Guest): So, yeah. They can actually in some ways present similar to adults and how Dr. Chauhan said that our patients may not have a lot of symptoms and that's oftentimes what happens in pediatrics. There are either no symptoms and we are actually referred for kind of non-specific symptoms, similar to what he had mentioned because you know, a lot of children can come in with a bellyache and have different things going on that we don't automatically think of as cancer.

And so, oftentimes these patients end up in different sub-specialists before we diagnose them with the cancer. Now there are a subset of patients who can be completely asymptomatic and are actually being followed by us because they have a family history of a certain syndrome that we know puts them at risk for developing these types of cancers. So, one of the syndromes is something called MEN1 or multiple endocrine neoplasia one syndrome. And we actually, in our clinic provide routine surveillance care for these patients because we do know that over their lifetime, they are at risk for developing these tumors.

And because we know that some of these can present as asymptomatic, we do surveillance with certain laboratory tests and sometimes we even use imaging to ensure that we are picking these tumors up early, sometimes even before they cause symptoms. And so that's one of the unique things that we do here at UK for these patients.

Host: Let's stay focused on the unique attributes of Kentucky Children's Hospital and talk a little bit more if you would, about the care children receive at the facilities.

Dr. Sorge: Sure. So, I am a pediatric oncologist and I've been at different institutions and I think UK really has done some wonderful things. And the unique thing that we have available here at UK is that we have a great relationship between Kentucky Children's Hospital and the Markey Cancer Center. So, fortunately for children, cancer is a rare entity and neuroendocrine tumors is even more rare. However, our adult counterparts do see a larger number of these patients because it does occur more often in adults. So, we are able to combine our expertise and have the expertise of Dr. Chauhan help provide that neuroendocrine expertise and we can provide the pediatric oncology expertise. And so we kind of joined forces and are able to provide excellent care to these patients and get the treatment that they need in the pediatric friendly atmosphere that I think all children deserve to be treated in.

So, we have pediatric child life specialists. We have pediatric nurses who are trained to you know, get the labs and the IV pokes and all of those things that are necessary to treat these patients in kind of a family friendly kid friendly center, which is wonderful. And they're still able to receive the excellent care that they deserve and need.

Host: Absolutely. Let's talk about some of those treatments. Dr. Chauhan, can you talk about what we have available for patients who are diagnosed with a NET?

Dr. Chauhan: So, unfortunately, neuroendocrine tumors being rare cancer, we do not have many treatment options. The needles have moved a bit for adult patients, but for pediatric neuroendocrine tumor patients, we still don't have a whole lot of treatments, especially when we are dealing with metastatic or advanced disease. Lot of our treatment paradigms are based on how we treat neuroendocrine tumor patients in adult patient populations. But this is an area of critical unmet need. The way we treat early stage disease is with help of surgeries. If the tumor can be resected and patient be made clear of disease, that's considered always an ideal goal and the right way to go.

But, if the tumor has already spread and what we call stage four or metastatic disease with involvement of other organs besides the primary tumor, then the treatment can be challenging. Some of the treatment options that we can try include somatostatin analogs, certain targeted therapies, and most recently peptide receptor radio nucleide therapy, which is a targeted form of radiation treatment. What makes these cancers somewhat unique is presence of somatostatin receptors on its surface. And we have made a drug which targets these somatostatin receptors. And irradiates them. So, we will be talking a bit about one of such therapies, which we are bringing to University of Kentucky via a clinical trial.

Host: Well, let's switch to that clinical trial. Now, I know that Kentucky Children's Hospital is one of six hospitals nationally that's involved in the NET or pediatrics clinical trial. Can you tell us more about that trial and also when will it be available?

Dr. Chauhan: Yes. So, we are very excited to be selected as one of the select sites in the country for this very unique clinical trial, which Dr. Sorge and I would be leading at University of Kentucky. This study is looking at a drug called lutetium 177 dotatate, which is a targeted radiopharmaceutical drug, an IV form of treatment, which is injected and given every two months for a total of four doses. And it's already FDA approved based on a similar named trial called NETTER1 in adult patient population. As I mentioned earlier, since we do not have FDA approved treatments for metastatic diseases in pediatric NET patients, this is a very significant step forward towards improving therapeutic options in pediatrics.

We expect this clinical trial to open at our center for enrollment in next one to two months. They're in very advanced stages of getting the protocol open here. And we feel that this is a very patient friendly treatment considering the treatment schedule and schematics. And we're just very happy to collaborate with our pediatric oncology colleagues and be able to deliver these cutting-edge treatments to our pediatric NET patients.

Host: It definitely sounds like a much needed clinical trial and hope that it provides some relief for everyone dealing with NETs right now. And that wraps up another episode of UK HealthCast with the University of Kentucky Healthcare. Find more information on pediatric NETs and how we care for them on our website, UKhealthcare.uk.edu. Please remember to subscribe, rate and review this podcast and check out all the other University of Kentucky Healthcare podcasts. I'm Evo Terra. Thanks for listening.