Pediatric Hepatoblastoma

Podcast Transcript

Dr. Scott Steele: Butts and Guts, a Cleveland Clinic podcast, exploring your digestive and surgical health from end to end. Hi again everyone, and welcome to another episode of Butts and Guts. I'm your host, Scott Steele, colorectal surgeon and president of main campus here at Cleveland Clinic in beautiful Cleveland, Ohio. And today I'm very pleased to have Dr. Gloria Gonzalez, who's a pediatric general surgeon and leader of the surgical oncology practice here at Cleveland Clinic Children's. Gloria, welcome to Butts and Guts.

Dr. Gloria Gonzalez: Oh, thank you very much for the invitation.

Dr. Scott Steele: Can you first give us a little bit of background for our listeners out there, where you're from, where'd you train, and how did it come to the point that you're here at the Cleveland Clinic?

Dr. Gloria Gonzalez: Sure. I'm from Chile. I train in the University of Chile a long time ago and after that I did a fellowship in transplant, solid organ transplants, liver and kidney. And after that I trained like a fellow in children's at St. Jude Children's Hospital for two years and that was in 2009. After that I came back to Chile and I worked there like a leader in oncology surgery. I was the chief of one of the biggest hospital in Chile, Hospital Luis Calvo Mackenna. And after that they invited me to join Cleveland Clinic Pediatric Surgery Department and that's why I'm there.

Dr. Scott Steele: Well, we're very glad to have you here. And so today we're going to be talking a little about pediatric hepatoblastoma. So to start with, can you explain at a high level what pediatric hepatoblastoma is for our listeners?

Dr. Gloria Gonzalez: Sure. First just you need to know there are some like 500 new cases a year of malignancies, roughly 1% of those tumors are liver tumors. And the most important or the most frequent tumor in children is hepatoblastoma, followed by hepatocarcinoma. Hepatoblastoma is a really special tumor in kidneys, in children because it's related with some genetic disorders and some other diseases.

Dr. Scott Steele: So what are the common symptoms and signs that parents should look out for?

Dr. Gloria Gonzalez: Most of the times these tumors present like a mass. So this is a right quadrant abdominal mass, and that is the most important thing that the parents has to look at. And they're not so much other symptoms, maybe they look a little sick, but most of them are just an abdominal mass.

Dr. Scott Steele: So are there any known risk factors or causes associated with pediatric hepatoblastoma?

Dr. Gloria Gonzalez: Yeah, there are many. For example, preemies has a more hepatoblastoma than that term baby. And there's some genetic diseases that are related to hepatoblastoma like polyposis, and some other overgrowth syndromes, also with Wiedemann disease. So there are some syndromic genetic disorders that they're related with hepatoblastoma.

Dr. Scott Steele: So how is pediatric hepatoblastoma diagnosed?

Dr. Gloria Gonzalez: First with the parents, they ask for an abdominal mass. The first thing that we are going to do is say ultrasound to be sure the abdominal mass is from the liver. And after that you have to do a workout. There are blood exams, labs. There is something that it calls alpha-fetoprotein and that is a tumor marker that will be really high if you have an hepatoblastoma. After the ultrasound you have to do some better images like a CT or an MRI and a lung CT also to see if the kid has the abdominal mass or the hepatoblastoma just in the liver. Or there are some metastases in the lungs that there's the most frequent organ affected like a secondary elision.

Dr. Scott Steele: So before we get into treatment, let's circle back to the pediatric hepatoblastoma itself. So where is the origin of this from? Is it from the liver cells in general? Is it a metastasis to the liver? Is it from the bile ducts? Tell us a little bit more about this tumor.

Dr. Gloria Gonzalez: This is a primary liver tumor and it comes from the cells, the hepatocytes. So it originates in those cells in the liver it cells, and it comes from the embryonal cells of the liver. That is what is called hepatoblastoma. So it's not secondary. It origins in the liver.

Dr. Scott Steele: So what are the current treatment options available for children diagnosed with this type of cancer?

Dr. Gloria Gonzalez: When you have this cancer you have to have two things. One is medication, like I mean chemotherapy, but also you have to have the tumor removed. So you will need surgery and chemotherapy to treat this cancer. And it has a really good prognosis in overall prognosis is 80% survival after these treatments.

Dr. Scott Steele: So when you take this out, do you just take out the mass itself or do you have to take out half the liver, all the liver, certain segments of the liver?

Dr. Gloria Gonzalez: Yeah, when we start we have to know where the tumor begins. It can be in the right liver or the left liver or the left lateral segment of the left liver. So there are different places where this hepatoblastoma could be located, and it's much more frequent in the right side. So when we operate, these kids usually have to take half of the liver. The most usual surgeries is a right lobectomy or left lobectomy. But we can also take out just a part of the liver that is involved with tumor. And sometimes if the four sectors of the liver are affected by the tumor, sometimes we have to do a liver transplant to resect the tumor completely and give those patients the survival that I told you before that is 80%.

Dr. Scott Steele: So truth or myth? Truth or myth, pediatric hepatoblastoma is always caused by genetic factors?

Dr. Gloria Gonzalez: It's myth. There are genetic factors that can relate with hepatoblastoma. Like I told you before, there are some genetic syndromes that has more frequency of hepatoblastoma, but you don't have to have those symptoms to have an hepatoblastoma. There are kids that they're healthy, totally healthy and they develop the hepatoblastoma.

Dr. Scott Steele: So as a pediatric general surgeon, can you share a little bit more about your surgical approach for hepatoblastoma? And what I mean by this is do you do this robotically, a large incision on the belly, laparoscopically? Are there consequences to taking significant sections of liver for these age group patients? Give us a little bit more here.

Dr. Gloria Gonzalez: Sure. When we approach these patients like a pediatric oncology surgeon first, these tumors are big. So when we give them the chemotherapy, this tumor we expect will shrink, but we need to remove a part of the liver that is most of the time half of the liver. So we usually do it open, like an open laparotomy.

There are some few cases that we can do it laparoscopically and of course if we can do that, we are going to do it minimally invasive surgery. But most of the time we have to take half of the liver. And like I told you, it's better to do it open except if the patient is big enough to do it robotic or laparoscopically. Now when you have to take the half of the liver or more than half, we take care that the liver we leave behind is enough for the patient to live without any problem in the future. So we can remove half of the liver and you will not have any problem in the future. So you need just 1% of your kilograms in grams to live. For example, if a patient has 20 kilos, we can live behind 200 grams of liver and that will be enough to live a long life without any problems.

Dr. Scott Steele: So you mentioned before about a liver transplant can talk more. When is this an option for patients with hepatoblastoma?

Dr. Gloria Gonzalez: Yes, like I just said, we have to have at least 1% of your weight in liver to leave behind to live a long life without any problems. If we cannot do that, we have to do a liver transplant. And most of those cases are tumors that are in all the liver. Like a surgeon, we anatomically divide the liver in four sectors and if you have the four sectors with tumor, you have to have a liver transplant. But liver transplant is a good alternative. Those kids live a really good life and the survival is really high. So it's a good option when you have to do it.

Dr. Scott Steele: So you talked earlier and said that it was about an 80% survival for this disease process. So when these patients do recur, do they recur locally in the liver or do they spread to elsewhere in the body?

Dr. Gloria Gonzalez: Most of the kids will recur in the liver, but some may recur in the lungs. That's the most frequent site when they recur in a secondary location. But we can give them treatment again for the recurrence and we can remove that secondary location or we can re-operate in the liver if the patient recur. So we have more options.

Dr. Scott Steele: So how important is early detection than in the prognosis of these patients?

Dr. Gloria Gonzalez: I think in all pediatric malignancies it is important, the early diagnosis. What we can say to the parents first, if you have a genetic syndrome that is related with the pathoblastoma, you have to do for example, like serial ultrasounds to try to look after the tumor and do a early diagnosis. And if your kid is healthy and don't have anything, remember this is a really rare disease, so we don't have to be scared or afraid all the time thinking having a malignancy in a kid. But if you notice the belly of your child is growing or you see your kid being sick, remember you have to ask your primary doctor to try an ultrasound. That is a really simple thing to do and just rule out if you have a malignancy or you have a tumor or you have something benign also. They're much more frequent than a pediatric malignancy.

Dr. Scott Steele: So are there any advancements on the horizon when it comes to the treatment of pediatric hepatoblastoma?

Dr. Gloria Gonzalez: Actually we're trying to learn more about the hepatoblastoma. One of the most important things that we are doing right now, we're trying to work worldwide together with all the groups, Japanese, German, European, and of course COG to try new protocols that gives this patient much more survival, trying to understand a little more the disease because it's infrequent, like I told you, and if we work like a group together, we are going to have much more things to do or offer to these patients in the future.

And now what we are looking is at molecular biology is trying to see how these cells specifically behave dependent on the genetics of the tumor and try to get medication for these patients. Also, of course we are trying to improve the surgery and we're growing in technology and trying to work with that with the kids also, meaning robotics, 3D images. There are some medication that we can give to the patient before the surgery and we can see the tumor during the surgery to try to take it out and have negative margins. So we are doing really rapid knowledge about it and I think in two more years we are going to have much more understanding of the genetic of the tumor.

Dr. Scott Steele: So now it's time for our quick hitters, a chance to get to know you just a little bit better. So first of all, what is your favorite food?

Dr. Gloria Gonzalez: I think they're vegetables. I'm not vegetarian, but I enjoy vegetables.

Dr. Scott Steele: What was your first car?

Dr. Gloria Gonzalez: A Mini Cooper.

Dr. Scott Steele: Tell us something that you like about your home country, Chile.

Dr. Gloria Gonzalez: Wow, landscapes. Chile is beautiful. It has beautiful landscapes. We have desert in the north and in the south we have to the Antarctic. So I think if you come here to Chile, you're going to enjoy beautiful places all around that is breathtaking.

Dr. Scott Steele: And then finally, if you could have one superpower, what would it be?

Dr. Gloria Gonzalez: You know what? I don't remember the name, but what was this clock that Hermione Granger has in Harry Potter where she can be in different places in different times and come back and be in different places in once? That would be my superpower.

Dr. Scott Steele: Absolutely. So why don't you give us a final take home message for listeners about pediatric hepatoblastoma?

Dr. Gloria Gonzalez: I think one of the most important thing it's devastating when you're talking about malignancies in general. And when you're a parent and you know your child has a malignancy, it is really difficult to see the future. But we have so many things to offer these kids. And of course if we have an early diagnosis, it's much better for them. Don't lose hope. We can help them. We have all the technology and all the knowledge to try and give them the best chance to be healthy.

Dr. Scott Steele: Well, that's fantastic. And so for more information about Cleveland Clinic Children's Hematology and Oncology Department, please visit clevelandclinicchildrens.org/cancer, that's clevelandclinicchildrens.org/cancer. You can also call us at 216.444.5437. That's 216.444.5437. Dr. Gonzalez, thank you so much for joining us on Butts and Guts.

Dr. Gloria Gonzalez: Thank you very much.

Dr. Scott Steele: That wraps things up here at Cleveland Clinic. Until next time, thanks for listening to Butts and Guts.