# Hepatoblastoma with Dr. Greg Tiao — GCMD Library

Hepatoblastoma is the most common malignant liver tumor in the pediatric population but treatment strategies have changed dramatically over the past couple of decades so today we will review the basics of hepatoblastoma with Dr. Greg Tiao, MD - pediatric transplant surgeon at Cincinnati Children's Hospital Medical Center.

Minimal adjuvant chemotherapy for children with hepatoblastoma resected at diagnosis (AHEP0731): a Children's Oncology Group, multicentre, phase 3 trial
https://pubmed.ncbi.nlm.nih.gov/30975630/

The paediatric hepatic international tumour trial (PHITT): clinical trial design in rare disease
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4660185/

Type: podcast · 10 min · posted 2022-02-03 Canonical: https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960 ## Chapters - [0:00](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=0) Introduction and Differential Diagnosis - [2:08](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=128) Clinical Presentation and Diagnostic Workup - [4:00](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=240) Liver Anatomy and PRETEXT Staging - [6:34](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=394) Treatment Algorithms and Transplantation - [8:46](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=526) Current Research and Conclusion ## Statements - "Hepatoblastoma is the most common malignant liver tumor in the pediatric population" (epidemiological) [0:00](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=0) - "There are around 250 new cases of hepatoblastoma per year" — Greg Tiao (epidemiological) [0:15](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=15) - "Treatment algorithms for hepatoblastoma have changed dramatically over the last 20-25 years" — Greg Tiao (clinical) [0:15](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=15) - "Neuroblastoma is considered by most to be the most common abdominal wall malignancy in children" — Greg Tiao (epidemiological) [0:51](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=51) - "Neuroblastoma is the most common solid malignancy in the pediatric population" (epidemiological) [1:13](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=73) - "The differential diagnosis for liver tumors includes hepatoblastoma, hemangioma, hematomas, focal nodular hyperplasia, and hepatocellular carcinoma" (clinical) [1:28](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=88) - "Hemangioma is the most common liver lesion in children" — Greg Tiao (epidemiological) [1:41](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=101) - "Hepatoblastoma can present as an asymptomatic abdominal mass, tumor rupture, respiratory distress due to tumor size, or as a paraneoplastic syndrome like precocious puberty" (clinical) [2:08](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=128) - "An elevated alpha-fetoprotein (AFP) is a key component in the workup of hepatoblastoma" (clinical) [2:08](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=128) - "AFP is critical for the diagnostic algorithm and is a guide for treatment response" (clinical) [2:08](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=128) - "MRI is used by most experts to delineate anatomy and evaluate the extent of liver involvement in hepatoblastoma" (clinical) [2:39](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=159) - "EOVIST is an MRI contrast agent that is taken up by hepatocytes and then excreted, providing the best definition when looking at the liver on imaging" — Greg Tiao (clinical) [3:00](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=180) - "To establish a diagnosis of hepatoblastoma, four things are needed: history, abdominal imaging, AFP check, and liver biopsy" (clinical) [3:17](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=197) - "The most important intervention for survival in hepatoblastoma is to achieve complete resection" (clinical) [3:17](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=197) - "Aggressive resections for hepatoblastoma include trisectionectomy, mesohepatectomy, resection with an additional procedure in the remnant liver, and liver transplantation" (clinical) [3:17](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=197) - "Couinaud labeled liver segments in a counterclockwise fashion after the districts of Paris by injecting portal vessels" — Greg Tiao (clinical) [4:08](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=248) - "The right hepatic vein differentiates anterior sections (5 and 8) from posterior sections (6 and 7) of the right liver" — Greg Tiao (clinical) [4:45](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=285) - "The right portal vein separates superior segments (7 and 8) from inferior segments (5 and 6) of the right liver" — Greg Tiao (clinical) [5:20](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=320) - "The PRETEXT staging system classifies tumors based on the number of contiguous liver sections free of disease, calculated as 4 minus the number of free sections" (clinical) [5:53](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=353) - "PRETEXT 1 has 3 contiguous sections free of disease, PRETEXT 2 has 2 contiguous sections free, PRETEXT 3 has 1 free section, and PRETEXT 4 has no free sections or diffuse tumor burden" (clinical) [6:34](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=394) - "PRETEXT 1 and 2 tumors with greater than a centimeter margin from the middle hepatic vein and portal bifurcation should be considered for resection at diagnosis" (clinical) [6:34](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=394) - "The change in practice for PRETEXT 3 and 4 tumors improved survival rates for hepatoblastoma from less than 30% in the 1970s and 1980s to between 80 and 90% today" — Greg Tiao (epidemiological) [7:15](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=435) - "PRETEXT 3 tumors are generally biopsied at diagnosis and then started on neoadjuvant chemotherapy" — Greg Tiao (clinical) [7:31](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=451) - "Indications for liver transplantation in hepatoblastoma include unresectable disease, unsafe to resect, or resection would leave inadequate liver remnant" — Greg Tiao (clinical) [7:31](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=451) - "It is important to consult a transplant center early for PRETEXT 3 or 4 liver disease" (clinical) [8:11](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=491) - "Teams are now trained to recognize tumors and send them to centers that offer transplant or can do aggressive resection" — Greg Tiao (clinical) [8:19](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=499) - "The Pediatric Hepatic International Tumor Trial (FIT) is an ongoing multinational trial aimed at better delineating treatment algorithms and identifying high and low risk features for each PRETEXT stage" (clinical) [8:46](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=526) - "The FIT study has been ongoing for three years" — Greg Tiao (clinical) [9:06](https://library.globalcastmd.com/watch/hepatoblastoma-with-dr-greg-tiao-4960?t=546) ## Transcript Hepatoblastoma is the most common malignant liver tumor in the pediatric population. But treatment strategies have changed dramatically over the past 25 years. That's why today we're going to talk about the basics with an expert. So, Hepatoblastoma, there's around 250 new cases a year. Huge changes in terms of the treatment algorithm over the last 20 years. That's Dr. Greg Tiao. He is a pediatric transplant surgeon at Cincinnati Children's Hospital. He's going to walk us through some of the basics. So stick around. This is the Stay Current in Pediatric Surgery podcast. Okay, so I'll start by setting the scene. A child comes into the hospital with an abdominal mass. We're talking specifically about Hepatoblastoma today, but we should still have a very wide differential diagnosis. That's right. Hepatoblastoma is the most common malignant liver tumor in children, but it's nowhere near the most common liver tumor or the most common abdominal tumor. When I'm recalling the most common abdominal wall malignancy in children, I recall this being somewhat controversial, but most would consider it to be neuroblastoma. It's a toss up between neuroblastoma and Wilms, depending on who you talk to. Now neuroblastoma is the most common solid malignancy in the pediatric population, but that's because there are tumors everywhere in the body. So you could make an argument that Wilms is the most common. So we get our imaging and we figure out that the tumor is coming from the liver. So on our differential diagnosis, we should have hepatoblastoma, hemangioma, hematomas, focal nodular hyperplasia, and hepatocelular carcinoma. Common things being common, hemangioma should be pretty high on the list. Unfortunately, I'm not sure if Rajni jumped on or not, but she'll see many kids with liver lesions and their hemangiomas. The most common lesion is hemangioma. Even though hepatoblastoma is the most common malignant liver tumor in children, it's still pretty rare with about 250 cases per year. So you got to be pretty in tune with the wide range of presentations and have a high index of suspicion. Like Rod said, neuroblastoma has a wide spectrum of presentations. It can present as an asymptomatic abdominal mass, but on the other end of the spectrum, Dr. Tiao has seen it present as a tumor rupture or as a patient in respiratory distress due to the tumor size. It can also present as a perineoplastic syndrome like precocious puberty. But keep in mind that in any situation, an elevated alpha-phetoprotein, or AFP, is a key component in the workup. AFP is critical for the diagnostic algorithm, and it's a guide for treatment response. Okay, so to summarize, I see a kid and I'm suspicious because he has a large abdominal mass and he has an AFP in the 10,000s. Next we need to get axial imaging. Most experts are relying on MRI to delineate the anatomy and to evaluate the extent of liver involvement. MRI with EOVIST that we got today. What was that? What's EOVIST? EOVIST is an MRI contrast agent that is taken up by the hepatocytes and then excreted. It can provide the best definition when looking at the liver on imaging. If you're listening in the Stay Current app, scroll down under the media player. We're going to give you some images of EOVIST so you can take a look. I mean, it really does distinguish biliary anatomy. So to establish a diagnosis of hepatoblastoma, we need four things. We need to start with the history. Then we need to get our abdominal imaging. Then we need to check an AFP. And finally, we need to get a liver biopsy. The most important intervention for survival is to achieve complete resection. The way you get there can be varied. Primary goal is to achieve complete tumor removal. There's conventional resection and then there's more aggressive resections. Aggressive resections are when you're starting to think about a trisectionectomy, a mesohepatectomy, a resection with an additional procedure in the remnant liver, and of course, liver transplantation. As you're probably starting to figure out, a lot of the resection algorithms are based on liver anatomy and the pretext system. But before we get to that, Dr. Tiao, can you walk us through how to find all eight liver segments on axial imaging? That was correct. One is Caudate. Two and three are left lateral segments, superior and inferior. 4A, 4B is left medial segment. 5, 6, 7, 8 are the right side of the liver. Do you guys know how Cunode labeled them in this clockwise circle? He injected them into portal vessels. And so that's really how these are labeled. But he labeled them in this counterclockwise fashion after, I can never pronounce this, but the districts of Paris are done in the same fashion. Hmm. That actually makes sense in a strange way. When we look at the right side of the liver here, you guys see this here is the vena cava right here. So Al, how do we differentiate 5 and 8 versus 6 and 7? What's the anatomic marker that allows us to differentiate between the anterior and posterior sections of the liver, of the right liver? That looks like the right hepatic vein there. You see this right hepatic vein coming out here? And so what we're actually seeing that track out. And then how do we separate between superior and inferior? That looks like the right portal vein coming in there. So right anterior is going to be segment 8 and that is going to be anterior to the right hepatic vein and superior to the right portal vein. 5 is right inferior and that's based off the right portal vein inferior and anterior to the right hepatic vein. 6 and 7 in that same fashion, 7 superior and 6 inferior. Okay, now would be a really good time for a diagram of the anatomy. Conveniently, we've put one in the media player for you. So scroll all the way down in the app and you'll find it. And the reason that all matters is because there's something called the pretext staging system. We need to classify our tumors in a more anatomically understandable fashion. And so the sections of the liver, the right posterior section is 6 and 7 and the right anterior is 5 and 8 is considered the right sections of the liver. And segment 4 is considered the left medial segment. A section in the segment 2 and 3 is the left lateral. And depending on if you had tumor in those stages, in those sections, the number of free sections without tumor is a minus 4 is your pretext stage. We've also included a table of the pretext system to review. So pretext 1 has 3 contiguous sections free of disease. Pretext 2 has 2 contiguous sections free of disease. Pretext 3 has 1 free section and pretext 4 has no free sections or diffused tumor burden in all sections of the liver. Additionally, this staging system is the framework for the treatment algorithm. Pretext 1 and 2 tumor, if there was greater than a centimeter margin from the middle hepatic vein and the portal bifurcation, you could offer, you should be considered for resection and diagnosis. Patients with bigger tumors that did not get resected would then be risk stratified to intermediate, low risk or intermediate risk. And then depending on annotation factors, if they had a positive, they could be in high risk. Pretext 3 and 4 is the big game changer. This change in practice is what improves survival rates for patients with hepatoblastoma from less than 30% in the 70s and 80s to between 80 and 90% today. And we had surgical guidelines for pretext 3 tumors and teach these patients to generally be biopsied in diagnosis and then started on neoadjuvant chemotherapy. And then depending on how they responded, be ready to send them to a transplant center or a center that has expertise to do more advanced liver resections. And then pretext 4 patients had a much more complicated schema. And the reason we had to do that was some of these patients needed to be transplanted. So if you have pretext 3 or 4 liver disease, there are specific indications to transplant, mainly if it's unresectable disease, whether it's unsafe to resect, or a resection would leave the patient with an inadequate liver remnant. But the main thing to get out of this is it's important to consult a transplant center. And the earlier the better. You refer these patients to liver transplant programs much earlier. And this is why we see so many more patients nowadays than perhaps when Fred and Maria were running the program 15 years ago. Because teams are trained to recognize tumors and send them to a center that offers transplant or can do aggressive resection. And if you'd like more information on the survival benefits of liver transplantation, you guessed it, it's in the link below. After clarifying the pretext stages you're not quite done, there's an ongoing trial to better delineate the treatment algorithms for each of the stages of hepatoblastoma. Sheik is responsible for an ongoing multinational trial called FIT, or the Pediatric Hepatic International Tumor Trial. The aim is to identify high and low risk features for each of the pretext stages. The FIT study has been going on now for three years. We've had the privilege of contributing to it in terms of its development. That's why we see a lot of tumors here. The treatment for hepatoblastoma has really evolved significantly. This is the schema for the study. This is the schema for hepatoblastoma and we'll break down this down a little bit more in just a second. No worries. Details for this study and others can be found in the links below. Management of these malignant liver tumors continues to improve through improved treatment, multidisciplinary teams, international collaborative efforts, and research. Thank you for joining us for this update on pediatric hepatoblastoma. And remember to check out the link below for the full lecture. Follow us on social media. Subscribe to our YouTube page. Download the Stay Current Pediatric Surgery app. It's in the Apple App Store. It's in the Google Play Store. Or if you're listening to us on an audio podcast, leave us a comment, a suggestion, a rating. It helps with the algorithm. And remember, knowledge should be free. --- Not medical advice · citation policy: https://library.globalcastmd.com/ai