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Hepatoblastoma: Update Course 2014

Video Published 2019-01-11 Updated 2023-07-27

Timestops (7)

Introduction to the goals of the presentation

Introduction to the goals of the presentation: staging, resection recommendations, and controversies.

Presentation of the first scenario

Presentation of the first scenario: a 2-year-old boy with symptoms and imaging findings suggesting hepatoblastoma.

Explanation of Pretext staging and how to correctly stage th…

Explanation of Pretext staging and how to correctly stage the described scenario

Explanation of Post-text staging and when this would be appl…

Explanation of Post-text staging and when this would be applicable

Audience poll and discussion on appropriate management strat…

Audience poll and discussion on appropriate management strategies for a Pretext 2 tumor, including when to proceed with …

Discussion about the importance of margins during surgery an…

Discussion about the importance of margins during surgery and the controversy surrounding what constitutes a negative ma…

Brief mention of differences in philosophy between approache…

Brief mention of differences in philosophy between approaches in the United States versus Europe for treatment.

Topic Overview

A surgical education session on hepatoblastoma staging and management, focusing on the PRETEXT/POSTTEXT staging system based on segmental liver anatomy and imaging prior to chemotherapy. The discussion covers current COG recommendations for resection timing, the role of neoadjuvant chemotherapy (with most tumor shrinkage occurring within two cycles), and the critical decision points between extended hepatectomy versus liver transplantation. Key controversies include the definition of adequate surgical margins, the timing of transplant referral, management of pulmonary metastases, and whether complex liver resections should be centralized at transplant centers.

Key Takeaways

Most hepatoblastoma shrinkage occurs in first 2 cycles; if not resectable then, evaluate for transplant rather than more chemo. (10:26)
Chemotherapy shrinks tumor bulk but does not improve vascular margins—tumors remain adherent to vessels despite size reduction. (10:26)
Failed resection followed by rescue transplant has worse survival than planned upfront transplant; refer early to transplant centers. (14:52)
Extended hepatectomies with vascular reconstruction have inferior survival compared to transplant; heroic resections now discouraged. (12:33)
PRETEXT staging tends to overstage due to mass effect mimicking vascular invasion on imaging; true resectability determined intraop. (1:41)

Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Speaker 1 — guest
Speaker 2 — host
Speaker 3 — guest
Speaker 4 — guest
Speaker 5 — guest
Speaker 6 — guest
Speaker 7 — guest
Speaker 8 — guest

Chapters

0:00PRETEXT Staging System and Case Presentation — Introduction to PRETEXT staging for hepatoblastoma based on segmental liver anatomy prior to chemotherapy, with discussion of a case involving multifocal disease and staging criteria including vascular involvement.
3:52Management Strategies and Chemotherapy Response — Debate over upfront resection versus neoadjuvant chemotherapy for PRETEXT stage 2 tumors, discussion of tumor shrinkage patterns after chemotherapy, and the observation that most volume reduction occurs within two cycles without improving vascular margins.
12:08Transplant Versus Extended Resection — Discussion of survival outcomes comparing extended hepatectomy to liver transplantation, the poor outcomes of rescue transplants after failed resection, and debate over whether complex liver resections should be centralized at transplant centers.
18:56Surgical Decision-Making and Biopsy Practices — Practical discussion of when non-transplant center surgeons should refer patients, criteria for proceeding with resection, and varying practices regarding pre-treatment biopsy (core needle versus open wedge).
24:49Pulmonary Metastases and Future Research — Controversy over timing of pulmonary metastasis resection relative to hepatectomy, management of stage IV disease with lung involvement, and introduction of the PLUTO international collaborative study.

Key claims

1:41PRETEXT staging stands for pretreatment extent of disease and is based on segmental liver anatomy prior to chemotherapy — Speaker 1
1:41PRETEXT staging has a tendency to overstage because it is based purely on imaging and bulky tumors make it hard to assess true vascular invasion versus mass effect — Speaker 1
1:41POSTTEXT refers to extensive disease after neoadjuvant chemotherapy has been given — Speaker 1
3:52Key staging annotations include involvement of the retrohepatic cava or hepatic veins and the portal vein bifurcation — Speaker 1
7:30COG recommendations state that PRETEXT stage 2 tumors with no vascular involvement and achievable 1 cm margin can be resected upfront without neoadjuvant chemotherapy — Speaker 1
7:55COG feels that PRETEXT stage 1 and 2 tumors do not necessarily need referral to centers with liver resection expertise if the local surgeon feels competent — Speaker 1
10:26Most tumor shrinkage from chemotherapy occurs within the first two cycles — Speaker 1
10:26Current recommendations are that after two cycles of chemotherapy, if the tumor has not shrunk to a resectable point, the patient should be evaluated for transplant — Speaker 1
10:26As hepatoblastoma tumors shrink with chemotherapy, they do not shrink away from the vascular supply, so chemotherapy does not improve vascular margins — Speaker 1
12:08The goal surgical margin for hepatoblastoma resection is 1 cm — Speaker 1
12:33Survival in patients undergoing extended or heroic resections (tumor liver explants with back table resection and reimplant, portal vein reconstructions, hepatic vein reconstructions) is not as good as transplant survival — Speaker 1
12:33There has been a move away from heroic resections for hepatoblastoma — Speaker 1
14:52Patients who undergo failed resection followed by rescue transplant have worse survival than patients who undergo planned transplant upfront — Speaker 1
15:48Patients should be referred to a transplant center early even if they may not ultimately need transplant, to have pre-transplant evaluation completed and be plugged into the system — Speaker 1
16:38Primary transplant patients with hepatoblastoma do surprisingly well despite immunosuppression against rejection in the setting of cancer — Speaker 4
18:56For non-transplant center surgeons, criteria for proceeding with resection include feeling 95% confident of success and ability to perform an anatomic resection with good margin — Speaker 8
19:54Disease close to hepatic veins, disease extending across the liver, or involvement of the portal vein should prompt referral to a transplant center — Speaker 8
21:52The truth about resectability is determined at the time of operation despite all available imaging — Speaker 1
22:13In Europe, all liver tumors receive chemotherapy upfront before surgery because it results in smaller, easier-to-resect tumors — Speaker 4
23:53Core needle biopsy for hepatoblastoma typically requires about 10 passes through an area that includes normal parenchyma and tumor — Speaker 1
24:49There are two camps regarding pulmonary metastasis treatment: one advocates resecting metastases upfront before hepatectomy, the other suggests waiting until after hepatectomy because liver regeneration growth factors may stimulate previously unrecognized lung sites — Speaker 1
24:49There is no good data on either side of the pulmonary metastasis timing debate, with both approaches limited to a handful of patients — Speaker 1
26:11Papers on stage IV hepatoblastoma with lung metastases are limited to cohorts of less than 20 patients, making it hard to draw good conclusions — Speaker 1
26:11There may be reporting bias in transplant outcomes because successes are reported but not all failures — Speaker 1

Cases discussed

0:00Two-year-old boy with hepatoblastoma presenting with multifocal liver disease
3:52Hypothetical PRETEXT 2 hepatoblastoma case for management discussion

Points of disagreement

5:03Management of PRETEXT 2 hepatoblastoma: upfront resection versus neoadjuvant chemotherapy
- Speaker 1: COG recommends upfront hepatectomy for PRETEXT 2 with clear 1 cm margin and no vascular involvement
- 60% of audience favored giving chemotherapy first
17:05Whether complex liver resections should be centralized at transplant centers
- Speaker 4: Complex resections should be done at centers that do pediatric liver transplants, similar to UK approach
- Speaker 8: Non-transplant center surgeons can proceed if confident of success with anatomic resection and good margin
24:49Timing of pulmonary metastasis resection relative to hepatectomy
- Resect pulmonary metastases upfront before hepatectomy
- Wait until after hepatectomy because liver regeneration growth factors may stimulate previously unrecognized lung sites

Open questions

What is the optimal timing for resection of pulmonary metastases in stage IV hepatoblastoma - before or after hepatectomy?
Should patients with stage IV disease who have complete response of lung metastases to chemotherapy proceed with transplant or resection?
How should patients with stage IV disease be managed when all disease responds to chemotherapy except one or two lung nodules?
What is the true significance of microscopic positive margins in hepatoblastoma resection?
Do patients who receive neoadjuvant chemotherapy have equivalent survival to those who undergo primary resection?
Should all complex hepatoblastoma resections be centralized at liver transplant centers?
What is the optimal imaging modality for PRETEXT staging - CT with multi-phase contrast or MRI?

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Written for:

Hepatoblastoma Resection: When Chemotherapy Helps and When It Doesn't

The episode's teaching points arranged as a structured lesson, building from the basics up to the finer points. Written by Kai from the episode transcript and reviewed before publishing.

For trainees · Teaching arc · AI-written, human-reviewed

Staging Drives Early Decision-Making

PRETEXT staging—pretreatment extent of disease—is performed before any chemotherapy and relies entirely on imaging of segmental liver anatomy 1:41. This creates a predictable problem: PRETEXT tends to overstage because bulky tumors create mass effect that can mimic true vascular invasion on cross-sectional imaging 1:41. Understanding this limitation matters because it affects the threshold for attempting resection. A tumor that appears to encase vessels on imaging may prove separable at operation, but only if the surgeon has the experience to recognize when imaging has overcalled the invasion. POSTTEXT staging, performed after neoadjuvant chemotherapy, reflects the tumor's response to treatment and provides a more accurate picture of what the surgeon will encounter 1:41.

Chemotherapy Shrinks Volume, Not Vascular Margins

Most hepatoblastoma volume reduction occurs within the first two cycles of chemotherapy 10:26. This is the window that matters—if the tumor has not become resectable by cycle two, current recommendations are to evaluate the patient for transplant rather than continuing chemotherapy in hopes of further shrinkage 10:26. The reason is anatomic: "as the tumor shrinks it doesn't really shrink away from the vascular supply" [q4]. Chemotherapy reduces overall tumor bulk but does not improve vascular margins 10:26. A tumor that abuts the portal bifurcation before chemotherapy will still abut it after four cycles. This means chemotherapy can convert an unresectable tumor to a resectable one by reducing volume, but it will not convert a close vascular margin to a wide one. The goal surgical margin remains 1 cm 12:08, and chemotherapy does not reliably deliver it where it was absent at presentation.

Heroic Resections Have Been Abandoned for Good Reason

Extended resections—tumor liver explants with back-table resection and reimplantation, portal vein reconstructions, hepatic vein reconstructions—were once attempted in an effort to avoid transplant 12:33. The survival data ended that era. Patients undergoing these heroic resections do not survive as well as patients undergoing planned transplant 12:33, and there has been a clear move away from such procedures 12:33. More importantly, patients who undergo attempted resection that fails and then require rescue transplant have worse survival than patients who undergo planned transplant upfront 14:52. This creates a bright-line rule: if you are not 95% confident of achieving a complete resection with adequate margin, refer before attempting resection 18:56. The penalty for a failed attempt is not just the morbidity of two operations—it is measurably worse oncologic outcome.

Early Transplant Referral Is a Hedge, Not a Commitment

Patients should be referred to a transplant center early even if they may not ultimately require transplant 15:48. The goal is to have the pre-transplant evaluation completed and the patient integrated into the system so that if resection proves impossible, transplant can proceed without delay. Primary transplant patients with hepatoblastoma do surprisingly well despite the theoretical concern of immunosuppressing a patient with active cancer 16:38. The key judgment for the non-transplant surgeon is recognizing which cases warrant referral: disease close to hepatic veins, disease extending across the liver, or involvement of the portal vein should all prompt early consultation 19:54. The truth about resectability is determined at operation despite all available imaging 21:52, but the decision about where that operation should occur must be made beforehand.

The Margin Question Remains Unsettled

What constitutes a true negative margin is more ambiguous than the 1 cm goal suggests. Cautery artifact may obscure margin status. CUSA devices create 5–6 mm of tissue division width that may be aspirated rather than submitted for pathology. Surgeons describe shaving tumors off vessels, raising the question of whether such a dissection plane represents a true negative margin or simply an absence of gross residual disease. These are not semantic distinctions—they reflect genuine uncertainty about what degree of microscopic involvement is compatible with cure in a chemotherapy-responsive tumor. The discussants emphasized that the significance of a microscopically positive margin in hepatoblastoma is unclear, particularly when the tumor has responded well to chemotherapy. This is not an invitation to accept inadequate resections, but rather an acknowledgment that the binary language of "negative margins" may not capture the biology of this disease.

Takeaways from this story

Chemotherapy shrinks hepatoblastoma volume but does not improve vascular margins—tumors remain adherent to vessels despite size reduction.
Failed resection followed by rescue transplant yields worse survival than planned upfront transplant; refer early if not 95% confident.
Most tumor shrinkage occurs within two cycles of chemotherapy; evaluate for transplant if not resectable by then rather than continuing chemo.
PRETEXT staging tends to overstage because imaging cannot distinguish true vascular invasion from mass effect in bulky tumors.

Hepatoblastoma Resection Strategy: When to Operate, When to Transplant

The points where the speakers disagreed, with each position presented side by side. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Points of disagreement · AI-written, human-reviewed

The Central Question

For PRETEXT stage 2 hepatoblastoma with no vascular involvement and an achievable 1 cm margin, should the tumor be resected upfront or should neoadjuvant chemotherapy be given first? COG guidelines recommend proceeding directly to hepatectomy in these cases 7:30, yet when the audience was polled during this discussion, 60% favored giving chemotherapy first.

The Case for Upfront Resection

One discussant argued that COG recommendations are clear: PRETEXT stage 1 and 2 tumors without vascular involvement do not necessarily require referral to centers with liver resection expertise if the local surgeon feels competent 7:55. The rationale rests on avoiding the toxicity and immunosuppression burden of chemotherapy when a complete resection is technically feasible. Patients who undergo successful primary resection without neoadjuvant therapy may avoid both chemotherapy exposure and the risk of tumor progression during treatment.

The threshold for proceeding is high. For non-transplant center surgeons, the criteria include feeling 95% confident of success and the ability to perform an anatomic resection with good margin 18:56. Disease close to hepatic veins, disease extending across the liver, or involvement of the portal vein should prompt referral to a transplant center 19:54.

The Case for Neoadjuvant Chemotherapy

The European approach treats all liver tumors with upfront chemotherapy before surgery because it results in smaller, easier-to-resect tumors 22:13. Most tumor shrinkage occurs within the first two cycles 10:26, and current recommendations state that after two cycles, if the tumor has not shrunk to a resectable point, the patient should be evaluated for transplant 10:26.

The critical limitation of neoadjuvant therapy, however, is that as hepatoblastoma tumors shrink with chemotherapy, they do not shrink away from the vascular supply, so chemotherapy does not improve vascular margins 10:26. The benefit is volume reduction, not improved resectability at critical vascular structures.

The Rescue Transplant Problem

Both positions converge on one point: failed resection followed by rescue transplant produces worse outcomes than planned transplant upfront 14:52. One discussant noted that attempting resection without achieving complete removal and then requiring transplant does not benefit the patient because survival is worse 14:52. This creates asymmetric risk. The surgeon who attempts resection must be nearly certain of success, because the penalty for failure is not simply proceeding to transplant—it is proceeding to transplant with diminished survival.

Survival in patients undergoing extended or heroic resections—tumor liver explants with back table resection and reimplant, portal vein reconstructions, hepatic vein reconstructions—is not as good as transplant survival 12:33. There has been a move away from heroic resections for hepatoblastoma 12:33. Yet primary transplant patients do surprisingly well despite immunosuppression against rejection in the setting of cancer 16:38, which shifts the risk calculus toward earlier transplant referral for complex cases.

Where They Agree

Both approaches recognize that "the truth is at the time of the operation whether it's resectable or not" [q7], despite all available imaging 21:52. Both agree that patients should be referred to a transplant center early even if they may not ultimately need transplant, to have pre-transplant evaluation completed and be plugged into the system 15:48. Both agree that the goal surgical margin is 1 cm 12:08. And both agree that attempting resection without high confidence of success is a serious error.

The disagreement is not about whether to attempt impossible resections—it is about whether chemotherapy should be given routinely to PRETEXT 2 tumors that appear resectable on imaging, knowing that imaging may overestimate vascular involvement 1:41 and that some tumors will prove easier to resect than anticipated.

What Would Resolve It

The discussants did not identify a specific trial or dataset that would settle this question. The challenge is that PRETEXT stage 2 hepatoblastoma without vascular involvement is uncommon enough that single-center experience cannot answer whether upfront resection or neoadjuvant chemotherapy produces better long-term survival. The international PLUTO consortium (Pediatric Liver Unresectable Tumor Observatory) was mentioned as an effort to pool data across centers, but whether it will capture sufficient PRETEXT 2 cases with granular surgical decision-making data remains unclear.

What is clear is that the 60% of the audience who favored chemotherapy first were not following COG guidelines as written, suggesting either that the guidelines have not persuaded the field or that practicing surgeons perceive risks in upfront resection that the guidelines do not fully capture.

Takeaways from this story

Failed resection followed by rescue transplant yields worse survival than planned upfront transplant—confidence threshold for attempting resection must be very high
Chemotherapy shrinks tumor volume but does not improve vascular margins; most shrinkage occurs within first two cycles
Extended resections with vascular reconstruction produce inferior survival compared to transplant; field has moved away from heroic resections
COG recommends upfront resection for PRETEXT 2 without vascular involvement, yet 60% of polled audience favored chemotherapy first

Keywords

hepatoblastoma jaundice abdominal pain lethargy biliary atresia hepatectomy liver transplantation right trisegmentectomy tumor liver explant with back table resection portal vein reconstruction hepatic vein reconstruction central hepatectomy core needle biopsy open wedge biopsy laparoscopy

Hashtags

#hepatoblastoma #pediatriconcology #childcancer #livercancer #pretextstaging #posttextstaging #COG #childhoodcancer #LiverTumors #PRETEXStaging

Transcript

So, um, yeah. Here we go. Okay. So, um, my goals with this um were first of all, just to sort of blow through some quick stuff which is sort of an update on um staging because I don't know that um everyone necessarily is well informed about pretext and post text um staging of hepatoblastomas. And then after that get into um some of the current recommendations from COG in terms of um resections of tumors based on that staging. And then hopefully at the end, um get into the area of controversy and with hepatoblastoma, we don't really have um the the areas of controversy aren't don't have studies that are well-powered like you know Wilms tumor. And so, um it really is I think um maybe an area for more debate and um probably has a lot of opinion built into it. So um, so this is the first scenario that I um put up to cover this um staging issue and um so you have a two-year old boy who presents the emergency room with abdominal pain, lethargy, five pound weight loss, jaundice and dark urine. Um on exam has a large abdominal mass and uh CT AWB pelvis is done with IV contrast. shows the image, it shows multifocal disease affecting um all sectors of the liver with an elevated AFP. So the presumptive diagnosis of hepatoblastoma is made based on that. Um how would how would people um uh stage this patient? So 75% are saying four. Okay. 80%. Okay, so so yeah, so um so the the issue with staging so so pretext staging um sorry. Pretext staging stands for pretreatment extent of disease and um this staging system is based on segmental liver anatomy and and the important thing about this is this is staging prior to chemotherapy has been given. So so this patient is definitely a pretext and um and then you know I tried to make it relatively straightforward has one or four and you know with multifocal uh disease you're clearly not going to be a one. Um the interesting thing about pretext staging is that um it has a tendency to overstage. Um because this is based purely on imaging and many of these tumors are so bulky, um it it's hard to assess whether or not there's true vascular invasion sometimes or if it's just big uh mass effect of tumor so pressing on portal uh vein, hepatic veins, things of that nature. Post text uh in contradistinction refers to extensive disease after neoadjuvant chemotherapy has been given. So um these are just some images that I borrowed from um COG website you can see that pretext stage one involves one sector of disease. Um two involves uh two sectors the disease of of the liver. Um three is three sectors. Um one specifically spared because it's not um necessarily three contiguous and then four is um the the image that showed which is multifocal disease. So um so the next question for the audience would be which of the following other considerations are not factored into staging. there. Going to pull up in a second. We can blast through this. I guess there's an issue here. Yeah. Yeah, okay, all right. So basically um extramedullary hematopoiesis is not involved. I mean there are a bunch of additional annotations that are made. I think the two key ones that people look at are involvement of the retrohepatic cava or um hepatic veins and then um the portal vein uh bifurcation whether or not that's involved. Okay, so this image isn't coming up clearly but um for some reason the slides not loaded right but the the image that I have um is to say that you have a tumor that's classified as a pretext two, there's no involvement of the retrohepatic cava or the um hepatic veins and the portal vein confluence is spared. So appropriate management um strategies for this patient would include the following. Is that up? People can actually pull off of it or Yeah, Mark, can we get these polls or We're not seeing them. Sorry Matt, we'll try to get the. So that's okay. So so we can just sort of go through it. I mean um the the question is really I think boils down to um what would people do in the setting of a pretext two with no involvement of the um hepatic veins or portal veins. So maybe while we're waiting for that to come up we could just. Yeah and and Mark if you can't find the polls at least just write A B C D. Even if you can't. They're right next to you but the one that's just right here, that's not opening. Um the last two we don't see. There we go. opening. You have to scroll up to it. So you had commented before about the fact that the the pretext overstages uh patients and uh in a case like this would you base this on uh what kind of imaging studies do you get to make this kind of a decision? Yeah, so that's a good question. So I think I I think there's um people favor different things. Some people look at um CT um with multi contrast phase and other people favor MR. Um and I think that uh I I don't know that one's been shown to be superior. Um but I think clearly cross-sectional imaging is absolutely critical to to staging these. So does anybody get other studies other than would you get a CT or an MR? Steve, would you use CT or MR in your institution? We're using more and more MR. MR. MR. Both. Both. Yeah, well that's one of my concerns is frequently we end up with. Yeah they all end up with both somehow. Any role for pet scan? I don't know that Pet's really I mean I don't think Pet's going to be useful to to to use. Got to have one available in your hospital. However the converse is also true if you have one available then everybody. Right well I mean our we don't even have the oncologist are just getting pet scans in more and more patients and diffuse area and and it's like you have information and kind of what do you do with it type of thing. Because if you're going to make a decision based on the pretext uh staging you have to be able to trust your imaging uh or your imaging may play a factor in the answer to this question as to whether you think you would proceed directly to surgery or not. Absolutely. And so the the audience 60% is going with B. give chemo first. Give chemo first. So yeah, so this so this is sort of an interesting thing because if you look at the um recommendations um by COG, they would say that you know if you can get um a clear margin um on a pretext two, um you know a good margin a 1 cm margin doesn't involve any of the vascular structures that you just go for the um hepatectomy up front. Um and and you know and and I think that they're pretty clear also that um they feel that this is not something that necessarily gets referred to an area of you know expertise and liver resection that the ones and twos um if the surgeon feels competent should be resected locally which is sort of interesting. Well your diagram showed the tumor pretty close to the portal vessels. So I think that might have scared some people. You want a margin. Yes, absolutely. So so and and this issue of margin I bring up later on um because I think it um there's some controversy there too um in terms of what people refer to as a negative margin. Um people talk about shaving um tumors off the vessels and whether or not that's a negative margin or whether um you know cautery artifact actually obscures the fact that you may or may not actually have a negative margin. People use the cusa to come through the um liver that there's actually, you know some width there probably, you know five, six millimeters of tissue division and so does some of your tissue margin actually go um up the suction device and so you know. But some of the some of the decisions on this may also be uh related to differences in philosophy in the approach in the United States versus the approach in Europe. Is that true? Um certainly for Wilms tumor there is the European approach is to get chemotherapy first no matter what. Yes, um I think that's true. I think that um interestingly the um it's from from what I've uh read it seems to me that the um North American and European um approaches seem to be converging similarly. I think that's true. I'm people like Rebecca Myers who's has spent years getting those uh getting the two groups to come together on uh on an approach because as you mentioned at the outset they're very the what 100 hepatoblastomas in the country. You're so the only way uh to make progress with this is to to have a combined approach with the European groups and Rebecca spent a lot of time getting that organized. Um so another thing I think that's worth mentioning here is just um this issue of tumor shrinkage that we were talking about chemotherapy effect and um with hepatoblastoma I think the the recommendations are pretty clear now that most of the shrinkage of the tumor volume that you're going to get is going to be within the first two cycles. And so there are some, you know groups that in the past have advocated for giving more chemo before proceeding with resection and I think now the the recommendations are pretty clear that after two cycles if you haven't shrunk to a point that makes the tumor resectable that the patient be evaluated for transplant. Um there's been some nice studies done on that. The other interesting thing about that um I think the group out of Vanderbilt looked at this but the the the ways in which the tumors shrink is um something to consider because um you know some people would would say well you know if you give more chemo maybe you get more of a vascular margin. It turns out that as the tumor shrinks it doesn't really shrink away from the vascular supply um all that all that much and so um so that's sort of you know shrinks down in terms of size. It doesn't give you more of a margin. Exactly. So maybe maybe you preserve but not vascular margin. So don't do so don't do chemo for the vascular margin. Exactly. Wow. So so when you evaluate imaging do you want a 1 cm margin? Is that what you'd recommend to the audience? Yes, I would say a 1 cm margin is is the goal, yeah. Now, if you if you look at the survival results I'm sure you're probably going to show the data if you can get you can get the thing out. Don't those patients do better? They tend to do better and you know and we'll we'll go through a little bit of this because I think some of that controversy exists on who has better survival? Um somebody who has a very extended resection like you know a right trisegmentectomy or somebody who goes on and just gets a transplant. And I think um in years past it was felt that um very extensive or quote unquote heroic resections um were undertaken you know things like um tumor liver explants with back table resections and then reimplants, portal vein reconstructions, um hepatic vein reconstructions and I think the the take home message from that after it having been done for years um is that the survival in those patients is actually not as good as the transplant survival. So there's been sort of this move away from some of these certain heroic resections. But I think the other the other question is chemo, you know, if you if you're going to commit them to two rounds of chemo, those patients they get chemo don't have as high survival as a primary resection or do they? That's the question. I was just going to say prior to that with the previous discussion about you should if you can get it out you should try to get it out because they have a better survival. But if you try to get it out and you can't and they require then you have not done them a favor because then they have worse survival. So you have to be pretty confident that you're going to be successful in getting out out with a margin is that what you're saying? Or with a margin although I you know the data better than I but the the significance of a microscopic margin is unclear. What's the incidence of the ones that uh they recently out for a liver transplant, getting a liver transplant. Right. Uh what is the availability of organs because you have to factor that into which course you're going to take. Yeah, and and I think that that's an important um point is you know when you're looking at well I'll put it on this but um wh when when you're looking at patients that are going to go the route of transplant versus a right trisegmentectomy, you have to say well, you know if we put them into the transplant group, number one, what's organ availability like? Number two, you've committed now a very young child to lifelong immunosuppression which is not um an entirely benign thing. So um but to Dr Van Almon's point, you know these what they call rescue transplants that there's a failed resection that then goes on and needs a transplant. Those patients it's pretty clear do fair worse off than patients that are a plan transplant up front. Yeah, so that's been my rule of thumb. If I don't think I can get a margin and they don't respond to chemo so I can get a margin, they should be referred for transplant before resection. Is that still true? Yeah, so so the the It's that's a critical uh point. Yeah, I I think the current thinking is that you refer to the transplant center early. Doesn't necessarily mean that the patient's going to go towards transplant. You still might give two cycles of chemo and see where you end up, but at least you want the patient plugged into the system, have all the pre-transplant evaluation done sort of have them sort of plugged in in the event that they need to go that route. I think the concept is that the patients in whom transplantation is done as salvage therapy, don't do as well as a primary transplant. That's very clear. Yes. The primary transplant patient does surprisingly well. You know doing a transplant and immunizing somebody against rejection who has cancer has always been a a major question. Is that a safe thing to do? And it seems as though uh the liver patients do extremely well following the transplantation despite being immunosuppressed. And so so should all these complex liver resections for hepatoblastoma be done in here we go back again. You took word right out of my mouth. I was going to ask exactly the same question. Should they be done in centers that do pediatric liver transplants? Uh I think that's the way they do it in the UK. I mean I I think that's a critical obviously a very complicated uh question in terms of Same with biliary atresia. Yeah. But biliary atresia is different. Uh that's that operation it's not a big operation. It's easy to do. But the transplant is. So they they all those patients who have biliary atresia are referred up front to a transplant center. See, but they have time. I mean biliary atresia is is slowly get sick over time. This is different. These people, these children are going to get sick really quickly. If you're in a place that doesn't have a transplant program, you can't do all the uh arranging uh for a potential transplant. I'm not advocating total uh specialization for everything we do, but these are issues that are in the current year and put in a look at. So, so for the people who are at non-transplant centers, do you have criteria that you use to How would you manage a patient who has a questionably resectable tumor? Would you do it? Appears to be inactive and will be ended soon. you're on. Saved by the bell. I'm back next week. any key your telephone. Are we off the air? We just got a message. Audio feed. I think the audio's down. Okay. Okay. So so non-transplant center surgeons, I don't even know how many we have. Oh, that's that'd be yeah. So so what how do you manage? Because you do liver liver resections. So I I I think if there's I I need to feel very comfortable that I can do the resection. I mean, you know, first of all you have to decide are you comfortable doing major liver resections. assuming that then I need to feel very comfortable before I start the procedure that I'm going to I'm going to succeed and that's not that you couldn't have a complication or problem but that within my skill set there's a 95% chance that we're going to we're going to do that. And if I don't feel confident with that then I will refer them to a transplant center because to be in between is I think you've you've done a significant disservice. Risky business. Yeah, for us I think if you can do an anatomic resection with a good margin, I think that's fine you would proceed down. But if it's anything close to the hepatic veins, if you have disease that extends across the liver or anything in the portal vein, you should send it. And would you give pre-op chemotherapy to get to that point or would you send them before? Well you know we've we've actually had three in the last two months and then we go six months and don't really have them. I think it's case by case, but we have sent sent them early when we just as surgeons felt like um they couldn't be resectable and I I would say we've had a couple that we sent to your institution for that and you guys were able to safely resect it um because you probably have a little bit more confidence and do a lot more uh liver resections and the patient never did need a turn. So I think that's another value. But but Deb brings up another I don't want to be creating all these paradigms, but brings up another situation that's real and that is that the liver transplant surgeon has a lot of experience with operating on the liver. And so if there are issues being close to the portal vein, being close to the hepatic veins, it's not so bad to have somebody like that that might be available to help the pediatric surgeon who's done a lot of liver resections do it even better in this day and age. And there's no reason that the scans can't be sent to somebody from that center to have a look at to help you make the decision if that's somebody they should see earlier. There's nothing like looking at the anatomy with a belly open. we do all these laparoscopic ways. Touch. Too short. So yeah, well despite despite all the imaging that that that's available uh I think anybody who's done a fair number of liver resections will tell you that the truth is at the time of the operation whether it's resectable or not. And despite of all that. And uh people in Europe early on will give all liver tumors chemotherapy up front and then do the surgery because it's easier it's smaller tumors. think perhaps can avoid trans. Yeah. And that was their reasoning for it. But in our country avoiding a transplant and lifelong immunosuppression, you know is something if you can avoid it, it's probably okay. So I I I would not hesitate doing a trisegmentectomy for cure. And perhaps in certain centers I know Mike has had some experience doing central hepatectomies, which is a difficult operation, but he's done them successfully and the patients have been cured without requiring a transplant. So the you know there's a lot of thought process that goes into it. But certainly those uh procedures should be done by experienced personnel. We've had a couple kids who are over the age of three, like eight and nine with hepatoblastoma. Likewise we've had hepatoceller. Do you biopsy kids outside do you have a do you biopsy before you treat with chemo just to be sure it's really a hepato one or the other and how do you do that? Do you do it open or do you do it Yeah, so so I think um based on what I've read, um it seems that the field is sort of divided. So there are some people that would say you could do core needle biopsies and they usually recommend I think about 10 passes of the needle and that you're supposed to go through an area that includes normal parenchyma and that um and obviously you know get tumor uh as well. Our practice has not necessarily been to do that. We typically do an open wedge if we're you know to to get tissue but I'd be interested to see what other people do. I think we got to we're going to have to. Yeah is there any final final things that you wanted to hit before we move? But you've got you know in the last final 60 seconds. Well, the only other thing I would say is that I think a a topic of debate um is really this other issue of pulmonary metastasis in hepatoblastoma and the treatment of the pulmonary mets because um there are two different camps on this issue and one would say you know if you um you should resect the metastasis up front and then go on to do your um hepatectomy after. There's others that would say um well, you know the the growth factors that are secreted by the liver as it regenerates are going to stimulate growth of other sites in the lung that may not have been previously recognized. And so that maybe there was a benefit to waiting and doing it after because then you can actually clear them of disease. But I I don't know that there's good data on either side of this. all of it's limited to a handful of patients in in both sets. Matt, one last question on that. The the thought had been that they treated with chemotherapy and have stage four disease and the tumor in the lung disappears on chemotherapy alone. Do you go ahead and do the transplant? Or do you do the resection? And what happens then if you have everything that responds but you only have one or two nodules left in the lung that didn't respond to chemotherapy, would you operate on those patients? Right, and so um so again, that's um a small subset of of patients and the the papers that are out there are limited literally to a cohort of I think less than 20 um where this has really been looked at. So it's hard to really draw any good conclusions. Um the the other piece to this is that there may be some selection bias in what's reported or reporting bias because if you have a good response to chemo and you go on to do a transplant and your patient is a long-term survivor, that's a patient that gets reported as a success. But I don't know that the um all the failures are being uh reported. So to that point, um there's um a group and I think this would just be the last little thing to mention. there's a group um called um let's see if we can go back to it. Pluto, um this pediatric liver unresectable tumor observatory and this is um an international group and and they're really seeking to answer those questions because it's not answerable at individual centers or even multi centers within I think North America or Europe. This really takes, you know a global initiative to answer some of these questions and so they're looking at all these things, but I think it's probably going to be several years before we can really answer any of those questions. Okay. Thank you very much.

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