All right. Um, we're gonna move on to our next session. Which is on Wilm's tumor. Uh, Justin Huntington is gonna be moderating, and Justin's from Akron Children's, and we have Bargava Mulaui from Kansas City Mercy, um, City Mercy Children's Kansas City. I always get it backwards. Children's Mercy Kansas City. Children's Mercy Kansas, and, uh, we have Doctor, uh, Michael Nightingale coming beaming in from the Royal Children's Hospital Melbourne, Australia. Australia. So, um, this is something we've been, for those of you who have been with us for 12 years, you know how many years we've been trying to get the audio to work where we can truly bring in all the international speakers as if they're here. We're we're trying it today, um, hopefully it'll work because it changes everything if we can bring in international faculty, uh, for the future courses. So, uh, Justin, it's all you. All right, um, you wanna start us off. All right, let's start with the next slide. Let's start with the uh first case to warm us up. Um. So we have a 19 month old male present with a newly noted right-sided abdominal swelling, decreased PO intake, ultrasound, CT scan, very suspicious for Wilms. This is what the scans look like, um, pretty classic. Um, next scenario is an audience poll. So nice. Next slide. So, uh, we can, while we wait for that, go with some audience questions, uh, and also online if, uh, what would differ from CIA protocols from what we do with our quag protocols. Thank you for doing 3 choices cause that increases my odds to 33%. So what Any comments while we're waiting? the chest. Question was negative imaging of the chest, negative. No, nothing else in the abdomen. Any other Information anyone needs. Strong opinions? And remember The the polls will keep coming in over the next 20 seconds, but you can, it looked like we could tell there was a split. Oh, we have a question, legitimate question. Not to sound smart. Any extension of any tumor into the IBC no tumor thrombus. Great questions. No tumor thrombus. We'll, we'll get there. All right, so I think we can look at those. OK, let's look at the audience poll. Next slide. So it's back there. It looks like. Um, Most would do a. Close 2nd chemotherapy pretty split, yeah, really close, and then 3rd would be biopsy with chemotherapy. Yeah, this should be pretty straightforward where we should proceed with resection. Um, in this particular case, but let's look at the questions and see, um, uh, why that would any of that would change with different scenarios. Uh, so looking at the clinical scenario, so we did successfully resect that margins were negative, mass weighs about 728 g. Path is also, you know, triphasic but still has more blasphemy predominance, kind of stage two because of the invasion of the renal, sinus, lymphovascular spaces, favorable histology. But there's some cytogenetics that we also have. It's negative for loss of heterozygosity, 1P16Q. Now, this always sort of confuses me, so I want to, you know, target, uh, surgeons. What do we need to know about uh loss of heterozygosity and what significance of gain of 1Q. Any questions? If this were to be something different. If there is a. If it's positive for loss of heterozygosity, 1P16Q, did, does anyone want to know that information? What you would do next? Let's go to the next slide. Coca, no, no comments. Figured you'd be jumping off the table with comments, no. So based on that before you go further, I do think, you know, when you look at the audience poll, the reason it was pretty split is because this is an international poll, right? And so our European colleagues would oftentimes um offer chemotherapy, no biopsy, and of course, that's where the yelle and COG data differ. So I think as you look at that audience poll, it's a reflection of an international um audience so the end result is it's pretty split. I think if it was strictly in North America, um, or COG sites, then it would be very much a resection of diagnosis really great point. Wait, hold on, Dan, repeat that again in the mic. It also would impact the chemotherapy answer because you would add doxycycline for even for a low, uh, stage tumor. Exactly. And, uh, this is our next audience poll. Let's see what we do. So we have the weight of 728 g of the nephrectomy tumor specimen, age is 19 months. Now, what do you do? Close follow-up per protocol, flank radiation. You do EE4A, which is vincristine and deactinomycin, or DD4A. You add doxorubicin to that. Audience questions. This is gonna be a good poll. What, what do people here think? Any opinions? Or or send to oncology and Yeah. OK, alright, audience, um. Let's see what we have. C is coming in first, followed by D. Less for A and B. Yeah, I would go with C too. I think the one reason, uh, as Dan pointed out, you would add doxorubicin to keep in mind if you have if you have loss of heterozygosity at 1P and 16Q. That is important for us to remember because that adds some exotoxicity, but that's the one that shows higher, uh, recurrence, um, you know, when you, uh, have loss of heterozygosity. So, um, for this, uh, decision that it's like. Which chemotherapy do you use? Usually it's a multidisciplinary thing surgeon oncologist, or is the oncologist the only one who take it is definitely multidisciplinary tumor board meetings where you kind of present your cases. Surgeons has to be there to give in, uh. Uh, insight on what happened in the operating room. Make sure your lymph nodes are always out. That's very, very important to get 5 or more of lymph nodes, um, and just give the surgical perspective. And we have a question from the chat. Um, is anyone using ICG for lymph node deal? Uh, uh, we premature question. We'll also get there, uh, but yes, you can, um. All right, so what's next? Let's kind of change up the scenario a little bit. Next slide. So now, difference audience poll with the different uh parameters here. Now we have a stage one tumor. The weight is 450 g. Significance of loss of heterozygosity, 1 P16Q and also 1Q gain, and we'll go into a little bit more details on what that 1Q gain is. Um, let's look at this. Any preliminary thoughts from the audience? Stage 1 450 g. Somehow I think if the topic was appendicitis, we'd be getting so many more comments from the audience. Wilms, everyone's like, head down. Tim. Uh, observation. So I just wanna say I think the most important thing here is that there's a lot of stuff that's rapidly changing with the biology here and a lot of open study questions and so for the surgeons, there's gonna be a lot more of waiting for the biology before you determine if chemotherapy is gonna be needed. So as we move. Move into the next round of COG studies on this, um, the, the group of patients who are gonna be eligible for, um, surgery is gonna increase and so there's gonna be, you know, as you're thinking about placing the port, there's gonna be a lot more of wait and see what all these biologic markers look like. Absolutely. And that's how your patient discussion or family discussion should continue, uh, not just say, if you're less than 2 years of age, if you're less than 550 g, you will not need chemotherapy because that's not accurate based on, uh, your biology studies. You will have to add chemotherapy, uh, if you have loss of heterozygo state 1P16Q. Uh, so this is the update from COG, um, just from the last, uh, fall meeting. Uh, so this is adverse biologic factors like we discussed or with worse prognosis and stage two, but not that much seen in stage one, favorable histology, William's tumor. But if you have some of the loss of heterozygosity, we're still adding chemotherapy. Um, next, um, based on the current enrollment, age and tumor nephrectomy weight was not associated with the event-free survival. This has not made into mainstream yet. So will there be a time where you have older patients than 2 years, higher weight than 550 g, that with good biology that may not, uh, need any more therapy? Very possible that, uh, we have to be on the lookout for. Uh, next is that's exactly to, uh, Tim's point is to, uh, new certification and modeling treatments for that. Uh, next clinical scenario, this is more on a, uh, surgical side of things on what people do. So we have a 10 year old male with long-standing abdominal pain, presents with what appears to be a right upper pole mass with the IVC thrombus extension, lungs are clear, um, and, um, there's some enlarged lymph nodes in the abdomen. Audience poll Still 3 questions, Todd. I'm kind of, I'm kind of interested in the crowd. Anyone do surgery for any IVC thrombus up front depends on level where people are at. Any comments? Uh, yes, of course, it depends on the level. If it's, uh, until the kind of the atrium, I might go and check it out at the beginning. Anyway, we are, uh, following, uh, umbrella protocol in Chile, so I don't do that anymore, but, uh, I did before when I, when I was following in COG. So yes, it depends in the where is the thrombus in the up to the hepatic veins or into the atrium into the hepatic veins. Is that pretty much where everybody is. Below the hepatic veins. OK, when would you biopsy? Well, again, I, I guess if you're following COG protocol and you want, uh, to give him chemotherapy first, uh, you should do a biopsy. But in umbrella and it might change at COG at some point, uh, we do chemotherapy up front, uh, unless the patient is, uh, kind of older, you know, like 6 year olds, uh, or really small, uh, so they are another histologist other than William Williams. So we've been sometimes avoiding biopsy if you have family history, or if you have some other diagnosis, that's very consistent with Wilms. Imaging, that's pretty consistent with WIMs. If you, if you don't have a question on what kind of tumor it is or any other issues with you're worried about uh uh anapplasia or something different, then uh we'll proceed with biopsy. But if not, Under 10 years of age, if you're pretty sure it's worms, uh, we're avoiding chemotherapy. I'm sorry, uh, biopsy upfront. So that I think the, you know, the COG approach is to avoid biopsy upfront, but the other indication for biopsy is if you do give chemotherapy and it doesn't respond as you expect it to, then you would do a biopsy to rule out anoplasia, because that would change your chemotherapy protocol. Absolutely, yeah, great point. Right, uh, let's see the poll results. All right, looks like most people are a biopsy chemotherapy followed by resection, #2 chemotherapy, then resection. Yeah, so that's what we did here is chemotherapy and uh resection. I wanna get to one more question before we have some surgical specific questions on this. Uh, so similar scenario, now it's a 12 year old female, uh, same presentation, chronic abdominal pain, now has, also has IVC thrombus, lungs are clear, some lymphadenopathy in the abdomen, uh, initially managed at an outside hospital with an IVC stent placed. Uh, let's go to the next picture. I don't know if the next bitch showed up Anyway, there's a picture with a stent in there too. Uh, go back please to the audience, uh, uh, pool. All right, so what would you do? How would you, uh, manage, uh, this particular patient? Uh, she also had a IR guided, uh, uh, stent placed, um, and if we should do that, if, uh, there is tumor thrombus in the IVC and what's the downside of that, and, um, looking at the questions, any preliminary audience questions? I was just curious where the stent goes from and an IVC. Yeah, I don't, there should have been a picture in there. The stent goes into the atrium. Are you asking them to advance the slides when you say there should be a. Back to the clinical scenario. There was an overlap picture maybe it got cut out. Yeah, there's a stent in the IVC plus going into, uh, halfway into the A frame. So I think that there's, there's going to be some discussion about just collaboration with other services. I think certainly this is one where you, you, you phone a friend, you get your interventional radiologist, you get your cardiothoracic surgeons, you get everybody loaded for bear after you've gone through the, uh, neoadjuvant chemo, and you go in with scenario 12, and 3, and especially, especially anesthesia. You know, you're, you get into a mess, you know, at the retropatic IVC, you're gonna need an anesthesiologist who really knows how to keep that CVP as low as possible and then flood them as soon as you get things under control. Um, that's a very dicey situation. So I think it just highlights the need to make sure that everybody is on the same sheet of music and you're not talking about this 15 minutes before the case. Yeah, can I ask a follow-up question to that? Uh, so how would you, uh, approach this, uh, for vascular isolation? I think you, you may, in fact, need to do a sternotomy and get, uh, control at the level of the supra diaphragmatic IVC right at the right atrium and just at least have that controlled. Obviously, not, uh, actually, uh, Clamped, but the ability to do that and, um, obviously, you'd have a Pringle maneuver, you'd get, uh, retro cable, IVC control at a place just below the renals. And, uh, you know, you would just have everything completely exposed for yourself. I think one of the questions is how much tumor is invaded into that stent, how sticky is that stent going to be? You obviously worry about embolization of the tumor. That could also be something that would be pretty scary. Uh, so there's a lot of different things to think through, and I think that's where it just making sure you have solid relationships and understanding the capabilities of your partners and your specialists and what they think is possible. Absolutely very key to have the collaboration, multidisciplinary, so audience pool. And the answers, can we see the poll answers and then we're gonna bring in Doctor Nightingale as well. He's, uh, here to make some comments. OK, it looks like, uh, A and C are pretty and equal. We may have to cut out a couple of your poll questions because we're running kind of late. Um, let's, uh, Doctor Nightingale, I don't know if you can hear us. Um, good morning. Good morning. We'd love to hear if you have any thoughts or comments on all this so far. Oh, well, I'm conscious I'm, uh, living in the future because it's Wednesday morning here. I think you're still on Tuesday morning. Um, that, and I'm also conscious there's a group of COG surgeons there where I'm a psyop surgeon, and good to see someone from Chile who's also a psyop surgeon. A really interesting discussion. Um, most of those patients would, of course, receive preoperative chemotherapy on psyop protocols, and we would have no qualms about biopsying them. Um, the 10 year old patient now, I think would definitely get a biopsy in our institution. Um, so some very interesting differences between the two protocols. Awesome. Um, we're gonna keep going. Yeah, yeah, let us know. You know, don't be shy and, uh, can he just talk when he, yeah, so just, Michael, just talk when you want to and we'll go right to you. So just interrupt anytime you want, OK. No problem, Todd. Um, I'll, I'll try to be fast because I understand you're running over time a little bit. So can we put up the first slide? Yeah. All right. So, I'm speaking as a psyop surgeon, um, and I'm just going to talk about your standard unilateral non-syndromic wound. And I think in, certainly in psyop, there are 3 areas that we're, we're looking at innovation, um, nephron sparing surgery, minimally invasive surgery, and lymph node dissection. So, we go to the first case. Um, I'll just remind everyone in signup protocols, all patients under 6 months of age are treated with surgical intent. They don't get preoperative chemotherapy. So, this is one that perhaps bridges between the two groups. 66 week old female, a solitary mass on the right upper pole of the kidney. So it's, you know, it's well, a discrete, right at the top, separate from vessels, no evidence of rupture or lymph node enlargement. Next slide. So we do a poll, what is your surgical approach to this tumor? You biopsy it? Do you do an open radical nephrectomy? Do you do nephron sparing surgery, or do you do a laparoscopic nephrectomy? All right, and while we're waiting for that be interested in it, yeah. Go ahead, my thoughts from the audience. Yeah, any comments while we're waiting for the poll to, uh, generate? Nobody? None of the oncology surgeons? Coco, come on, you go. Dan. All right, go ahead. Um, She has the two kidneys. No, yeah, she has normal kidney and on the other side. Yes, I guess I'll do a laparoscopic nephrectomy up front because of the age of the patient. To see the, the histology, you mean. Any other comments. So I'm assuming that the patient has no underlying genetic predispositions or anything like that. So, so just to be controversial, I would suggest that there's the possibility you could do a partial nephrectomy, do a nephron sparing, because of that upper pole lesion would be very amenable to a, uh, partial nephrectomy. Yeah, I'm wondering how this patient even was diagnosed because um you don't usually pick up such a small tumor in a small kid, but this seems like a perfect situation for nephron sprain surgery. It's probably a rest also, it might not even be a tumor. This is an ultrasound done for possible polaric stenosis, an incidental finding. Let's look at the poll results, yeah. Oh, there we go. It looks like nephron sparing surgery was number one answer. Yeah, and that's what we did with, with this patient, um, and I think that is the priority for SIA protocols, um, is to focus on nephron sparing surgery over laparoscopic or minimally invasive surgery. Um, so, I think in these patients, we're weighing up the, Oncological risk of nephron sparing surgery, so it's certainly not something that the occasional surgeon should be doing, but we are getting the benefit of trying to preserve nephrons, and we all know the risks over time of developing renal failure later in life and those patients who had nephrectomy early in childhood. So it's a controversial topic, um, and it's interesting that there was a majority of support for nephron sparing surgery. So we go to the next slide. any other? I can't see the audience, so yeah, Michael, uh, we have one question for you. Go ahead. Yeah, uh, um, I was, uh, asking myself because I, I might do like a nephro surgery, but do you do something different in the surgery like if you're doing a testicular, uh, kind of partial surgery, I mean. Uh, do you, uh, wait for the frozen section or something to decide to go for all the kidney, or you, you're doing nephrone sparing surgery upfront? uh in Wilms. Um, so most of these patients are over 6 months of age and will have had preoperative chemotherapy, but this particular patient did not. The approach that I use for nephron sparing surgery is the same that I use for syndromic patients or for patients with bilateral disease. So I, I don't do laparoscopic surgery for partial nephrectomy. I do an open operation. Um, I immobilize the kidney, so I have control of the vessels. I don't occlude them for a polar tumor. I simply compress the, um, the kidney between fingers. Um, we do intraoperative ultrasound to mark the margins of the tumor, uh, um, and we try to leave a safe cuff around it. And of course, we would only do this knowing that we could leave at least 2/3 of the kidney behind. Alright, let's move on. OK. We probably have to cut it short. So move on. We're gonna, um, hold on to the questions because we're pretty much pretty over time, so we'll just keep going. Go ahead, Michael. OK, a, a quick 1. 18 month old male, um, imaging showed a right mid upper pole mass. Um, this patient was treated on a site protocol, so he received 4 weeks of VA therapy, um, and had this preoperative imaging. So, aware this is a group of cog surgeons, um. You know, what would your surgical approach be? Further chemotherapy, an open radical nephrectomy, a laparoscopic nephrectomy, or a robotic nephrectomy? OK, OK, Jose, you wanna make a comment while we're waiting? It's OK. On, on the last thing, yeah, I was just, um, thinking about the previous case, and I see a lot of hope in nephroim sparing surgery for unilateral wms and non-syndromic patients, but I'm just glad that you mentioned that this should be super selected patients because. Um, it's going to be very difficult to demonstrate a benefit from that surgery, I think, uh, this group of patients only has a 1% of end stage renal failure, and we know we we quote to ourselves the rates of hypertension or cardiovascular disease they get, but that might be the consequence of chemotherapy and not necessarily the loss of nephrons, so. Just, just a note of caution and I'm happy that you, um, as a really skilled surgeon within a protocol are bringing that up, but I'm, I, I'm have the fear that it's gonna take a long, long time before we can just generalize this knowledge to everyone, just an opinion. Yeah, great points. Absolutely, Jose, I think it's a, a murky area. I think this is not a nef, I mean, we have to weigh the oncological risk here. We know that this type of Wilms tumor is imminently, imminently curable with standard therapy, um. And you know, uh, an open radical nephrectomy, um, is, is a very good operation to cure it. Um, so we have to be cognizant of that risk. Can we see the pole? Uh, You could just, what were the percentages so we can just read them off. Oh, here we go. 64% had um open nephrectomy. Yep. OK, and, and that would be entirely inspected and obviously a, a very reasonable approach. um, but the next slide. One of the advantages of being in a, in a psyop center is that with patients treated with preoperative chemotherapy, it does two things. One is that it shrinks tumors, so they're obviously smaller. The second is that it definitely makes them more robust. Um, they become a lot more rubbery. They're a lot easier to handle. Um, and that does open up potential for minimally invasive surgery. Um, so we all know the advantages of minimally invasive surgery. We also know the risks. Um, these are the standard site criteria for minimally invasive surgery in Wilm's tumor at present. Um, I think when we're doing minimally invasive surgery for Wilm's tumor, there are two main risks. The first risk is the risk of rupture. Um, and it can be difficult to know how much you can move tumors or kidneys around, uh, with minimally invasive surgery when you have a tumor that you desperately do not want to get any rupture from. Um. But they are remarkably robust after chemotherapy, and I think, you know, in our, our series here at the Royal Children's, we haven't had any, uh, any ruptures during the laparoscopic nephrectomy. I think the second issue is lymph. Go ahead. I after you to this point, we're out of time, but what I would love, and I know you have, I know you have a bunch more polls, can you do us a favor and give us the upshot? Give us what are the high yield points that you were going to be making that we need to know with Wilms. Right, so minimally invasive surgery is a good option for patients treated with pre-operative chemotherapy, but it's difficult to do an adequate lymph node resection, and I think you have to be doing a lot of, uh, a lot of procedures to get good at that. That the third case that I was going to show um was about lymph node dissection and the areas of importance for taking them out. Um, I think that's the crucial message. Do you want us to show that because that's like a visual thing just show to the picture. Yeah, and then we'll, he'll tell you which slides to go to Isla. There's a picture with ICG if you keep, yeah, go ahead. Yeah. Perhaps we'll just go to the 2nd to last slide. Keep going. Yeah. Uh, this, uh, sorry, the previous one. So the simple thing about, um, this is a really elegant study from India that looked at which nodes are important to sample, and it highlights the importance of taking the aortic caval nodes. And I think that is the difficulty at laparoscopic nephrectomy. And, you know, most, most surgeons who do minimally invasive surgery can do a, a, Laparoscopic nephrectomy, but it's the nodal dissection that is challenging, especially in that aortic pal area, and that's why this study has shown is very important to show that that's actually a crucial area. Um, in terms of the number of nodes, um, I heard, I think, mention of 5 earlier in the, in the session. Um, in studies, it's a minimum of 6, and going forward, we really aim to get more than that, so 7 to 10. Um, and I think that's just because with pre-operative chemotherapy, some microscopic metastases can actually disappear. So we need to really, perhaps a slightly better, even a greater number really than cog, um, and. I just the last, just keep, yeah, I like, keep going, keep going. Yeah it's just about IC, yeah, sorry, 2nd to last slide. Uh, this is just to show some work about ICG which I think is a real adjunct to show the location of lymph nodes during dissection for Wilm's tumor. Um, so I don't know how many surgeons are utilizing this. This is something that we've started using, and I think this is a nice demonstration of how important those aortic cable nodes are. I can see the, the green lining up between the aorta and the cava. So, Michael, what dose are you injecting and where are you injecting this? We have only just, well, it's expensive equipment, so we've only just purchased it here at, at RCA so I have not used it for this. Um, but I know that, you know. We have, uh Yeah, so, um, the ancient group is doing a clinical trial called Visceral X that we're about to start enrollment on, but it's, you use an angio cath without a needle to infiltrate it into the soft tissues of the kidneys, so it's a direct injection into the soft tissues to do it. Some people will do it with the needle into normal parenchyma around the tumor if you have a rim of normal tumor, but probably the safest thing is to just infiltrate with an angiocah, uh, a small. Do that I don't remember off the top of my head. So, um, what I would suggest there's gonna be when we go this fast rapid fire, a lot of questions pop up. That's what chat's for. So if you could put in there some of the details you were just describing, so if people have specific questions, keep the discussion going in the chat. This does typically happen if there's details you want to know, the chat can continue on about these topics while we move on. Do you want to make some final, yeah, the, the only other thing I was gonna say is like of all the topics in our field, there's, um, amazing resources for oncology. So there's seminars in pediatric surgery, have clinical guidelines. I'll give a shout out to Jenny Aldrich. She did the Wilms tumor one. All the major tumor groups are there, the stay current app, apps, the library. There's some really good stuff out there to review. That's a, that's a great point. And we have to be better with making sure we know where all these resources are and And get access to them. One final plug-in for the surgical, uh, just so we push ourselves some more for IVC thrombi and thrombosis that's, that's extending into the area, you can mobilize the diaphragm from the suprahepatic cava. You can make a pericardial window. You can put your clamp up there. You've, you've done a transplant rotation. This is just a routine clamps, uh, vascular isolation, so not everyone needs to. Go on a bypass for uh atrial thrombus unless it's attached to the valves or something, uh, hopefully we'll have a collection of patients, uh, soon, uh, but we've done extensive diaphragmatic, uh, mobilizations and put in clamps, uh, up in the pericardial area and we can put the ICG information in the chat. Yeah, if you could do that, if everyone can just any resources anyone has, this is a great pool to throw your resources, be, be generous with your, uh. You know, anything you know that would help the rest of the world, we, we'd love those. Phenomenal session. This is, this is one that probably needed twice the amount of time. Michael, I don't even know what time it is there. What time is it in Melbourne right now? Uh, it's 12:300. 0 man. All right. Thank you. I hope you can sleep in a little bit tomorrow, but thank you so much for joining us. This was so I know it's been, it's been fun. Thank you for asking me. I think this is a great event and it's really good to sort of open it up to the rest of the world. Thank you so much. Thank you. Thanks, Bargava and Justin. Thanks everybody. Great session.
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