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"The role of a Paediatric Surgeon in the management of neuroblastoma" by Dr Hafeez Abdelhafeez, a Paediatric Oncologic Surgeon from St Jude's Research Hospital, Memphis, USA on



This engaging and interactive on-demand teaching session is led by pediatric surgical oncology expert, Dr. Hafiz Abd Afis. Focusing on the critical role of pediatric surgeons in the management of neuroblastoma, Dr. Afis shares his vast knowledge and experience in the field. With roots in Africa and numerous fellowships under his belt, his expertise in Pediatric Surgical Oncology adds a unique perspective to the discussion. The session would be especially beneficial to those attending the pan-African Association of Pediatric Surgeons meeting scheduled in Lusaka, Zambia. Not only does the presentation provide a comprehensive overview of the disease, its presentation, and the common sites for metastasis, but it also addresses specific clinical and biological prognostic factors. This session is a must-attend for medical professionals keen on expanding their understanding of pediatric neuroblastoma as well as its diagnosis, preoperative evaluation, and postoperative management.
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This is an invited talk by Dr Hafeez Abdelhafeez, a Paediatric Oncologic Surgeon from St Jude's Research Hospital, Memphis, USA on The role of a Paediatric Surgeon in the management of neuroblastoma". This is part of the Zoom academic meeting of the Department of Paediatric Surgery in East London, South Africa.

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Learning objectives

1. Understand the role of pediatric surgeons in the management of neuroblastoma, including aiding in diagnosis, conducting preoperative evaluations, and ensuring optimum postoperative management. 2. Gain insights into the diverse clinical presentations and metastasis patterns in neuroblastoma cases. 3. Become familiar with significant prognostic factors in neuroblastoma patients, including age, disease stage, primary disease location, and resectability. 4. Recognize the variables involved in image-defined risk factors (IRDs) for neuroblastoma, including degree of blood vessel encasement, involvement of renal vessels, and tumour location. 5. Develop knowledge about the involvement of blood vessels and potential for spinal cord risks in neuroblastoma cases involving the costo-vertebral junction.
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The following transcript was generated automatically from the content and has not been checked or corrected manually.

Can you see my slide very clearly? Yes. Ok, excellent. Uh, greetings to everybody and thank you for attending the Zoom academic meeting of the Department of Pediatric Surgery in East London, South Africa. And uh, today, uh we are really, I'm pleased and I'm honored uh that we have Doctor Hafiz Abd Afis who is a very personal dear friend of mine. Um, and he is really a pediatric surgical oncology expert and he's going to talk to us about the role of pediatric surgeon in the management of neuroblastoma. Uh Doctor Hafiz looks young but he is quite educated. He is the director of pediatric Surgical oncology Fellowship program at Saint George Children's Hospital Research Hospital, which is one of the pioneer, uh pediatric surgical, uh pediatric oncology institute in the world. He's also the Oncology group lead of the Global Initiative for Children Surgery GS. He is on the Education Committee of the International Pediatric Surgical Oncology Society. Um, his roots are in Africa. He's originally from Sudan and he told me that only for about a decade and a half, he has gone out of Africa trained in the UK Ireland and the US. He has got multiple fellowships. He has critical care diploma from cardiff cardiothoracic intensive Unit fellowship from Southampton pediatric surgery fellowship from Wisconsin and pediatric surgical oncology from Saint Jude uh Memphis. He has also received multiple awards from Saint Jude and also in Wisconsin. And we are really honored to have him. And I take this opportunity to uh inform uh all of you about the pan-african Association of Pediatric Surgeons face to face meeting, which is happening almost exactly in a month's time in Lusaka in Zambia on the Thursday and the Friday 14th and the 15th of September. And uh you can submit your abstracts up to the 21st of August and don't worry about this early bird registration. Anybody who wishes to register can register uh with s straightforward fee for African delegates $200. So I welcome you and invite you to attend the pep sa meeting. I will stop screen share and I will invite doctor office to share his experience with us office. Thank you for the kind introduction. And uh it's very accurate. I'm actually old way older than I look on that picture. But let me see if I can share the screen you can see now. Only one slide. Correct. Yeah. OK. Fantastic. So, so we'll um start. So obviously, the topic of, of today is the role of the surgeon in the management of neuroblastoma. And uh we'll try and make this interactive. So I'll shrink the time for the, for the um presentation, we go skip some slide, go fast on some slides and then allow more time for, for the discussion. We can go back to the presentation at the time of the discussion that shows specific slide. A and, but I think it will be better to have it. Um You know, I have nothing to disclose. But generally speaking, the role of the uh surgeon in the management of neuroblastoma. It will be either to aid in diagnosis, you know, complete the preoperative evaluation and uh adhere to the principle of uh uh local control and then the postoperative uh management of these uh complex patients. But overall, you know, one can summarize the the the role uh of the um of the surgeon into those three phases that you know, the the phase of the evaluation, a phase of the pre planning and and postoperative uh uh management. And we can also specifically talk about the about the principles of of surgery. We can start this with a, with a question which maybe we can answer at the end of the session, which of the following patients with neuroblastoma would benefit from a section of the primary tumor most so infant is an MS disease B is an unit with a 15 mills adrenal mass seeing is an infant with stage four disease that is and mek nonamplified and he is a four years old with stage four disease and a celiac access primary, you can uh uh write in and I'll sit on the chart and uh and then we can look at, at that at the end of the uh of the talk. All right, moving along. So start about the uh evaluation. Uh you know, just looking at how would they classically present patients with neuroblastoma uh in an overall uh um uh sort of uh uh a review of this tumor. It's, it's uh it's the commonest, maybe uh intraabdominal a solid tumor of childhood. It constitute 8 to 10% of all malignancies. Uh And um uh in the US, maybe here we see about 800 new cases per year. It's the most common uh uh malignancy in infants, more than 40% of patients present by one year in. Um it has although um and I can see how, how, how many neuroblastoma constitute the, you know, the percentage of a total MG but it causes 15% of cancer related. So it's, it's uh it's a very um heterogeneous tumor part of, you know, as some type of it is not as aggressive but some type of it. The risk is extremely aggressive. 65% occur in the abdomen, ha half of those are uh in adrenal and 40% are localized at presentation. So it does have a high uh uh you know, incidence of metastatic disease, obviously for the high risk. And this uh diagram shows, you know how it's really primary disease of infancy and within the year, you, you, you do get AAA big percentage of the of the patients. Obviously, the the origin of the tumors from this uh symp sympathetic chain and the um adrenal gland. Therefore, these are the common side based on the uh on the origin and uh retroperitoneal side including the adrenal side. Uh A thoracic uh posterium in interspinal, obviously, uh uh as we mentioned earlier on the, the retroperitoneal can be a para paraspinal or uh an adrenal. The renal is, is uh it's pretty common. Uh less common sides include the pelvic, which you know, originated from the organ of zucker candle and cervico. Essentially, the, the common sites for metastasis for neuroblastoma are bone and bone marrow. There is some other less common uh sides, you know, including uh the, the liver. However, it's obviously common in, in stage um for S and skin. Similarly, it, it does occur in stage four S but the, it rarely metastasized to the lung and we're only in 3%. So this is like a contrast to the, to the other uh uh type of uh So, in pediatrics that it's uh has this characteristic metastatic uh pattern and the common presentation is a mass in the abdomen uh and with metastasis at the bony side. So it's uh and that's the, the difference bet between uh uh an abdominal neuroblastoma and uh uh a Wilms tumor that generally uh patients who who Wilms tumor are. Well, they have just an incidentally palpable mass that was felt by the parents during bathing or that is sort of a typical scenario for neuroblastoma patient because there is a really high incidence with acidic disease and high c catecholamine levels. They don't look well, they look anxious. Uh they have uh s signs of metastasis, either bone pain uh or you know, hemorrhage and emos in the eye scenario and all and all that. So, obviously, there is some other less common uh scenarios including of clonus, myclonus and diarrhea. For uh for example, uh the uh VIP secreting uh neuroblastoma. This is um a, a picture of a lacuna. Again, another one of it thi this is the uh the skin metastasis that is seen in uh stage for in the, talking about the, the ca the rare scenario of clonus myoclonus, which is essential an and, and really an immune mediated uh paraneoplastic phenomenon. Uh sis is rapid eye myoclonus is uncontrolled movement of the extremity. Uh and um and some uh some other associated symptoms. When uh looking at the clinical picture of the patient, there is some specific um findings that have uh um a significant uh prognostic. Uh I in the II, you know, um uh implementation to summarize them there, you know that they are biology, age of the patient, uh stage of the disease, the primary site of the disease and resectability. We can uh divide these these, these factors, the clinical prognostic factors and biologic prognostic factors. So, you know, from the uh um from the uh from the initial evaluation and on the clinical evaluation, it becomes apparent uh what risk of neuroblastoma are we dealing with? Um obviously, uh the biology factor comes more uh later with the laboratory investigation with age that is more than 18 months and the presence of I found these factors which is basically the neuroblast being in case in case in blood vessels, major blood vessels is uh too poor clinical um uh factors. The biology factors include uh uh uh MN uh amplification, which is the strongest factor in prognosis uh comes uh um Also we start the uh 11 Q deletion and blo you know, is uh le less powerful than uh and uh amplification. This is the, the really an image to define what is meant by image defined risk factor versus just a tumor touching of blood vessels. You can see on the uh on the a side of the screen. Uh if I may use uh pointer that uh this is there is a less than 50% engagement uh or just that, that, that you were touching the blood vessels that's not considered an E virus factor while when there is more than 50% casement uh or I in the venous uh scenario when there is a effacement of the of the vein. Uh then that is considered an I virus factor. Obviously, the case of the renal uh vessels currently uh just involving the renal vessels. Not necessarily, in case it by more than 50% is considered an IV factor. I'll go through this image pretty fast that these are just like they published uh seminal paper, but image found risk factor in different uh part of the body. These are in the cervical neuroblastoma and see how it's increasing the um carotid artery uh and uh moving this cervical thoracic uh as as seen here of how the tumor is really causing sig significant. Also uh airway um uh uh compression or tachy racy plexus, you know, major blood vessels as well for the uh thorax. Um Again, the major uh airway compression or the major blood vessels will constitute an imi risk factor. Add to that is the location of the neuroblast. So, it is involving the cost of vertebral junction uh between T 12 and and uh and uh T nine and, and T 12 are all the way maybe to L1 because that area the uh intercostal uh vessel uh um basically supplies the anterior spinal artery through the uh artery of a books. So this uh in reto at this stage can really uh pose a risk to the vascular supply of the spinal cord. Uh Again, uh uh more images uh uh uh pictures of images uh find this fact that in the um uh thorax with a vascular in in involved in the abdomen. As you can see uh uh in this side of the, of the, of the uh image that here you can see a large tumor. It's if maybe touching the blood vessels, but it's not really encasing them. Or here you can appreciate that this tumor is, is crossing the midline is and is uh encasing the major blood vessels. Similarly on this thoracic tumor, it is uh encasing blood vessels. So these are the summary of all the uh um I define risk risk factor that you can see in, in the abdomen. And different example of it from portal HEPA is the AORTA and SM uh renal artery and IVC effacement to celiac access engagement that can be seen here to uh a renal artery uh engagement. I there's some other additional uh criteria criteria when some critical organs are also uh involved. This slide really talks volume about the heterogeneity of, of neuroblastoma as if we're dealing with two different disease. As you can see in the uh Kappa Mara curve here for uh event free survival. That low and intermediate risk neuroblastoma has really good outcome is, you know, they, they, they generally the they're maybe, yeah, low is a little bit better than when beat it. But the two outcomes are are close and they generally do well. While the high risk glioblastoma is a very aggressive tumor with really poor uh outcome and low event free survival. And that translate to how the philosophy of how we treat all these two tumors. But you can see, uh for low risk surgery only can suffice and, and be, you know, uh the only therape mot that is required while for the intermediary surgery and chemotherapy, uh both are used for aggressive and multimodality therapy where we throw everything to a, you know, a surgery, chemotherapy, immune therapy, targeted therapy, radiation therapy. And my therapy was bone marrow transplant. So everything is thrown to uh this aggressive and you know, to try and and solve it. Uh the the situation moving to the preop planning and uh uh management for the workup of a neuroblastoma or a suspected neuroblastoma should include tumor markers, catecholamines and imaging cross section, imaging of the primary tumor uh of the CT or MRI, maybe MRI is better when there is uh uh a uh an uh um suspicion of i intraspinal extension for the chest since it's really rarely if ever metastasized to, to the uh chest, only 3% of um uh incidence of me that uh metastatic disease in the chest chest x-ray only will suffice. Uh uh The workup should also include metastatic workup for the most common side of metastasis, which include bone marrow assessment, bone marrow aspirate and biopsy. And uh also assessment of the bone uh uh for metastasis M IG is more uh specific and it will work uh really well to uh evaluate these metastatic sites. And then uh the, the, the step to uh confirm the tissue tissue diagnosis is biopsy in which we will come and discuss that later. But this is sort of the algorithm that we have discussed uh for um um diagnosing your breas orma. Well, this is the the most common uh scenario we see on CT scan. As you can see in this image, the uh celiac uh a uh artery is really e encased. So in case of media is a common thing. Uh here, you can see an M IBT scan showing the primary is avid uh in 10% of patient, the neuroblastoma primary may not be of it. So, I mean, you know, 90% they, I mean they are. So it's it's pretty spe uh specific in here in this image of the M RB, just kind of showing um a distant metastatic disease as well in the bone, bone scan can also s uh uh work to um show the distant metastatic disease in the bone. But you know, in summary, the assessment of o of the disease extent should include uh imaging of the primary. As we I mentioned, the CT and M IPG imaging for metastatic uh asides including the bone marrow and the bone, uh an assessment of the uh lymphatic spread by imaging and and by clinical examination, obviously, e eventually by examination at the time of resection and uh and uh resection of involved lymph nodes. Um also uh imaging of the uh abdomen and liver should be included as pa part and parcel of of for meta disease uh for the chest um chest X ray, as we mentioned, is it to establish the diagnosis of neuroblastoma. One of the two you know, um can establish there is unequivocal pathologic diagnosis made from tumor tissue by laparoscopy. That's a needle, uh an image guided needle biopsy of the primary tumor that is e examined histologically will confirm the diagnosis. The the other scenario is if you have a bone marrow or to, to find biopsy that contains an agro VC tumor cells. Example, uh you know, uh with all the features that you see in the in the neuroblastoma, um tumor cells together with decreased urine and serum c catecholamines or metabolites. This also can confirm the diagnosis. So if you have this, you probably don't need um the uh uh either the primary tumor uh biopsy. So this table really uh shows what what is the most, you know, important aspect of um as evaluating neuroblastoma. Uh essentially by um uh risk strate strategic those tumors into very low risk, low risk, intermediate risk and high risk. And essentially the therapy is tailed based on this. You can see some powerful uh um uh prognos, uh prognostic biology uh markers that will, you know, change any uh a combination of things to high risk. If you look at a and make amplification, if it's amplified even an L1 here will go to uh a tumor will be, will become a high risk. Uh Similarly for an uh L2 tumor. And obviously just to explain L1 is not encasing blood vessels. L2 is encasing blood vessels or you know, or has an image defined risk factors. M is metastatic and, and MS is that metastatic special that happens is in, in, you know, less than two, less than 18 months uh of age with, with less than 10% infiltration of the bone marrow and specific pattern of metastasis in the liver and the skin. Again, if the nic is amplified, this takes it uh straight to high uh a high risk. And you can see that uh uh uh ganglion neuroma which is, you know, a very uh um you know, favorable histology and neo rest inter intermix are ve uh are very low risk um to uh go and summarize. Um what the implication of this obviously a very low risk is a really uh uh excellent, predictable five years event free survival in the 28% of patients. The low risk constitute 27% of patients. They also do very well uh in the combining those two, the very low risk and, and, and, and low risk is a substantial percentage of patients. But again, uh uh the high risk is also uh uh quite prevalent up to um 36% in intermediate risk is a little bit not as common. This is the same thing we show just in a, in a, in a three format to show how, what, how, you know, everything start, start by first. Uh uh You know, um uh uh basically determine whether the tumor has I found factor or not, whether the tumor is metastatic, whether it's a special type of metastasis. And as soon as this is determined, you can see the fact that that really change the outcome is and make umbilical except for metastasis. Uh uh arm. The age of uh uh works as a as a as a first step to to determine metata is a metastatic. Older patient has a really poor uh uh prognosis as mentioned earlier on therapy for low risk is is is minimal therapy, surgery alone will do intermediate risk, require surgery and chemotherapy. While high risk require all sorts of things including surgery to receive chemotherapy, bone marrow transplantation, radiation therapy, acid immune therapy, uh you know, everything uh that is thrown to it. Uh talking about some other specific scenarios where the surgery can also play a role. Obviously, the low risk can be, you know, um a very low risk can be treated with observation as well without sur surgery, we will go into into detail that later. But uh uh a tumor is showing significant uh growth and uh you know, observation can be removed um uh or any tumor that progressed from L2 to uh from L1 to L2 or, or, or to M on observation. Uh then you should also be uh addressed the special scenario of state. Uh MS um in the uh younger age with a specific, you know, a metastatic um uh pattern can be treated with uh conservative uh measure measure. Obviously, this is in the absence of adverse biological uh factor like anemic. And in terms of when the tumor has an intraspinal uh extension, if it is not, if it is un and as asymptomatic, then, you know, nothing needs to be done specifically for the uh uh intraspinal extension. And then based on the tumor stage and based on the tumor risk uh group, then the therapy is tailored. But if the patient has chronic symptoms and chemotherapy, um uh uh will be um the uh modality of choice for patients with acute severe symptoms and urgent uh uh decompression may, may be uh the therapy of choice. Um moving more to, you know, the the principle of resection, as you can see in this image, which really shows the uh celiac axis in the sma being skeletonized and the tumor is in the middle of the tumor was encasing these tumors, these vessels uh completely as it does in, in many times. Uh And um you know, surgery uh of WS tumor is essential uh of uh neuroblastoma, essentially a vascular surgery where the vascular tree is identified delineated first, and then the tu and obviously preserve and then the tumor is uh it, it will be the second phase of um of the resection. But in the, in the first phase is delineating the vascular artery and protecting it. So, you know, the tumor is generally removed in segment. Uh And uh there is some uh important principles when dealing with with this major blood vessels. For example, the renal artery is extremely sensitive to uh aggressive manipulation. It can, you know, if it, if it is to, it can go to spasm, it can affect the kidney significantly. Uh Or uh if I if it, you know, uh a dissection occur in the vessels, then this will lead obviously to thrombosis that can be missed at the time of the surgery. The role of uh of uh chemotherapy, you know, it plays a role is in a new adjuvant phase to, to shrink the tumor and also in, in, in intermediate and and high risk uh patient. It also works as an adjuvant uh uh part of the multimodality therapy for and, and obviously for, for higher risk, it, it is part of am multimodality therapy. And this is a diagram showing how chemotherapy can really decrease the imac risk factor and shrink uh the tumor uh and minimize uh the L level uh of complexity of resection level of o of blood vessel involvement. But uh you know, uh the effect of chemotherapy sort of start plummeting after the second to third cycle. So giving more and more and more chemotherapy wouldn't necessarily translate to uh further decrease of the I defined risk factor. Therefore, general speaking, we do perform the resection at around uh after uh cycle four or so. But you know, any time, I think after cycle two and three, it can be um acceptable. Again, radiation therapy then plays a role mostly in the um a high risk group uh in uh as part of the multi mod mod therapy, but also works to address specific metastatic sides. Uh When talking about the uh a complication for, for uh neuroblastoma. They are uh variable in, in, in, in uh um incidents. But the most important common issue is hemorrhage is that it's a very vascular tumor and really involve major blood vessels. But um uh a rare uh uh injury but significant and have significant implication is a, is a major vascular injury that should be avoided at all costs. Um in terms of urinary uh um complication, um obviously, the kidney loss can happen at the time of the surgery. If at the time of resection, a nephrectomy is performed which should be avoided, but it happens in about 11% of resections or if an a subintimal dissection occurred in the renal artery or so, that was missed at the time of of resection, obviously and gradually on followup. Imaging. Uh 1 may notice the atrophy of the kidney uh for pelvic area and neuroblastoma, bladder and u ureteric injury. It can also occur bowel injury. Uh uh is also a risk diarrhea can happen in the um secreting neuroblastoma, VIP secreting neuroblastoma, bowel obstruction can happen for multiple reasons either from the, the, you know, the adhesions after uh that sort of major surgery or in the suction that can happen after um retroperitoneal dissection. Ascitis is common but most of the time it's self resolving, we generally don't leave drains after resection of major neuroblastoma. The perineum works as a good drain. Generally speaking, but some patient may uh very few patients develop significant ascitis that require um uh intervention, corona icu uh admission and death. Uh All right, common common complication. I for a specific type of uh of uh neurosurg, you can see in this image uh a a child with Horners syndrome and this for the apical neuroblastoma that involve the uh the ganglion. And uh you know, the parents should be warned preoperatively that this is, you know, the surgery is not gonna improve the uh Horners syndrome chylothorax. Uh I is another uh uh not uncommon complication and one should have a low threshold for operative duct ligation for high volume chylothorax. But, you know, initial conservative therapy is always uh uh uh indicated for the kind of societies. Again, um conservative therapy works in the most of the time, but sometimes it, you know, uh uh in operative uh approach is required. Uh when dealing with uh renal artery, uh intraoperative uh spa, spasm, topical papaverine with uh from our anecdotal experience is basically seemed to be uh effective low dose do dopamine infusion. Uh And we are not sure, we're not very sure about how effective uh this is but, but um uh you know, maybe to see a, a more of an immediate effect, uh topical papaverine may help. Uh but obviously, most importantly is preventing torque of the blood vessels or any aggressive dissection. Uh The any vascular injury can directly be repaired or grafted, uh uh or occasionally um um uh you know, transplant uh and vascular heart uh may be required. So they, they take a whole message from uh from what, what, what would cause is accurate. Uh uh in uh particular staging is it better, is it is really important because it tailors uh the therapeutic approach in patient with um uh uh nonhigh risks, tumor need as little treatment as appropriate and they will do very good. But the majority of patients with the highest tumor will eventually relapse intensive therapy is aimed at giving them the best overall. Um a chance this, this uh slide will, will summarize um some of the trial that are uh um you know, uh some of them are, are, are completed. Uh But, you know, for L1 less than 11 less than five centimeters in a patient who is less than one year, this can be observed um uh safely. And there is a criteria of when intervention is, is indicated, the tumor increase by 25% or the catecholamine increased by, by the same uh percentage for uh if the L1 tumor is large, it can be resected uh primarily and surgery may be o the only thing that is required for um tumors with vascular in case or image virus factor but no uh adverse biologic uh factor where the nick is not amplified and the age is also favorable and they have generally favorable biology. Th this, this patient can be treated initially with observation and further treatment if there is an increase in uh volume or o on in the cat colony. Again, for uh the, the tumors that are encasing blood vessels but still don't have any ification, still have a favorable uh age less than 18 months. But unfavorable biology then chemo uh uh plus resection to achieve at least uh uh a partial response of more than 50% reduction in volume. Obviously, we, we al almost always try to aim for 90% resection or or more. Uh because you know, the, the, the principle of resection is the same identify blood vessel, identified blood vessel first, protect them and then, and then um essentially remove the the tumor in, in peace me. But for in intermediate risk, you know, uh one can, can, can still do well without going too too aggressive with a resection. Uh However, in, in patient with uh L2 but non, non make amplified but unfavorable that are uh uh more than 18 months, those patients should be treated with chemotherapy resection to achieve uh you know, um uh A A AAA, you know AAA good, a good uh reduction in volume of the tumor for uh uh the an anemic um nonamplified also in case of blood vessels that are both older than 18 months in which and fes um they tho those patients require uh high risk therapy and obviously that will include multimodality therapy plus resection. Thank you very much for your time and uh we can go back and see uh the um the question that you asked at the beginning and maybe I'll go and see the chat but uh or, or somebody can just answer the question because uh hi doctor, this is Doctor Moni. Uh health fitness has, uh will not be able to join us right now. He's got some problems at home. Um Thank you for that talk. I think everyone would be agree that it was very informative, especially from a surgical perspective. Um I'd like to invite some comments from some of the consultants that are um uh that are listening. Um uh Derrick Harrison, Doctor Derrick Harrison is uh from uh Johannesburg from I'd like him to um give some comments. Mhm. Hello. Hello doctor. Hi, how are you? Yeah, good evening doctor. A good evening. We met, we met in Prague during the first meeting and I presented Surgical principles in neuroblastoma. Yes. Yes, I remember very well. Yes. Yeah. So I thought I should attend your this presentation. It's very nice. Thank you. Thank you very much. Thank you. Thank you for your time and hopefully will for arranging this one. Ok, doc. Thank you doctor. Do you have any questions and other comments? No, no, I just wanted to compliment doctor. That's all. Ok, thank you. Thank you. Sorry, my video is not working. I don't know how that's why I am not able to switch on my video. That problem. Ok, no problem. Uh Doctor Derek Harrison. Uh Do you have any comments? Ok. Uh I don't think Derek is, he was here but he, I he was wearing scrubs so I I'm he may have gone to the operating room or? Ok, but he was here. He, he had this came on and then I think probably he he went to to the operating room. Yeah, I OK, he's unmutated. So Derek, we invite you to make a comment with your extensive experience. The audio is not working. Derek, I can't hear you. We cannot hear you, Derek. Maybe I'll Yeah, I think there is a technical issue. Yes. Um We can't hear you, Derek. So yeah, uh maybe Derek can write his comment in the chat box while sure that can ask for other comments. Yeah, so Derek will come back to you if you, if you can sort your mic out. Um And then I'll ask Doctor Sello Mataya who is one of our consultants from East London to make any comments and ask any questions. I uh thanks. Uh Thanks for uh a great overview on this complicated topic. The one thing which um hopefully Derek will be able to come on to highlight or make um like the proper statement a about it is resecting of tumors because um in your setting, maybe you see quite a lot of neuroblastomas in our setting in East London, we see way more uh nephroblastomas than neuroblastomas. Um As if I can recall in the last 56 years, you probably only operated on two if not three at most of neuroblastomas versus the nephroblastomas where we almost do one every single week. Um So the stats internationally versus locally are a bit different. We're seeing a whole lot more afros than Euros. But my main thing concern was that maybe um I was concerned that we were palliating these kids or not giving them enough opportunity. Um Because there was this old notion that um you have a neuroblastoma with more than 10% bone mirror involvement, then you kind of withdraw. I know about the image defining factors. Yes, which would make your operating more interesting. But um I'm just trying to get a feel from you. Um Should we try to resect tumors? Um I know you even said that if uh it's intermediate risk and has less than 50% uh volume shrinkage on chemotherapy, then that's good to mean that you can go and operate. But um we strictly stick with 50% on tumor volume shrinkage. And also a response maybe to how the bone marrow is involved or what specific factors should we consider um in the light of who should we operate and who we can't operate on? That's, that's a very excellent but very complex question. Uh to answer, uh I think, you know, the the answer for it uh again, has to be stratified for all different risk risk groups. As we have seen that some very low risk patient can even be treated without surgery just by observation and they will still do very well. A and um you know, these are obviously patient who have, you know, a favorable age, they're very young, less than one year, no metastatic disease, uh L1 disease less than five centimeter. Now, those those can be um just observed, but obviously, observation can also be challenging and difficult and require um resources. And um and one should also in this category, weigh the balance between observing the tumor and resecting, resecting it in it. Different setting has different answer. Maybe the answer would be just to resect the tumor. And then that way, you know the biology of it very well. And um and you know, um the the follow up can be uh easier for the intermediate risk. The important principle, uh although these patients do require multiple diab that are, they require chemotherapy and surgery, but their outcome is really good. So at the time of and surgery does uh benefit them. So, but at the time of the surgery, once we put in mind that these patients do well, so um the uh prioritization of uh uh you know, organ preservation, vascular preservation is a is always there whenever we do uh an uh neuroblastoma surgery. But it should be even, even uh a more uh prioritized for intermediate uh risk patient that you know, if, if one felt this last diel of tumor left in this very dangerous blood vessel, uh that it has a really high risk of injury, then um you know, leaving it maybe will be uh you know, based on, obviously, this is based on the the volume of o of, of the surgery that the the patient the surgeon does and uh the level of, of, of expertise in, in, in the room with it. But it will be quite acceptable for intermediaries to avoid uh major bleeding or major complication at, at all costs because, you know, they will do really well in terms of high risk, multiple studies. Uh Now, I think it has settled uh the gross total resection for high risk, more than 90% resection does improve outcome. Although overall the outcome of this patient is really poor and they require uh um essentially a lot of um multimodality uh therapy, multiple uh modalities, you know, as we, we, we went through. So it's not only surgery that they need, they need uh you know, any other uh modalities. So, again, uh the organ preservation comes in the prioritization because those patient needs their kidneys, they are gonna get heavy chemotherapy, they're gonna get really prolonged uh uh therapy, really toxic therapy. So um a a nephrectomy or a major injury to uh blood vessel will set them back uh significantly. What they, what they really need is the chemotherapy, the multiple the therapy and they need both kidneys to be functioning. They need to, you know, you know, um uh to minimize the chance of major complications. So, yeah, it's, it's a really difficult balance. Thank you. Thank you. It's, it's, it's, it's challenging particularly when you try to assist. Um But you, your hands are tied and you feel like you might be doing more harm than that's because that's the thing. I, we want to go ahead and resect all of it if, if possible. But then you find yourself in a situation where you're resecting the primary, but the Mets didn't really change much or it's a high risk uh classification. And you said they like what's, what's the real end goal here? But sure. Thanks for your explanation. Yes, that, that's an excellent point for high risk. Yes. High risk are tricky and they require high resources for sure. And, and um and it should be clear on the multidisciplinary discussion based on the tumor biology and the available uh uh therapies. What is the goal of therapy? Uh uh But you know, um aiming for a therapeutic goal uh is, is, is, is achievable. Obviously, it's not the, the cure is not high in this category, but it is still uh uh you know, potentially can, can, can be uh controlled. OK. Thank you doctor for that um explanation. I just wanna give my comments um from our setting. Definitely the most common solid malignancy we see in childhood is nephroblastoma. We see maybe two neuroblastomas a year like Sello was saying, and the difference in the presentation of these patients is that they're usually older, so older than 18 months, definitely and usually stage four. So they're already high risk patients. I would say majority of the patients that we're seeing and that's why we have these conundrums. Uh coupled with that is the lack of other biological markers um to stratify them. Uh but we are able to do and mix stratification at least. Um And then, so with these patients, our uh most guiding factor is metastatic, complete remission after chemotherapy. So we would look at the imaging and if there are image defined risk, risk factors, we would uh probably contact a larger center where there are more experienced surgeons. Uh There's uh more ICU um capabilities and more theater time because these cases last probably more than, you know, more than 2 to 6 hours. So we can't spare that theater time for a stage four neuroblastoma. Uh and you know, the pediatric anesthetists also um are needed. So there's many factors that go into deciding whether we can get them to resection, which is why we don't do a lot of resections of neuroblastoma in our setting in South Africa, I think we're moving more towards um sending these Children, especially with Idr Fs to um bigger centers with those, those resources that are um you know, more abundant. So, yeah, that's, that's our challenges on our side. Um And yeah, I don't know if you have any comments about that, but uh we, I know Idr give me palpitations. So I'd rather just uh because of the lack of experience that we have here, the, the low numbers, we're not getting the experience in doing this very intricate vascular um surgery. Absolutely, I am very supportive of your approach. Actually, it's, it's, you know, there is evidence to support this approach uh will improve care, uh improve outcome because, you know, for something that is that rare, that complex centralization is, is key. Uh because you know, the, you know, you, you're basically benefiting the surgeon at the, at the, at the Major sur center as well to accumulate the, the experience and, and uh uh and improve. Uh basically the, the um the surgical outcome. So I think that's, that's, that's fantastic. That's, that's how it, it, it should be uh um done in terms of the, I'm, I'm really happy that you are, y you know, you have the most important uh uh you know, bi biological uh marker that you can measure the a and make. And I think that's, that is wonderful. Many other sides don't have the capacity to do the uh and make measurement. But when you don't have that uh the C PPO DC, probably some, an alternative uh surrogate marker uh that can be used uh to rest 35 which is essentially LDH and ferritin, you know, LDH of more than 750 International Unit and uh um A and also af uh significant race F I think um about more than uh 100 and 20 nanograms. Those are like cut off point if, if the of the fri uh the LDH and ferritin are, are higher than that, they give you an indication that likely this tumor is fine. It's like an aggressive, you know, tumor was very high turnover. OK. Mhm So this test can be used in lower resources. Yeah, we, we have um been using them but uh yeah, lack of, lack of experience. But um uh I know we did publish uh I was involved in Doctor Van Heerden um publication where he did uh look at the those values as well. Um It's a shame Derek uh is not able to talk to us because he is. Can, can you hear me now? Yes, we, we're all looking forward to your insight. Thank you. I'm glad that the, the issue is resolved. So II had to be unmutated by the host. Oh, that's, that was the me. So sorry. I actually II don't know what the question was because I was so busy trying to play with the it um, did sell have a question for me or was it just a comment or it's just your insight? I think, you know, on the um, you know, OK. So, I mean, we have, so, I mean, I mean, just one thing. So your shower was talking about Jacques Van. He's um research on neuroblastomas in South Africa. So probably out of our solid tumors. We've got the most South African literature on that because he's done his phd in it. And, and that paper that your show was talking about. I presented that at our, our recent conference um a couple of months ago. Um But that was if, I mean, that was purely on high risk and effectively, you know what I'm actually looking at these slides, those slides. Now, you know, if, if someone had metastatic, complete remission, they did, you know their survivors was 40 so for high risk was 45%. But if they didn't have metastatic remission, it was 1 1%. So, I mean, there's a huge difference but he also looked, you know, if you had, you know, if you had remission and you operated, then your survival was up to 30% but no operation was 7%. So, I mean, that's four times, four times the survival if you operate. So, I mean, effectively, I mean, effectively what we are advocating is these patients should be operated on. Um And then, and then you, you basically just bring you bring up the problems of um sort of expertise, icu anesthetics theater time and all of that. I mean, and that, I mean, that is a problem, especially when you pushed for other, when your hospital is pushed to do other cases. So you can attend to three or 45 other cases in that time and, and not doing your neuroblastoma. Um I mean, the, the, the one way around that, I mean, obviously you get experience. Yeah, I mean, the more you do, the more experience you get, I mean, you know, people advocate well, in the meantime, send them to a more experienced center until you build up experience. But it's difficult to get experience if you're sending them elsewhere unless you go and work at other centers for a while, but it's also institutional experience. So it's not just yourself, it's the institution that's the, the, the anesthetist, your theater staff, et cetera, um your POSTOP IC and all of that, I mean, it, it's quite funny that um that actually is giving this talk today because actually tomorrow at our oncology MDT, we've got three neuroblastomas to discuss and actually one is 15 days old with bilateral adrenal masses and metastases. So 15 days old, still a neonate. We've got another one in our ward, a nine month old that's got a pelvic neuroblastoma that came with the intestinal obstruction that we have to divert. And then there's just another post neo adjuvant one for treatment that's just over two years of age, but with multiple sort of image defined risk factors that are that we need to review and then discuss with anesthesia and ICU regarding doing it in the next two or three weeks. Um But yeah, I mean, the just the the bottom line is if you look at Jacque's work on, on neuroblastomas in South Africa and his surgical work is he, he's an advocate for, for surgery for people to do surgery because he's shown on all the, all the South African patients. I mean, and he included patients from 2000 to 2016 that the outcomes are better if you operate and you get your, so it's for high risk, you're going through a 90% reduction if it's your intermediates between 5090. And then obviously your lowest patients generally by the nature themselves, they, they are resectable and, and that's that there is a, a request in the chat, uh uh for good eye for you to share the, the, the paper you referred to. Uh but that was called neuroblastoma and the role of surgery. And um also a question uh uh from Saha Hi uh about the uh the um the challenges with neuroblastoma um encasing celiac and sm Yeah, it's uh it's a, you know, maybe II can um just summarize the uh the approach and, and please have, you know, have your input on that. Obviously, this is very, you know, a very challenging um anatomical ar area a and really taxing if, if injury to occur on the sme uh or the celiac. Um So, uh when approaching such, uh uh you know, uh a, a tumor in that sort of uh difficult anatomical area, one normally start from uh below uh the tumor where you see the vessels clear and not encased by the, by the uh tumor and then work uh the way either up or down from that clear zone of the aorta. And, uh, and then if you're coming from down uh up, uh then obviously the first thing that uh one will find following the order is the um, um inferior mesenteric artery and you would expect its location based on what type of bowel rotation are you doing? Are you now doing, uh, rotating the bowel to the right and approaching the art from the left side or you've done some dissection or you're rotating the bowel to the left and you're approaching the aorta from that difficult, uh, um, you know, altogether window. But let's say, uh, the bowel is rotated to the, to the right, uh, then, you know, you'd expect the, uh, the, uh, inferior in artery to be, uh, pulled and pointing a little bit to the right. And, but, you know, uh, when with, as you're marching up, be cognizant of its location, it's not very far away from the bifurcation And then when it's diff uh identified, cleared and you march up, then the next, uh you know, stations the renal arteries and the crossing renal vein. These are all, uh you know, sh she would be expected and then cleared, uh all the way until you, you reach that critical area. You asked about the uh sma A and the celiac and obviously, the bowel is rotated to the right. The SMA also is expected to be looking slightly uh midline but slightly rotated to the right. So when, you know, that's, that's when you, you fashion your, uh your 12 o'clock by section of the tumor a little bit to the left to avoid the artery, see the artery, clear the artery and then, uh, you know, it's, it's uh uh march to the, to the um uh celiac axis and so on and so forth. Some people also, uh uh uh you know, go to the, uh a cruise and, and find out today and then follow it from, from top down but uh as we mentioned there, it's a vascular procedure. The first part is to delineate the vascular tree, preserve the vascular tree, make sure that you see it all it is not injured. Then you start thinking about where is the tumor? OK. Thanks doctor. Um I think uh there are a few other questions. Um There's a question from Mansour. Uh I think you kind of explained this anyway, but his question was uh the surgical technique. Uh if it was occupying the IVC and AORTA vessels in inside the tumor. So I think you've covered that. Um how you would approach it. And then again, it s MA NC like axis I oh it was, it was doctor Saha. So you answer that, but for sure, maybe I'll, I'll, I'll uh after greeting Monsour, I'll go a little bit of further details about uh you know, uh for branching blood vessels that are going into the tumor. And maybe a safer approach, if one can delineate the aorta really well, and then be able to see the origin of these blood vessels is 10 to dissect the tumor on top of the on of the origin of the, of those blood vessels and marsh all the way to its distal um distal um uh you know, uh extension. Uh e essentially that, that is the how I uh dissect the tumor on top of, of the renal artery. After identifying, clearing the aorta, seeing the origin of the renal artery, then by bisect the tumor on top of the renal artery and then, you know, follow, follow that. Um And you can follow it so far. But obviously, when you get really close to the small branching uh uh vessels from the renal artery there, uh you know, that it gets more difficult uh and down there and maybe, you know, sometimes if there is tumor in that territory, it, you know, you can leave some microscopic tumor rather than injure the, the, the, the, the blood vessels. Ok. Thank you. I think that was um nice and detailed. Uh There's one more question by Doctor Gautam. Can you give more info about 15 miles of adrenal mass? But she's, she's referring to the, the question that you asked in the beginning. Yes, that is actually that question is really good question for this discussion. Maybe I'll share it uh back again and then we can answer it together. Do you see it on the screen? Yes. Yes, we see it on the screen. So let's go one by one. So an infant with a MS disease likely, you know, it's not gonna benefit from surgery much, especially if it is, you know, a a and make an amplified and conservative. The therapy uh will be the for the, for, for this special type of, of metastasis. The MS which is, you know, we talked about the less than 10% infiltration of the bone marrow and a spe specific pattern of metastases that are uh sort of liver and skin but not bone if you have bone metastasis, this is, this is not MS for A N unit with a 50 mills adrenal mass. There is a Japanese study that's really started the discussion on this category of patient where the Japanese were trying to improve the outcome of neuroblastoma and thought maybe uh uh like AAA surveillance ultrasound will, will help uh uh diagnose them early. So they did the se the se uh uh ultrasound, they identified a lot of patients with this uh you know, possible masses in the adrenal and observed them and many of them, you know, did well and the mass actually resolved spontaneously without, without uh surgery. So then it, you know, this led to a trial question that included patient who was, you know, less than uh less than one year, less than three centimeter tumor and they were observed and, and that showed that they did really well and the tumor resolved spontaneously. And then, you know, the second trial asked for another question, so on and so forth. But this is the like I in some, this is the very low risk patient that can also be treated conservatively with observation. This is a tiny, small uh uh uh tumor uh I in a very young uh baby, there was no adverse uh uh uh factors, no metastasis in terms of the image with stage, uh patient with stage four disease that is uh anemic nonamplified. Uh And uh in comparison to the one who is stage four and at the celiac uh primary. So the, you know, the patient with celiac primary tend to, to do um uh uh a bit worse and many of them are anemic amy actually. So I would, I would select the, the, the, the, the c for many reasons for it. It, it's an infant, it's not a 40 years old, obviously, the uh the older age, the worse, the prognosis. So this is an infant and has a and, and, and non me amplification. So, you know, these are all um a factor that will um point that this patient will benefit from surgical resection the most of, you know, uh uh in comparison. OK. Thank you. Um I don't think there are other um questions that I can find on the chart. Um So I think we can start closing off. Uh Thank you, Doctor Hubs. This was a really informative, really thorough talk um for our surgeons to start attempting these. Um and um to keep in mind the anatomy as you explained it and um got quite a, a good technical approach. Um And uh yeah, I think it, I'll let you make last comments and uh pro will be loading this on youtube for anyone who wants to uh have AAA Relook at the recording. I know I will before I attend to another neuroblastoma section. Uh So last comments from you doctor. Well, I just wanna thank you all um for your time and for the invitation, it was a great discussion and, and thank you all for your input. And uh yeah, we'll, we'll hope to continue continue this. Ok? Thank you so much and thank you. Thank you. Thank you. Thank you. Thank you very much. Thanks. Thank you. Ok?