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Summary

This on-demand teaching session is led by a specialized orthopedic surgeon, Dr. Yoko Yun, who offers his expertise in understanding and dealing with bone tumors in pediatric cases. He covers how doctors can identify aggressive bone lesions in children, the significance of various signs like the Codman Triangle, and the implications of pathological fractures. Using real-world case examples and X-ray analyses, Dr. Yun explores pre- and post-operative strategies, including neoadjuvant and adjuvant chemotherapy. The session delves deep into how to approach cases of osteosarcoma in children and offers tangible guidance for non-orthopedic surgeons. This teaching session offers crucial knowledge for medical professionals seeking to improve their understanding of pediatric bone tumors.
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Description

Summary

Of the Zoom academic meeting of the Department of Pediatric Surgery in East London, South Africa, with guest speaker Dr. Jaco Viljoen presenting on bone tumors in children.

Key Takeaways:

Topics:

Introduction

Typical Osteosarcoma Case

Periosteal Osteosarcoma Case

Ewing's Sarcoma Case

Soft Tissue Sarcoma Case

Principles of Ewing's Sarcoma

Challenging Cases

Audience Questions

The next meeting is on January 16th with a talk by Dr. Giulia Brisighelli on “Acquired anorectal problems in children in LMICs”.

Supporting media

Learning objectives

1. Understand the common symptoms and typical patient profile related to bone tumors, particularly osteosarcoma. 2. Recognize key radiological and MRI features indicative of aggressive lesions or processes in bone tumors. 3. Learn about the different investigations and procedures involved in the diagnosis of bone tumors, including CT scans, MRI, biopsy, and chest x-ray to check for metastasis. 4. Gain knowledge about the standard treatment protocol involving neoadjuvant chemotherapy, surgery, and adjuvant chemotherapy. 5. Understand the implications of pathological fractures on treatment options and become familiar with approaches to limb salvage surgery versus limb ablation.
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The following transcript was generated automatically from the content and has not been checked or corrected manually.

Hello. Yo. Hi, how are you? Good. And you? Good, thanks. Can you hear me well? Yes. Yes, I can hear you. Nice and clear. I just Oh, wonderful. Uh Give you um a right to share and then you can share your slides and we will, OK, let's quickly see we can go forward and backward. Perfect. Let's try that. So we'll sit here. Thank you. OK. I'm trying to share the powerpoint itself. Is that working now? Yeah. Uh We can see your first slide if you can just go maybe two slides. Oh OK. So the X rays. Yeah. No, no, we can see all three. OK. Yeah. Um If you don't mind uh what bios you have sent, I will introduce you and then you perfect directly from uh I think this is this slide. That's perfect. We can do that. Not a problem. Thanks. We can start there. Actually my privilege privilege to introduce all invited speakers. That's wonderful. Perfect. Yeah. So, yo, I'm just keeping my video off because I'm uh uh conducting the meeting from home and I was perfect. Walk in the background and there are still like almost 10 minutes. We will start just after five o'clock. Perfect. Yeah, that's wonderful. Thank you. Looking forward. Yeah, thank you. Thank you. Bye II. Just want to thank you. Oh, fronting video that you will ever say I have made an incredible sac something that you should ever. I made an amazing investment in the most vegan activist that has ever been the personification like Jesus, I thought. Yeah. And let me just say this, I got a bit of a sleep thing and it doesn't disappoint ii way too. The question. Which a meeting? Yes. Uh Yes. Yes. Um Yeah. Can you see my first light more? Yes, I can. Thank you. Ok. Um Hello. Uh good afternoon, good evening. Um Welcome to the last Zoom, a academic meeting of this year. Um the Zoom academic meeting of the Department of pediatric Surgery in East London, South Africa. And, and we are really uh happy and um and grateful that uh doctor Yoko Yun uh has agreed to talk to us about bone tumors. What do we pediatric surgeons need to know about this? Um uh Doctor Fo is a highly qualified and specialized uh orthopedic surgeon. He is a tumor sepsis and limb re reconstruction orthopedic surgeon who practices in neck care unit at Montana Hospital in Pretoria. He did his undergraduate training in 2000 four and a master's in medicine and Fellowship of orthopedics in 2015. He for six years, he was the head of tubal sepsis and limb reconstruction unit at Steve Beco Academic Hospital in Pretoria, uh affiliated to University of Pretoria. And now he has been in private practice. He is married for more than two decades to Melissa and he is father to four kids. Uh So he certainly understands what it um uh uh is important to Children. So, yo, thank you for, for agreeing to give a talk. I will stop sharing and you can start sharing and then share your expertise and knowledge with us. Thank you so much for having me. OK. So I just wanna make sure everyone's seeing the main, the first light. Yes, we can see it. Thank you. Uh did something now. Got it. OK. So, um fortunately for you, um this is not something you will uh deal with often. Um So I kind of wanna try a different approach to this. Um So please just bear with me. It's the first time. Um I've given a talk like this to non orthopedic surgeons. So I'm gonna try and, and, and get a, a middle path where there's some overlap or where it might be something you get exposed to. Uh just to give you some guidance. So I just want to start with a textbook, typical case. Uh so typical age group, 10 year old boy complaining of a left distal femur uh pain or discomfort with swelling. And what I'm about to say is critical is for some weird reason, there's always this trauma uh history, um which obviously throws a lot of people uh off because they'll start with the initial assessment. They'll always think it's a sports injury and, and start with physio and so on and that's often where the wheels come off. So this, this trauma history is, is very uh uh um um uh we call it a red herring. Uh it just throws you off track, so just always keep that in mind. Um So just typical X ray features. Um So you can see there is a, a lesion, a distal femur, I am going to point with this laser on the AP here. You can see there is this li lesion over here. Um So it is a mixed li sclerotic picture. So this is normal bone, the distal femur, you can see it is a immature bone. So it is a young person or immature bone person. Um What's interesting though is, do you see this periosteal reaction approximately? That is what we call a Codman triangle. Um That is a feature of a highly aggressive lesion or or process. And then what is very interesting as you see on this far left uh picture, you see the soft tissue mass uh or uh soft tissue calcification. And you can see the soft tissue um expansion here as well. And on the lateral, you can see the li picture again, you can see there is a cortical uh break anterior cortex and then there is this posterior component and anterior as well. This is a typical feature of aggressive lesion. One we would be very concerned. Um not something that we'll just leave and we will investigate further. So one of the biggest investigations to to do in any child is always a chest X ray, make sure there is no metastasis to the lungs, but generally the push is to get a CT scan. Ok? MRI scan apologies. So on the MRI, you can see typical features. So on the T one sequence, it is always dark um which is not specific, but it is just a feature. We we generally see what's textbook of uh osteosarcoma is. Do you see how it's grown all the way to the growth plate and then it stops, you can see the tumor expanded, it went through the cortex. So it's a, it's an extra medullary expansion which is already a worse prognosis for this child. But you can see it's extra uh compartmental already, but you see it doesn't go past the growth plate, which is a typical textbook feature of osteosarcoma. And the reason for that is it needs cells that replicate quickly to, to actually grow. And when it hits the growth plate, the growth plate is a cartilage and cartilage is very slow to grow. And that's why it's a kind of a barrier. It's what we call AAA natural biological barrier. But I'll show you advanced cases where it does eventually go through if you just wait long enough, this is just a post gadolinium scan just to show you the uptake. So you can see it's a high signal intensity and then the typical feature that is heterogenous. So you can see it's kind of dark here and brighter there. So it's just areas where there is necrosis areas that's very vascular and growing and on the lateral. So I sort of move the this picture, minimize it. There we go. Then if you look at the lateral, you can see what we saw on the X ray, the soft tissue component posterior and anterior. And that's just a bad prognostic sign that the fracture of the, the tumor is already outside the, the, the uh bone. So it is not in C two anymore. It is already extracompartmental and already on the grading 12 and three, it is already a grade two and it is actually a grade two B already. Um So I quickly go to the later. Here we go. This is an actual view to show you. So this dark black thing anterior is the patella, this is the distal femur and you can see this large extracompartmental soft tissue mass. And what I just wanted to highlight is to see how close it is to the vascular structures. And that is why we also MRI S to see if it's involving or encroaching on the neurovascular bundle because that often hinders uh limb salvage surgery. Um And then the other reason for the MRI is just gonna go back, I'll show you some examples later just to show you that we often find what we call skip lesions in the same bone. And that is a form of local metastases. Um And that is also relevant for um, limb salvage surgery and also for prognosis. So what generally happens is with these kids is that we do a biopsy, we confirm the diagnosis. We get the oncology, pediatric oncologist involved. They start with what we call neoadjuvant chemo. So it's chemo before surgery. That's usually a 10 week process. Then I'll do surgery whether it's limb salvage or limb ablation. And then they go back for adjuvant chemotherapy, which is about six months. That's the principle. We just want registrars to understand. Um if they do know about the actual um agents that are used um like methotrexate, um then obviously they are more your claudi type candidates. So if you do know the, what the oncologists give, that's great. But generally we just want them to know the basics. What happened to this child is unfortunate. As you can see, he was, he finished his 10 weeks and he was about to go home. He got up from the bed and went to brush his teeth and then he sustained this pathological fracture. Now, this used to be a AAA nightmare uh previous um or a few years ago, we believe that uh as soon as a person has a pathological fracture, then that means they automatically have to get an amputation. Fortunately, there's enough evidence that even though there is now a fracture hematoma that once this heals, we can still go and do limb salvage surgery. So I kept him in above knee cast. I'll show you the next one here. Um And you can see that's how he healed and you can see how well his chemo worked because you can see the tumor itself kind of uh uh ossified completely. So the tumor itself stopped growing and it made for a relatively easy uh excision. And I then offered this child uh limb salvage surgery where I did a distal femur resection and then I did a, a total knee replacement uh tumor prosthesis. Um I've got an example of one later. This reason I show you this one is, it's a bit above I think your age group, but it's still in the typical age group, second decade. This is an eighteen-year-old boy. And the only reason I want to show you this is just to show you the other very common side. So you saw the first one is distal femur, which is probably the most common we see. The next common one is proximal tibia, which is this one I want to show you and I just want to show you this as well because this is what we call a textbook osteosarcoma. OK. So if you look at this, you can see it's a bone forming tumor, you can see it's aggressive. The way we say it is aggressive is, is do you see how it's expanding outside the cortex there? This is the Codman Triangle I mentioned earlier and this is uh what we um say it has got a wide zone of transition. So you can't really with a pencil outline exactly where this tumor is. You are not sure how far it is extending into the lateral compartment here. So that's a wide zone of transition, bone forming tumor. And once again, do you see how it's going all the way to the growth plate and kind of sparing the phys of the? Yeah, the phys distally um of the epiphysis distally, it's gone up to the five CS. And whenever you see a case like this, this is an osteosarcoma. Um we always joke and say differential one osteosarcoma, there is only one aggressive bone forming tumor and that's uh osteo sarcoma. So just to show you the other common side, just an MRI just to show how aggressive this is, you see the soft tissue expansion posteriorly anteriorly, almost going through the patella tendon here, which is a big problem with surgery. And often the only option here is an above knee amputation, which is quite sad. This is just an example. This is something I think we uh maybe get to, to you or you'll be consulted about is this girl presented with an upper limb swelling. That was it. She's 14 years old. And if you look at her, her upper uh or her arm was circumferentially swollen um around the uh uh midhumeral region. And once again, I've seen it, if this was not x-rayed, uh it would have been missed. And that's why the principle is to please, whenever you have a mass, always X ray, at least because you wanna make sure it is not bony. And if it is bony, you want to make sure it is not involving the bone. Even if even if there is a soft tissue tumor, make sure there is no infiltration into bone. So if you look at this, I am going to show you the next uh picture as well. So the AP and the lateral. So you see on the ap how this could have been missed. It is very subtle. But you see at the distal end on the lateral side, there is a bit of a periosteal reaction there, you can see a bit of sting speculation over here. But you do see this large soft tissue component to this. If you look at the lateral, this is what we call soft tissue calcifications. You can see a large uh uh posterior periosteal reaction. And very importantly, even on the ap uh this indentation in the bone called scallop, uh well, not scallop. It is scoping, but scoping is generally from the inside out, this is more compressive uh uh feature of something pressing on the bone. Now, if you look at the Mr just to show you quickly here. Um if you look at the MRI, you can see that it is largely a soft tissue component, but it is circumferentially around the bone, but it's also intramedullary. Now, this is what we call a periosteal osteosarcoma. Now, they are less aggressive uh but they are still uh uh malignant tumors and they are uh surface osteosarcomas. So the one Children get is called periosteal osteosarcoma. So it's literally in the periosteum where it starts and that's why it generally goes circumferentially around. So this child went for her neoadjuvant chemo and then I did surgery. Now, the problem was is this tumor. You could see it extends quite, very proximal and quite distal. I only had three centimeters, um distal bone and six centimeters, proximal bone. So I had to get a custom made prosthesis done for her. The prosthesis itself is, is, is the same as all other patients. But the stems are the problem. I'll show you now. So you can see here the proximal stem they had to make for me because the normal stem is 15 centimeters. This is six and distally, they had to make one that's three. And then also they can't take screws. I asked them to make holes for me so that we can get some stability. And initially, it looked great. She was very happy for about a year and unfortunately, she had local recurrence and the arm had to be amputated. Um So just very briefly, I'm not yet to give you too much info. But osteosarcoma, the incidence is quite low. It's about five cases in a per million people. The prevalence is about 2.4% of all pediatric cancers. The age bracket is, is very critical for you to understand it's called a bimodal distribution. So it's in the young kids in the second decade, generally between the age of uh so it's usually 10 to 18, which is the most common. 10 to 14 is the most common age bracket. We do get, I've had an eight year old girl with osteo sar and obviously we go up to 21 and 22 with the adults. And then the second pick is more the older patients that's above 65. Um And that's usually after radiation or after something that they get a osteosarcoma, the male female, slightly more dominant in males and then something very uh uh typical you'll see is it's much more common in our black or Hispanic patients compared to white patients where Ewing sarcoma is the opposite. Ewing sarcoma, we see more frequently in, in the white patients, which is quite interesting to see primarily affects the metaphyseal region as you saw distal femur, proximal tibia. Um and then other sites which we see a lot is the proximal humerus um proximal femur. I've seen a few pelvis is fortunately very rare because that's a horrible presentation. The joint skull is not my field. So I can't give you more on that. Um Just a little bit on genetics. The reason I just wanted to add this is I think this is more where you might get exposure to this. So I'm sure you are aware of something called a retinoblastoma. I'm sure it's not something you personally treat, but it is something you might pick up or get consulted about because there is a pediatric tumor. Um And but once they have the RB gene, uh so if they present as a, as a young baby or, or, or a young toddler with this, we will keep an eye on them because they have a higher predisposition to forming an osteosarcoma later in life. And then the other one that's very important is what they call ap 53 tumor suppressor gene. Um The syndrome it's linked to is called leaf from Many Syndrome. Um and they have a high incidence of breast cancer and um osteosarcoma. So just quickly, the four syndromes I wanted to uh mention to you. So lef from many I just did, but then Rothman Thompson syndrome is also also inherited autosomal resist uh uh recessive inheritance. All three of them, the reason I added these is if you look at the Rothman Thompson one, they've got an increased risk of gastric adenocarcinoma. And that's something you might be uh exposed to and maybe even the cutaneous uh uh uh uh basal cell carcinomas and squamous cells. And in Bloom syndrome, they also again have high gi tumors which is more your, your field. And then the Wagner syndrome is the one, it's uh adult Progeria, which is um uh uh premature aging. Um and these kids have a high risk of osteosarcoma. So, just to mention those, it's usually something that might pop up in AMC Q. Um just to be aware of it but not, not to stress too much about it. This case is um very interesting, very sad story. So a seven year old boy, he is adamant that um he was at school and he bumped his hand against a chair and uh the swelling started and within two months, fortunately, they ended up with us, they were referred to to our pediatric orthopedic unit, but they could easily end up with you because uh pediatric surgeons also treat these young kids and he just had swelling of the hands. So it could easily, easily be mistaken for a, a soft tissue mass or soft tissue tumor. Um On X ray, you can see uh if you just look at the middle uh metacarpal, the index one is normal um with a bit of pressure effect from, from, from, from the uh soft tissue component. So if you look at the metacarpal, it is very sclerotic and you can see the thin pero reaction on the side there. Um but a large soft tissue component and this is when we realized you look at the lateral, look at the dorsum of the hand. That's what he had. It is a soft tissue uh mass. Now typical of Ewings is is this where they, the bone involvement is not really impressive but the soft tissue component of it is is very bad. And these Children often present with fever, they often present with um high white cell count, high infective markers like CRP. And that's why they are often misdiagnosed as Osteomyelitis. So just keep that in mind, this is just to show you the the the soft tissue component. So here you can see the the metacarpals on the actual view and this is the massive, I mean significant soft tissue component to this tumor. Here you can see uh on the um uh coronal view, you can see the uh metacarpal and then with these large soft tissue components, let uh both sides to it. Now, the fortunate thing for us um is these, these tumors are fortunately very chemosensitive. So this child was sent for, for chemo and just to show you. So on the top left is the prechemo and look at the right top right post chemo, you can barely even see a soft tissue component. And again, on the coronal view, the large soft tissue component and bone involvement and look at the post chemo minimal and almost the bone almost looks normal. So this child, I did a um middle finger ray amputation and he did so well. Um he had no other Mets um and I'll, I think there's another picture of him later. I'll discuss it with you later. So case five is a fourteen-year-old girl. Very sad story. She um is adopted, doesn't have a uh uh don't have parents. She was adopted very, very um involved uh uh uh uh adoptive parents. And they felt terrible when she came in and we told them she's got cancer because, you know, initially for a few weeks, you know, a child complains of leg pain. You kind of think, ok, it's nothing serious, just keep an eye on it. But when I noticed swelling, so if you look at the X rays, she came to casualty, which is not a common occurrence, they usually go to the clinic, she came to casualty because she had this large swelling on the, on the left leg. If you can look at this, you see this large soft tissue component. And then on the X ray, she was done diagnosis, having uh chronic Osteomyelitis, uh which is a common feature. If you look at the X ray, do you see the bone that's destructive? And then you see a space and then a a AAA sclerotic persea or bony pers reaction. Then another gap and another line, this is the textbook feature of what they call lamellated um periosteal reaction or onion skin reaction. This is a uh a typical feature in, in Ewing sarcoma. Um The reason is as the tumor rapidly grows, the periosteum doesn't have time to, to ossify. So it get, gives you that uh gap and then the tumor set and it can throw down a piece of bone and then the tumor grows rapidly again. So it's got a stagnant period of a few days uh where it stops and it grows again. And that's why you get this laminated picture. Once again, she had no other Mets on pet CT. It showed no other meds. Um She had a chemo just want to show you the prechemo MRI. So as you can see, this is the tibia. Uh So look at the right leg that is a small fibula and the big tibia, it's a, I mean, the tibia is about three centimeters and look at this large soft tissue component to this tumor, which is quite aggressive. And at this stage, I would almost say irresectable. And then she uh sorry, I did not show the post one now, then post chemo completely resolved and I was able to excise only the fibula. Um And she also had a full year of a happy life when she came back, she had suddenly had me meds all over. Um And the same thing happened with a five year old boy. So, um it's quite sad how these kids do so well for a year and then they suddenly come back with meds all over even though it was so chemosensitive. Once they come back, the chemo doesn't work. So it's something we are uh quite uh frustrated about this case. II put in because this is something you definitely will get exposed to. So this is a child. It's a I think it is nine year old. So I can't see. There we go niney old uh male that presented with this right thigh, um soft tissue mass. So this is something that will definitely be referred to you as well. Uh If you look at it to me, obviously, it does not look benign, it does not look like a ly poma. It is a bit more aggressive, skin is quite shiny, uh happened over a few uh uh months. Uh and you can see the skin here is changing colors as well. So to me, this was not something uh something benign. So we did an MRI that show, sorry, it doesn't want to advance. There we go. So just once again, basic principles, x rays which confirmed there was no bone involvement. You can actually see it is a clear soft tissue mass um on the AP is over here. Um MRI was done and you can see it is definitely not lipoma because it is not, it is a different color to the fat. Um We were thinking maybe like a liposarcoma which would be quite typical in this area. The age group didn't really fit in. I was not expecting what I found. So what I, why I wanted to show you this is, please be careful with biopsies. Um generally with soft tissue sarcomas, sarcomas. Uh we want to do what we call core needle biopsy. So through this just a little uh neck in the skin and then a biopsy, I just want to show you what can happen um with it. So this is how the child came back. And I mean, once you break uh that tumor, um the capsule um and you and you and you kind of aggravate the cells, this can happen. And I mean, this changed the whole picture. This suddenly became quite aggressive, almost irresectable. Fortunately, I was able to still excise that it's a, it's a literally from the, from the hip to the knee large excision, fortunately clear margins and the child went on to do quite well. Uh I just couldn't close it obviously. So I had to do a skin graft um just on Ewings very brief. It's very little. We actually have to know or understand about it. But Ewings sarcoma, it's always in the articles you you read Ewings and then this uh dash and then pet pet is peripheral neuroectodermal tumors. So they think we're not sure they think Ewings um comes from a peripheral primitive neuroectodermal tissue. Um So these both fall into what they call a family of small round. Uh uh we always say small round blue cells. So small round cell, uh neuroectodermally derived tumors, they have overlapping entities with same histological origin. So the only different, they have different degrees of differentiation and that's the only difference there is. So P net has neuroectodermal differentiation and ewing sarcoma doesn't, but they look on a slide. So similar that he can't really differentiate. So the only thing you have to know is is especially in the M CQ. They often ask this, what is the mutation in Ewings or PT? And that is T 1122 translocation. That's what I always taught my registrars to memorize. So they are present in 85 to 95% of cases, which is, which is the majority. This leads to the formation of a fusion protein between Ews and fly one. So it's so Ews is easy. It's like Ewing sarcoma. So that's easy. And then fly one, you just have to try and remember. So Ews fly one. So in the M CQ, always remember that and this is identified with PCR of fish. Um And this is how they differentiate between Ewings and other round cells like lymphomas and things. Um And, and uh rhabdomys, uh myosarcomas and uh uh uh uh what's the other one? Uh small cell osteosarcoma also is a small round blue cell. So, less common translocation is still t but it's 2122. So, it's quite easy to remember. T 1122 is the most common and then the other 10 to 15% is T 2122. So it's not the worst uh thing to remember. And then the proteins between EWS and E RG. So not fly. One. Um Metastatic disease quite common. About 50% go to the lungs and a quarter to bone and another 20% to the bone marrow. And then uh 26 to 28% present with actual distant macro me when they come in. Um I want to show you it's adult cases, but the principles I think is relevant for you. So I'm gonna show you two examples. This is the scary thing where patient presents with a mass. Uh and a sonar says that is a hematoma. Now, I don't know, but when this picture was shown to me that does not look like hematoma. OK. So I'm very sorry, but that is very worrying. And we also teach that any mass um larger than three centimeters uh should not be excised and needs a biopsy at least first. Um a massive hematoma obviously is something that just gets drained. But this person um does did not have a medical aid, uh did not want to go to state. So he went to a private surgeon. Um and the surgeon told him uh this is a hematoma. Uh I'll be in and out quickly 45 minutes. They paid private fees. Uh, just to give an example, private surgery fees in theater just being in theater 440 rand per minute. Now, this, uh, if you book an hour is 18 grand just for theater time, the surgeon fee anywhere between 20 30,000 rand, depending on what he did. Uh, a fee, anything, anywhere between 10 and 15,000 rand. You get my drift. It is an expensive thing. And what happened is, is he jumped into that, got into it and realized it is a sarcoma. Uh So this principle, I really want to hammer on because that is wrong. Jumping into tumors is wrong. Its principle is wrong. You need to investigate further. You need to do an MRI to make sure it's not infiltrating uh deeper or to see the whole extent. And then you do a biopsy and then you get a diagnosis and you plan the surgery. Ok. So anyway, he jumped into that. It took him 3.5 hours. So because when he opened it, it wasn't a uh a hematoma. He realized it's a sarcoma. So they then tried to excise it, um which took 3.5 hours. You can imagine the cost for the patient in the end and then he had a large drain in. So this is what happened. He came back with this. Ok. So this is what I tried to tell you earlier, these tumors if, as soon as you start getting into them or near them, they get very aggressive. OK. And this is typically what happened. It, it looks similar to that child. Correct. So this is what happened. This is how it grew. This is when he was eventually, uh he ended up with me. Um, and it was via Facebook, uh not via Facebook, ironically, sorry, apologies. Another patient. This was uh actually through uh uh uh uh a friend that realized uh this patient needs help. And I'm a tumor surgeon and they tried to get into me and I was able to help. Unfortunately, it was so bad. The only thing I could offer him was a hip dis articulation. Um He already had metastases um And I just tried to make him a bit more comfortable um just to try and help him with personal hygiene as well. Um And this was a picture sent to me. Five months later, I tried to hide his face, but he's got a massive smile and uh he passed away in a comfort of his own home without this fungating mass. A few months later. This is another one where um it is quite scary. So this is a patient that presented with this lytic proximal tibial lesion. And the biopsy was done. Now, if you look at the X ray, it looks like a giant cell tumor called a G CT, which in theory is a benign tumor uh can be very aggressive but uh locally aggressive, but it is a benign tumor. Um This case was done by my uh uh uh my professor a few years before I was there. Um and he did uh an MRI MRI. I can see it is quite aggressive, but the histology confirmed it is just a giant cell tumor. So this patient then had this resection prosthesis done and you can see it's completely resected. So everything looks great. And then this patient, when I took over the unit came back and he had this and uh sad to say, but it was obviously not a giant cell tumor, but it was something called a giant cell rich osteosarcoma. And uh this patient refused amputation and uh subsequently passed away where the only thing I could offer him was an above knee amputation. So just be very careful. Um The principle from this case, I wanted to teach you or, or discuss with you is we always approach tumors as a multidisciplinary team. Um So it's the surgeon, it's the uh radiologist. Um it's the pathologist for the slide. Um And then it's off for the biopsy and then it's often even the oncologist that get involved. So please just be aware because cases like this do happen where you think it's something benign and it ends up being something quite aggressive. And then um I told you earlier that the principle is that if you get a child with a mass, you have to do a biopsy, confirm the diagnoses. And then afterwards, generally with osteosarcoma and Ewing's, they do neoadjuvant chemo, then um surgery and then adjuvant chemo. That's the principle. Unfortunately, we live in a, in a, in a interesting country where we don't always get that privilege. I'm gonna show you a few cases. So I wanna ask you honestly, how do you send this child for chemo? She's got obviously a large um septic wound where the tumor has grown so big, it's actually killed off the skin, the skin has become necrotic. The tumor is peeling through that and this child presented to, to the emergency unit um in a hospital um um 50 kilometers from us um with an HP of two. Now, I was quite um ii wouldn't say um not, not, not sympathetic, but I mean, I told the cash doctor that they are not transfusing her unless the parents agree to an above knee amputation. Because otherwise, what they do is they just delay the suffering of these kids. OK? Because if you not transfuse her back to an HP of 10. Um and they refuse the surgery within a few weeks. HP is back to that and she's busy dying slowly from that. So fortunately, the parents accepted the amputation. I'm going to show you that. X uh sorry. Uh that's another case. So um they accepted the um amputation, they brought her across, I did an above knee for her and she had such a big smile because this horrible painful tumor was gone. Um, and she survived a few more months because with a tumor, this big there's already mets in the lungs, uh, before they passed away, just another example. Uh, another twelve-year-old boy. So, do you see this fating septic wound? Look how swollen his thigh is compared to the right one. Look at the extent of this. There's no way you can do neoadjuvant chemo and then do surgery. So I've often been forced to go straight to surgery to try and just give these kids some form of comfort. So I do in this kid, I had to do a hip articulation to try and get some control. Um and also uh gave him a little bit of this uh relief until he passed away also from the disease. What I'm about to show you might disturb you. Um This is a 10 year old girl that was referred from uh a peripheral hospital called Rob Ferrero Hospital, which is 300 kilometers away. Uh We are there referring it's a different province but there is no one in Mpumalanga that does any tumor surgery. Um and uh we have an arrangement with them. So they call me directly. I accepted the child, child arrived and you can see it is bleeding profusely. I am going to show you a horrible video uh that was sent on the night. Just give me a sec. So sorry, let's take this. Uh oh. Mhm. Ok. Now it's not working. It's gonna set to uh oh there we go. OK. So you can see there it's eroded um into the uh popliteal artery and it is bleeding profusely. Um This was sent to me at night by one of my registrars on call. Um and even with this, the parents still refused an emergency amputation. Um and we just put a proper compressive bandage on and she subsequently passed away three days later. Um This is an example of where we actually do send the child for neoadjuvant chemo trying to do what the book suggests. Um And then the child comes back, no metastasis on the lungs. And then uh this is MRI this is what I mentioned earlier. If you look at the second picture, do you see this dark lesion? So the femur is of the, the, the the shaft is involved with the tumor, then there is a normal tissue and then the suddenly the skip lesion. So this would be a grade three tumor which is uh um a local metastases and then unfortunately doing an amputation and then the child comes back with these horrible local recurrence. So where I feel if we had done the amputation earlier, we probably would have saved her. But for 10 weeks with this large tumor, she had to still wait. Um So I think our presentation in this country is much later and we often have to make decisions. That's not always textbook. Um This is another example uh of quite a sad case. It's a 12 year old boy presents with this textbook, distal femur bone forming tumor, large uh expansion outside the bone. Um He had his neoadjuvant chemo and then refused amputation, which is quite sad, came back. And if you can show the X ray, you can't really even see what's what uh that's the femur. I'm gonna try and get my laser pointer again. Apologies. There we go. So you can see the distal femur or femur then distally, there's absolutely nothing. It's just this bone forming large tumor and then somewhere over here there is a proximal tibia. Um And unfortunately, he, he passed away soon after this. Uh we did not even offer surgery because he was so hectic. Um and um literally be uh um um um on his deathbed. If you look at his chest X ray, you see what we call cannonball, uh metastases so large. Um It, it is in the lungs and I am not sure if you can see it, but look at his left shoulder. So look at the proximal humerus that's normal. Look at the right. He's got burning metastases to his own humerus, which is quite a poor prognostic feature. This is a good one. So this is a nine-year-old boy um where he presented once again to our pediatric unit, not to the tumor unit, which is interesting. Um Just shows you the delay because even from the peach unit to us, it can take a few more weeks to see us. So fortunately, the professor was there on the day he saw this called me immediately. I was three doors down. I went to see the child. We, we got an urgent MRI we um and I discussed with the dad and I said, listen, I am not going to take another 10 weeks for him to get chemo. Um I would suggest early surgery, get rid of the tumor and then do chemo. And that is what I did. If you see here on the actual view, you can see it is literally encroaching onto the um vessels and I just decided I am not going to give this child um 10 weeks of chemo knowing this tumor is going to get slightly bigger and risk, he is going to end up with an amputation. So we did surgery immediately. Um I did a distal femur resection, total knee replacement. Um And what is wonderful to share with you is three years later. Um He had now a short leg and I had to go, if you see this link, it's a three centimeter link. I had to open him and put a new link in and I saw him again now a year later, he's doing so well. And we are already planning his next link for when he's 16 years old. So he's really, really doing So well. Um and after this case, I had a long discussion with a pediatric oncologist that we should maybe consider in our specific unit to consider this when we get these massive large tumors and we actually get a child that might be salvageable. This is just to show you an example of what happens with these kids if they don't come early. Um This unfortunately is not the child's fault, um or the system's fault, it's the parent's fault. So this is a child, seven years old that presented with this uh proximal tibia bone forming, aggressive tumor. Um at a, at another um tertiary hospital, uh academic hospital near us called uh it's the Sefa Makato University and the hospital is called George Maka. And they also have a professor there that he's retired now, but he was still consulting at towns. So they got this child, uh diagnosed osteosarcoma and then wanted to start chemo and do ablative surgery. The dad refused. Um The, the doctors actually got a court interdict against him. The dad is a lawyer though, it's quite important info. And um, he went to court and said, listen, you know that the he wants a second opinion so he knows the loopholes and immediately the court said, oh, yeah. Yeah, you, you've got a right as a patient to um a second opinion and he, they weren't allowed to keep him against their will and they discharged him. The dad never took him for a second opinion. He took him home, they went for uh different things other than what we would feel is necessary. And then he presented to our casualty one night and this is what he came with. He had this large uh uh uh tumor. I'm just going to show you the picture. It's fungi, it's coming through the skin. Um And he, he actually was, they were suggested that he comes and sees me and the dad promised to um we had a colleague that spoke a half an hour with the dad and explained to him what needs to be done. And unfortunately, he still, he said he agreed and never came back. So one night, the mom brought the child to casualty, she brought the child without the dad's knowledge. She actually went to uh T's University. We've got uh the lawyer department. They've got uh obviously like registrars uh in training, they've got students that are learning uh law and they do a free clinic. So she went there for some uh legal advice and the lawyer said, listen, you take the child, we will get a court order to do this. Um The child arrived and I saw them and the lawyers told me they're getting a court order and then the dad arrived and he told the court the tumor is getting smaller the moment he said that the court said, listen, you know, you can't go further. Uh if the dad says it's getting smaller. So the lawyer called me and we had to within three hours give evidence that it was getting bigger, which wasn't difficult. We had the X rays I showed you before that one and we had this one and anyone with two brain cells can see it's getting bigger. Uh We had given the latest literature which was easy. We just googled osteosarcoma found the latest article and printed it. Um And fortunately, the court phoned me um and said, I'm pleased to an amputation which I did and the child subsequently did well for another year, unfortunately, because the dad left it for a year. Um He passed away with lung meds just to show you the tumor. Uh It's quite a gritty appearance when I cut through this. It was, it, it's like uh cutting through uh uh uh your uh gritty tissue, it crunches as you cut through it almost like uh um uh what's that honey honey comb that sweets the like a um uh uh uh what is the sweet, a chocolate, sweet, that yellow honey comb stuff? It, it breaks like that when you cut through it. Uh So quite, quite an aggressive tumor, quite a nasty tumor. This last one I'm sharing with you is to show you something that might uh be referred to you or you get exposed to be very careful with um Children that present with a history of uh uh sports injury. This child thought he, he sprained his hamstring. Uh He went to a, a physio, physio was massaging his hamstring for two months. Uh uh uh Even a GP referred them to the physio for that. The GP unfortunately is one of those that never even touched the child's style, never examined him and never did an X ray. So for two months he was doing that, it wasn't getting better, it was getting worse. Eventually they went to another GP. This GP actually did an X ray uh and showed this. Now, if you look at it, you can miss it. If you look at the AP you can see on the lateral side there is the soft tissue calcifications. If you look at the lateral again, there is like minimal going on post here to the uh femur femur itself looks fine and you get the soft tissue calcifications going on, but look carefully at the hamstring. You see there is large soft tissue component to it and that was initially missed. Um And this child had um at that stage, no lung meds um did an MRI and you can actually see it's an intramedullary tumor that posteriorly broke through and created this large soft tissue mass. Um At this stage, I offered uh ablation um chemo and everything. And unfortunately, the mom um whose father is a GP. So it means this kid's uh granddad is a GP. The mom is what they call a Reiki healer. I had to Google that it's part of the homeopathic medicine stuff and she believed that that would heal him. And then four months later, she brought him back when he was gasping and we just gave him oxygen and he passed away. So she uh was part of his demise because he had no lung meds. And then four months later, he was gasping on his own uh meds in his lungs. Um So that is me. Um Are there any questions? Um Yako, we will certainly invite comments and questions, but I would just say if it, it was absolutely wonderful. It was uh you, you are a born teacher. Yo. So, so thank you, sir. I appreciate that. I hope you still have some teaching attachment. Um And, and your teaching registers because II can see it is in your blood and uh and before I invite questions and comments, can you give us a take home message? Uh You know, we, it's uh we uh don't see them often but what would you tell your pediatric surgical colleagues? Uh just take home message, be very careful for the, it's just a lipoma. Um I think it's where majority of the mistakes I've seen have been made uh is with assumptions that uh it's just a lipoma. Um We'll, we'll quickly just excise it. Um The principle remains, um The guidelines are quite clear. If it's, if it's bigger than three centimeters, you should actually be. Um um doing investigations. Um, MRI would be, would be the best. Um, I know, um, we had struggles at the BCO even where the MRI was down for six months, which was an absolute nightmare. I know, uh, um, not all facilities have it. Um, so at least then use ultrasound, which is helpful. Um, but that one case I showed you was an example where, um, ultrasound, uh is operator dependent where they missed a clear tumor. But my advice would be is um always investigate all tumors with, with x-rays just to make sure there's no bony involvement, whether it's coming from bone or uh in or infiltrating into bone. Make sure you always biopsy lesions larger than three. If it's less than three, you can do an excisional biopsy. Um that is completely acceptable. Um And, and please don't be scared to ask advice. Um There is very few of us doing this just in orthopedics. Um in the country we have, I think less than 10 uh dedicated orthopedic surgeons doing, doing tumor work. So it's a very rare uh field and in private um um all the surgeons are referring to me as well. They don't want to do any soft tissue sarcomas. Um And even the pediatric surgeon has told me, uh fortunately, she doesn't see a lot of them, but if she does get them, she'll refer to me. So I think it's just important to you have open communication and make sure don't don't put your foot into it. Uh rather ask advice. Uh I mean, these days with technology, uh I mean, I'm a whatsapp away. I'm an email away. Um um even though we are in two different provinces, um I'm always willing to give advice or help. So I think that's the key message is, is never be afraid to ask for help. Yes. Uh Thank you. Yo, I think that is so important to be humble and to ask for advice. We are uh blessed in a way. Uh You, I'm sure you know uh your colleague Koshi Daniel, who is a very orthopedic surgeon, but he is our pediatric orthopedic surgeon. So he's here. He's always available and we are also blessed that we have a fully functional pediatric oncology unit. So, so they look after all the chemotherapy. Um uh Yes and, and that support is great for us but uh just uh uh uh uh um uh something to substantiate what you have just said that today in our clinic, uh me and my register, we saw a little girl who is about uh not even two years old and she has had a swelling on her scarp, which initially we thought it was a hemangioma, then we thought it was maybe a fibroma or something like that. And it suddenly a follow up today, the mom showed another lump on the scar and a left supraclavicular lump. So I said, no, this is not just something simple. This is very likely to be malignancy. We have MRI but not that easily accessible. So we are just going to get a ct scan of the skull, brain, chest and abdomen and pelvis done and and uh then we will decide further. We will certainly need to do a biopsy as soon as possible. So I think that just to substantiate what you have said is something more than three centimeter. Just don't think that it is something simple. Think about it. Can it be malignant, take advice? Do investigations do biopsy? Uh Thank you. Um I will. Uh, now, um, uh Daniel Vi is our senior medical officer. She has a question, Daniel, go ahead. Thank you prov, can you hear me? Yes, yes, yes. Um I just want to ask, we actually got today a va referral um, of a patient that I just want to show you the X rays for. Um, it's an 11 year old boy had a history. Let me just start sharing my screen so that you can see there. Um Can you see the, the my screen now? Not yet done yet? Not yet. Let me just uh hm. Were you given permission by the um, mine organize of the Zoom meeting? Otherwise you won't share any, anybody, anybody can share. Uh, anybody can share. OK. Yes. Yes. I've still multiple participants can share uh simultaneously. OK? Cause there's a message from that said pro now to screen sharing Um Yeah. Uh my my here it uh still says uh sorry, uh multiple participants can share simultaneously. So try again. Yeah, I will try again. Prof ok. In the meantime, del uh I'll just ask for comments from um uh other consultants who are here. Um I know uh Carla Van and Professor Saro have have commented but uh Carla, anything else you would like to add? Uh Carla was our register and a consultant for some time. She's in, in northern Ireland. So Carla, anything else? Um, hi, good afternoon. Can, can you hear me? Yes. Yes. Ok. I don't look very presentable. So, um, my, my camera was erroneously on previously, but I've switched it off now. Um No. Um, thank you. That was really a fascinating presentation which actually just makes me miss home. Um, so much more, um, just because of the, you know, the pathology that, that we see there, but really fascinating. I am a bit rusty on my orthopedic. So I'm very useful to um, see those images and just get a refresher. I mean, where I am now, um, most sort of uh any lesions on, on the legs don't even come through pediatric surgery. So we wouldn't, we wouldn't see that it goes straight to, um, orthopedics. Um, so which is probably a good thing. Um, I suppose, um, because they'll probably have a, you know, a higher threshold to, um, or a lower threshold to suspect something sinister when it is something more sinister. Um, yeah. So I don't really have much to, to add, I, I'm, I'm afraid, I suppose I can add that in, in, here in northern Ireland, people present really early. Um, so we definitely don't see advanced pathology. It's, it's so, so rare and, and that is for all other tumors you do not see massive tumors like that. Um, people present early. So, outcomes are generally much better. Um, it would be unheard of for a child to be admitted here for, for palliation, you know, it just absolutely. And they've got dedicated, uh, sarcoma units in the UK, um, that, um, that are quite, um experienced and they've got world-renowned surgeons working there and they've got their, uh, referral systems down to the key. So, no different, different, completely different to what we used to hear. Yes. Yes. No, it's, it's, it's true. Um, interesting that you mentioned the sarcoma surgeons. Um, my, my very first senior registrar when I did my general surgical training. Di Strauss, um, is one of the Sarcoma surgeons at the royal, you know, he trained in Stellenbosch. He's a South African too. But yes, all sarcomas go, they go to, to London. They not done locally. Yeah, but a very good presentation. Thank you. It was excellent pleasure. Thank you. Thank you doctor. Uh, maybe it's not here. Uh Are you able to share your screen? I think she is off prof Oh, ok. Uh, we just might be trying to re reenter again. Oh, there bottom there done. I'll see her. Oh, there we go. Oh, good, good. Yeah, we can see your screen now. You can share the image. Oh, there we go. OK. So I know what that is but you can ask, you can hear me. Yes. Yes, we can hear. Now you can give your diagnosis and then doctor will, will correct us or guide us. No. II want to actually ask um what it is because it was basically an 11 year old boy. He presented with a history of falling while he was playing rugby yesterday at three o'clock. Yeah. Um and went to the local hospital with pain of the left leg. He couldn't walk and no fever, no wound, sustained, no other comorbid. But in clinical exam, he was febrile tachycardic, um anti antalgic gait with a swollen in the left leg, he's warm to touch um and further neurovascularly intact. So I did tell them to speak to orthopedics because I'm not sure. So I want to know um what, what do you think this is and how to make it? So I know what it is. Uh But I'll run through your approach. So if you look at that, do you see on the ap uh we're dealing with a lytic lesion. Um If you look at it, you see how, well it's a sclerotic margin around it. So it's a, it's a narrow zone of transition. Um It's got a sclerotic border which means uh it's been there for uh some time. Uh If you look at it, it's sitting poster. So it's what we call centric. So it's not centric. And um this is a textbook example of a non ossifying fibroma. So it's called A NF. Um It's got other names when you Google. It, it's uh O CD, it's a uh AO FD. It means it's an osteo uh uh uh C FD cortical fibrous defect because often on the, on the one view, it looks like there is no cortex and it's just because it's fibrous tissue. These are always coincidental findings. It's the most common tumor in, in all of us. It's just in adults. By the time we see it, it's already ossified. So it's healed. So in kids, it's often a, a minor sports injury. The bone is obviously weaker there where the fibrous tissue is compared to the bone. And then this is when it's picked up. So there's most likely a little fracture. That's why it's so sore. Uh Once you splint him and uh it heals within six weeks, he will be completely fine and then a few years if you x-ray him again, it will be completely gone. Um So no need for a biopsy. No need for MRI. No need to investigate further. I think the febrile part might be completely coincidental, but um definitely nothing to stress about completely benign tumor Ok, perfect. Thank you so much pleasure. It was quite interesting ve ve very nice, the very appropriate case for, for his advice today. And um I saw Doctor Uz Juma um in the meeting there. Are you still around anything from Red Cross or from your overseas experience? Um Hi there prof thank you so much for um allow for the meeting and thank you for a great talk. Um I just wanted to ask with regards to the principles of biopsy. Do you suggest if we have a soft tissue lesion to do what, how we would normally approach the soft tissue tumors and and do a true cut biopsy uh to and ensure that whatever skin that we go through is included in the uh eventual excision of the lesion. Yes, absolutely. So, I mean, true cut biopsies um are completely acceptable. Um The, the, the the tissue amount is just very small. So we, when we have our multidisciplinary team meetings, they, they often complain that they don't have enough tissue to actually uh investigate further. So, um I have um core needle biopsies that I use. Um So I've got different sizes, 345 millimeter size. So I take a large core of tissue um and you do it through a small incision and then you can uh just put a suture into that and the principles remain that never violate more than one compartment. So let's say it's on the thigh and it's in the anterolateral aspect, go from lateral because when we go and excise it, you have to excise that whole compartment uh uh or have a wide excision um of that muscle. Um And if it's lateral, you can do that. If you go uh biopsy anteriorly, uh you have to excise the quad uh and then you lose your quad mechanism. Um There's biopsies that's been done through the patella tendon. Um So then when you, if it comes back as malignant, you are in deep trouble because now I have to excise that compartment, which means the extensor mechanism will be gone. So, never violate more than one compartment. Um The other principle is to uh have a hemostasis uh because it's that bleeding that actually seeds it to try and always maintain hemostasis. Um If you do an incisional biopsy, um always make sure your drain comes out in line with the uh uh uh excision, not to the side of it. Uh Because if you, if it comes back malignant, you have to where that uh port also came out. And that will become quite an extensive soft tissue reconstruction procedure. And if you do come out of uh just below your uh suture line, make sure it's within a centimeter or two, not four centimeters away, even though it's in line, it's going to be a large excision to cut that out. Um Always make sure um you find the easiest lesion to biopsy um And this is where it's kind of changing the field for me a lot is where intervention radiology comes in where they can do ct guided biopsies. So, if I can, I always try and go for that, especially your difficult to reach places like your pelvis, um obviously vertebral uh lesions um and uh even, even uh hip lesion, um if they can do a CT guided biopsy, it's much uh safer and, and more correct or, or uh uh uh more accurate then uh when I go open and look for it. So I think those are the principles. If you stick to that, you'll be safe. Thank you. It's a pleasure. Thank you, Echo Neha. Um Has uh has this uh advice answered your question or do you want any more cla clarification about biopsy? No problem. It's perfectly fine. Thank you very much. OK. Um Now I think last two comments I'll ask is my consultant colleagues. Uh Doctor Nikou Mohave is one of our consultant, pediatric surgeons. NC. Any comments, anything to share? Um Thank you, pro thank you, Doctor Felo. That was um a fantastic presentation. Um So, so recently, um we've had um a case of a, of a KD. It's, it's a, it was a, the lipoma, but since it's so population, it um not just a lipoma, but it was so large on the, on the left um anterolateral aspect of the thigh. So we decided to biopsy confirmed that it was a lymphoma, um, but it was quite unusual to get a lymphoma in the pediatric population. But a more interesting case is, um, we also had a kid d who had, um, this anterior abdominal wall mass that we initially, um, did suspect that, um, it was malignant. We did CT scans, um, ultrasounds, all the relevant investigations took him to theater. Um, and when we trialed to do a biopsy, the mass was essentially mostly cystic. And when we tried to biopsy from uh the solid aspect of the, of the mass, um we couldn't really get much of it out. Um So it came back as no malignancy. So we decided to take the child to theater for an excision only to find later on histologically that it was a confirmed ewing sarcoma. So the has been initiated on chemotherapy. But um it was also um I guess an interesting, an interesting presentation um because we've um I've had, we've seen them on the anterior abdominal wall. Um and just, you know, the, the, the diagnostic approach and um all the interventions that we had to go through just to get to that diagnosis was also interesting. Yes, very true. So, II think I actually missed it. So this child that I presented earlier, this extra uh uh of this soft tissue mass, this is an, this was an extra skeletal uh Ewings. Um So it, it, it does happen um always keep in mind that's one of the issues with biopsies, um, is you can easily take a biopsy of a part of the tumor that is not representative of anything. Um, and that's why, um, if you do take a bigger specimen or a, or a larger piece of it, um, it's less likely to miss it. Um, so especially if, if you have a mass that is, has got necrotic areas, be very careful to take only necrotic tissue because that's just dead cells. They won't see anything. So always take uh um that part plus uh uh a part that's more, almost more normal tissue. If I can put it that way, then your, your yield will be a bit higher. Mm OK. No, thank you doctor. Fully pleasure. Yeah. Is it safe to say that if it's a large mass, rapidly enlarging solid, rather think of malignancy uh in a child. Is that safe? Absolutely. Yeah. Yeah. Absolutely. Yeah. Rapidly growing. Um uh skin that's shiny uh vessels that are dilated around it. Those are all features that this thing is rapidly growing. That's never benign. Yeah. Yeah. I think the final comment I would ask is uh our senior pediatric surgical consultant doctor Sell Mataya Sello. Uh Any comments. Hi, thanks. Uh Thanks Hiko. Uh Good, good, good talk. Um The it's, it's good that you are actually seeing soft tissue masses because unfortunately, where we are, if it's not involving bone or directly into the joint, it's not an orthopedic problem. So it's good that someone is actually taking initiative there. Um My one question has to do with particularly with the recurrence uh rate where they the the kids come back um with metastasis. Um because I know the principle is limb sparing, uh avoid any mutilating procedures. Um But last case scenario, worst case scenario you amputate. Um And with some of the kids you presented today, they had limb sparing. And I always wonder for me personally, my margin is it clear enough, particularly when you have a very big tumor where you unless if you have frozen section on, on on hand, how, how, how confident are you that you have clearance of the tumor at the primary site without actually doing an amputation because I personally always worry that I might be leaving something behind. Um by not just removing that appendage. That's a problem particularly I think it was case number three or four, the one with the upper arm kid where you, it was a quite significant tumor where you remove the tumor and they put in the orthopedic gadget. Um And unfortunately came back with metastasis. Um How do you kind of decide? Do I sout, do I try to do limb salvaging procedure or so on? Yeah. So just ironically, it's a bit of a mind shift thing because if you go look at the literature, um there's actually less recurrence with uh limb salvage than with amputation, which is quite uh mind boggling to get your head around it. But if you think about it, um, amputations are done for more aggressive, more advanced cases. So the chances that you're gonna uh go through uh uh a vessel that's got Mets in it. So, one of my worst cases is a child with distal femur osteosarcoma and I did a high above knee amputation. I was 17 centimeters away. Now, I mean, if you talk about margins that is far away and in the amputation specimen, they could see tumor in the veins going back to the groin, they could see tumor cells. So I mean, that is scary as can be. So the reality is, is um the the guidelines for tumor surgery has changed a little bit. Um There used to be this thumb suck. Um You need a centimeter of normal tissue, you need three centimeters of normal tissue. That's all very thumb suck. Guidelines. Um There's much more clearer uh guidelines now where they talk about a biological barrier. So if you have a biological barrier, so that's uh argument's sake, let's say um a child has um a distal femur tumor. Now that's in the bone. So when we do an excision, we leave the anterior muscle um on top and then excise above that. So we take the quads, but there's still a muscle anterior to the femur that we leave and that is seen as a biological barrier. So if it hasn't gone through that yet, that's your barrier. If you excise near vessels or nerves, if you can excise the uh uh the uh a neural sheath or the fascia, that's a, that's a barrier, then that is seen as, as, as still a, a clear margin of tissue because for the, for the tumor to go through that barrier takes time. So if you have a clear barrier, so normal looking tissue around it, that's your guideline. And that's why we've been able to have literally one millimeter uh uh margins with no recurrence in the kids. So there's always a risk, there's a 25% risk of recurrence, which is high. But um um um we are definitely moving towards limb salvage surgery more than ablation. If you look at international literature, obviously in South Africa, uh and more African or third world countries, the, the amputation rate is still very high because the kids present late, but we're definitely going more towards limb salvage. Perfect. Thanks. Thanks. Pleasure. Yes, I think it is also um eye opening uh to see how cultural beliefs uh uh they, they sort of mess with the management of these Children and they are actually denied. Uh You presented at least a couple of cases where there were court orders necessary uh to do the right thing for the child. And I think that also delays their presentation and, and complicates their course and and decreases their prognosis. Uh Am I correct Absolutely. Yeah. It's probably the biggest challenge we have is, um, and it's, it's, it's whether it's cultural that you believe in, um, your own type of, uh, medicine, whether there's now homeopathy or traditional healers or whatever, uh, you have to respect that because patients, that's how they grew up, that's what they understand. And they'll go for that for a few weeks, months even. And it's only when they realize the tumors are getting bigger and bigger that they actually present to us. And we can actually see the traditional cuttings on the tumor and they can actually see how they actually marked how it's enlarged over the few weeks. Um So we have to respect that. We've got religion issues where patients uh believe they're gonna be uh faith healed. So they go to church, which, which is fully understandable. You have to respect that. Um So, yeah, it is challenging. Um I think as a surgeon we always just feel, you know, it's obvious, you know, cut it out or cut it off. Um Unfortunately, it's not that simple. Uh We deal with, with a, with a, with, with a human being with a different belief systems and cultural beliefs and we have to respect that and kind of work around it. So, um I've learned in the years not to, you know, oh, you don't want it. I'm gonna chase you away. I try and work around it. I try and tell them, go for it. But please just realize we also wanna help. Um I don't cut off legs for fun. Uh I do it to save a life. So we say sacrifice the limb to save a life. And unfortunately, if they don't go for that, they often come too late and then, and then they still feel that they just died at the hospital, uh which was the kind of belief from the start and that's what we want to try and break that. Uh um um uh what's the word um stigma? We want to break that stigma about, you know, kids coming and they're gonna go home without the leg or dead. Um And the only reason that happens is because they present too late. So that's what we kind of, that's why we try and teach registrars to pick these things up. I teach uh I taught undergrad students um what features to look out for. And that's kind of, I think the only way we can move forward is to be aware and to have uh easier access. So that's it. OK. I think that was very uh uh interesting and, and very, very educative. Yeah. Thank you very much again for sharing your pleasure and, and we know where to ask for advice if we, yes, obviously we will go through Koshi. So that, yeah, going to be easier. And uh yes, thank you, everybody who attended and contributed. And uh this is the last meeting of the year and I hope all of you have a peaceful Christmas and, and uh healthy and happy New Year. And the next talk will be on the 16th of January Tuesday. It will be by Doctor Julia Bri pediatric colorectal surgeon at Baragwanath Hospital and she will be talking about acquired colorectal problems in the LMS in Children. So, thank you again, yo. Um and uh with your permission, I will share the video on my youtube channel on various whatsapp group uh if you don't mind. Not a problem. Ok, thank you so much. Ok, bye bye bye.