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Hello. Hi, Sonia and Jessica. This is, hi. Can you hear me? Are you able to join the stage? Yeah. Hello. Hi, Sonia. Yeah. Hello. Hello. I don't know. Yeah, it's saying that I, that it doesn't let me use my camera and I have never used this platform before. So, mm, what do I do? So, just check on the screen for the main stage and try putting your camera on. Are, are you using a phone or a laptop? A laptop? Yeah. So just down below you'd see the camera button. Yeah, which is in red. So just tap on it and you should turn on your camera. Yeah. So it's, it's saying, um, an exclamation mark, an orange exclamation mark and I don't know why it doesn't let me use my camera asking me to turn off my, anything that is using my camera. But the thing is I'm to know what technique would you want to exit and rejoin. Yeah. Sure. I can do that. Uh, let's see how that goes. Yeah. Sure. I'll exit and rejoin. Thank you. Ok. Wonderful. It's much better. Um, still can't see you but that, that's fine. Are you able to share your screen now. This is uh yeah. How do we share a screen there? There must be some option here present now, more options. So what should we choose to? So present now allows you to share your screen? Ok. Present now. Share your screen, share my presentation. Yeah, share. Yeah. Slight show. Is that ok? Um Yes, it's just loading up, editing, continue to load. Would you give that a second shot? Hi, Jessica. Welcome to the stage. Can you hear me, Jessica? Yes, I can hear you. Can you hear me? Mhm. And that's great. You have challenges with your camera as well. Uh Is it necessary to have the video? No, not, not, not entirely. Um The most important thing is knowing how to share your slides and move them accordingly. So um Sonia gave it a shot and she's still trying to set herself up. You could try sharing your screen and presents for us to do the test one. Yeah. Hello. I can either share my screen or I can use my camera. That's a problem. I mean, I don't have enough memory in my laptop. That's the problem I'm having. All right. Um I think we would prefer you the screen to share the screen. Ok. Sure. I'll just share my screen now. I don't know what's happening now. This I refresh this page. OK. I'm just trying to share my screen, share your screen, open slides and share. Can you see my screen. Um, it came briefly but it's, yeah, wonderful. I can see it. Are, are you able to move through the slides just to show to be sure that it's moving quite well? It, yes, it is. Yes, it is. Um Yeah. Yeah. Yeah, I'm happy with that. Um, should we let just give it a test? Ok. So is it be my, my screen has been shared. Hi. Can you see my screen? Hello? Yes. So um yes, I can, I can see your screen. Is it going smoothly? Yes, it is. Yeah. And how can I um monitor chat because I will be asking question in the middle of the session. So how would im monitor the chat? Um There is a chat sign. Let me send a quick photo on the group page. You maybe uh and people um sorry, can people um unmute the microphone and talk to me is an option here? Uh Sorry. Yes, I think so. Uh Let let me ask, we have some members in your teams. Um Hi views. I can see Mohammed. I can see you can see Ahmed. Are you able to speak if possible? If not just type in the chat box? So we we can get a feedback if someone needs to tell me about it's in the chat box because I think I can't see the chat box here. I need to leave this and then go to the chat box, right? So sharing your screen for a moment. I'll see whether we can sort that out challenge is to stop sharing my skin. Yeah. Sure. Yeah. Yeah. Just are you able to try sharing this me? Yeah. II can see your screen. Um Are you happy to put it in flights? Share mode? Wonderful. Yeah, and you can try moving through just for us to see if it's all good and oo Yeah. Great. Great. Mhm. I just confirmed quickly on the cat box and the speaking of participant, I just need to check you. Oh, thank you very much. Uh Thanks. Uh Yeah. Yep, I could hear that log and here we did your place quite well for. Mhm. Ok. So I'll start off by sharing my screen and I then stop for us to have the first presentation. Ok. I'll just do the basic introductions and talk about RJ CFP and we can have the first presenter taking Uber after I stop sharing my screen. Would that be all right? Yeah. Yes. Sorry. One more question. Can the audiences speak to us um by? Ok. Fine. Yeah, thank you. Um Not currently. Um I'm still trying to get that um sorted out. I don't think the audience is able to speak. Can I confirm that from the audience we have? Are you able to speak? Just type in the chat box if you are, we don't have any messages yet in the chat box? Yeah. Right. So we shall be boosting the messages around so we can get more people starting to join and then we commerce usually five past seven. So see you guys in a bit. Hello guys. Um Good evening. My name is Hamid. I'm the chairman for the British Radiology Journal Club. Welcome to the events tonight. We are looking to have a fruitful session. We have our presenters, Sonia and Jessica with us. I ha I'll be happy if you could come to the stage as we plan to commence. So this general club has been and in activity sense, may we've aimed to discuss and properly appraise journals and researchers in the field of radiolog. The teaching is an experience that we aim for everyone to have and some of our past presenters are here with us. We can talk about how much experience it gained from joining these sessions. We typically have monthly sessions sometimes twice in a month and we aim to have a second session this month to increase the chances for more people to take up presentation rules and build their experiences. You can follow us on our various social media. You can subscribe for our monthly newsletters as well and join our pages on Instagram, join our whatsapp communities and follow us on Facebook and stay up to date with all our activities. Just do so by scanning these links, we shall be putting in the links into the chat box. So keep an eye out for them. We are having a very exciting session today, looking at interventional radiology, both presentations will be on the field of interventional radiology. Over the over the past couple of months, we have done something on GI radiology. We have done discussions on neuroradiology as well as respiratory radiology and taking a look at the use of A I in radiology. So monthly, we take a topic and try to analyze two articles on them. Our presenters would be happy to shed more light. We will be starting off with um Doctor Sonia and I shall leave the stage for her to take over and we can commence today's session. Thank you. I do have a wonderful session. The chat box is open. You can be interactive, you can share your questions and your thoughts and let's have a fruitful day. Um Thank you, Haid. Um Just one question. Are we recording this session? Sorry. Hello. Hi, Sonia. We we can hear you. Would you be happy to share your slides and take over? Yeah, just one question. Sorry. Are we recording this session or I mean, we shall we be, I mean, see you afterwards just for a feedback that how we performed and or how we presented? Yes, the sessions will be recorded and we will share with you as well. All right. Thank you. I'm gonna start now. Share my screen. Yeah. Right. So, hello. Hi. Um um Can you hear me just confirming? Yes, we can. It's quite a lot in care. Yeah, thank you. So, I am Sonia and uh to uh and um today, I'm going to present um an, a paper about desmoid fibromatosis is an interventional radiology sometimes to the rescue for an atypical disease. So this paper was published some somewhere in July 2024. It's a recent paper and it is in British Journal of Radiology and I have just pasted my link over here. So the learning outcomes for today's session would be we're gonna analyze the role of IR to treat desmoid fibromatosis. And uh so we will start with describing the incident and i aggressive demographics of desmoid fibromatosis. We will touch base a little bit genetics and pathological features of desmoid fibromatosis. And uh we will summarize different studies to determine initial treatment. We will roughly discuss different studies. And then we will compare the um non Ir versus IR treatment options and their efficacy. And uh what's the outcomes for the? Uh Then we differentiate between C A and he four, which is cryoablation and high intensity focused ultrasound. Um There will be a section about different images uh pre and pro procedures. So we're gonna have a roughly, I mean, briefly, we will um have a look at those images and uh we will see the guidelines as well in the end and briefly assess the recommendations and guidelines. In the end, there will be a little quiz just to um check the feedback from the from the audience and what they have gathered from this uh from this um lecture or this presentation and uh and how they're gonna build up their knowledge onwards moving forward and it will give them insight to study more about ethmoid fibromatosis, which is quite rarely um investigated topic and result. And there is, there isn't any very little amount of knowledge available in the literature. So first we'll start the presentation from just asking all from all of the audience that what is desmoid fibromatosis, any idea or anything that come to your mind? Have you ever come across this um this terminology? So I will ask Hamid if he can have a look at the chat and just to um because I can't see chat at the moment. So if he can just let you know, let me know that what people think about ESMO fibromatosis and uh and have you heard about it? What is it? And if you, you can use your microphone, then you can speak to me. So feel free to share your thoughts in the chat box. I'll be happy to read it out for soon. Yeah. Yeah. Thank you. Yes. The waiting, I'm sure people are typing. Um Yeah. So we have a Yeah, we have, we have um circulated this paper, I mean, just as a preread. So people might have some idea if they had a they have had a look. So I'm hoping. Right. Um So far. Hello? Hello? Hello. What's wrong? Uh, Ami. Hello? So, hello. I don't know if I lost her. Uh, I don't know what's wrong. Hello. Uh, if someone can just heads up or say something, shall I just continue when you hear me? We, ok, thank you very much. Sorry? Yeah, I've just lost her. I don't know where is now. So, yeah. So, um, so I'll just keep jumping just to, um, check the chat myself and going back to the presentation. So, yes, someone said in the chat that it's a desmoid tumor. Yeah, you are right. And it can present as mesentary mass. Yes, of course. Yeah, you are right. So, yes. So this is a little bit of interruption which I found in the paper. So it's quite a rare um locally aggressive soft tissue tumor, which is um look quite aggressive and it tends to occur isolated uh isolatedly and, but this cannot metastasize so on. S for wh O it is a clonal fibroblastic proliferation that arises in the deep soft tissue uh which present as infiltrative growth and has a tendency for local reoccur, but it doesn't metastasize. Um So location wise, um it occurs sporadically, um extraabdominal in the extremities in the trunk. But in 10% time it occurs syndromally with the other conditions called familial adenomatous polyposis and that tends to occur intraorally. But the majority of the time it occurs sporadically as an extraabdominal. It is associated with pregnancy, trauma, surgery oral contraceptives and it constitutes about 3% of all tissue neoplasms. It's incident incidences three and five cases per million and ps 30 to 40 years and it tends to happen more in females than in males. So these are a coup uh couple of pictures. Um It have, I mean, it looks at the back. So this is how it present genetics and pathological features, which we're gonna discuss here briefly. So, pathogenesis wise, um it happens with the dysregulation of Wnt pathway that regulates beta catenin. So, beta catenin is a kind of a proto oncogene coded by CDN N B1 gene which is implicated in the onset progression and malignant transformation of several tumors. So this is a common gene which can be found in several other tumors. So, the concentration of uh Kenin is down regulated by a PC protein coded by a PC adenomatous polyposis coli. So in sporadic desmoid fibromatosis, CT NN B1 K mutations are found and it tends to happen in three different mutations. T 414, S 45 FSF and S 45 P, which is which considered the 10 person. But that is the one which is quite, has got like a very malignant potential and a very poor outcome in patients. The syndromic syndromic one, the 10 person one which was associated with FA it, it happens because of the germline mutations of A PC. If looking at the histology of uh this um desmoid fibromatosis macroscopically, it is like firm, grayish white, like a scar. And as you remember, in the previous picture, it is like a swelling and there is a scar mark around it. So microscopically, it presents like that and microscopically um histologically, it is like a collagenous stroma filled with long physic cycles of elongated thin spindle cells which, which will look all exactly the same. If you see the first picture, it, it's just like a spindle cells and they all look uniform in appearance and it tends to, to show prominent but blood vessel as, as well under a microscope. So this is a microscopic pictures. These three. So moving forward, how do we treat desmoid fibromatosis? So any idea about the treatment options or have you ever come across a patient getting treatment for desmoid fibromatosis? So, in, so this, this question basically aimed towards the non ir treatment. Do you know any of the name of the drug on any treatment or any treatment modality which you have came across um, to treat desmal fibromatosis? I'll jump back to the chat to see if someone you can take your time. Yeah. People. Yeah. Any idea. Any, I mean, yes, someone said radiotherapy. Yeah. Yeah, you are quite right there. Yeah. Radiotherapy. Anything else that, that's, do you think that it can be treated? So I'm just trying to get answer whether it's the right answer or wrong answer. I just need to get some responses to check prior knowledge or yes, it's been mentioned in the re Yeah. Surgical resection. Yeah. Surgical resection is the one. Yeah. R therapy is what you're right. NSAID has been used as well. Um Yes, steroid injections. Yeah. Chemotherapy. Yeah. Yeah. This, this has always been mentioned in the paper. Yeah. All right. I think I got enough responses. Thank you. Um So, so these are the non ire options that has been described in the paper which has been recently used in 2024. I have just tried to briefly touch it because there were quite a lot of discussion in the paper that has been published. So they are the active surveillance which has got the key role in the management. So active surveillance has been describing the guidelines as the first line option or front line option. And um it requires like a watchful waiting for a couple of years, like 2 to 3 years and which which requires the patients being clinically um patients being in the clinics for assessment and uh radiological assessments like MRI or CT uh every 3 to 6 months during the 1st 2 to 3 years during that watchful period and then 6 to 12 months for uh afterwards. And the main aim for active surveillance is is to look for other problems like the pain management and um uh patients having any other problem or any infection or anything or I mean, if, if it's growing or if it's a small in size. We are just checking it some size as by the radiological investigations like Mr MRI or CT. And if there are three consecutive progression within those three periods, within those three sorry three year period, then we will offer treatment modalities. And our main focus for this imaging is just to reassess the critical areas like neck mesentery thorax because we have got vital organs there. So that's the point and we just need to make sure that it doesn't lead to any complication or like a like a complication which requires hospitalization, local treatment, um which includes surgery and radiotherapy no longer first line treatment. So surgery um reported like poor outcomes like a reoccurrence and uh and if someone who, who has like lots of comorbidity, so their outcome is not very great. So surgery was found to be useful when there is a significant progressive disease with acceptable morbidity and best outcome was only found in abdominal wall, um desmoid fibromatosis with regards to radiotherapy. So, radiotherapy has been performed by two ways. One is the standalone treatment. So, and another one is the as an achievement after the surgery. So, standalone treatment is radiotherapy is just used to treat it like uh primarily on the on the mass A phase two trial was uh done which is studied the efficacy of mo moderate dose radiotherapy and this was the dose 56 g in 20 fraction. So what they found that the three-year local control rate was 81.5% which is quite a good response. And uh as an achievement after surgery, there was a meta analysis which uh which targeted 22 different resection, which was the R zero resection and then one of the R one resection. So this R zero resection is just kind of staging of this tumor. So what they found out that there is no reduction in the rate of local reoccurrence with this R zero resection surgery. But with the R one resection, it showed it reduced, I mean reduced in the rate of local reoccurrence. And uh when they found out um in the la in the next three years, complete response in the first three years. So six out of 44 patients, they showed complete response, which is not too bad. It's 13.6% wise. And uh what they found out in the um in the young population is to started producing radiation induced sarcoma and the surgery. Sorry, the radiotherapy was unfortunately limited to symptomatic progressive desmoid fibromatosis. There was a trial for chemotherapy. So low dose methotrexate and vinBLAStine plus vinblastin or vinorelbin. The this is um a chemotherapeutic regimen which is, which is uh which was, which had a systematic review in 2023 and it shows 71 to 100% of uh um uh tumor response rate. I mean disease control rate that oral venelbin alone, it control like 64% of the uh of the patients. And there was also um conventional dose anthracycline regimen which is a conventional chemotherapeutic. Uh But there isn't any data available for that. Then, in this paper, tyrosine kinase inhibitors were described. So, tyrosine kinase inhibitor um inhibit tyrosine kinase which uh which is implicated in the progression of many tumors. And but there isn't much uh the the whole patho pathogenesis hasn't been described in the paper, but they said that it targets um platelet drive growth factor receptor beta, these tyrosine kinase inhibitors. So they mentioned four current TKI S which are Sorafenib, PAZOPanib imet and SUNItinib, which can be used lifelong and studies. They, they supported uh its efficacy as well. Side effects were not bad hypertension thyroid disorder. But the, the, the one thing and even on tyrosine G is they required regular reviews while on the treatment because these side effects that need to be sorted um when they present with it. Another one, another method is hyperthermic isolated lymph perfusion, but this is quite a complex procedure. So it is not included in current practice. Um So I'm gonna move forward now. Yes, someone mentioned NSAID and NTI nsaids. Yes. So, NSAIDS were also been um used in NSAIDS, been used as an observational study, but there were variable results and there wasn't much literature available, just case reports but not a proper study, but there isn't any availability of any proper study. Um So historically and said that anti estrogen was suggested as a first line systemic therapy. Uh But in 2011, systematic review, anti-estrogen showed some effects in half of the desmoid fibromatosis cases. But the data was the same was moderate. Go outside. Sorry about that. Sorry, sorry. Uh sorry about that. So, anti estrogen has some effects in half of the desmoid fibromatosis cases. But data was moderate quality means that uh there's just case reports and uh not any metasis was available were available. There were other W NT inhibitors, um sirolimus and immunotherapies, but they all showed like unsuccessful results. So that was all about the non IR treatment. Now, what another question which is for the audience. Now, what is the role of IR in the treatment of desmoid fibromatosis? I mean, anything that come up to your mind thinking about Ir and desmoid fibromatosis or have you been to an I Iri? Mean Ir Department have seen anyone getting treatment for desmoid fibromatosis or any answer? Radiofrequency? Yeah. Ablation. Yeah. Yeah. That's right. Well, yeah. So the next uh discussion uh would revolve around these two intense ultrasound scan. Yes. Yeah. Yeah. That is high focused ultrasound. Yeah, that's right. So our our next discussion will be uh will revolve around these um these answers that you have posted here in the chat. So the role is. So surgery was um not suitable for high morbid patients. Radiotherapy was um quite toxic for in some cases. So the benefits of going towards the IR is the reduced morbidity and reduced toxicity because these procedures which has, which has been been mentioned here, the side effects were quite few and they were quite suitable for any population with any other underlying background problems. So the first one is chemical ablation. Second one is heat based ablation which can be given either radio frequency, microwave or high intensity focused ultrasound. And then we have got cryoablation, then we have electrochemotherapy and then transatrial treatment. So we'll briefly touch these um uh your modalities. So first is chemical ablation. So in 2003, quite a long time ago, two patients with unresectable desmoid fibromatosis, they were treated with acetic acid as a chemical. So they used acetic acid to, to dissolve or to I mean to destroy that desmoid fibromatosis mass. But after that, it hasn't been described in literature. Another one which is like a heat wave type which is radio frequency ablation. So there is one case report mentioned in 2007 by TS KL. He used radio frequency obligation and produce a report. And uh the good thing is there wasn't any reoccurrence at follow up after three months. However, the complications for cellulitis and soft tissue necrosis and since then, it has not been um published in any literature. Then there is one called microwave ablation ablation. So the one retrospective studies which involve nine patients, it has doesn't show any details of the procedure, how it was performed, what type of um um thing, what type of microwave um waves they have used, but it reported significant reduction in the tumor volume at mean follow up of 3.71 months. Um And it also showed an improved quality of life in majority of the patients, which is again a good thing and a nerve palsy was reported only in one case. So I think it looks really good, but I'm not sure why it was, I mean, it has not been used up to now. It can can be, but I am not sure because I haven't found any of the, I mean, in the recent study, there wasn't anything mentioned about this. Then we have got high intensity focused ultrasound. So the principle of this high intensity focused ultrasound was they use ultrasound beam to produce coagulation necrosis, noninvasively to reduce or shrink the tumor. Um It was done either through ultrasound or MRI. So the goal is standard one they have mentioned in this paper was the um Mr GH Ih Ifu, which is like using MRI for soft tissue masses. The reason being it has great visualization. And so that's why it's been termed as a gold standard. Um So the first study that was published using ultrasounds um um like a high intensity focused ultrasound, using ultrasound beams was um done in 2011. That was the first published study So what they have done, they took like 25 extraabdominal tumors in 10 patients. And they used this ultrasound guided one high intensity focused ultrasound which shows 50% shrinkage at the follow up of 30 months, uh which is really, which is really, I mean, um an impressive result. And then another series which was the largest series using ultrasound was 100 and 22 tumors. And they used nine patients who has got 100 and 22 tumors. And they targeted all those people who was treated prior with surgery. And then they had like a reoccurrence of the tumor and they targeted like all the tumors less than or more than 10 centimeters. And the response rate was 47.3% by using ultrasound and all. And the disease control was 96.7%. Complete response was 15 patients shown complete response out of 100 and 22. So it was 12.3%. Complications were 20 skin burns. I mean complications looks like it's quite high in 100 and 22 patients. You're having 20 skin burns and 10 nerve injuries. So the complications were quite, quite high. So, hence, um this one is reserved for only for intraabdominal desmoid fibromatosis because we don't want to um damage the nerves and underlying other uh um other structures. Then there was a study which was published by using this um uh ultrasound. Uh uh he fu um for intraabdominal tumors which shows the tumor volume reduction. It was 58.2% in 12 months. Follow up in seven patients and 59% reduction in mean follow up on 29 patients in 15 patients in sorry, in at 29 months in 15 patients. The complications that it shows were bowel perforation. So, yeah, so this is ultrasound and the other one which I have just mentioned like Mra G HIV, which is like MRI Hiu. Um It, this one is the gold standard in reality and twen in 2024 largest multicentric study was performed which used in 105 patients having extraabdominal DF, which is like um tumors as small fibromatosis in extremities in the neck back and they were followed up and they were treated for 10 years which uh shows the reduction of size from 100 and 14 mL to 51. I mean half of the size has been reduced at the mean follow up of 15 months. Pain score was massively reduced on R criteria which is like um response evaluation criteria in soft tissue tumors. It shows disease control of 86%. So that was really good and complications were like less grade skin burns. But the one reservation is this cannot be used for intraabdominal DF. It can only be used for extraabdominal um desmoid fibromatosis. Now, the the core of this presentation is the cryoablation. Um So cryoablation is um generally, it's um a gold standard for in musculoskeletal oncology. So what they do in cryoablation, they monitor eye spot, they inject um uh for to cryoablate, they inject like a freezing material and then they take series of cross sectional images and then they take um I mean contrast and MRI at the follow up after a couple of months or a couple of years and then they monitor it um which is quite easy to monitor the mass. And it is, it is efficient for large desmoid fibromatosis near nerves, bones and viser. So we can safely use it near nerve, near all the optimum, like I uh important organs. And uh it's, it's shown like if in in the process, if any nerve damages, it shows a regrowth, neuronal regrowth and healing. And the first study that uh was that was published about cryoablation was in 2011 by called Kuk ETL. And he, he uses five decal fibromatosis patients. So, I mean, luckily all papers were cryoablation safe and effective and it's quite ef effective treatment modality to achieve, to achieve the disease control. And it also very good for pain control. And there were a couple of studies that was done by other the, these three like this one like Bahama ETL, which is the largest literature in the largest study literature. Having 84 patients with extraabdominal desmoid fibromatosis. They were um cryoablate and uh the survivor, the three year progression free survival was 68% which is a really um good achievement. And then in 2021 VA L he published a systemic review which shows a similar rate like 84.5 to 78%. 3 year progression free survival rate. And then we have got C AOL and um in 2022 that shows um survival rates between 85 3 year progression free survival rate, 85.1% to 85%. And another one around 77 to 82. So that was a gold standard. And uh another study over here, they use um they studied efficacy and safety of uh cryoablation in soft tissue tumor. So they did like 393 session of uh cryoablation which reduces the pain like up to 79% of the cases and volume reduction. Um So, the recent study, which is called cryo desmal using cryoablation for the treatment of progressive extraabdominal desmoid fibromatosis after medical treatment is a recent prospective observational multicenter study and it um mean diameter of tumor was 8.9%. And uh 50 patients in this study were followed up clinically and radiologically using emre cyst criteria. So the one year nonprogressive rate was 85.8% which is a good number. Complete response were found in 28.6%. Patient, partial response was a slightly lower like 26.2 and stable disease. In 31 patient a couple of minor pro complications were noted, but that was symptomatic problems, which was, which was um treated symptomatically only 79%. But they were all minor complications, electro chemic chemotherapy and trans arterial treatment also being discussed in this paper briefly. Um So these electrochemotherapy used for superficial skin tumor and it's used for to ablate malignant tumor in your spinal cord in a recent case study. But um but there is only one case report that but that showed that it was managed. The D TF and Adema fibromatosis was managed. And this trans arterial treatment which is called trans arterial chemoembolization case. Um It has also been used um in 2022. So one of the drug which is a common chemotherapeutic agent and is used in all as a chemotherapeutic agent. Doxorubucin. It was used in this and, but the side effect was it, it has like a potential cardiotoxicity. So if someone, if, if in any case can or someone cannot have cryoablation, so we can use this option. It can be a primary ir option via optimus target drug in case one C was not ineffective. So if we can, you cannot use cryoablation, then we can use this case. And these two studies in 2018 and 2022 they used in tace. So the 2018 1, it used like for pediatric Children and they observed 97% of shrinkage of the tumor in at follow up of 6 to 32 months and in 2022 11 extraabdominal desmoid fibromatosis was covered and it shows like um 18% shrunk uh in one month. And then last follow up, 38% of the tumor has been shrunk. So I'm going to discuss this um couple of um images. So this, this is the image of a cryl of a pelvic desmoid fibromatosis. If you see over here, these three arrows, this is show the tumor over here in B image. Is it still there? Then they started to uh this um tumor size is 4.5 by five by 11 centimeter. They have viewed eight chop probes and uh this, they achieve this hydrodissection by incl by injecting a mixture of saline and contrast. So it just, it just like a gel which is um which is engulfing this tumor. And uh it's an hypodense ice ball covering. If you can check, this is a hypodense iceball. That's what it is called hypo um hypodense ice ball. This is the ice balling effect and covering the tumor without extension into skin or nearby hip joints. So if you see it is um like a little ball and this is the ice balling effect. Then after a couple of months, I think uh they have just followed up on this G and H images. So this is all hollow now. So it shows the tumor um resolve. Um tumor has been resolved. So this is a contrast enhanced MRI which is another one is this um desmoid fibromatosis in the shoulder girdle. If you see in this image, it's a quite a massive like four by six by 11 desmoid fibromatosis tumor in the shoulder girdle. And it, this one shows um after six months, it has been resolved. And uh this one is six months and then these are the images for 18 months after treatment shrinkage and complete resolution. So they have just cryoablate it. Another one is this one is the this mass over here. If you see with the white arrows, it's located in the neck. So they have just inserted the probes over here and then they achieved the out balling effect if you just check here. Yeah, this one in the image. So they have just almost um cryoablate this tumor. And uh the pro the the problem this patient had in this um article, it says that this patient suffered from laryngeal nerve, be palsy, Claude Burn and Horner syndrome and C five palsy. But that's the these symptoms were resolved at a one year. Follow up. Another image was this one in the abdominal wall. Uh the black. Yeah, it's over here. The if you see the black asterix within the colon. So um and then this one is that they have inserted, the cryoprobes, achieved the hyp um achieved the uh cryoablation and the hypodense and they created a newer peritoneum. If you see this black area in the colon and then they just in the emergency, it's al already been gone. All the mass. I think that's the last image. Now there are a couple of more. So this is the infraclavicular one if you see and this is the brachial brachial plexus. So they have achieved this in B image, they have achieved this hypotense hydrodissection and freezing. And this one is the contrast and MRI the C image, it shows the devascularization of the tumor after one month follow up. And then this one is a large, very large desmoid fibromatosis 11 centimeter tumor. So they have just used cryoprobes and the um and the, the hypodense ice ice ball. If you see this one, see image, it achieved that this is the cryoprobes. This is the hypodense eye swelling. But it, it shows that um it you can see on the ski, it's it comes up to the skin surface. So this patient later developed skin fistula because of the skin freezing and it was still lasted at follow up. And in its images D and E you can see complete resolution. It started to resolve here. Yeah, these were the images. That's a treatment algorithm. We're just gonna have a brief look diagnosis by co needle biopsy and then frontline approach active surveillance 1 to 2 years. And if there is a three consecutive progression, then we start deciding uh either surgery, radio therapy, medical treatment. So this is a really good um treatment alga in here, it shows abdominal wall, what to do with if someone has abdominal wall, it says surgery, intraabdominal retroperitoneal. So this is all like a treatment algorithm. So current gold standard is cryoablation and high intensity focused ultrasound. In 2023 a meta analysis was done supporting these three cryoablation, high intensity focused ultrasound and microwave ablation. But uh but cryoablation is the first line option, but it requires anesthesia support, cross sectional imaging and patient's preference. So this is a first line option. And however, high intensity focused ultrasound covers larger masses and ultrasound is available worldwide. And uh it is a treatment can be performed anywhere in the world. Taste is a valuable option as well. Trans arterial, which uh local options, sorry when C is not applicable. So we can use this taste. And uh but if we are ablating, it's important that we um we follow up with contrast enhanced MRI. This is the treatment algorithm in 2024. So that's my tumor working group 2020. They have um published surgery as a first line option provided. Morbidity is limited. If morbidities in the favor are acceptable, then we can use the surgery. But algorithm says 1 to 2 years of active surveillance doing like a follow ups for three years by clinical follow up radiological follow up in April 2024 National Cancer Network. They have uh that they have um publish the sarcoma guidelines. So they have dedicated a section for the treatment of desmoid fibromatosis and it says that it must show progression before treatment. If not progressing, then we have to actively survey to do active surveillance for three years before offering any treatment. So in a nutshell, intraabdominal and retroperitoneal um can be treated retroperitoneal and intraabdominal DF by sys systemic therapy that is chemo or, or tyrosine kinase inhibitor or surgery if possible or if it's in patients favor while the other, if the tumor is at the loca extraabdominally, then we will ablate it emboly or use systemic treatment. And um plenty of observational studies are in the favor of uh cryoablation as the first local option. And if they have completely ab um ABT then cryoablation, we can use it. We need to be sure that yeah, we can completely ablate it. So we can go for cryoablation in intraabdominal ones. Cryoablation is contraindicated due to the presence of viscerous. And uh they have briefly discussed cryos mo O2, which is, this is a, this is the study which has been, which has started enrolling the patients but it's not been published yet. So we are still awaiting for it results. Um So that was all about my presentation. I would request the audience if they just, if they have their mobile phone or something in their hands, can you just go on mentee.com and type this code over here, cement.com. And then they will see a link which is, which will, which will be asking you a box, which will be asking you for a code. And if they type in there, so that will take you to a little quiz and that will be very interesting. So if people like to join it, then that would be great. And if the people can confirm in the chat that they have, they're happy to join um to do this quiz for me, please. And that would be really interesting. Yeah, II think we can do this in just a couple of minutes to wrap up. It will be a nice exercise. Kindly do it and let's have that done. Yeah. So it will take like three minutes maximum. So I'm just gonna go there just to check if people are voting. So I'm not sure if I can see. So we voted for the first one, but it says you are yet to change slides. So CTN and B1 mutations associated. Oh, I'm just changing it. I don't know. I'm not sure. Yeah, I'm just gonna change it. Uh Sporadic. OK. Heart we change like OK, fine. Sorry. So people would, I'm just need to check. So sporadic, I got three responses and four for Syndromic, I think CTN and B1 KININ mutation associated with the sporadic form Syndromic was with FAP. So I'm gonna change the slide. Now. Can you see the slides like uh the second one? Not yet? It's not showing. Ok. Um I think I should stop uh sharing my screen and I'll check then. So can people see the second slide? Um Not yet. No, I don't know what's wrong. Hard weight change, slight um to get. Oh, no. So first one is done. I have changed the slide here. No. Um No, I'm just gonna wait. Right. Uh No. Do you see any arrows on the lower left corner? Yeah. Lower left corner of my screen. Yeah, it's just present now, you mean? Yes. Yes, that lower leg. Yes, that. Ok. So I have to share my screen. Uh No, I mean on the beer. Yeah. Yeah. It, it says share PDF, share your screen. That's all right. Try sharing screen. Let's see what happens. Yeah, it shows this and I don't know how to change this. Uh No, we have this. I think um I can share this. Can you guys see now? No. OK. That's fine. I think this um metal doesn't support uh the meter so I don't know or maybe the internet quality is quite poor. Yeah, but that was really interesting. It just to check the um at how much people has gathered from this session. So, right. Um Do you want to restart the meter to see if that would work or you could try um sorting that out in the background with me and Jessica can start her presentation if you'd be happy with that. Yeah. Hi, Jessica. Would you be happy to close room. Yeah. Sure. I can start. Ok. All right. Hi, guys. Um, I'm Doctor Jessica Wilson and I'm gonna speak on a very interesting topic and we can take a more interesting procedure. I actually had the privilege of seeing this procedure myself during my case that I undertook a few months back. Um, I hope the guy is trying to stop it. As interesting as I so, um, uh, sorry, Jessica, there appears to be some noise in your background, some sort of fee that is it still present? Yes. Yes, it was. Um Are you using the audio device? Yes. Um Is it possible to use your speaker? Is it better now? Yeah, much better. It, it's back again. Sorry, it's back again. It's a mix it um Yes, I'm afraid it's still there. I am not sure. Would you consider changing your location? Maybe it's from some nearby device? Is it better now? Yeah. Um and I have some feedback in the box. II can still hear aches. I don't know about others. Yes. II think it's, it's quite ok if, if you can speak more clearer and louder. Ok. Um Is this better? Yeah. Yeah, that's fine. Ok. So the article that I'm gonna speak on uh reduces the clinical and imaging outcomes of 100 patients from one facility treated with transvenous embolization for a condition known as cerebrospinal fluid to venous fistula. There currently there are surgical and minimally invasive interventions um For uh treatment options. In case of surgical interventions, we have no flu and venous ligation. But to access the area surgically, we need to perform a laminectomy or a facetectomy. And they are inherently longer procedures and need a longer recovery time. So, it's not as preferable as for minimally invasive techniques. We currently have epidural blood patch or injection of a fibrin glue to occlude the fistula. However, it's only effective in a minority of patients. So what is a CSF venous fistula? And what is it cause? Uh the subject of this study is a condition known as spontaneous intracranial hypotension. Uh loss of CSF is what it leads to this debilitating disease. And the symptoms may range from headaches and vertigo to vision in uh vision changes and uh cognitive implant. Uh This loss of CSF can be due to several reasons including spinal trauma, anything that leads to ablation in the hiccup seen in cases of spinal surgery or lumbar puncture, uh inherent weakness in the dura as seen in patients with uh conditions like uh uh connective tissue disorders like LS down. Those also leads to uh this loss in CSF. But the most common cause of, of such a loss of CSF is an aberrant connection between the fecal sac and the adjacent epidural venous, which is the CSF venous fistula. So this is an illustration of the anatomy of the spinal cord and the subject of interest. This is your spinal cord, this is your nerve root, dorsal root ganglion and this is the area of interest. This is your venous plexus. So this is a normal finding, an arachnoid granulation. The light blue thing is the, your CSF the purple is your dura. And these arachnoid granulations are what allows for the exchange of nutrients and gas exchange of nutrients. And so this is a normal finding, but it's a proposed mechanism that this rupture is what leads to the formation of CSF venous fistula leading to the outflow of CSF into the venous system. So, um this is a retrospective study. So they had to make sure that there was a standardized diagnostic workup done by a neurologist, specifically, those who were specialists in CSF dynamics. Um The only selected patients who had a comprehensive imaging workup including pre and post imaging uh post procedure, MRI and those who were diagnosed as definitive CSF venous fistula by neuroradiologist that is seen as a washout of CSF on dynamic digital subtraction. While review now about digital subtraction, myelopathy, it is a technique done to assess the site and level as well as obviously the presence of uh fistula. It's done by injecting a contrast that is the om 300 into the fecal sac as usual before via the spinal needle. And then imaging is done after the procedure and the site and the level of fistula is. So to evaluate the imaging, they had a baseline. MRI done within three months prior to the procedure. And the follow up MRI done at least three months post procedure. They used a scoring system known as the burn I score to evaluate these images. We will talk more about it. But before that, we need to know more about the Monto hypothesis. Um We all know the mon hypothesis in terms of a mass or a bleed in the brain. The cranium is a fixed space. Any extra fluid that takes up space results in the exp expulsion of other uh elements inside the cranium, which inherently leads to a brain herniation. In this case, we are dealing with a loss of CSF. And since the vacuum can't um be present inside the cranium, what happens is and the brain can't well have more brain tissue and the arterial blood is not as compliant. It is the venous blood that takes up the space that is blocked by the CSF. And these are the findings that we have to correlate with MRI S. So, um this is uh illustration of the brain before and after uh the formation of si. So this is your normal brain and this uh normal CS premium and this is your after development of si So as far the hypothesis, you will see a loss of CSF and an increase in we. Um So uh there are several findings that we see but the most um good dys power and the most specific signs are the uh venous engorgement of the severe sagittal sinus. As you can see here, there is pachymeningeal enhancement and it is proposed that pachymeningeal enhancement is seen because of the dilatation of meningeal veins leading to um increased capacity of the meninges itself. So you can see pachymeningeal enhancement and you'll see subdural fluid connections. And all of these are because of the increase in venous um venous blood. And the other findings that you'd see are because of a loss of CSF. So these spaces that are usually occupied by the CSF grow less uh are decreased in size. So what you want to notice here is the suprasellar cistern. The this area here, the mammal pontine system, mammal pot distance and the prepontine system, all of these will be reduced in a case of S Rh because, well, the C uh CS that was supposed to be there is not there anymore. OK. And uh these were the findings and these are the signs that have a good discriminative power and they have formulated the B high score recording. So, uh according to this score, patients are classified as low intermediate or high probability of finding was spinal CSF leak. So less than two, less than or equal to two points means a low uh probability of finding AC 3 to 4 is an intermediate and greater than equal to five points is a high probability of finding a spinal CSF is uh it's on a scale of nine possible points. So uh moving on on clinical evaluation, the factors we had considered included demographic details, obviously specific si symptoms and any other issues like presence of or connective tissue disorders or any primary headache disorder history like migraines, cluster headache and any prior interventions. Um surgical or minimally invasive and their efficacy were reported on follow up. They had categorized the patients into those who had complete resolution of all symptoms, improvement of some symptoms or, and those with no improvement or worsening of symptoms. The intracranial hypertension were also recorded along with any uh any very or postprocedural complications like um pulmonary embolisms, neurodeficits severe postoperative pain and any negative outcome like recurrence or the need for the treatment for. Noted. Now on to the front with the IR and the procedure itself, the procedure is technically catheterizing the culprit paraspinal vein that was identified on the myography and embolize it with a liquid embolism agent called onyx mix. And uh this is in brief, the uh procedure itself as with most IR procedures, the groin axis is the favorable root, common femoral vein axis is obtained done with a sheath. The Azy vein is catheterized after passing through the IVC, then the S VC, then the level of the target vein is reached PY is done and it's embolized with THS then postprocedure CT X rays done to confirm the chronic level. This is an illustration or a schematic diagram of the entire pathway. So you enter through the common femoral vein and uh uh get into the IVC, then the S VC, then you identify the right main stem bronchus, which is your landmark and then you turn and then this is your arch of the si the reason that we reach the SVC before anything else is all of the veins converge at the SVC. So if you want to get to any part of the body, you can get that through the S VC and then you catheterize and pass the uh guide wire through the Azy screen and reach wherever you need to go. These are the common drug uh roadmaps to each foramen and you can see from the SVC Azy to T five to T 12 SV Ci Azy to right lumbar, then you have on to the left side through the hemiazygous and the accessory hemiazygous means now we have, um this is our author himself who has a youtube video on this procedure and the condition. So I'll just play the procedure. But so you can, here, I'm gonna show you one more thing and then I'll go into some of our results. So here's just like a little uh video uh showing the procedure. So, you know, this is, you know, typically these are 20 minute, you know, 20 to 30 minute procedures. Uh We're here, we're getting a, a wire in the AZT vein. Uh Then we're advancing a, uh you know, a diagnostic catheter uh deep into the, the Azac vein uh that gives you like a, a little bit of stability. And then, um once we're inside the Azac vein, uh we proceed with doing the uh Azac Vein venogram and that kind of just gives us our roadmap and shows us what the venous system looks like. And it's actually so interesting, you know, having, you know, done a lot of these now, like over 70 maybe close to 80 patients. And there is a lot of variation in, in the AZ and he a uh venous system, but typically this is what it looks like. So once we're in there, uh we take what's called a microcatheter uh and actually get into the paraspinal veins. And, and one of the reasons I do like, you know, this technique is I always know that I'm in the vein um by cause I've accessed the vein, you know, I'm not as concerned about, you know, stuff getting into the arteries or, or, or intrathecal or, or anything like that. But that, that's just me cause I do neuroendovascular procedures and you know that that's the world that I know, right? So once I have my microcatheter um inside the vein here, I have a cat inside the paraspinal vein and this is something I just wanna point out um when the wire goes medial, uh then you know, you're at the spot. So this is like the pedicle right here. The wire going medial means that the microcatheter is basically in the fora foraminal vein. So now I have my, my micro catheter here, my wires going medial. So I know that I'm, I'm pretty much where I wanna be. Um And then once I'm there, I do a uh venogram. So here's a microcatheter. You can see it's positioned very nicely at the level of the foramen here, you can see on the lateral right next to the foramen. OK. And once I am a Mark Catherine position, I do the uh venogram to prove that I'm exactly where I wanna be. I'm in the vein, I'm not extra dural. Uh And then uh after that, we proceed uh with the uh onyx embolization, you know, generally under, you know, full arrest with the, the balloon with the onyx is this black tarry stuff and, and Mark is right. You, it does create, you know, artifacts and issues on postoperative imaging. But typically, if you get a cast like this, you're not gonna see, I mean, the these veins are, are permanently occluded and I just want like this is intended to show just how many veins are in that area. And you know, when you do AAA DSM or CTM, you can see how complex some of these fistulas are. Uh and just one of the things you know that I do like about liquid embolic agents in general is that they do find um pretty much every nook and cranny uh all the different uh venous uh outlets that could potentially be used uh for a CSF fistula. You can see we have some epidural here as well, which is, you know, something that I like to see uh in the final result. So our first case series, you know, we probably, ok. So do you know what? Getting back to the paper, the results, the median duration of the procedure was 40 minutes. Patients did experience intraoperative pain during the injection of the onyx, but none of them had to be converted into a general anesthesia. There were no cases of prolonged hospitalization but we did see five cases where t onyx emboli was seen in the lungs on the post uh embolization ct, but it did not develop into a fullblown um symptomatic urary embolism. Um Severe postoperative pain was noted in three patients which did require medical management including opioids. Uh One patient did have radiculopathy and numbness postoperatively, but it resolved after a year. There were no patients who had any sort of motor weaknesses. Uh 19 patients uh developed rebound intracranial hypertension after the occlusion of the fistula and required acetaZOLAMIDE therapy. Median duration for was three weeks. So these are the variables associated with the complete partial no uh treatment response. So, as you can see here in each patient with no improvement was just five out of 300. That is it 95% of improvement in symptoms, which is a good sign. And this is what is essentially important. The three was a post treatment um BSI score and I'll discuss about this shortly. So subgroup analysis was done to determine the variables of patients with resolution. So it was seen that male patients were more likely to have resolution of symptoms, patients with no prior history of headache. And patients who had uh MRI findings like pa pa meningeal enhancement or venous sinus engorgement prior did show um better resolution of patients with shorter duration of symptom history were more likely to have um resolution of symptoms. And um for the cementing the accuracy of the burn si score. All patients with clinical improvement except for one showed an improvement in burn si score as well. That is the true positive and all patients with no improvement showed no improvement in bone I score. So it just tells us the high score is highly accurate as well. So this is your uh MRI findings before and after the uh procedure. So here, if you can correlate, if you remember the side score, you can see the distance between the mamma mammary bodies and the um pons. And you can compare that to the post where you can see an increase in the distance and the prepontine system and the prepontine system, the suppressella system is not very evident to my unchanged eye at least. So I'm not able to discern it as well as well. So you can see pachymeningeal enhancement here, which is relatively resolved in the post. And these are your contrast uh uh the emboli onyx embolization. Um MRI S moving on um like with most of the things, nothing is completely perfect. So in this study, it was noted that 17 out of 100 patients who needed to undergo retreatment, um 10 of those were noted to have a new fistula at a new spinal level and uh repeat embolization were done for those uh 17 patients, but only resold. About 13 out of the 17 patients and out of the remaining 42 patients had to undergo a surgical treatment. And yes. So as stated previously, si patients uh can have a varied symptom presentation. Transvenous embolization is both safe and effective and the lack of 100% resolution was um more associated with longer duration of symptoms that is greater than four years of symptoms were associated with lesser chances of uh resolution. And uh if there were a history of headaches and rebound intracranial hypertension, they were associated with less resolution. So, um of the 17 patients that underwent retreatment uh then had a new fistula to get a new level. It suggests that the patient uh may go on to develop new leaks, new fistulas, but it's also possible that these additional fistulas are present from the beginning and just not detected. Um And it is confounded by the fact that 21 out of these 100 patients included in the study had multiple fistulas at baseline itself. And the two patients who unneeded to undergo surgical management proves that surgery after embolization is still safe and effective. There were limitations to the study. Um The study was a retrospective study but all efforts were taken to minimize the errors and biases. Selecting only those patients with a standardized diagnosed, diagnostic and postoperative workup. Follow up evaluation may have been short but they have mentioned that they are still following up with these patients and there are no patients that are lost to follow. Um Another problem is that since all of these patients were taken from a single facility and treated by a single operator, the findings can all be generalized. Uh Some additions that this paper provides to parent is proving the efficacy and the safety of transvenous embolization. 95% treatment responses impression. Um and this may lead to an increased utilization of such procedures in the future as well. It also highlights the need for further large studies to evaluate the generalisability of the procedure. In fact, the the same author um had published a systematic review of nine studies with 77 patients done on this very topic, published um December 2023 and it had concluded that there was an 84.4% of significant improvement after transvenous immobilization of the CSF venous fistula. And that's it. Thank you. If there are any questions I'd be have. Thanks so much Jessica. Um I think we've had quite an insightful presentation. We've gone way beyond our usual time frame and that means we may not be able to go through the Menter as well as Q and A s. However, we will be very happy for you to subscribe to our newsletters and receive all the updates on upcoming events. I'll just share with you our website, just give me a second. Right. And we've shared feedback forms um via emails. So you can quickly share your feedback for the presenters. It will be a good learning opportunity for them. And following that, they would be able to use that as evidence for their teaching. 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