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Management of Craniopharyngioma: Open vs. Endoscopic Techniques

Fred Gentili

January 18, 2021

Transcript

- Hello, ladies and gentlemen, and thank you for joining us for another session of the Virtual Operating Room from the Neurosurgical Atlas. My name is Aaron Cohen. Today our guest is Dr. Fred Gentili, from University of Toronto. Fred, is one of the members of the great crew of neurosurgeons at the University of Toronto, amazing group of neurosurgeons. All of them are dear friends. Fred, specifically, you and I have been great colleagues along the years. It is truly an honor for us to have you today. I know you are one of the founders of the North American Skull Base Society. You're a Professor of Neurosurgery at University of Toronto, truly a world renowned skull base surgeon who has experienced the transition from open skull base surgery to endoscopic skull base surgery. Very nicely embraced it, and truly demonstrated that flexibility in evolution of approach in skull base surgery is so important. So with that in mind, I'm so excited to learn from you, specifically regarding craniopharyngiomas that are such challenging lesions. And so, let's please go ahead and jump in, and very much looking forward to it. Again, thank you, Fred.

- Well, thank you very much. And the first thing I wanna say is to thank you for giving me the opportunity to participate in this virtual series and really acknowledge the significant contribution that you are indeed doing. I think I said at the best of times, these educational videos educational prestige are very important, but particularly in this tremendous, tumultuous, craziness of this pandemic. And I think, for young trainees, very young neurosurgeon, these are very, very, very useful. And I wanna thank you for all the efforts you've done in putting the virtual Neurosurgical Atlas on. I really think everyone appreciates that.

- Thank you.

- So my talk today is on, The Management of Craniopharyngiomas Open versus Endoscopic needs and looking at the current state of the art. Now, it's very, how do you say... People like the idea of open versus, at meetings, the idea of controversy and I think controversy is good. I think controversy promotes ideas and in fact helps with progress and innovation. What I would really like is dogma. And I think that dogma is basically Steve Jobs said, "Is really thinking in someone else's thinking "and in someone else's mind." So when it comes to open versus... I think now we're beyond that. And looking at really more, and I believe now after a decade or more endoscopic that we realized that endoscopic surgery has been a significant contribution to skull-based surgery, is here to stay. It's not gonna replace open skull-based surgery, I will say. But no question and it has a significant role to play. So I think with that, there remains a need for both endoscopic and open techniques in skull-based surgery. However, I have to say that my own experience now is that the endoscope approaches are indeed superior most, but not all of the time. Now, I just wanna say that for those watching that the October edition of World Neurosurgery as part of it, has its some of the annuals of the series. And this one was from the University of Toronto for my group, that has three or four nice publications on craniopharyngioma. So it's just to let you know that everything I'm gonna say today can probably also find in all of these papers, that's come from our a great team in Toronto, our fellows and other staff. Now almost 90 years ago, Harvey Cushing said that, and he was speaking about craniopharyngiomas "That this is the most formidable of intercranial tumors, "the most baffling problem, confronting the neurosurgeon." Now not that I wanna have any say that I'm anywhere near Cushing, that this is a Fred Gentili's quotation in 2019, "Craniopharyngioma still remain "a significant surgical challenge "and management problem confronting the neorosurgeon today." 90 years later, why? Well, these are rear lesions. They're benign lesion. Most of them are WHO grade one, but they can have significant different in terms of biological activities. They're very surgically challenging because of their close proximity to visual endocrine, hypothalamic and vascular structures. And while long-term survival is high. So we're not talking about a GDM, here that is very high and we have over 90% survival at 10 years. There is significant morbidity and poor quality of life associated with both the tumor itself, but also its treatment. And while surgery is a primary form of treatment, total removal still remains very difficult and some would say, 50% to 60%. This may be even optimistic. And this of course, then results in higher recurrence rates, even in so-called radical total removals. The other interesting thing is if you look in the literature, you'll see that, the optimal management of craniopharyngiomas remains controversial after all these years with no consensus statements or Standard Treatment Guidelines. We're all aware that the bimodal distribution of craniopharyngiomas in the pediatric group of course, and in the adult group. In terms of the pathology, there's two major pathology, and this has become a little bit more important now with the issue of targeted therapy, et cetera. So we have the adamantinomatous, which are the majority in childhood and the papillary mostly in adults. And of course the characteristics, the majority are mixed solid cystic with very few purely solid. Now, in terms of the presentation, of course, it depends on the size, the tumor, the location that they can present with visual disturbance. Hypopituitarism, memory and cognitive disservice. So obviously a full history and physical examination is very critical in these in all patients, of course. The other important thing, these patients require a full endocrinological evaluation. So we refer these to our endocrinology colleagues because many of them will have subtle endocrine deficiency when they present, subtle DI and so forth. They need a full ophthalmological evaluation, including OCT and Retinal Fiber Layer Assessment. It gives us a better prognosis in terms of the improvement in their vision. Of course, neuro-psychological evaluations necessary if there are neurocognitive issues. And of course, radiological assessment. There are a number of classifications and top of the classification, this is the classical Kassam classification. The preinfundibular, trans and retro. This is our classification has not been published yet. Where we wanted to look at the criteria, the anatomical criteria, and maybe then see which ones would be best in terms of the surgical approach, whether it's endoscopic and so forth. And we're still working on this, and hopefully this will be published. The management of the craniopharyngioma, of course, is surgery, radical surgery, subtotal conservatives function sparing surgery, conservative surgery with installation of chemo, radionucleides and a conservative surgery with various different radiosurgery techniques. Primary radiation has been used. And of course, now we have target medical therapies. The different surgical options are of course, the open transcranial, the bilateral options that we have. And in fact, this is my preferred open approach is a bilateral transbasal interbimesphic approach. And then of course, there's all the unilateral approaches that everyone is aware of. And now of course, we have the transsphenoidal pure endoscopic approach. Now these are some that controversies in craniopharyngiomas that I'd like to today touch on. Some of them, I will not be able to, but the majority I will. Looking at what is the best approach? What is the best surgical strategy? Is it possible to remove a craniopharyngioma totally and spear all pituitary function that is stuck? And then I don't have time to look at pediatric, are they different from the adult? And then I'm gonna finish off by looking at the management recurrent craniopharyngioma, which are a real management problem. So what is the best surgical strategy? Open versus or endoscopic? Well, if you look at my own series over the last decade, my own experiences. You can there's been a general tendency towards the endoscopic approach. If you look at the last lesion, these are about 30 patients that I'd done. If you look at the primary lesions, 14 endoscopic, only two craniotomy recurrent lesions, basically three to one endoscopic. So total is you see almost five to one. The question here is, why? Well, this is a reason that they... So here's a gentleman, he's in his 70s with presented with visual disturbance, severe memory and mild endocrine dysfunction. Obviously, it looks like very typical for craniopharyngioma. What is the best approach in this patient? Well, initially this was a decade ago, the open approach was approach of choice, whether you use a terrioral approach, super orbital approach, or sub-frontal approach and dermis ferric. The problem with these approaches are good approaches. We all use them, but for craniopharyngioma particularly retro-chiasmatic infundibulum it relates to basically the access. Very limited access to the pre-chiasmatic and the carotid optic space was just not occurring in this patient. And this is the problem in accessing these patients. This patient was done that using an open approach, as you see, there was not a significant removal of the tumor. They were worried about injuring further his visual apparatus. So this is now basically the endoscopic approach in this patient, there it is. So this is the endoscopic approach, and you can see the significant difference in the visualization. Here we're taking off the tumor of the medial wall of the hypothalamus. Here we're taking it off the chiasm and of course it's stuck because of the previous open approach. This is after the gross, radical removal that you can see here. Obviously, this is the component of the hypothalamus and this patient was a 69 or so, and we're not gonna be totally that radical on him. So this is a patient post-op, we gonna see, we left some tumor in the hypothalamus, but his patient improved in his vision cognitively. He was panicled pit over now almost a decade. He remains very stable. So it's this type of thing that basically brought me over to the endoscopic approach. The advantages of the endoscopic approaches, a more direct, midline approach for these midline lesions. It gives you that wide, excellent illuminated visualization of the anatomy with reduced manipulation of the optic nerve, chiasm and vascular structures. And it allows for this easier access and removal of the infra, retro-chiasmatic, and retro-sellar interpeduncular tumor extensions. There are disadvantages, and these are very restricted approach and workspace. And while we made significant advances in CSF leak, repair and reconstruction, we still, it's not totally assault. And of course there is a learning curve to do this. And like anything in terms of the endoscopic skull-based approach, you need a knowledge of anatomy. We all know the surgery really is anatomy, anatomy, anatomy. Here, we need an interdisciplinary team and I do these with my rhinologist and discovery rhinologist. We need a bimanual, binostril technique because as surgeon, we are bimanual animals and we have to be able to do the same, same technical dissection that we would do under the microscope. Instrumentation of course is critical. And as I say, here is training. You have to have a learning curve. So that's the just briefly the anatomy go through the anterior skull-based endoscopy. The classification, of course, this is for the central skull-base, the transcribriform, transplanum, transturberculum, transsellar, and transcriber that we use. And here, basically for the craniopharyngioma, we were really using a transsellar with a little bit of transtubercular and transplanum. Here's the cadaver view here, is some nice cadaver dissection done by one of my former fellows. And this is a again, a cadaver, and here's same anatomy that you see in the patient. And this is after the removal. And this is what we do, basically it is we remove the sellar and we do a Transsellar, transtubercular and a little bit of transplanum. Now the degree of transplaner, removal depends on the degree of anterior extension of the tumor. Which we assess using the interoperative image guidance, of course. And so basically what we are looking at is the same anatomy in this area, the supersellar region from below the same anatomy that is from above. It's just the same anatomy, but from two directions. So this is a patient that I want to discuss presenting with a six-month history of headache, some confusion, weight gain, some visual dysfunction. If you can see here and on examination, he was somewhat confused, no motor sensory disturbance. You can see that he has this solid cystic lesion, with associated hydrocephalus diagnosis, likely craniopharyngioma. And now what is the best surgical approach? Well, we go through what I showed before. We have all of these options for this tumor. In my mind, the best approach for this patient would be the endoscopic approach. And I wanna show you here, the video showing the actual approach that we use. So this is a middle ethmoidectomy. We do a middle ethmoidectomy. here we're doing uncinectomy. It's just a, you open up the backstory sinus. And then this is basically the, maxillary sinus you can see over here. And this is a piece bipolar, which is the anteritmoise, basically that we take that off. And now there's the, basically the ostia and the sphenoid ostia we're into this sphenoid sinus now. Now very important now is the nasoseptal flap for the reconstruction. And you can see basically the inferior cut and this can vary depending on the width that you need and can go right down to the hard palate. And it comes interior. You wanna get a very nice long flap that will fill out the entire opening of the skull base, almost out to the mucosal cutaneous side. Here's then the inferior superior cut that we try to keep below, at least a sonometer or a half below the skull-based to try to maintain, preserve the olfactory function. And, the key here now is to find that mucosal, semi-causal crane. And there you see going right down to the Spinoid rostrum, the flat is this a very nice pasteurized flap, that we put down the nasal pharynx, and here's now the sphenoid. Posterior septectomy and to allow for the bimanual binostril technique and the microsurgical technique. Here's a Sphenoid ostia here. Now we do a very small little flap on the other side to allow us to do our binostril, bimanual technique. So here we are looking at the face of the sphenoid, and here's now the drilling of the intersphnoid septation that you can see here, and we can already see the roof of the sphenoid. So here basically is the tuberculum that's being drilled down. Here's the carotid, parasellar carotids that you can see very nicely. Here's a sellar here, and that tuberculum, that we're drilling down there. And the key here is to get as wide an exposure as you can, here is the medial optic Rotter recess over here, over here, you wanna have is as wide an exposure as you can, to allow us the ability to go as far laterally as we can. So now we're cutting the, and just the superior part of the pituitary gland. And here we are going laterally, right over to the optic canal. And as we do now, the arachnoid, and here it is basically the tumor. And now really the tumor resection, as you can see, is done a bimanual technique as we would do under the microscope. And this patient, we saw had a solid component anteriorly, and then this large cystic component, more posteriorly, and then dissection is basically done as you would do it under a microscope. And here we are gigging out the solid component and the typical machine oil type of craniopharyngioma fluid that you see, and here's a pituitary stalk over here. There it is. The stalk, pituitary stalk mammillary bodies. We see here in the basil artery back here, we're seeing. So we attempted to spare the pituitary stalk. This patient was elderly, and this is after the removal of the cyst was obviously evacuated to here we are. After looking inside, here's a basil artery. You can see these, the third nerve down here, and here's a tumor, a capsule stuck to the third ventricle hypothalamus. So, and because of his age, we're not gonna try and remove this totally, which caused significant morbidity. Reconstruction, multilayer, inlay, fascia lata, then onlay, fascia lata. Then this is followed by the nasalseptal flap that we bring in. And here it is a Uranus here and serge it out and a tissue reserve, that's I think the end of the video. So this is the patient's post-op, no CSF leak, some cognitive improvement, length of stay five, seven days, pathology adamantinomatous, and is a patient's postop CT scan one day. And now at three months, you can see the patient came in. He was cognitively normal. He was panhypopit on full replacement, and you can see now also the decrease in the hydrocephalus we chose to then observe this patient for the residual that we had in the wall of the third ventricle. And he's now three years stable disease, but we will watch him very carefully because we know there's a definite a risk to this patient to recur. There's another patient, 45 years old with a virtually blind. As you can see here, finger counting on one eye, 2,800 in the other eye, what approach? Well, now in my mind is no other approach than the endoscopic approach. And here's the video shown here. This is a shorter video. Here's the exposure, same exposure we showed you before the transsellar, transtuberculum, a little bit of transplanum. And here we are taking this out. I just want to do this video because of this, this patient you see was virtually blind. Any manipulation of the optic nerve in this patient or vascular disturbance is gonna make him blind. And the beauty of the endoscopic approach that allows you to spare these important vessels that are not as easy to spare from above. And this is patient, and this is basically a true is not me, but I could not believe this myself, that university from finger county to 2020, 8 years at this patient, you can see here, no evidence of the disease recurrence. And then I'm gonna bore you with these, but you can do different size of tumors and you can see in different locations shown here. Now there are limitations like any techniques to the endoscopic approach. And of course, you're not gonna do this patient endoscopically. So, large tumor, lateral extension, there are significant neurovascular, encasement, and purely intraventricular tumor. And I don't believe are good candidates for the indescribable approach. And of course, surgeries experience and preference, that's very important. So this patient here is a patient that presented with visual dysfunction. What is the best approach? Well, I think some of my more aggressive endoscopic college may consider doing this endoscopic. I don't think so. This goes very laterally, superiority, very narrow stalk. And for me, this is a impartial, an open approach. I didn't answer Ms. Spheric Trans-callosal approach that you can see here, and I'm not gonna show the video, but this patient, as you can see after the surgery, you got a very nice resection of this tumor, but left a little right at the very bottom of this tumor patient did initially weld requires some cognitive rehab, but as you can see here, he presented subsequently with rapid visual decline and what happened? Well, that cystic component that we left, the little component expanded, and now what approach? Was no question now at the endoscopic approach is really the approach of choice. And we go in here and you can see very nice and they mobile. In fact, we did an endoscopic third ventriculostomy from below doing this, and that's fine at this stage. And you're used postoperatively. You can see another patient, I guess, is 71 year old patient, progressive visual loss. What is the approach here? Again, this patient... let me go back to this to show you that this patient would also, because of his lateral extension, I would know to do this and just topically in this patient. I did a Lipterional approach that you can see here, a nicely decompress that and put in a Ommaya reservoir. And this is another approach. Another patient, I was gonna say that, with tis craniopharngioma, a young man presenting visual decline, what approach? Well, initially I did this endoscopically and that was a mistake. And I'll show you why, because of the fact, and this is the tied surgery. She had a prefixed chiasm. He had a free prefix chiasm, and you can see with very limited exposure to the tumor. And this is very minimal manipulation that you can see. And then if you look at the preoperative assessment, you can see that in fact, this patient had in fact that a prefixed chiasm. So this was not the best approach with this patient. Next slide. And so this patient, in fact his vision was worse just by that new manipulation post-op then I basically offered this patient a repeat surgery and open approach or radiation. He felt he did not wanna consider another surgical procedures. So you underwent IMRT radiation, 54 degree, 30 stretch, and he's three years now with stable disease. So what is the best surgical strategy? Radical excision versus total excision and rediotherapy. It's a lot of controversy in this, and there has been a significant pendulum swing over the last decade from a rather radical resection at all costs to a more conservative function, sparing multimodality therapy. And again, the question is why? Well it's because of this. This is a series of Yasser Gill back in 1990, his cases, DI an 86% permanent and 87 regarding placements therapy. Post-op obesity, 50% in pediatric cases, severe obesity. This is from another very prominent where we shoe a pediatric neurosurgeon that even after significant follow-up, even after supposedly a total excision significant recurrence rates up to 38%. And we all became aware of, at least it became aware of the mental disturbances in adults and including and in children, the morbid obesity, the cognitive functioning. And so this really has led to a more, as I say, the Pendulum swing to a more conservative function sparing. So where we deal with the problem they have, is it vision? Is it, whatever it is, and not be as aggressive that you're gonna cause significantly quality of life issues. Well, does that work? Well, this is from the pediatric literature, if you look at the progression free survival and overall survival of gross total resection and subtotal resection radiation, and you can see in fact that there's not a lot of difference, and this is also true in adults, we don't have as much data, but this is also a tendency in adults. So we have to be aware of this. When we make a desertion decision-makers, surgical decision-making and discuss these with our patients. So what approach this patient? This is a patient that, and then I was just gonna go back here to say that it's important in that it brings on basically patient involvement in the decision-making, this patient presented with this tumor here would approach. You don't have to show the video in this. It was felt that we could not do a total removal. We did a sub tool removal. You can see some residual here, you had improvement his vision. He was hyperopic, residual disease. What do we do next? What we observed this be subsequently three years later, you can see it progression of his disease. What now? So there's patient then underwent radiation therapy 2014, and look at this now, 2018 significant responses, including a decrease in the size, four years, post-op radiation. And this is a serious thing to then 2014, looking at the issue of radiation and craniopharyngiomas. And we have to not ignore these at the local recurrence rates. Local control rates was 95% at 5. 92% at 10, and 80% to 20 years. The overall survival you can see at 10 years 83%. At 20 years 67%. Concluding that no radiation has a role to play in craniopharyngiomas where there's gonna be significant issues with regard to quality of life at attempted radical removal. Now, this is just my own new caveat. Is it possible to remove a cranepharyngioma totally and spare all pituitary function I.e stalk. My own belief was prior to endoscopic that really rare. If this can be done, the question is now with endoscopy, better visualization can we do that? Here's a patient with progressive visual deterioration with this tumor. We tempted and the just bear the stalk and we did, but this patient was, it remained panhypopit, and you can see the tumor here. But then here's a patient that I wanna show you this. And this is a patient after we thought we had, we moved it all and spared the stalk with a recurrence three and a half years later. And then we went into a complete hypophysectomy. Here, this patient is now with no evidence of recurrence. And this is if I can show this video, I think it's an important video. This is a patient that with a small craniopharngioma that we, the endoscopic approach. And you can see here, we spared the stalk and to show you here, this is the removal. Now here's a tumor that I think we all would always like to have at the cranium. This is a soft tumor, very nicely, no significant calcification. And exophytic 'cause you can see of the stalk over here. This is again being very careful to spare all the best restructures. And here we're looking inferiority, chiasm. Here's the chiasm, and here's the stalk over here. As we think that off, look at the preserved stalk. It's great. Say here now I said, well, now with endoscopic approaches, maybe we can spare the stalk and, next slide. And here it is, here's a nice view here. Total removal. This patient didn't have a CSF leak required a lumber drain because we did very dysfunction and three years. So we've cured this patient now three and a half years recurrence, where did the recur? Right in the region of the stalk. So microscopic cells on the stalk, because we know the origin of these pouch, and so this patient, so what next? This patient, we then did a full hypothesis ectomy, second surgery. You can see here, this patient now remains a stable, I think, four or five years later. So my conclusion is still now. And in medicine, you can never say never, never say always, but I think it's rarely, very rare that you can remove a total, craniopharyngioma totally and spare the stalk. So here, as I said, you need patient engagement. You have to talk to the patient. And this patient is a young man, with this craniopharyngioma that I talked to him and I explained that we could go in and this totally, but he'd likely will be, have to have full replacement of endocrine replacement. And we know that endocrine replacement long-term is really not as good as your own hormones and our endocrinologists tell us that, or we can be less radical, try to spare the stalk, but then there's a risk of recurrence. Well, this young man had, it was very fearful. Did not want in any way to have the possible recurrence. He says, "Do whatever you have to take it all out. "I don't care what you have to do." So this makes it easy for the surgeon because they find, so this patient here, I don't think I'm gonna show them the video here, but we took the stalk in his patient and it had no CSF leak, panhypopit, full hormone replacement. He remained stable at this stage. Here's another patient. However, he's an older patient with rapid vision loss. And again, we gave them the same option. And which is interesting in terms of patients, because a younger patient had issues of fertility. You think in one who can have hormonal stability, this patient at 51, did not wanna take replacement. He did not wanna take those. And he said to him, it doesn't matter. Take, remove whatever you can try to maintain my hormonal function. So we did, and he was this patient at Transit DI, no hormone replacement there. So finally, I wanna look at recurrent craniopharyngiomas, as I said, these are real issues. These are common. As you can see here, some up to say 60%, there are difficult management problem. The median time recurrence, you can see here, they can vary as you signed my patients from two years up to 10 years. So you have to follow these patients. The growth rates are very low in these patients. Some would say 60s, I think more realistic. You're looking down at the 20 or 30%. And the surgery of course is more difficult with increased treat morbidity as well as outcome. And again, like the version cranio, there's no standard treatment guidelines. These are the factors, social with recurrence, of course, the size of the tumor, biology of the tumor, the degree of resection, and the adherence to critical structures that I showed you in one of my videos. And we know that up to 2/3 of patients can have some degree of hypothalamic involvement. And of course, a length of followup is very important. So here's a patient 52 year old patient that with a history of visual dysfunction, what is the best approach? And it just want to show you here, why craniopharyngiomas recurred at least in many of them. So here's the same approach. You're getting very used to this now. And I wanna take you through, and again, this is a transtuberculum, transplanum approach, transsellar. And, this is a video look, I guess, that we could speed up, by a minute or so. So here's what we're looking at. As we open the dura, this is the craniophryangioma, look at us adherence to the carotid artery. And these InComm, as you can see here, the adherence to the stalk, the adherence, basically to the optic nerves and a while you can do a significant dissection here, and you'll see very careful dissection. We can take this off and decompress the optic nerve, at least the optic chiasm. And here's the stalk, as you can see, you're very careful dissection of the optic chiasm. We're allowed to do that. But the idea that you can remove this totally with this degree of involvement, attachment to these critical structure is really not possible. And this is one of the reasons that craniopharyngiomas, and you can see recur, and I think we can stop the video now, as we remove this, and here's this post-op, you can see improvement in his vision, which is really what his problem was, no hormonal replacement, because we speared the stalk residual disease, what will we do? We observe these very carefully and the first sign of progression. We consider radiation. These are the, what we, the management options for a recurrent craniopharyngiomas the same as the, for primary, but we do know that the treatment for recurrent often requires a multidisciplined process. Surgery alone is usually not gonna be the answer. And I'm just gonna go quickly through this to show you what could have an 18 year old gentleman with this craniopharyngioma it had an open approach. We had trans, open craniotomy, intermuscular transfusional approach as shown here above the chiasma and below postoperative that has some residual disease down here. As you can see, we watch this had decreased vision and evidence of a cystic recurrence out here. So this patient then subsequent and disruptive approach with pituitary transpositioned, but now learned that pituitary transposition really means basically no further pituitary function. So they issue of being able to transpose a pituitary gland and maintain function is really not feasible. Then he has had some further recurrence years post-op did you see here? He then underwent a transventicular endoscopic reception and ETV rexydrocephalus, and two years later remain stable. So all of the options that you have need to be considered when you have these recurrent, this is just showing you a recurrent tumor 47 year old gentlemen with a cranial, had previous craniotomy and radiation. And so this is a Virgin approach from below. And so you can do a nice decompression in this patient. Obviously not get a tool, removal will be impossible, his adherence to the carotid artery, but five years after you can see your patient had improved vision, no CSF leak, length of stay. And five years later, he has stable disease. This is a tumor on the carotid artery. Next. And of course, this is not gonna be a patient that you got to do endoscopic, I'm just gonna quickly go over because of the problem with 2007, you can imagine with a craniotomy then radiation, then right frontal inter-mospheric approach, then decreased vision and a recurrence craniotomy plus ommaya. And then September 2012, blind one eye. He had a recurrent repeat craniotomy, blind with significant residual and to went through the radiation out there at 2012 and now 2013, stable with some reduction in the disease. So very, very, very problematic in some of these recurrences. This is our series that has been published as a back in 2019, looking at our senior 40 patients, just to show you a very briefly our results. Gross total removal was only 44%, but in the primary lesions, not the recurrent lesion was 73%. And when the objective was gross removal, that is like an older person. Your objectives, not that up to 81%, which was reasonable. However, in the recurrent lesions, only 10% did we feel we were able to achieve a gross total resection, also very, very satisfying those visual outcomes. And this, as I say, one of the advantage of the endoscopic approach, 95% stable improved, only one patient had visual versioning, and this patient had multiple previous open operation and radiation. The results, no mortality, CSF leak initially very, quite high as you see 21%, but with our a nasal septal flap, this is now less than 10% in our recent things around five to seven 75%. What we did in this series before we published it, it was very interesting. We waited and follow the patient. So the first medium follow-up was 56 months. You can see the re the recurrence rate was 14%, but we waited another two years, three years, another nine patients. So the overall recurrence rates was 35%. And this is a very important lesson in terms of following your patient, the longer you follow up, the more recurrence you're gonna see. And then we also just to let you know, this is our publication of the manage of recurrent craniopharyngiomas here, and I'm not gonna deliver this. And then just looking in the literature, if you look in the literature, you'll see the interim of the endoscopic approach is a valid strategy. This is a number of papers, systemic reviews, and meta analysis, trying to compare craniopharyngioma open and endoscopic, showing them the more complications, the open in the scopic. But my issue is that I'm not really keen on these meta analysis and reviews because they're unreliable. And I think for the younger neurosurgeons or fellow residents, you have to be very careful when you read these there's often case selection bias with significantly greater numbers in the open series longer follow-up, et cetera. So in conclusion, I think looking at the literature and we have to try as to use as much evidence-based specimen as possible. Unfortunately, the agreed to recommendation is no better than four and then shown here. And so in conclusion, basically the management craniopharyngiomas remain a significant surgical challenge with high rates of recurrence, regardless of the approach. Craniopharyngioma should be considerably a chronic disease. If we think of it that way. And gross total resection may be associated with reduced recurrence rates. The focus should be on quality of life issues and long-term survivors. You just got to be approached as a valuable addition. It provides a very useful complimentary transcribing procedures and you'll role management of both primary and recurrent craniopharyngioma. And with careful patient selection, I believe can achieve comparable to better results than the best microscopic series, especially in terms of less morbidity and higher rates of visual disfunction. And there's no question that subtotal resection plus radiation is a valid strategy, especially in those cases where attempts at Grosso removal carries significant risks. So no one surgical technique can be considered the best approach for all patients. Each patient is best served by the formulation of a tailored individualized surgical plan taking into account both patient and tumor factors. So what about future trends? Few slides. We all are aware of the issue of a molecular profiling of different tumors, whether meningiomas, and now we have this in craniopharyngiomas and we all are now aware of the BRAF mutations that have been identified in craniopharyngiomas particularly for papillary. And we saw this republication a number of years ago, and our experience has been very limited, but here's a young man, 35 years of age, visual dysfunction had a craniotomy radiation, repeat craniotomy for recurrence, repeat recurrence that an extended and disrupting approach residual disease, what next? So here's the residual diseases was growing, further surgery, repeat radiation, which he could not have, but about medical therapy. So this patient was underwent basically a course of targeted therapy. And here's this patient is six months. You can see a very dramatic improvement. What we don't know is the longevity of these treatment, and we just need further studies to assess this. So in conclusion, the evaluation management of patient with craniopharyngiomas require a multidisciplinary team approach with the active participation of an experienced surgical team, radiation, medical oncology, pathology, neuro-ophthalmology, endocrinology, and psychology. So thank you very much for your attention.

- Thank you so much, Fred. Spectacular talk really appreciate very illuminating curls off technique. Craniopharyngiomas so challenging because although it's still logically, we've not been looking at malignant, they recur because they're so sticky. There's such a vital location that prevents their gross total resection, and therefore there's such a challenge and affect the lives of our patients so adversely. One of the things that I wanted to discuss with you is, and I know triple H in the great triple H in Toronto, I think Hoffman was the guy who did the craniopharyngiomas, didn't he?

- Yes, Aaron. And I have a story about that though, once you finished. Yeah, so it's a Harold Hoffman. Very, very famous pediatric neurosurgeon that I was fortunate that I had in my residency that tells you all that I am under him at the sick kids.

- They called him the great three H's is that--

- Yes, it was Humphrey, the pediatric surgeon who basically brought pediatric era surgery as a specialty. And then Hoffman, and then Humphreys was the other neurosurgeon.

- Right, and I know that Hoffman was very much sort of liberal in terms of just pulling out a tumor through the floor of the third ventricle and the outcomes were fine. And to be honest with you, I have also been a little bit more aggressive than usual. And I've tried to really tame sorta, not just necessarily, I've host the tumor, I don't think Hoffman did that either, but just really make sure all of the discolor tissue along the floor of the third ventricle, all removed for craniopharyngiomas and the outcomes have been fine for us. In other words, I guess started just occasions where maybe eye could have been prevented or other minor issues, but overall, a slightly more aggressive strategy to remove the tumor. Again, this is something that has to be judicial, not just very aggressive has been successful. What is your experience in terms of extent of resection craniopharyngiomas right at the border of the third ventricle.

- So just a story about Harold, Dr. Hoffman I was gonna say, when I graduated and I did adult neurosurgery, and he did his gross total removal. I often saw many of his recurrences that regrow total removal that years and years later, we would see the recurrences. So, as I mentioned there, you have to follow the patient they're recurred. Now I think, Aaron, every case, whether it's a craniopharyngiomas is a separate case, you have to look at each case. I think the one that I showed you there was gone through, into the third ventricle that is stuck to the wall of the hypothalamus. There's no advantage to doing that, but in the floor, yes. I think the floor of the third ventricle, it goes up, you can be more radical there, being very careful and trying to take that off. And often, as you saw, as I took it off the optic nerve that one video, you sometimes can get a nice plane and take that off. So I think it's an interoperative strategy that you have to look. And every case is very different. You can not assume I'm gonna go there and to be able to do this, you think you're able to do it. You have to have a plan. I always say to my fellows, you have to have a battle plan, but unfortunately, sometime the plan does not work and you have to adjust your plan when you see the enemy.

- Well said. And I really refer to the fact that when you're looking at, from bottom endoscopic approach, there's really not a great border between the floor of third ventricle and a tumor. There's always that degradation of discoloration that you see, and you're always left with this idea or question where, what am I gonna do the cut? And I would say staying a little bit on the lighter side of the discoloration, it can be very effective in terms of achieving more gross, total resection. Don't you agree, Fred?

- Yes, I think that's a... I think a definite option and strategy.

- Yeah, thank you. And the other thing I wanted to say that is I think, for years pituitary adenomas were standard of care to have removed them via the endonasal approach, transcranial surgery, for cranial pituitary adenomas, but just really not considered a standard of care unless absolutely need it. And I really think for many craniopharyngiomas, not for all you should, many of them that are so large, they have to be approached transcranial. Really, they are the lesions just like pituitary adenomas, where the sizes right, can be approached in nasally. And really the preferred approach is the endonasal route. Just because of that a sub cosmetic lesion is being approached by a sub cosmetic approach. And therefore visualization is so much better and retraction of the brain is so significantly less. So I completely agree with you for the majority of craniophangiomas that are on the mid to smaller size. The endonasal approach provides significant challenges. Obviously there is a slightly improved rate of visual recovery after endonasal route at the expense of maybe slightly increased risk of CSF leak. However, I think there is other advantages that may not be reflected in the current studies. Any other pearls of technique you have as a closing statement for us today. Fred, could you please share with us?

- I'm not sure exactly it was specific questions, Aaron. I think what I've learned in years is that the ability to see better allows me to be at certain times more aggressive, but I have to look at the patient. I said in my talk, patient engagement is very important in this to find out what is the patient really want, because as we've seen here, the issue is not necessarily longevity here, they survive, but if they're surviving in a significantly compromised quality of life state, and as you know, Aaron, quality of life matters, and as surgeons neurosurgeons, as physicians, our job I think is to not necessarily prolong life, but maintain, improve quality of life. And so I think we have to always keeping that in mind when we are doing resections of whatever lesion that you're doing at that to me has become very important as I've gotten older, the issue that to see a patient they've operated on, 20 years ago come in and totally quality of life, very poor, heavy bleed leach, blind, and whatever. I don't think that's not a good outcome to me anymore. One time it's a patient survived. That was a good outcome, no longer. We have to look at quality of life. You have to look at patient engagement in decision-making. And those I think are important.

- Very well said, which eventually translates into quality is more important than quantity. So with that in mind, I wanna sincerely thank you, Fred, for any mass work in skull-based surgery, truly mentoring so many great individuals. And you're one of those integrates university of Toronto Neurosurgery Program that all neurosurgeons across the world have so much respect for Jim Rothko, you, so many of other colleagues in the cranial aspect of things in the university of Toronto, we really respect you what you have done, all of you. And thank you again. I look forward to having you with us in the near future.

- Aaron, it's my pleasure. Thank you for those kind. I'm honored and humbled by those. I'm not sure whether we're the right. I deserve all of that, but I'll take it anyways. Thank you very much, my pleasure.

- Tanks, Fred, thank you.

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