 So I'm going to present a case of lipoma, this is an oculoplastic scorbid conference case from November that we had to postpone to be running out of time. And this is a patient that I saw in Dr. Patel's clinic actually in my first year of residency. So she was a 52 year old female and she was first seen in 2006 and noted to have right upper eyelids swollen. At that time a biopsy was performed and demonstrated external marginal zone lymphoma and she was treated with external beam radiation. Subsequently she in 2008 developed a left orbital mass as well as a retroperitoneal mass and at this time it was biopsy began demonstrated similar pathology and she was treated with rituximab. And then in 2013 she noticed additional swelling up around the left eye and found that she had some left upper eyelid edema and erythema as well as a superior subconjunctival infiltrate. So here's a photo from her second presentation in 2008 with the subconjunctival infiltrate there. And then here are the photos from 2013, you can notice maybe some fullness there. And then here's her, you can definitely appreciate that there's a lot of material under her conjunctiva. And then we'll go to Dr. Davidson to go over the imaging. I pulled up the 2006 and 2008 as well as 2013 but I think I saved her more recent imaging for the very end. Sure. Imaging of conjunctival lymphoma of cross-sectional MRs is really reserved for assessing their inforableness. Because we don't see the real superficial mass as well, obviously it's something you need to feel valid and you can see pretty easily. So this is a teaching fat suppressor image. It looks like there is a little bit of signaling in this orbit. This doesn't look like a mass, but I think this is probably some fat suppression artifact which we commonly get, depending on what's in a person's face, if they have a lot of dentition or dental work on one side, if they have orthodontia or something like that, you can get the whole side of the face can cause a distortion of the fat suppression. And be a little careful about reading this. And if I remember right, at this time it was just this little bit of stuff on the right, is that correct? And so it looks like what we have is an isolated preceptal mass corresponding to what you saw. Lymphoma is a very cellular neoplasma. So on T2 it tends to be darker than a lot of other pathology. So the kind of things that are dark on T2 that are pathology are cellular tumors like lymphoma. And also we have sclerotic kind of inflammatory conditions. That allows for relatively dark on T2. We didn't see any post-epidline extension. The lacrimal glands obviously are sweet spots for lymphoma, so we looked pretty closely at that. And other than that, pretty limited evaluation. At least we were able to confirm that there wasn't anything post-bobar or intra-orbital. And then coming back at, I guess this is the same time. So here we see a solid enhancing mass, pretty typical of lymphoma. Describing lymphoma and imaging as being plastic. Meaning it likes to get into and lose around and infiltrate amongst the anatomical structures. Which is one of the reasons why part of the differential with lymphoma is always going to be something inflammatory or possibly grammed and limitous. Again, you can see there's the same issue with fat suppression on this side. Looks like it's been failed. The amount of thickening, and now in 2006, they didn't have any right in the left side of these, right? So this is where imaging, it's easy to look stupid if you don't know exactly what's going on clinically because you might be tempted to say, oh look how thick that eye lid is, but there was anything there. So we have to be a little careful. I tend not to be too definitive about eye lid pathology on anything. She had this large perineal mass that was biopsy, and that was part of her, and she was treated for that as well as she had some recurrent eye disease at that time. And then you want to go back to, go ahead and hear. Okay, so a few years later, she was having disease on the other side. And now we can see, I think, probably pretty confidently that this is not merely thickening of the eye lid. We actually have an enhancing mass that is, I guess, from the point of view of imaging, we don't know if that's the conjectival or if it's eye lid. If you see air between the surface of the conjective and the eye lid, then you can tell which side the mass is on. But apart from that, this could just easily be something that's infiltrated the eye lid itself as opposed to the conjective, so you have to tell us about that. Again, we look very closely at the large perineal glands. There's a place where Lymphoma likes to go and see anything there. So it seemed like it was pretty well isolated to the conjective. And here we see a kind of a satellite showing that thickening of the conjective. We didn't see anything big going for a Ulvar or Pocetal. Lymphoma is going to usually be fairly hot on pet. In this case, we do see that it does show up. I'm not sure that's all that specific in this instance. And the pet was probably more useful for making the rest of the body anyway. Let's see, so that was from June now, a few months later in November. You can see that she has responded. We still see some thickening that's a little abnormal, but it's quite a bit less than we saw before. And we didn't see anything else that looked like it was progression or occurrence. And the same thing of pet, really very little uptake in this thickening. And so it looks like there was a favorable response. Can you get those sagittal and axial images with any pet? Uh-huh. In fact, I probably didn't have to do any special processing to get this. This is usually started with the studying. Usually we just see kind of on the imaging, the bone scan kind of appearance, and then there's a black flaw in the head. Right. Yeah, so one of the things about pet scans is when you look at it on the packs, there are probably 15 or 20 different sequences. Sometimes you have to go all the way to the end of the list. And some of them look like black boxes. When you start to scroll through, you realize that these are what they call the fused images. So it's got the CT data, and then the pet data is superimposed on it. And we'll almost always get the fused data in free planes along with everything else. You kind of have to look for it. If you have faith that it's there, you can look until you find it. But it is sometimes with... We have the same problem with MR. There are so many sequences that are either preliminary or preparatory, that all get dumped in the packs that by the time you get through them all, you think you've seen everything, but there's actually more waiting at the end. We discussed the pathology. So she had a biopsy again in early 2014. And this was the official report. I'll just click on the arrows down here. I've got also the clicker word. So the surgical pathology showed a dense confluent lymphoid infiltrate with smaller, immediate-sized monocytoid cells, basically consistent with the previous biopsies she had in previous years of Marshall's own lymphoma. And they also did inflow cytometry to help further characterize that. And again, that was consistent with the previous biopsies she had in her clinical history. And so this is our low-power H&E steen of the biopsy. And really here, big thing to remember for a surgical pathology, for Dr. Manlis is blue is bad, right? And that's really all we can see here is essentially all these blue cells. It's hard to really get any more information, except there's some scattered areas of heme as well mixed in with the blue cells. Same thing, a little higher power. We don't really get any phenotypic information, just a large confluent amount of blue cells. And then one thing to point out, obviously, higher differential at this point, looking at this pathologically is something of lymphoid origin when you see all these small, round blue cells. And so when we're looking at it at low power here, one thing we want to look at when we're thinking of lymphomas and other things of lymphoid origin is the organization of the tissue. And here, there's really no organization. It's just a bunch of confluent cells. There's no normal architecture, no lymphoid follicles, that we would expect to see in like a normal lymph node biopsy. And so that's important for us to look at at low power here. And then at higher power, we can see clearly these are lymphocytes. They're small, round blue cells. They have very minimal cytoplasm. And then beyond that, looking at this really, that's going to be the expertise of the hematopathologist. They can look and say, oh, these have a more monocytoid appearance or these look more follicular. And they use that phenotypic information in addition to all the immunohistochemical stains and the flow cytometry to help determine exactly what type of lymphoma this is. And it's important to know that it's not always exact. There's a lot of kind of crossover. And you can talk to some of the oncologists about this. You know, it's not always 100% clear exactly what type of lymphoma it is. But in this case, it was consistent with the martensile lymphoma, which was also consistent with her previous history. And so, again, what we talked about, so we look at the overall structure first. And then one of the big things was to determine that it's a lymphoid origin. We want to say, is there a normal architecture or not? Here there was not. And so we're very concerned that it is a neoplastic origin, so lymphoma as opposed to, like, a reactive lymphocytic infiltrate. And so once we know that, we want to decide, is it truly clonal? Is this a single, you know, neoplastic cell that's multiplied? And we can do this by a flow cytometry, which is what was done in this case. And so you do light chain restriction. We'll go over that a little bit. And then we want to determine if there are B cell, T cell, natural killer cells, and that's all some of the special stains they use and then use that pattern to determine exactly which cell type it is. And so it's just a diagram of how we do the light chain restriction. And so you can see all the way on the right, the polyclonals are just going to have a 50-50 mix of the light chains in the B cells. If you have a monoclonal, it's going to be primarily one or the other. And I don't know what the exact percentage is, but in our case, it was essentially 98% Kappa. And so it was pretty clear that this was a monoclonal neoplastic process as opposed to something reactive. And this is just a control slide showing the CD20 positivity. And I think it's important to really know the different stains. That's more for the heme path guys. The CD20 is a B cell marker. And here in the control, you can see how it forms those nice round follicles, which is in contrast to our slide, which is next where it's just this confluent infiltrate. But you can see these are all pretty much CD20 positive. So that tells us it's B cell origin. And CD3 is a T cell stain. This is the control, again, showing some scattered T cells around these more normal follicles. And this is our patient, which just shows some scattered CD3 positivity, which can be pretty normal to some reactive T cells in addition to the actual neoplasm. Let's review a few things about ocular and nexol lymphomas. So this is just all the different types of lymphomas. T cell, B cell is more common in the orbit and congenitiva and lacrimal gland. And it's most often a non-Hodgkin lymphoma. And the most common type of non-Hodgkin lymphoma in the ocular nexa is the marginal zone lymphoma as this patient had. Next most common is follicular and one of the less common types is mantle cell. These classifications depend on the differentiation of the cell origin, those B cells, and their different stages of maturation to kind of enter the follicles and exit. And that's how that classification comes about. So we talked about the typical pathologic findings of marginal zone lymphomas, which is here. And then we talked some about the differential diagnosis, a reactive hyperplasia, which would not be monoclonal. And the CD10 and CD5 on the flow cytometry in this case to distinguish the case from the other types of lymphoma. So a little bit about the clinical presentation of ocular nexol lymphomas. So on average patients have symptoms for about eight months before diagnoses. These tend to kind of grow slowly. Most of them present with a mass. Pain and systemic constitutional symptoms are relatively rare. Most often the marginal zone lymphomas are in the orbit, but they're also reasonably frequent in the conjunctiva. 10% of them are bilateral. The recommended staging workup includes some blood work as well as CT. And biopsy is recommended to establish the diagnosis if it's necessary. Most of them are localized to the ocular ednexa, 76%. Only 19% are disseminated. Some risk factors include autoimmune diseases and HIV. So we mostly have, I've mostly been referring to these as marginal zone lymphomas. So elsewhere in the body, the most common type of marginal zone lymphoma is a malt lymphoma. And that's classically associated with H. pylori in the gut, and it's thought to be due to chronic inflammation from that H. pylori infection, and it resolves with antibiotics. However, in the eye, the prognosis is actually different compared to these gastric malt lymphomas. And it looks different on histology. So, most likely these marginal zone lymphomas are different from malt lymphomas elsewhere in the body. Some people have hypothesized that the ocular marginal zone lymphomas could be reactive to some sort of ocular infection. And there was one publication that associated with this, Climidia, I don't know how to pronounce that species, but with this type of Climidia, however, subsequent studies didn't really confirm that. So it's thought that I think the evidence supports that these marginal zone lymphomas of the eye are different from malt lymphomas of the gut that can just be treated with antibiotics and are very unlikely to have systemic involvement. So does anyone know what's happening with the malt of the gut? Because I was just reading some literature that they don't feel that it's related to H. Pylori anymore, that they feel that it's just inflammation in the doxycycline, so treatment was actually decreasing inflammation and not the H. Pylori treatment. So regarding treatment, localized disease is usually treated with radiation, and disseminated disease is usually treated with chemotherapy. Rituximab can be used as a single agent. This patient received radiation when it was just in her right orbit, and then once she had it in her left orbit and her retroperitoneal area, then she got Rituximab. Sometimes these have been treated with steroids, with surgery only, and with observation. Response to radiation can be pretty slow. In terms of the prognosis, it's relatively good. Overall survival at 10 years is 89%. Progression-free survival is 57%, and death due to lymphoma at 10 years is only 3%. So after the one orbit is radiated, the most common site of recurrence is the contralateral orbit, as occurred in our patient's case. Usually the orbit was affected in one side, the other side orbit will be. And there is this international prognostic index that can be used to determine the prognosis for an individual patient. So for this patient, she still had those, that residual ocular disease and the retroperitoneal lesions. Although the ocular disease was biopsied, she was recommended to get Rituximab, but then subsequently did not follow-up and get that. She got imaging two years later, despite not having any chemotherapy, both the congenitival and the retroperitoneal lesions had partially regressed. She didn't follow-up with Dr. Patel, but she did see Dr. Mamelis, who's her anti-carot segment doctor, her congenitiva and cornea were grossly normal at that time. And this is just some imaging from more recently, just since its past fall, I think it's pretty benign. But they did say that there were multiple sites of recurrent tumor, including the superior aspect of left blow and the left peritoneal region. So a little bit about the natural history, since this patient regressed somewhat, even though she didn't get any treatment. Even though these lesions are usually treated, this one study was published on 36 patients who declined treatment or had reassuring histopathology or their lesions were completely resected. Two of these 36 patients notably did die from their lymphoma and 17 of them, about half of them, eventually did progress and 11 of those were treated. But on average, they didn't require treatment until about five years later and 25 of them were still not treated even after seven years. This other study was published on eight patients who had congenitival lymphoma and that was incompletely resected and seven out of the eight lesions spontaneously regressed. So it has a relatively benign natural history, but it still can be fatal in some cases. So it's probably a good idea to treat if the patient's willing to go for it. Questions, thoughts? Dr. Malis? You know, when you're looking at the association with systemic lymphoma, you've got to be really careful to look at the study, how long the follow-up was. Frederick Covey had a good group of patients with ocular lymphoma in New York and they had about a 25% incidence of systemic lymphoma associated with it in five years, but then he followed them and in ten years it went up to 50% and in 15 years it was like almost two thirds and so depending on how long you follow them there's more and more of a chance of systemic lymphoma showing up. So you really want to keep a close eye when you're looking at studies, you want to see how long the follow-up is because that is really going to depend on how many of the patients you'll see that have the disseminated or systemic lymphoma. To the rest of the board, I don't know if you may have any interest rather than Mr. Covey had co-opism when he wrote this out years ago. You'd think hormonal lymphoma would be worse than contractile lymphoma, would be worse than iodine. It's interesting, it's the opposite. One of the initial studies, highly lymphoma worse from just the contractile lymphoma, contractile was worse than iodine. It's interesting. I always assumed that these must be multi-centered. There was a vogue in the 1990s, early 2000s just to inject the steroids blindly. I thought a big fan of that because you really don't know what you're doing. I'd rather resect these maximally because they're happy with this vogue sort of question. Quite a few lymphoma patients refused treatment. It's interesting, I'm not sure why. Because localized radiation seems to melt them very well in the localized. But the iodine wants otherwise, you've got to be careful about it. I mean, if you take this. My question is due to Dr. Tilt's point about the iodine versus the contractile lymphoma. I always kind of thought that it was, I thought it was worse. Yeah, I didn't include that here in the study. But yeah, there were some papers I looked at, but I can't recall at this moment which site was worse in terms of how this is. And then generally, I think from the radiation perspective, we always feel like the orbital lymphomas are more responsive to radiation compared to the margin of skin or the walls of the stomach. But then again, it could just be because it's easier kind of to follow some of the more superficial contractile lymphomas to see their clinical response. And then like a big question kind of in our field right now is whether to do like super low dose, like the boo boo 2-grade times 2, just going to call those a 4-grade versus doing kind of more standard of 24-grade and 12 treatments. And like part of how we decide on that is like a lot of it has to do with kind of patient preference even. A lot of times I leave it up to even my patients to see like what they prefer. Like if they're really reliable, we'll come to follow up. We'll do kind of sequential imaging with studies at 8 and 12 weeks and then kind of reliable then we would even try the 2-grade times 2. Not that 24-grade really has that many more side effects, but it can potentially and part of the reason is that the higher kind of 24-grade dose still doesn't really necessarily have super high rates of complete response after radiation alone. It's like, you know, in the 90%. And so when you compare that the 90% kind of complete response rate to something like a 70% with just the 2-grade times 2 then it's kind of like why not just try the low dose first and see if it responds and then kind of keep following. So where I tried to end the address and actually almost all the patients would just get the 4-grade and then would be followed because it doesn't, if there is still just kind of partial response or you're just not super comfortable with the response then you can just add up the 20-grade later. Thank you. But I mean, of course, we just kind of go crazy over these like not really relatively low doses on radiation. Could you change the dose? Yeah, that's a good point. No. Generally, not really. We kind of lump all of them together. I think that in general the kind of hormonal sites in Hyva and Isla are pretty sensitive. Thank you for joining us. Oh, yeah. No, thank you. That's good. Thank you. Talk about watching these over time. Do you think that's the end for most of the career? Do you have some choice for those? Do you want to follow up on the manuality or watch it or something? I know that the surveillance in the abdomen is something that we'll do with Pat and probably CT as well. And I'm probably to refer to the oncologist as overcurrents. For the ion orbit, I would think that your clinical exam is probably usually going to be your drive-out on the screen for that unless you think it's recorded and you want to see what might be behind that behind the orbit. Are you watching Chris' stomach film? Yeah, I'm watching Chris' stomach. We don't really see surveillance as part of either just CT or PET CT. I have to check with the body and the folks, but I think that would be pretty routine. Pat is part of that surveillance. What's the cost of this? Separate or CT versus PET CT? Let's see. It's probably a chest abdomen, probably going to be 1,000, 1,200. And PET might be a little less than double that. Alright, so this is... So I checked the reference that I have looked up and it did confirm what Jacobia had found earlier that conjuctinol has the most benign prognosis, at least like a little bit of conjuctinol in the eyelid and the eyes. So this is my fifth presentation so far this month, but I think some of the people in this room kind of make me the most nervous out of all of those, so I'm going to talk kind of fast, I'm afraid. So this was kind of an interesting patient that I saw actually when I was in clinic with Dr. Patel last year. So she had come to us initially from outside hospital out of state. Around age 36, she began developing an increasing prominence of her right eye. She's kind of followed for a sense like about a year and a half. She got in with an acute plastic surgeon in her home state and initially under an orbital decompression in 2008 and at that time was diagnosed with fibrous dysplasia for the outside reports. We didn't have any of the outside records and actually in reviewing our stuff it looks like several people have tried to get ahold of them as well and have been unsuccessful in getting those. So she came to us about three years later in 2011. When we saw her at first she was doing quite well. She was 20-20 in both eyes. She had no APD, a full color place, but she didn't have fairly significant right-sided proctosis. Her visual field testing showed mild enlargement of the blind spot on the right. And then she also had a little bit of fairly benign appearing superior optic nerve edema. Maybe these pictures just because there wasn't a really better way to do it, but this was when we first saw her and this was after a subsequent decompression surgery that she had. So it's hard to tell because I was trying to protect her identity a little bit, but it actually does look a little bit better. It's not quite as obvious here but you can see the right-sided proctosis. After she first got plugged in with us she developed a kind of acute subacute decrease of vision in the right eye. She had been stable for a few visits and then under what kind of a semi-emergent right lateral orbitotomy and decompression in April of 2011. And then that specimen was actually synthropathology. At this time I'd kind of like to just review the images that we've got. That's okay. So I'll get your stuff pulled up here. And then I put that other picture that you emailed me. So for starters I just put up a soft tissue windowed image of a CT scan showing that there is an obvious expansile primarily what looks like looks to be a bone based process. So you can see that there is this mark that gives lateral orbital wall a greater sphenoid and even extending into the central sphenoid. There is no obvious soft tissue mass either here or here. This is the non-contrast scan. This is actually an interesting study in diagnostic imaging because we probably could have done better than we did in terms of using diagnostic imaging to help you figure out what's going on. There are a couple reasons why people came to the conclusion that they did early on. But assessing for soft tissue whenever you see what looks to be a process of growth is a big part of what we're looking for in the head and neck. At this point I think people kind of focused in on what the bones look like which is I think very telling in terms of what you think this might be. And a series of images here from the level of maxillary sinus all the way up to the upper central skull base you can see that there is this fairly striking sort of homogeneous expansion process and it's pretty solid. You have some little logulations here at some parts of it. This is the level of frame rotundum right there. This is the level of the superior fissure. You can see that even though it's open eventually you might get some aspect of this. This is involving a broad region of the greatest human wing that are over the wall in the squareest portion of the temporal bone. And it's isolated and seems to be interested in that one location. A little higher. Narrow at the level of the optic chiasm. You can see that here's the optic canal here and here. Starting a little bit narrow. There is that expansile hyperostatic process surrounding the optic canal. So this is going to be one of your pressure points and one of the reasons why you're going to be thinking about intervening. And again we see this very dense hyperostosis, a little bit of logulations. There's no real central lucency in this. There's no areas, no pockets, no destruction, no litter component. A little bit higher. We can see this relatively homogeneous, solid process. Now one of the things that we try to do in this is assess, is this a dysplastic process or is this a reactive process? And it turns out in this case that sort of means everything. We're looking at this as a 36 year old female. When you hear somebody say skull base, expansile process fibrous dysplasia. And what's the buzz word for fibrous dysplasia? Ground glass bone. Is that kind of ground glass bone? Yeah, this is sort of ground glassy. Maybe the things about this that aren't as typical for fibrous dysplasia. These sort of logulations aren't quite as normal. And you see how you have these sort of bony excrescences. Like these little bits of bone that are growing off of the periostral surface. That's not typical of fibrous dysplasia. The other thing is, is there's absolutely no central areas of fibrovascular leucency. Usually in patients who have really active fibrovascular dysplasia, you'll see pockets of leucency that represent these earlier active fibrovascular phase and kind of the growth phase. Now you could look at this and say, well she's 36, maybe this is burned out. It's mostly all the expands and all the growth has kind of been done. And this is just a burned out manifestation of the dysplasia process. The other terminology you might use to describe this is hyperostosis. Which is not a dysplasia process, it is a reactive process. And when you think about what gives you hyperostosis in this location, by far the most common thing is going to be meningioma. A meningioma is hallmark inking characteristics is induction of hyperostosis in the adjacent bone. If you had a chronic osteomyelitis, you might get a reactive one like that. And I think at this point, the conclusion was kind of drawn based on the imaging and the clinical, I think they sort of just lab on this as fibrosisplasia. I think you got tagged as fibrosisplasia. If you look at one of the early scans, one of the readers, look at this and said the primary differential is two things. Fibrosisplasia or meningioma. We would use the term in this case, intraostasis meningioma, meaning there's not a big intracranial component to the meningioma, but rather the growth is into the bone itself. And we'll occasionally see meningiomas that do that. Instead of growing into the cranial cavity as like a mass protruding into the displacing the brain, it actually grows into the bone primarily and we use the term intraostasis meningioma to describe that. Sir, to Chris, can we get those bony expressions as you pointed out with meningioma? I will take those bony expressions as are typical of meningioma. If you look at patients who don't have an meningioma, but are older and they have these sort of dural, reactive calcifications that are very normal, I think how often those things look like excrescences. You get a 70, 80 year old patient with a headache and otherwise normal. And you might see these little dural calcifications that look just like that. These little bony growth excrescences and sort of a more or less normal aging, dural reaction of calcification. So I think that that appears maybe could have been a bit of a tip-off. Like right there too, these little excrescences. And then these that would not be typical for private dysplasia. So in the coronal plane we see simple stenoid, greater wing, a lot of orbital wall, crowding the apex as a bit a little bit farther. Here's your optic canal. And then we talk about it in Avenue 4 optic strut planet process defining the optic canal on this side completely surrounded by this bony overgrowth. A few months later I just had a follow-up. I hadn't really changed a whole lot, but again paying close attention to what's going on with that optic canal. A couple years later, June 2014 more of the same, but this had been growing a little bit not strikingly. But you have the same sort of solid hyperosotic bone surrounding the optic canal. Your foramen rotundum and bidium canal also could potentially be involved by this. This again is either going to be sort of maybe poly-oestotic fire dysplasia, but again everything appears to be in one locale. It's right there around the sphenoid wing the petroclival ridge where many humans are really well known to form. And then about this time she was probably having some more vision problems and so an MRI was obtained. We've talked before about how you look at MRIs as though you have a CT in your mind so you kind of go back and forth. There's your CT there's your MR so you can see that that is where the optic nerve runs. You know it looks like a reasonable amount of space there personally, but we have this very dense hyperosotic bone. If this were a fire dysplasia that had any active component you'd see little patchy areas of enhancement within it. And we didn't see that here. This is so dense that you don't even see any enhancement. This is a post contrast T1. You can see that there's enhancement of the vascular structures. You can tell that this is a T1 looking brain. So the most component of this is so powering under the signal you don't see any enhancement maybe a little bit right here, but if this were tumor, what is tumor enhancement? What is meningioma? Enhancing is really bright. So there is a competing process here between the dark signal of the hyperostosis and what is potentially a tumor in the same location. Series of images, T1 pre, T1 post and now I think for the first time we get really suspicious that this is not fire dysplasia. So on the T1 pre, you can see we have this expansile hyperostotic bone the mass spectrum of orbit we've already talked about and why that's important surgically. But now look at the the post congress image. And this is really interesting, this is now 2014 and there may be other images but in terms of looking for soft tissue mass, this is the first set of images we have that show us what's potentially going on and here we see a large thick plaque like enhancement right along the anterior margin of the temporal tip along the senoid wing and yet with a dural tail. When you see this that looks like, well now that that's an meningioma with a big interosseous extension and typical hyperostosis that we see as an meningioma. So at this point we're sort of saying, well now this is not fire dysplasia, this is definitely an meningioma. But early on with the CT, I don't think we really clued in on that very quickly and we didn't really ask for an MRI maybe we would have come to this conclusion sooner. It may be that there were other discussions where people were aware of this, but in the record it doesn't look like we were really on board with this being an meningioma early on. And then she went ahead and had a broad resection of this with some repair and here we see a portion of the roof lateral orbit and some cranial plastic assist. You can see that part of the senoid roof was taken out to help uncover and decompress it off the canal and had some follow-up imaging here in January 2015. You can see a plaque like enhancement along the medial aspect of the middle fossa right along the character of sinus. That's a very typical look for meningioma. So there's some residual tumor right in there and then I think we had another one in a few months. We see on the axial, post-surgically we see this thick plaque like enhancement along the anterior aspect of the character of sinus extending toward the overlay effect of the fissure. So there's a little bit of residual tumor there. I think in July 2015 it looked pretty similar. There's probably some getting through that superior fissure in the orbit. We have some artifact here. Maybe a little bit of residual right there and then February 2016. Pretty similar, but again it looks like we have some residual. Some of this could be granulation so we have to keep an eye on it. Is it growing? This would be pretty typical for some residual. And this picture is to show you what fibrous displays that really does look like. Here's a good example of polyaesthetic fibrosis plagia. It's not just along the dural surfaces. On our page pretty much you can see that where the dura was is where we were reducing the bone. Here you have polyaesthetic disease. You have these broad areas of multifocal expansion and then you see these lucent areas of this fibrovascular component. And when you give contrast with these, those areas really light up. On the MRI these look really bizarre. They look like big ugly malignant processes but it's just the active fibrovascular phase of these areas of activity. And this is more typical of the ground blast appearance. You don't get that libulation and that kind of bone ectrescence. Thank you. So it's hard to do these without kind of jumping too far ahead in time. So we kind of saw based on those last pictures what the ultimate course for her was. After her second decompression she saw neurophthalmology a few months later and she was again doing quite well. She was 2025 on the right. She didn't have an EPD at that time and then she had shown an improvement in her optic nerve, a demon. And then she was followed, you know, kind of every three months, every six months until June of 2014 when she started having these transient molecular blackouts in her right eye. And then those began to increase. Because of those vision changes, a repeat MRI was ordered based on July 2014 and those showed the changes concerning for meningioma. And so she ultimately underwent an optic canal decompression in October of 2014 with neurosurgery. So the wrong way. So here is the part for the pathology. I had an extra slide in here. Alright, so this first slide here displays the tumor itself. So it's a wing, a spinoid meningioma. And so with this aerial view it allows you to appreciate the tissue. You can see that it's definitely a hypercell there. There's compact, dense tissue here. Just a closer view. One thing that's unique about these meningiomas is that it's similar to squamous cell carcinomas in that you typically see worlds of cells that's characteristic here. What do you notice here? And I did also want to note that with these meningiomas the World Health Organization has typically three categories with them. So this category was on Grade 1. So there's Grade 1, Grade 2, Grade 3, Grade 1 being benign. So with this you typically don't see any mitotic figures and you don't see it here. Typically you don't see any areas of necrosis and it's not present here as well. And so with the Grade 1 subtype they typically have a 7 to 25% recurrence rate. And in the other grades Grade 2 goes from 25 to 50% recurrence rate and then Grade 3 is 50% to 94% recurrence rate. So here this is from Dr. Patelus sample and so this is when tissue was removed for decompression. What you could appreciate here is the bone bone structures and then down here is the fibrosis the fibrotic structures growing around the bone. This tissue was de-classified however there's still a large amount of bone that's present here. What's important to note is that within the tissue there was no atypical osteoblasts which is important because you want to try to see if there's any signs of recurrence. In terms of meningiomas for the most part they generally don't transform malignantly but if they do dissemination is believed to be through the blood and with the lung being the primary site. These are images from BCSE just to pressure to focus in on some key points. This first sample up here this is the optic nerve and you have the meningioma that is growing circumferentially around the optic nerve causing the compression and then here we have we have the optic nerve here this is the cancer itself the meningioma originating from the arachnole and then duramatter and then here also you see that characteristic world-like pattern that's very similar to swine cell as well. I kind of found this one interesting for a couple of reasons. One just because of the difference in the pathology just with kind of two reported processes and then also because we're getting close to OCAPS it was kind of useful to be able to review all this stuff while we were doing this. We had two path reports that showed fibrous dysplasia so one from 2008 or one reportedly from 2008 which showed fibrous dysplasia and then another one in 2011. In terms of fibrous dysplasia it's divided up into monostatic and polyostatic so the majority of 70 to 80% are monostatics where they're involving one bone and then the remainder 20 to 30% are poly. The poly austatic form is more likely to have skull involvement. It's associated with Albright syndrome so the endocrine abnormalities so you get precocious puberty, they have the fibrous dysplasia and then cafeoliomacules. The frontal and sphenoid bones are the skull bones most commonly affected and you can have optic frame involvement up to half of patients. So many of the orbital processes, proptosis is the most common finding and then just the changes associated with that. In the past it was previously felt to be self limiting with the lack of progression following adolescence so kind of burning out as the bones start to close but this is kind of falling out of favor as it's been shown in older patients. One of the papers I found from 1989 was they didn't have any histologic diagnosis but they had this patient that looked like fibrous dysplasia and ended up being meningioma when they actually biopsied it. At first they were saying that this was a fibrous dysplasia which transformed into meningioma but their diagnosis of fibrous dysplasia was based on nothing pathologic and it was felt to be meningioma later on too because the patient was past the typical age so they felt like it would have been too old to have fibrous dysplasia. So it was pretty common to kind of have that opinion but treatment is primarily surgical and it's been reported that radiation can increase the risk of malignancy. Sphenyl orbital meningioma is the other condition that we're seeing in this patient so it's a high female preponderance so greater than 80% of patients so even higher than with the optic nerve meningioma and again proptosis is the most common type of scien and up to 65% of these patients can develop vision changes and then for ocapsular association with neurofibromatosis type 2 so I just wanted to kind of just review the sort of NF1 so the greater than 6-caf.8 macules, greater than 2 neurofibromas of any type or greater than 1 plexiform neurofibroma freckling the axillary angloma region, optic leomas the lish nodules they get these distinct vascular regions such as sphenoid dysplasia and then family history so that's NF1 and NF2 is the bilateral acoustic neurofibroma any of the two so I mean angiomaglioma neurofibroma swanoma or PSC so for ocaps, type 2 neurofibromatosis is PSC cataract let's see that on the practice questions a lot and then the additional criteria as well so what the other kind of part that like I said that I found interesting was we had a diagnosis of fibrous dysplasia based on pathology here and then on the second resection it was shown to be meningioma so it's been reported in numerous papers over the years that it can be very difficult to distinguish between the two just based on imaging alone like we saw obviously and then it's been reported to transform spontaneously into sarcoma usually less than 1% of cases but it's also been reported to transform into fibromaxoma and meningioma in the past and then there's also reported association with formation of mucosil and then aneurysmal bone cyst so there wasn't a lot of stuff on the transformation into meningioma one of the papers was just kind of the pathologist were excited because it was it was in an area of fibrous bone dysplasia but then it was also transformed into a very typical form of meningioma as well but it's been documented in the literature too so I thought that was kind of interesting just to have kind of these two conditions kind of come one after another so that might account for some of the potentially some of the imaging changes too so what can be confusing with these is when you get the meningiomas especially back on this female ridge they can induce you know bomi changes and so the heart part differentiating is this meningioma inducing bomi changes into fibrous dysplasia with some fun things and I don't know that's really hard for me to tell the difference I kind of wonder is was this always meningioma? because we had the report of the path initial and then we can get the path in our hands until we had the meningioma so she began to seem to imagine in the early twenties which is more like fibrous dysplasia you can't see the meningioma until the 40s and 50s so it's easy to get psycho there are two reports which talked about fibrous dysplasia becoming meningioma I don't like that I think it's probably like archives and we will publish this and say our fibrous dysplasia became meningioma I'm sure I'd buy that I think you're right this has been meningioma all along as it progresses we start to see we've got suspicions I have to respect that's really hard differently the age group was wrong symptomatology what happens with meningiomas is you get a fibrotic change that leads to a foggy edema they just have a particular look and this was not fibrous dysplasia it was just a clean face with super ointment changes in the foggy change in the skin so that's a very clean, deep surface nothing to indicate a foggy fluid again it's supposed to show don't believe your mythologies 40 or 80 she had radiation along the way she didn't get radiation I mean probably her shape meningiomas they respond pretty well so like in most of the papers I saw between orbital meningiomas they didn't really talk about radiation for it most of the papers were really just kind of discussing timing for up to canal and roofing and then decompression but I didn't really see I couldn't find much on radiation for it so we reserve that for a black meningioma we have two or three questions right now where the tumor spreads along the dura it's very difficult to set a new rule like a wall a view of the whole canal we reconstructed the orbital roof so she doesn't pulsatide like twisters she's really done very well so we don't the vision's come back and there's no yeast to give her radiation unless there's recurrence and we were hesitant to operate because of certain risks operating in a reconstructed orbital area so just a quick word you know the BQ and Albright syndrome which Rhys mentioned is a precocious puberty virus dysplasia and then neurofibromatosis can be associated with meningioma and capiolay spots and so some people say that they sorry oh I didn't know that neurofibromatosis just the capiolay spots associated with neurofibromatosis tend to be smoother and smoother forms that's been likened to the coast of California whereas the capiolay spots and the BQ and Albright syndrome are more like sharper like the coast of Maine we'll take a look at the capiolay let's just study this for new caps