 Welcome and good day. I'm Dr. Steven Pomerance, neuroradiologist. I'm here with my partner, Dr. Malcolm Schupeck, neurosurgeon and neuroimager extraordinaire, and together we're going to tackle complex neuroimaging cases and problems with you from Saturday, November 22nd to Monday, November 24th at the Four Seasons in Chicago where we hold our annual neuroimaging and after that orthoimaging course prior to the RSNA. It's a great experience. People come from around the world. It's an international crowd and we absolutely love drilling into these cases, case solving both from the standpoint of a clinician with imaging experience and a radiologist with some clinical experience. So let's get started and look at a very interesting case together so you can see what the course and course reading is going to be like. Shall we? Sure. Well, what do you say we take a look at this case? Okay. Well, what we have is a 56-year-old gentleman who is having some neck and arm problems. And I think the first thing we notice is he's also had a little bit of surgery from a posterior approach. But even with that, it looks like there's still a little problem there that's apparent on this sagittal T2 image. And we also have some brain images. So we're going to be able to work this from a couple of different angles, I think. Looks like you got a little enhancing nodule on this contrast enhanced MR of the brain and the coronal projection. And then a somewhat liquid or cystic-looking nodule in the back of the cord, perhaps subpeal right underneath the pia. Should we take a look at some of the other images from the spine? Right. Let's check out some of the axioles and see if we can get a little idea of the morphology of this lesion because I think that looks like it might help us a little bit. Well, the mass is a little bit left-sided. It has a very slight hypointensity in the middle right there. And it would be nice to have a contrast enhanced portion of the examination. Let's see if we've got that. I believe we do. Here's some contrast. Let's see what happens to our lesion. It does enhance. Right. And it is posteriorly located. The cord's a little deformed, perhaps related to the prior surgery. So we've got a high signal mass previously operated on, enhancing within the cord and another lesion in the posterior cerebellum. So what's our differential, do you think? Well, I think that this lesion, to me, has a couple of interesting things about it that are going to help us, I think, with the differential. Now, first of all, we know there may be a surgery, but even with that, this is either recurrent or they didn't get it the first time. And something somewhere else in the body, so something possibly systemic. But what do you think of the cord? You know, to me, a cord lesion, you know, our foreign policy is don't do stupid things. We have a similar one in neurosurgery called don't do stupid things in the spinal cord. And this lesion, a stupid thing in the spinal cord is removing a lesion that doesn't need to be removed or removing it in a way that causes more deficit. So if we can get specificity, and one of the things that's always helped me is position a lesion within the spinal cord, because I think if you think through what the differential of spinal cord mass is, position within the spinal cord can help you a lot. In this case, dorsal, so there's a couple things that can be dorsal. But how about this exophitic that it's actually sticking out of the spinal cord? That is starting to ring a little bell with me. What do you think of that? Yeah, it's ringing a bell with me too. And, you know, the classic posterolato spinal cord lesion is an MS plaque. So you look at a lesion like this and you say, well, is it a mass or is it more plaque-like? Your comment that it's pushing out of the pia or pushing on the pia says that it's a mass. You alluded to the fact of where it is. You know, one of the first things any resident has first taught to do when you have a mass inside the spinal canal is, is it intradural? Is it extradural? Or is it intramedulary within the cord? And if it's intramedulary within the cord, is it in the gray matter? Is it in the white matter? And is it anterior? Is it lateral? Is it in the posterior columns? And this particular mass, I think you were alluding to this earlier, does like the posterior aspect of the spinal cord. It likes a sub-pia location. It may push on and even involve the pia. It's hypervascular. It's round. And that would be hemangioblastoma. That's right. And as I said, the actual, where it's actually coming out of the cord, you know, altering the contours as opposed to expanding or pushing the pia out of the pia intact, this is almost going to be looking at you right in the face. And so, hemangioblastoma is the thing that comes to mind that I have seen. And, you know, and then when you think about this other lesion that we saw in the posture fossa, that's sort of starting to fit together as well. Sure. And when you're talking about intramedulary lesions, you know, all-round lesions that are hypervascular, those are not going to be appendemomas. Those are not going to be, you know, astrocytomas or gliomas. And if you're thinking about intramedulary metastases, which occur most commonly from breast and lung, not unexpected, they produce a tremendous amount of cord edema, which this lesion has not done. There's also no known primary tumor. And you might think of metastatic disease, because you have a lesion in the brain. But the lesion in the brain is the same lesion as the one in the spine. It's another hemangioblastoma. And I know when you look at these, you know, surgically, they're sort of cherry red-looking lesions that are pretty superficially located, aren't they? Yeah, they are color-coded. And I keep using Play-Doh as an example of many things in some of our other cases. That's sort of what it looked like. When you mush together the red and the yellow Play-Doh, that kind of orangeish-clayish, that's sort of what it looks like. And it's great, because what you want to do, taking these out, is you want to get very close to the lesion, but without getting into it, because they are highly vascular. And a lot of them are cystic, so you work within the cystic cavity, but there's going to be a number that are solid. And in fact, I think in the adult posterior fossa, non-metastatic lesions, that's going to be the most common, or one of the most common in the adult, isn't it, the angioblastoma? The most common primary intraaxial tumor of the adult is hemangioblastoma. Right. And despite the fact that the classic teaching is they are cystic with a nodule, a hypervascular nodule inside, in my experience and I think in yours, in hemangioblastoma and in isolated ones. It's very common to see solid hypervascular nodules. They're frequently multiple, so you have to very carefully inspect the brain, especially the posterior fossa, because you want to get them out when they're small. You don't want to let them, you know, get big, because they're more likely to bleed. And now you're really obligated to check the entire neural and spinal axis. You've got to go all the way up and down, looking for these tiny superficial hypervascular masses. Well, you have to look even farther than that, don't you? You have to look systemically and for renal lesions, because there are some syndromes and I think probably most of the people watching might come to mind. We could be talking about a syndromic cause of this, such as on hypolyndal. Okay, in which case we have to really even expand our search to some other areas of the body. That's absolutely crazy. I mean, you've got a neurosurgeon that is willing to jump into the non-neural part of the body. That's fantastic. I read it in a book on the way up. Yeah, and you're absolutely right. You know, these patients can get adrenal adenomas, they can get renal adenomas, they can get renal carcinomas. In fact, they're sometimes phenotyped by, you know, what these other types of tumors are that they may get, so they have to undergo, you know, regular screening of some of these other organs as part of their further evaluation. And sometimes that has to happen if they have on hypolyndal for the rest of their life. They may have cysts of the lung, the liver, the epididymis is a particularly interesting one. The pancreas is another site where they may have cysts, true cysts as opposed to pseudo cysts, and of course they may get cysts of the kidney. But the big bugaboo one is renal cell carcinoma. Another lesion that is very important to exclude in this group is the achromocytoma. And once again, they're phenotyped by the category of accompanying tumors that they have and now these patients are genetically mapped. So they are going to have to have an abdominal pelvic CT or an abdominal pelvic MR or serial regular sonographic screening of the abdomen. So this is hemangioblastoma and it's interesting that you do not have a retinal hemorrhage. You do not have a bleed. I believe these signal alterations here are artifactual rather than real subretinal hemorrhages. Let's just take a look and be absolutely sure because one of the things you want to do is make sure you don't have a bleed in a patient with one of these lesions because that will absolutely seal the diagnosis for you of on hypolyndal because they'll get angiometous lesions in the back of the eye and if we just take the motion out of it, I think we're in pretty good shape here. There has not been a retinal or subretinal hemorrhage, at least not yet. So hemangioblastoma is the diagnosis. We've got at least two and there are certain very critical search patterns that not only have to be performed but have to be suggested in a proper and respectful way to the clinician so this gets done on a regular basis perhaps over an entire lifetime. Thanks and we hope to see you in Chicago November 22nd for the first part of our case reading course November 22nd to November 24th followed by our ortho course which will begin on November 25th just prior to the RSNA at the four seasons on Delaware in warm and sunny Chicago. Thanks and have a great day.