I, I think we make the world how much Gemma, Gemma wanted and they rejected it and, and then they would have surgery and you there's no one gets like mid mid off mid level on which is little. February, but it's just weird but they were not worried about in the morning. on on the show, I think they wanted to see if it's on there. It's there's a all right, we're gonna go ahead and get started back surgery so I use the buttons. OK. It's, it's I If anyone texts, let me know because we have no one on the actual Zoom except us, which makes me worry that the link and that there may be part of the one out there is a little different, but. Yeah, we'll see Somebody joins and. Probably probably won't. All right, so we're gonna talk about um, pancreatic tumors, um. And um I will freely admit that my uh knowledge base in this subject is not incredibly deep. So basically the information that I have presented is kind of the depth of my knowledge. Once we get past that point, um, I'll be guessing probably, um, yeah, I may defer to expertise, uh, here in the room. I do not have, um, well yes we do. All right, so basically, um, pancreatic tumors, they come in. Uh, kind of two major forms, uh, the primary pancreatic tumors, and there's some non-epithelial pancreatic tumors that we're not going to discuss a whole lot about. Uh, and then of those primary pancreatic tumors, there are two types, those would be, uh, exocrine and endocrine pancreas. Uh, we'll spend most of our time talking about the exocrine pancreas tumors and a little bit of time talking about the endocrine pancreas tumors. No, OK, um. Yup. So, first of all, benign lesions, um, so, uh, kind of One of the benign lesions, uh, a, a serous cystadenoma, um, and these tend to show up as kind of, uh, multiple small cysts or, uh, several larger cysts. Um, so that would be an example of what a, uh, a serous cystadenoma that's, uh, a several larger cysts, or all of the cystic, uh, cystadenoma might look like on imaging. Um, in terms of these, um, beyond them being, uh, uh, multiple small cysts versus larger cysts, there are, um, uh, uh, lesions that, uh, they are lesions that are, are, are benign, um, and they may grow and become symptomatic. So even though they're not necessarily, uh, cancerous, uh, they can become large enough that they Develop symptoms. Um, there are some rare case reports of them becoming, uh, malignant, um, and about 1/3 of these are identified while you're looking for something else. Um, this is the lesion we find when you're getting a CT scan in trauma. Um, and 2/3 of these, uh, are identified because of abdominal pain and bloating or Some kind of palpable mass and importantly these lesions are cured by removal. Um, so, uh, um, the mucinous cystadenoma, um, which is similar to the serous cystadenoma but different in the mucin in it, um, and they have mucin them because the epithelium inside of these things, uh, produces mucin. Uh, they are a little more common in females. Uh, they are, uh, majority of these are located in the tail of the pancreas. Uh, they're, um, obviously can grow large as well, similar to the serous variety. Um, and then they, uh, can be, uh, just single or a few large cysts, and of course they Contain thick stuff. Um, these are generally considered to be, uh, benign but potentially pre-malignant lesions, um, and they, are removed. Um, so here's, um, uh, kind of the next thing, right, which is a, uh, mature cystic teratoma. Um, and the difference here is that you've got, um, uh, some calcified structures inside of this. You'll notice this one, is not in the tail of the pancreas. This one is closer to, um, uh, it's in the head of the pancreas. So these mature um cystic teratomas, uh, these are different, um, in the sense that they can arise anywhere within the, uh, uh, pancreatic branchyma. Um, and, uh, they are oftentimes even adjacent to the pancreas and the retroperitoneum. Uh, they tend to be large, um, unilocular or multilocular cysts, and they also contain kind of a thick, uh, sebaceous type of material as seen in other teratomas. Um. There can be malignancy in these, uh, and these, of course, are also, uh, resected based on symptoms and on, on their malignant potential. Hey, David, when you're reading about that, did they talk about, I, I would assume that most of those would actually be retroperitoneal and actually behind the pancreas, just kind of, you know, given the fact that, you know, that whole gonad descent and the germ cells can be anywhere along that. Did they talk much about that? It seemed like you're probably more likely to get. Fooled into believing a retroparent again, I don't know. I just guess, right, cause you, you've seen them all the way down everywhere where the gonad descends, and I, I think you're exactly right. Uh, and it may be inappropriate to have included these in the pancreatic tumors discussion. Uh, the chapter did describe some of them as being intrapar theoretically. But I'm not sure if those that have been described as interrenkal actually just grew into the pancre, yeah, I just wondered seem might make more sense for it to be retroperitoneal too, right, right. Um, but yeah, clearly with those, it did, did describe them as potentially being outside of the pancreas and just kind of pushing into, into the pancreas itself. So at a practical level, probably be worthwhile if you saw that to see if it would separate, I would guess. I mean, I've not done one, but I've done other retroperitoneal ones, but yeah, I don't know, other people, yeah, I, I've never taken out a keratoma out of. I haven't either curious. Um, all right, so I got a question. Can I ask you, to go to, so if I, and I realize it's incredibly rare, but a cystic lesion in the pancreas, we say there's no history of trauma, um. You know, is this, you know, the algorithm in adults is kind of US FNA if you can access it, send CEA CA 199, is that Would that be kind of the same in a teenager with a, you know, that you were concerned of for one of those early lesions? Cause I mean it, the first lesion is something you try not to take out of an adults. Um, yeah, I think the As I read through these, and we've so far talked about the The tumors and cysts of least malignant potential, um, but even these tumors where there's considered to be very limited malignant potential, um, as I read through this chapter. With the exception of the serous cystadenoma, which is probably totally completely benign, um, the mucinous versions, the teratoma, uh, they, they have enough malignant potential with them, but I'm not sure it makes sense to do a bias, yeah, cause you're gonna have sampling bias, uh, or sampling error, excuse me, uh, sampling error issues potentially, and ultimately the, the concern for Malignant potential combined with the fact that these are most often coming up as being a symptomatic lesion, I think you're ultimately gonna. Yeah, independence, like, The, the serous cyst adenomas in adults that I remember, you have like a 2 or 3 centimeter. Cystic lesion you're trying to figure out. Whether it's where it is on the spectrum, right, of music production and or are any of those tumor markers positive within it. A lot of these in kids I think are bigger and and Causing mass effect, at least the ones that I've been a part of interacting with. All that being said, with Trevor here, sampling, it's really easy, especially if it's one of those big things. And, and then it just frames the discussion is if it's serious, if he sees mucin, if, if any of those tumor markers are positive. Because you're, I'm sure you're gonna talk about the pancreatoblastoma, um, you can have cystic components of those lesions as well. So it It doesn't seem like it's always crystal clear to me. What you're dealing with, yeah, with the, and, and we'll talk more about it with the pancreatic blast so here in a minute, but that's the situation sampling errors really uh a potential concern. Um, I was, I also think it probably depends on where it is in the pancreas, right? If it's in the body or tail and you can easily remove it with a distal pancreatectomitting that's a different conversation than if it's in the head talking about whipple operation, because I think in the, in the, the conversation at at least my Remembering the conversation is, if you're serious, you can just observe it, but in a, in a 14 year old or fifteen-year-old, what does observation look like? You know, is it yearly MRIs? I mean, it's a long time to survey for a, a mass. Yeah, so I guess the kind of the question here is, In a 1314 year old who's involved in a motor vehicle crash has a small um tumor seen on, um, on, on the CT scan. And the question is, You know she's, she's totally asymptomatic, um, and it's small, doesn't seem to have a mass effect on anything. Um, is that something you'd be willing to watch, um, and I, I think it looked really serious, and then you get in, in the US and get a sample of it and it's truly serious, um. That's a situation where I might be convinced to watch it. Yeah, I've had a few of these. I think if they're less than 2 centimeters. Uh, the, these are not all easy to access with that, especially if they're small, um. Um, ideally, if you can put a needle in, see if there's mucin, that's great, but otherwise, if, if, if it's less than 2 centimeters, there's an incidental illness, and you watch it, and then grow. It's like me, yes. Not pretty much. There you go. And It's not as though as an authority. My, my sister is. Some expertise in this, and then, that's where I've Derive this opinions in terms of, uh, what do you do with these ones that are incidental and small? Is that your question? Yeah, I know, I just, it's pretty, pretty well-defined in adult algorithms, um, for, for these things. Uh, the other thing, you, you mentioned the trauma patient. It's often, it's, it can be hard to figure out whether or not there's a history of trauma preceding this thing and whether or not you're like that big one that they showed in cross section, whether or not you're dealing with a pseudocyst or an actual neoplasm. So that's the other thing, if you are doing in the US you should send it for amylase and lipase, which Theoretically, in a pseudocyst should be elevated and not necessarily in those other lesions. Yeah, I guess the only other thought I'd have and with, with no data, right, is this whole question of how you do surveillance, right? My sense is that like a lot of what we do is surveillance is really that we're uncertain that we're correct on our diagnosis, right? So if you have a small thing that's Uh, serious, right? The idea that you've got image it for 40 years, I'm not sure I buy that, right? I think the reason you do it in an adult is you're making sure, I think partly that you're really correct that it's serious, right? So I think if you had 5 years it was stable for my kid, I wouldn't say, oh we gotta. MRI every year because I again, I, I don't, some of these things are benign. I, the idea that it degenerates, I suspect that you misdiagnosed in the beginning and I like just like CP. I'm trying to think those are probably that they were PPP in the beginning, uh, and I can't prove that, right, but because we know there's a lot of people out there with things that haven't been imaged. In the adult algorithms or uh butter thought series uh um are are those follow up you guys algorithms forever or do they taper down as 23 seconds. Anything with duct communication, would that be by an amylase in my case too, so you wouldn't know if it's like an IPMN versus acidosis. Yeah, I think that's, I, I don't, that makes sense. Yeah, IPMNs are not things we really talk to kids. Has anyone seen an IPM, you know, child or not, I've seen a bile duct equivalent. I can't remember what it's called. There's an IPMN biliary duct equivalent that I've gotten on a gallbladder, and, uh, that we just watch them and you know we're gonna get MYCD and. Fortunately she moves and somebody else has it. It's not about to do that, nor in real life. But in adults where presumably benign panenesis, does the imaging persist forever, or does it taper down over time. I don't think you even surveyed the serious ones at all. I think if you see it, then you're done. It's my understanding, you don't have to continually watch the serious system was the serious system I know you're just done. I know with the mucinous and the IBM and reserves a size criteria, right. With with serial follow-up. I mean they became so incidental that we had a cyst clinic, a pancreatic cyst clinic that people went to got their MRI in the morning and then um because it became, I mean, so much image you just find all these little No thanks, man. You say pine that was the that's right yeah yeah. sprinkle it. Alright, so we have a question. Um, 15 year old girl presents to your clinic with worsening, um, Uh, upper abdomen, I believe is what that is. Uh, uh, pain, nausea, and bloating on your exam you find left upper quadrant. Uh, mass, computerized, uh, CT shows, uh, a mass in the pancreas which you see there, uh, most likely diagnosis in this adolescent patient c pancreatic mass is what? We've got a bunch of things up there which we've not discussed any of those things other than a pancreatic cyst, pseudocyst we kind of obliquely talked about those. This is not a uh Systadenoma pseudopapillary tumor. All right, solid pseudopapillary tumors. What do you know about solid pseudopapillary tumors? Um, I know that they have and solid components. They happen in females like in their 20s and 30s, or 2nd and 3rd decade most commonly. Um, they're usually benign, especially in younger patients, but they do have malignant potential, and they can present with metastatic disease, I think, I, uh, fairly frequently. Um, and there's overall good survival if you get a complete resection. OK. So here's a, here's a girl who's a 15 year old history of big abdominal pain based on a CT scan. They took out the distal pancreas, um, and A final report showed that it was a pseudopapillotic tumor similar to the labyrinth, uh, and, uh, the ink margins were, uh, through a tumor, piece of arterial infection, um. 4 millimeters from the red section. One lymph node included was also negative and uh in discussions with the patient family informed them that the pseudoapillary tumor in the pancreas is what the patient needs chemotherapy. Um, monthly CA 199 levels. They need a, uh, a PET scan. Should we obtain genetic screening to rule out von Hippel Lindau, uh, or that basically she's cured. She's cured. She is cured, OK. Very good. So this is a situation where we've got a pretty good option, right? So this fits into these, uh, varieties that are called, uh, kind of borderline malignant, um, uh, of the pancreas, these Brand's tumors, um, and what's interesting about them is, is that. Uh, I read it. It seemed to me that they're actually still pretty rare. Um, about 700 or so have been described in the literature, although I suspect that that number is increasing time. Um, these are young women, more than young men, uh, 28 years old is the mean age of diagnosis. Um, they tend to have minimal, uh, but vague, uh, uh, clinical symptoms. And they have a uh fairly benign course with uh some malignant potential and then um a complete surgical erection, uh, surgical resection with uh uh clear margins uh is the uh uh treatment of choice and uh nucleation as opposed to the insulinoma, we'll talk about later. Um, uh, is not considered adequate treatment. Um, so basically if they're in the, uh, body and tail, uh, in the distal pancreas, if they're in the head, then that's what you're talking about. Um, Uh, overall survival, as we discussed, uh, is really good, uh, with complete resection, and they tend to not be more tempered, um, and then, of course, uh, lymph node metastasis are fairly rare. OK, so before we move on to, uh, malignant lesions, I'm assuming this is for us, right, right. Should we take a moment for food? Thank you for coming. Thank you. As far as, uh, follow up, um, I mean we've done 2, I think, to the Do they do kind of standard follow-up 36 month of interviews and then space it out to a year at like 2 years? Has that been what our oncologist, I mean, I'm just, yeah, you just, you just gotta work on saying that with a little more confidence and you're gonna pass your reports. There we go. But is that what we've done? We've, yeah, uh, yeah, there's no defined follow-up regimen that I'm aware of. So that's what we're doing with the, the girl who had. The pancreatic head tumor, right, OK. I had one of these. I follow it for 5 years and Oh, yeah. Yeah. I grab some breakfast? Take a moment. We'll get restarted here in a moment. And that's a bit came back completely benign go away. I can't get a solid pack. Whatever that means, OK, yeah, negative margins, OK. It's a very bizarre benign tumor, right? Because there is a relatively, I don't know if it is 15, 20% present with metastatic disease, but it's called a benign tumor, right? So even what do you do if, if this patient presents with lung metastasis? You resect the primary, yeah, and survival is still really quite good without any adjuvant therapy of any sort, as far as I know it's like a DFSP, yeah. Yeah. Do you go after that loving rifle? Uh, the cases I've seen reported, you don't have to. You just resect the primary tumor and let those. Metastatic deposits hang out. But I don't know. Any experience with that? Mhm. Yeah, 0. It's amazing to me that you could get nice just follow that given the number of granulomas that I've removed in wills tumor patients. That's exactly right. What's the idea dermatofibroma sarcoma for tuberant. It's like this low grade skin, skin sarcoma back and that you see on the that's more tumor, yeah, and, and occasionally they would. Have a lymph node they're always they're like an elevated or slightly, but. So like when they say they got the bourbon handle and they can really have like I don't know what this is. Oh, you did I guess I took it out with the baby share it. I, I don't know. I mean, yeah, uh, just. Think about it. You that. It's know that I'm gonna, I'm in the next year, uh, your medical protocols with me, um, so I mean, I, I, I thought we're doing that so. You know, Rob, I just got a, I got a message and I probably was I don't really she's called in, but nobody he's in the conference, yeah, I think, uh, I'll talk toless said hard Harley again, yeah, because this is the only way I can record it is if I go through this link this is the wrong thing about it. Like I said, it doesn't matter to me. It just, um, talk about it. On Sorry, excuse me, sir, I went opposite way last time you had to train them up and that's what I did. I opened it up and then they're also charging healthy I mean at this point, alcohol healthy as so do it because the other ones out, yeah. Um, yeah, I mean, I think it'd be good actually to make it. Britain tells him to do that too. Which Um I mean, you know, no, you were not doing right now, so. Yeah, I don't know maybe Eric about it and see what his thoughts are but. All right, we'll move on. So, um, uh, malignant lesions are these, uh, uh, we've already touched base a little bit on some of these, the pancreaticoblastoma, uh, the mucinous cyst adenocarcinoma, the asinar cell carcinomas, and the pancreatic ductal adenocarcinomas. Um, we'll talk for a few minutes about each of these, um. We're gonna start off with a question. So, you got a 5 year old boy who complains of abdominal pain, loss of appetite, uh, and, uh, can palpate, uh, an epigastric abdominal mass. The ultrasound shows, uh, a large hypophobic, uh, to heterogeneous mass between the pancreas and liver. Um, abdominal, the, uh, uh, abdominal CT shows a large multilobulated mass with enhanced seping, um, and internal calcifications within the body of the pancreas. And then, of course, um, Uh, in compressing the adjacent liver, uh, and the alpha-fetoprotein is elevated, um, more than 50,000, and, uh, with that information, so you've got, um, A mass with the uh mass effect, an ultrasound, uh, that shows both uh hypochoic and the, the heterogeneous components and, uh, kind of multilobulated mass with enhance enhancing septate calcifications and an elevated alpha fetoprotein, um. You're most suspicious of which are, which of the following tumors uh in the child of the pancreatic glands. So, anybody have an idea on this one? Pancreaticoblastoma. That's when, uh, pancreaticoblastoma. So we're gonna talk about these for just a minute. So the, the words on this are pretty small, um, but basically these, these are the most common malignant pancreatic tumor, um, in, in young children. Um, they tend to be younger than 8, mean age of 5. they are associated with Becker Friedman, uh, and FAP. There are some tumors that have uh genetic abnormalities on chromosome 11 P15, and, uh, They are twice as common in males compared to females, right? So as opposed to some of these other things that have been more female dominant, uh, these tend to be, uh, more male dominant, um, slow-growing, um, and they tend to be kind of Uh, difficult to diagnose, um, until they reach a relatively large size. Uh, they actually cause symptoms and there's a palpable mass. Um, and we talked about the ultrasound already being hypo hypoccor to heterogeneous, um, oftentimes. There's, uh, calcium within these, um, with, uh, multilobular masses and septate, um, and then, uh, the MR might be able to help you see where it's at exactly, um, but the critical thing with these is that, um, uh, in a fairly high percentage of the alpha fetoprotein is elevated, right? And that's distinct then. All of the others we've looked at so far, uh, the benign lesions and the benign to, to, uh, malignant lesions, uh, this is enough fetal protein, uh, elevated, and if it is elevated, of course, it can be used to monitor for recurrence after you've removed, um, the mass. All right, yeah, the, so the features look different between this and the solid pseudoapillary on imaging. Um, if you have a mass in the tail of the pancreas, solid mass tail of the pancreas. Any reason to biopsy it. I know we kind of talked about that for our last one, but if it's removable. Just remove it. Yeah, I didn't get an AFP and just remove it. Other than the potential ease of biopsy, right? So that's, that's kind of where I, I, I think there's an art to this that I think is worth discussing depending upon the center where you're at, and I'd be curious to get Zach's impression of what would happen here. Um, but the reality is, if you biopsy this and It comes back as Something other than one of these things, are you gonna be truly convinced that it's not this. I fall back on the buharism that got me through my boards, name it, stage it, treat it, and I don't know, in, in practicality, if there's a solid tumor in the tail of the pancreas that Our radiologists are confident in suggesting a diagnosis, and you can easily remove it. And the family's gonna be OK with that. It makes a lot of sense. Um, but it's also with endoscopic ultrasound, really pretty easy to establish tissue diagnosis on a lot of these things. I, I've, I've, we, we've been biopsying almost all this stuff in the last couple of years, but, you know, but, I guess I, well, I've never seen a pancreatic by somebody, uh, a lymph node mets. Common is seen, I, I think the chapter said you can do a spleen preserving. That's why I was that's why I was asking if if you need to take this spleen, then that would change your management if it's pseudocapillary versus the left no one but you just do a, um, a spleen preserving and you, you're, you're right, you probably should go. So that's what those drugs are, OK, absolutely. Absolutely. All right, uh, the next one in that box of, but can, can I ask a question about that, but if it's, um, so if it's solid pseudopathway tumor in, in that patient, I guess they, uh, were symptomatic, um. You're still gonna take it out, right? I guess you're, I, I guess the point was that the solid pseudopapillary tumor, there's a lower risk of malignant potential. So if you can't save the spleen, you might watch it, or if you need to do a liquid harvest, this pancreoblastoma, you need to do a distal pan and still have to be. Um, if you don't need to do liquid harvest, then you can just do a distalectotectomy on both. The pancreatic bus time doesn't that that was my question. No, no, not that I've seen, and I understand your question. Yeah, nothing in the textbook. Yeah, I know, I, I think that the question I asked was, was really smart, which is, you know, if you end up having to do the same operation either way, um, there's no reason to know the difference before you take it out. Um, but if there was a difference in what you're going to do based upon what it was, then it would be potentially that could be just do a biopsy. OK. All right, so mucinous cyst adenocarcinomas, uh, also uncommon, um, they can be challenging to differentiate from the mucinous cystadenomas, which is why a lot of those mucinous cystadenomas coming out. Um, they large, located in the body, tail of the pancreas, um. Again you're gonna see on CT scan a large multiloulated cystic mass with internal septum in the hands, uh, histologically, um, they can appear benign and frequently have fossa of dysplastic or frankly malignant epithelium. So, um, the situation we're sampling there is a problem, right? So we discussed the, the, the problem with doing, uh. Uh, endoscopic biopsies on mucinous, um, uh, cystadenocarcinomas and potentially feeling some degree of Uh, comfort if it, it doesn't show malignancy, right, it's because you have the potential risk of malignant degeneration or, um, as Doug, I think appropriately pointed out, malignant degeneration may actually be existing malignancy that we just didn't see initial, initially that we were wrong up by, um. And uh these are managed by um complete excision, usually, uh, consisting of a spleen sparing, distal pancreatectomy, uh, with adequate margins and then uh you can get into the realm of uh adjuvant chemotherapy, um, for unrespectable or unmetastatic disease, um, and we actually know very little about how well that actually works. Have we had any of those here that I'm aware of that I'm aware of. All right. Ainar, uh, cell adenocarcinomas, so these are also rare. Um, uh, they, uh, for in, in young children, that is 3 years old, they tend to be a well-circumcised mass. They occur commonly, uh, more commonly in males than females. Uh, they can be equally distributed throughout the pancreas. So you, you can get into a situation where you're doing a whipple for these in the head of the pancreas. Um, non-specific abdominal pain. Um, and if if they, they tend to have areas of necrosis, um, on imaging, uh, vascular invasion is rare. Um, can you remove these just like all the others, and it's essentially, um, Uh, complete cure, uh, adjuvant care, chemotherapy or radiation afterwards is not necessary, um, and, um, And these tend to be much better in terms of prognosis than a uh typical ductal adenocarcinoma, um, which are these guys, extremely rare, right? These are the ones that kill you in your 40s and 50s and 60s, um, and they just don't happen very often, uh, in, um, young people, um, unfortunately, um. When they do happen in young people, they're probably associated, often associated with something else. Um, FAP, um, uh, Per, uh, and then, uh, hereditary pancreatic cancer syndrome. Um, these tumors are firm, they're growing into things. Um, and they occur frequently in the head of the pancreas. So you're talking about a whipple. Um, they present with pain and weight loss in only to adults. Um, and, uh, it's a situation where you can have obstructive jaundice from this cause again, they are invasive. They're in the head of the pancreas. They can block ducts and, um, uh, slow down the flow of bile through the, um, uh, common bile duct. Um, and, uh, treatments depend, dependent really on At the time of the diagnosis, how big is the tumor? Where's it at? Uh, what's it growing into? Can it be resected? Uh, and, um, uh, of course, this gets you into a situation where you're talking about potential palliative, um, uh, bypass procedure similar to, uh, the adult patient population. Um, and that Complicated discussion. All right, so we've now talked about all of these tumors of the exocrine pancreas. Have we, have we seen a, uh, pancreatic ductal adenocarcinoma here? Is that, it seems. We've seen one of those. I don't know if we've ever seen one. I had a teenager with one in Birmingham, really. I had salt and peppers. Was, was it resectable or not? Uh, it was a wet bull. Did someone do it, yes, I'd do it if it's like an adult. Yeah that's interesting. Any kind of genetic syndrome, yeah, I think realistically the last one I, I did, I did was, was, was with Selo because he was there at the University of Minnesota when I started training, you know, did a bunch of these with him, um, all right, so then we talked for a few minutes about, uh, the, uh, tumors of the endocrine pancreas, so particularly insuloma. So this is your, uh, most common, um. Uh, PNT, um, and this is a, a beta, beta islet cell, uh, uh, neoplasm, um, and basically they just make a lot of insulin and have consequences that are associated with, uh, that insulin production, in particular low blood sugars. Um, they tend to be small, uh, relatively well encapsulated, and because they make insulin, it gives us a way to actually diagnose these fairly early. Um, and, um, The classic diagnostic findings uh of the Whipple's triad are signs, symptoms of hypoglycemia, documented hypoglycemia during the symptomatic period and the resolution of symptoms um with uh revision of, of glucose, um, and, uh, uh, basically, uh, you know, symptoms, Children tend to have these less than a year before we, um, uh, diagnose them. I will say that, you know, when I went through training, um, I feel like at that time, and it's probably still true today, um, this is a situation where a, uh, particular institution in the United States has somehow figured out a way to kind of corner the market on insulin illness. Um, at that particular time, it seemed like most of these were going to chop. Um, is that still true? I mean, it, it seemed like at that time I was in Ohio, if we diagnosed an insulinoma, that patient ended up in Philadelphia, um, even though there were several children's hospitals between that patient and, uh, and child. Yeah, I, I, I don't think that's true. I've not observed that for insulinoma. I think it's true for hyperinsulinism of the newborn. Yeah, but I mean, I, I've, I've taken care of insulinomas here. I mean. Um, but, you know, kids that come in through endocrine and researched them. Did you end up doing any of the venous sampling, any of that? Were they, they were, I mean, you could identify. They presented clinically with, you know, exercise-induced hypoglycemia and, um, the imaging, you could see a lesion on MR and and nucleated and so, OK. Yeah, so these are fairly simple when the biochemical diagnosis is obvious, right? So patients have low blood sugars, you give them some glucose, blood sugars come up, uh, and then you get imaging and you see a. A mass in the pancreas, right? That there, that's a, that's a fairly straightforward clinical scenario, um, where things get a little more fuzzy is when you're, um, uh, when you're, when you've got a good biochemical diagnosis, right, and this is a situation where, uh, you've got a, a high elevated level of insulin. And uh elevated C-peptide levels. Uh, why don't we check C-peptide levels, by the way? Make sure it's not exogenous, right, but make sure somebody's not giving the child insulin shots, right. Yeah, OK, that is true. That is why. Um, and then, uh, you, uh, look for the, uh, beta hydroxybutyric acid level of less than 2.7, um, and then you give them some glucose and their blood sugar comes up. So that, that's this whole biochemical, uh, diagnosis part of this. Um, and then the anatomic localization, so that's where the imaging, you're trying to figure out where this thing is in the pancreas and you know, if you do an, uh, a CT or an MRI or An ultrasound and um uh you can find it, then you're in pretty good shape. Where things become a little bit more uh confusing is when those things don't necessarily identify an obvious tumor. Um, because then you can get into the world of interventional radiology, uh, doing, uh, selective, um, Arteriography, um, with some, uh, uh, uh, localizing agents, um, to try and see where it is. Uh, you can do the, uh, uh, PET CT, the AFDA, uh, PET CT that might be helpful. Um, but you can also get into a situation where even that's not necessarily entirely, uh, identifies where, where it's at. So you may be into a situation where you're in the operating room and doing venous sampling, trying to figure out where this thing is in the pancreas. I've never been in that environment. I don't even know where to start with that. Um, but that's theoretically what this, uh, chapter talked about. But ultimately, if you can figure out where it's at, you scoop it out. Um, and, uh, they, you would think that something like this would be relatively vascular, right? That, you know, if it's, uh, uh, a, uh, endocrine, part of the endocrine function of the pancreas where it's actually secreting the hormone into the bloodstream, that it would be a, a fairly vascular structure, but they actually tend to be relatively well encapsulated and surprisingly not have, they do not have any blood vessels attached to them. So, I've never inoculated a pancreatic tumor. Is it, it just kind of shells out? Do you leave a drain, or is it, uh, they usually not connected shell out. This, this is something I have done. They, they, they, they just, it's like popping a grape out. I did a few in residency. I haven't seen one in a, in a child, but the biggest concern for us was always the relationship of the tumor to the duct and making sure you're, you stay right on it and it does kind of pop right out. The, the few that, that one of the two that I did had a pancreatic leak and we left a drain and because you, you are cutting through parenchyma, so we left drains. Um, I think the, the venous sampling and the interventional radiology stuff. Might be a, a, a little bit antiquated from an era when imaging wasn't as good, um, because these are pretty frequently identified on MRI and if not, endoscopic ultrasound is really pretty good at finding them as well. So you have a pretty good idea where you're headed, and then intraoperative ultrasound lets you really see the relationship of the, the mass to the duct. And that, that's. My end of 2, but you know, somewhere, I think that's the main thing is just that in the deep margin where's the duck. Just put it, you know, look carefully and put it in a drain. I, I, they, they talked about doing lap, but I, I, I think I've done two. I did them open just cause again, that thing I know I'm gonna be looking for something I don't wanna miss it. I, technically, I think you get a new grade and I'd worry about whether I can find it. You literally pop it out or are you like caught, is it like cautery like your cauterize it, it's like a lumpectomy. I mean, basically, OK. Yeah, it's um the one I remember doing were they were stuck to the surrounding tissues about the same degree that a gallbladder is stuck to the liver and taking a gallbladder, gallbladder so they don't just literally pop off, right? but there is a, there's a it's a, it's, it's clear where the edge of it is. It's not, OK. All right. OK, so we got a 15 year old girl, morbidly obese. She's undergone a subtotal uh parathyroidectomy after presenting for, uh, uh, nephrolithiasis. She has a workup which shows a fasting hypoglycemia, um, identifies inappropriate plasma and insulin and C-peptide levels and leads to a CT scan of the abdomen which reveals an abnormality in the pancreas and biochemical workups otherwise negative. So inclusion of which of the following is most likely to decrease the risk of recurrent disease in this patient with pancreatic tumor. All right. We talked about some of these um robot. We are, we are, you are an inner mountain, right? We love our robots. That's what I've learned. So an ultrasound, intraoperative ultrasound. Yes. You wanna do uh intraoperative insulin glucose levels, kind of like a parathyroid, PTH. No, you're not doing that. OK. Um, abdominal magnetic resonance imaging scan, you know, you got a CT scan, you need an MRI. Oh, so you're sticking with ultrasound, yeah, sticking with that, OK. All right, so intraoperative ultrasound. We do that here for lunch? Yeah, well, I've been, I've been doing it a lot lately. The radiologists are getting annoyed, but we also recently purchased this hospital, purchased a, a pretty high-end ultrasound that if anyone's interested, can get trained on, um, and it's really nice actually for liver and pancreas stuff. And, um, but the radiologist will definitely come up and, and do an ultrasound. All right, so insulinomas. One of the things to remember about these, they can be associated with uh uh multiple endocrine neoplasia type one, although gastronomas are much more common, uh, these uh sporadic insulinomas in children. Are often isolated, benign, while those with MEN one tend to be multifocal and have. A higher incidence of malignancy. All right, uh, gastronomas. Very rare, um, uh, situation where they have unregulated, uh, uh, secretion of gastrin, um, which leads to a hypergastronemia state, um, and then, uh, results in excessive stomach acid production. You end up with the, uh, ulcers, um, in the, uh, um, uh, stomach and, uh, duodenum. Uh, these are rare, um. Zollinger-Ellison syndrome. I feel like this is, uh, something that we all learned during medical school, um, and certainly a general surgery training, and, uh, they include abdominal pain, esophagitis, and secretary diarrhea, weight loss, and Uh, and the ulcers as I discussed, um, and then, uh, they are very rare in children, um, and 80% of these, uh, gastronomas occur sporadically, um, but at 20, 20% of them occur in setting of MEN MEN1, um, and we have those, those lesions that tend to be in. ME1 syndrome, um, part of that syndrome tend to be multicentric, malignant, and, uh, present at a higher rate of metastasis, um, and tend to show up at a slightly younger age, very similar to the insulinomas. All right. Um, this is the biochemical diagnosis these, um, which I, I don't think we'll spend a whole lot of time going over here, um, but, uh, the important thing to think about with these is that once you've got a biochemical diagnosis, um, About 60% of them are not in the pancreas, right? Most of these are in the wall of the duodenum and they tend to be between the, um, where the common bile duct inserts into the duodenum, the junction of the 2nd and 3rd portion of the duodenum, and, uh, then along the wall of the pancreas where it comes around the head and that sealed area. Um, and surgically, you can feel them. Um, I will say imaging is not super great for looking at these, um, and, but you can feel these, uh, when you're back there doing a, uh, Uh, uh, the, the procedure to remove it, which can actually be a, uh, a, a procedure in some circumstances. Um. A lot of these are, are, uh, metastatic, especially if they tend to be part of the ME1 syndrome as we discussed. And um, Uh, so you can use some other types of imaging techniques. So that's our gastronomy discussion. All right, so, That's it for um pancreatic tumors. Um, we got a little bit of duodenal tumors. Pretty much all of these are rare. Um, imaging ends up being super important now and there's kind of an evolving, uh, evolving, uh, role for endoscopic ultrasound in, in the management of these. That's, that's all I've got on pancreatic tumors. Anybody ever seen a gastronomer a kid? Oh, I've seen several in going on, but I've never seen a gastro. I tried to look for him did a bunch of adults with endocrine guys but. And the one thing I remember about that was that for the students and interns, right, they teach about this gastronomic triangle, which is basically the duodenal sweep in the head of the pancreas. At least what we do back then is we'd flay the duodenum open. So you could feel both walls, they feel, you know, bimanually feel the walls of duty and I don't know if that's what. I don't know, but you'd always find multiple little ones in these MEN one patients feel another little thing that you couldn't feel with the dude do them. But I remember Doctor Thompson would open it I'll be like, oh my gosh, we're just like laying this open like yeah that's where I learned the duty it's actually just a piece of intestine. Yeah, you can, you can just open it right up. Yeah, the, the duodenum is strangely, it, you know, you, you get involved with the duodenum and trauma, and, and it makes us all very anxious, but strains gonna, we get away with a lot of operating on the duodenum without many leaks, and I don't really have a good explanation for why that is. When you think about the duo, you know, do it in Austin as we do what we're doing in trees. This I always I probably get get away with that era. I don't know what. What you do Thanks like, oh, we'll need the doctor pick magnets right out of the river. Learn that the magnets who got the magnets magnets. OK, all right, it seems. Thank you, baby. That's an X-ray, OK, yeah. No, thank you. Off to the rib. So, Katie, were there any adventures that you cut out? Oh yeah. to the dentist.
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