Good morning, everyone. How's everyone doing? Coming on my way here, so first I'm Mohammad Atusham, I'm one of the faculty from Michigan State University, and we have a very excellent session lined up for you folks. So this morning coming here, the driver asked me, like, are you guys really going into the convention center? Like, you know, it's like one of those places where, and I said, well, this is our passion, we like learning, so we're gonna do that first, and then the reward will come later. So the session, Lung Pathology, it's really cool. We have some interesting cases lined up. We have about five presenters here, and we're gonna get started without any ado. So the first one is Hairspray-Induced Lung Injury, and Dr. Kamsenko, and we're gonna get started. Good morning, everybody. All right, so welcome to Hawaii. Thank you for coming. Today I'd like to tell you about a case where I saw a patient with progressive irreversible air restriction associated with chronic hairspray exposure. So my name's Cam, I'm from the University of Maryland, I'm a third-year fellow there, and I have nothing to disclose. So these are just some learning objectives. So I'd like to tell you about a 60-year-old female who presented to our Lung Transplant Clinic back in April 2022, after experiencing seven months of dyspnea exertion. She reports getting out of breath from walking from her bathroom to her bedroom, which is a stark contrast from before. When she presented to us for evaluation, her symptoms started seven months ago, back in November 2021, and she said she had some viral illness back then with rhinorrhea, nasal conjection, and some clear sputum production. She was tested negative for any virus at the time. When we talked to her to clarify, it seems like her symptoms were not entirely new. She did have years of chronic fatigue. A couple of years ago, she was seen by a cardiologist and she had an ECG, a treadmill stress test, which was negative for ischemia, but her test terminated early because of dyspnea. So just looking at her PFTs, so she came, she reported her symptoms started in November, and at the time, she had non-obstructive disease with a DLCO of 91%, but when she came to us, she had some repeat PFTs, and here we see some rapid decline in her obstructive airway phenomenon. So she went from a ratio of 48% down to 29, and she also had a significant drop in the DLCO. Past medical history, she had some cutaneous TCD30 non-Hodgkin's lymphoma back in 1999. She was treated with local radiation. She did not get any systemic chemo or systemic radiation. She's been followed up by her oncologist, and she did not have any signs of recurrence of disease. In terms of environmental exposure, she works at City Hall as administrator. She's been tired for a couple of years. She also had a history of smoking for about 10 pack years, but quit 30 years ago. What's interesting about her is that she has this lifetime enthusiasm for hairstyling. So she would get up in the morning since she was 15, apply her hairspray for a while, like 30 minutes to an hour each time. She consumes overall like two to three bottles a month, and she would buy them in bulk. Her family actually didn't report any respiratory symptoms because usually the odor is so bad for them that they would have to leave the house. So on physical exam, she was quite tachypneic. She was on four leaves of nasal cannula. We didn't really appreciate any crackles, wheeze, or squeak. The rest of her physical examinations were unremarkable. In terms of investigation, she had unremarkable autoimmune labs. She had a negative cardiac workup, and her baseline labs were also unrevealing. So here, I just want to show you the CT inspiratory scan. So overall, she had a pretty good-looking lung parenchyma. There was no signs of honeycombing, pulmonary fibrosis, ground glass opacities. She did have some irregularities of her small airways and some thickening of her small airways here. You know, we also got some expiratory phase scans. Here, we didn't see gross mosaic attenuation consistent with her PFTs. We did see some lower lobe hyperattenuation and very subtle mosaic attenuation here, too. So because of her rapidly changing course, we saw her in lung transplant clinic, and she underwent bilateral lung transplant a month later. Here on the right side, this is a gross normal histology. So it's like probably you and I showing you just what the normal bronchus and pulmonary vessel looks like. On the left, that's her lung X-plan. So under low magnification, we see preservation of her overall lung architecture, but the red arrow represents abnormal airways. So you can see that obviously from this low power anyways. So zooming in a little bit, what we see here are the typical signs of constrictor bronchiolitis. And what that is defined is peribronchial inflammation and fibrosis leading to obliteration of the airways, and it could be complete or partial. So the thin black arrow is actually epithelium. If I can show you with the arrow. Oh, the mouse is not working. So the thin black arrow is epithelium, and the black thick one is actually the smooth muscles. So in a normal lung pathology or histology, that should be very close up to each other. And what we see here is a thickening and separation between the two arrows, because there's growth deposition of collagen and extracellular material, which is a classical presentation for constrictor bronchiolitis. And we also don't see any airways here too. What we see here is quite interesting. So the yellow marks the airway wall, and the red actually marks the alveolar. And we see deposition of forming macrophages, which is kind of classical for constrictor bronchiolitis too. And we also see infiltration of lymphocytes, mononuclear cells, and some eosinophils. So overall, some inflammation here too, and an obliteration of your airways. Zooming in a little bit, we were curious to find this foreign material. So here, what we see is some wavy patterns of deposition of foreign materials, and it has a basophilic staining, which is consistent with PVP. So PVP also stands for polyvinyl pyrimidone. It's a common material that's found in hairsprays. And what it does is actually helps thicken the material. So it thickens the hair. And here, that's a quite classical pattern, which is this well-described early on since the 50s. That's when that was really commonly used. So here, we also see complete obliteration of the airways too. So overall, the diagnosis here is a chronic hairspray exposure and PVP deposition in the lungs. Thank you very much. Thank you. Okay, so this is obviously quite fascinating. Now, this has obviously not been reported the first time in the literature. This has been reported before with the hairspray or some other type of aerosol. So questions from the audience? Can you go back to that CT that you did? So, and then, so this is your inspiratory, right? Yep. And then go back to the expiratory. So you can also see that this patient also has some tracheal collapse also, both on inspiratory and expiratory, suggesting that there could be some tracheal malaisia. I don't know if that could be part of the similar condition because of the underlying pathology or just a different issue. We think it's a separate issue, but it's interesting that we also found she has tracheal malaisia. Right. Yeah, PVPs, those case reports are well-described in the 50s and then since the hairspray ban because of the CFC, because of the ozone layer, it's been really declined. But for some reason, she had kept using all these hairsprays for years. Do you have a question? Eric Lin on Morehouse School of Medicine. I'm just curious, how did you come up with the decision for doing a bilateral lung transplant versus a single lung? Our center usually favors bilateral lung transplant because of outcome. From what I know, we sometimes do single lung transplant for COPDs just because of lung sizing. But as far as I know, that's probably my limitation on my knowledge on lung transplant. Any other questions? All right, on to the next speaker. So Extramedullary Hematopoiesis with Indendiform Pulmonary Ossification, Multiple Myelomatization. Dr. Turbo. Hopefully that's the driest part of my presentation is the title. Hey guys, I'm Lindsay. I'm a Pulmonology and Critical Care Fellow at George Washington University Hospital and the DC VA Medical Center. I don't have anything to disclose. So we're just gonna jump right in. Today we're gonna talk about a 67 year old gentleman who's got a past medical history of ischemic stroke that's complicated by functional quadriplegia. This led him to have a pretty significant history of recurrent aspiration pneumonias causing hospital admissions. And he was also undergoing workup for monoclonal gammopathy of undetermined significance as an outpatient in this hematology clinic. During one of these visits, he was found to be pretty significantly anemic and with a new acute kidney injury and was asked to come in for, not only to have a transfusion, but just for a workup of what was going on with his acute kidney injury. So he's brought in to the emergency department and he is a patient that we've all seen many times before, cachectic, contracted, unfortunately nonverbal and very, very rancorous on exam. So this is just kind of a view of what he looked like when he arrived. He was slightly hypotensive, then improved with IV fluids. And then as far as his labs go, we do note that he has leukopenia and anemia, pretty significant derangements in his BUN and creatinine, elevated protein, elevate alkaline phosphatase and an elevated phosphate. Given his hypotension on admission or on presentation, he gets a full infectious workup. UA is dirty, but otherwise not floridly infectious. His blood cultures are no growth to date and his chest x-ray shows these very coarse by basilar predominant nodular interstitial markings that suggests an infectious or inflammatory process. However, when we look back, it doesn't look tremendously different from chest x-rays taken last month or even two months previously. However, there is suggestion that there may be a new right side of pleural effusion forming, therefore he gets put through the donut of truth. Where we see here that the patient does have bilateral, bi-basilar, dense calcified interstitial infiltrates, as well as a pleural effusion that is seen. Given his history of MGUS, his new anemia and kidney disease, he is also put through a PET scan, which here in the same cut demonstrates that he has moderate to high uptake in the bilateral lung bases. Again, suggesting that there is some kind of inflammatory process going on there, as well as the redemonstrated calcifications. And then what's not shown here is he has multiple axial and appendicular skeletal bone lesions and then mild or diffuse FDG uptake within the liver. And so with that entire picture put together, we come to the conclusion that something is wrong and we go to this patient's bone marrow, where we find many atypical or immature plasma cells with high nuclear to cytoplasmic ratio, prominent nucleoli, large nuclei, all findings consistent with the mature plasma cell myeloma. So now one of our questions has been answered. Why did this patient come into the, or what was the cause of this patient's anemia and his acute kidney injury? Obviously he has gone from monoclonal gammopathy of undetermined significance to a plasma cell myeloma. But our other question is, what is going on in this patient's lungs? So he's admitted to the hospital, he's undergoing continuous workup. On hospital day three, there's a rapid response called for him because he's requiring frequent suctioning, he has multiple hypoxemic episodes. And we again remember that he has this moderately sized pleural effusion. So it's tapped and we get about 700 cc's of red pleural effusion with red pleural fluid found to be hemorrhagic, largely segmented neutrophils. But from all other accounts and perspectives, his ADA is not terribly high, LDH isn't exceedingly high, culture is negative, and the cytology doesn't show that there is any other malignant process going on with this gentleman. Unfortunately on hospital day four, while the patient is visiting with his wife, he has witness seizure-like activity that initially responds to Ativan, multiple hypoxemic episodes requiring suctioning, he then becomes great cardic as respiratory arrest and then cardiovascular collapse. Unfortunately this patient did end up passing away. So my presentation is not to tell you about a poor gentleman that aspirated and died in the hospital. While it's unfortunate, it's not very uncommon, but it is more to kind of highlight the findings that we found post-mortem to answer the question of what was going on inside of this gentleman's lung parenchyma. So here is his autopsy report. His cause of death is listed as respiratory failure. Secondary causes are multiple myeloma and lung ossification. And so in the description of what we found in this gentleman post-mortem was he had extensive adhesions in his thoracic cavity which was consistent with his recurrent aspiration events. His left large bronchus was filled with thick mucous exudate. In his liver he had lobular and portal infiltration of plasma cells consistent with multiple myeloma. And in his lungs his histologic sections showed distended alveoli consistent with pulmonary edema, but also multiple ossification and occasional bone marrow island formations with dendroform pulmonary ossification. And if you're anything like me, you saw the first part and said bone marrow formation and dendroform what? So I kind of wanted to go back and talk a little bit about how calcium ends up in the lung parenchyma in the first place. So there's calcification, which is deposition of calcium ions within tissues in the body. That's either in a, I'm sorry, there's calcium deposition within tissues in the body. And then there's ossification, which is a formation of bone and bone marrow products within tissues of the body. And then there are two different types of pulmonary ossification. There is nodular pulmonary ossification where you find bone products, but not necessarily cellular bone marrow. And then there's dendroform pulmonary ossification, which was what we saw in this patient. It is an alveolar process that occurs in the interstitium. And it's thought to be due to repeated insults that turn the fibroblasts within the lungs into osteoblasts and bone marrow is then formed. The chronic repeated insults that are sometimes attributed to this disease process are either chronically anoxic environments, such as patients that have fibrosing lung injuries or patients who have recurrent lung aspiration and they're exposed to recurrent acidic environments. On the other hand, there's a completely separate process that's known as extramedullary hematopoiesis. And that is the formation of bone marrow outside of the bone marrow. And formation of bone marrow outside of the bone marrow in the typical adults, as we all remember back from embryology, we have multiple locations of bone marrow formation in embryogenesis, the spleen, the liver, and the bone marrow, which changes just to the bone marrow as we grow into adults. However, in times of stress, either malignancy or infection when the body is required, has increased demand, there's thought to be migration of stem cell precursors from the bone marrow to other places in the body that had previously had hematopoiesis occur and hematopoiesis does occur. So with that in mind, getting back to our patient. We see in his lung, in growth sections of his pathology, basilar predominant, very dense fibrotic calcifications. You can see some noted there in the arrow. And then I pointed out his hematoma on the left just because it was relatively obvious. Here's where the really interesting part begins. So the asterisk in the top left for you guys is normal alveolar tissue, fluid filled as the patient was extremely edematous. However, denoted by the blue arrow, you can see trabecular bone, and then immediately to the right of that, cellular bone marrow network. So we are seeing normal lung right next to bone marrow. And then on the right is just a highlighted image of the same thing showing cellular bone marrow. And here's where our case gets really interesting. Again, you can see the top left is hematoxylin and eosin staining showing bone marrow and trabecular bone within the alveolar network inside the lungs. And then the top middle is a high power view of the same. On the top right, we use myeloperoxidase staining. And myeloperoxidase staining highlights myeloid precursor cells, which you can see are lighting up in this image. The bottom left is CD61. And CD61 actually stains megakaryocytes, which you can see are present in the bone marrow. And then CD71, which is staining immature, amateur erythrocytes within the bone marrow. So not only have we demonstrated that in the lung parenchyma itself, we have the formation of bone and bone tissue, we also are demonstrating that there is hematopoiesis occurring in these tissues at the same time. So what are we looking at here? Unfortunately, this isn't a cause of death diagnostic mystery, but we're looking at a very interesting disease process that seemingly has either two separate diseases occurring or we have a poorly understood mechanism of one disease process. So why does this patient have bone marrow in the lungs? Clearly he was a setup given his fibrosing lung disease and recurrent aspirations for dendroform pulmonary ossification to occur as a result of repeated insults to his lung tissue. However, he also had a diagnosis of a monoclonal gemopathy and eventually a plasma cell leukemia that could in theory explain why he would have extra medullary hematopoiesis going on as well. And so what we tried to ask ourselves was, is this a functional process that's occurring in this patient? Meaning, was the bone marrow in this dendroform pulmonary ossification actually serving a purpose? And does DPO actually in patients form or undergo hematopoiesis? And is this truly just a spectrum of disease? And I tried to look through the literature and I didn't find anything that shows that patients with DPO actually have active bone marrow, just bone marrow formation. And so this kind of begs the question in this patient, does he have two different processes going on, two relatively rare processes going on, or are these two disease descriptors actually a spectrum of the same disease process? And I don't have the answer to that question, but hopefully have given you guys food for thought and maybe an area for us to look into in the future. So these are my references and thanks for your attention. Thank you. Interesting, because like plasma cell dyscrasias, we see them outside of bone marrow, but typically spleen or those type of organs, even CNS. In fact, in our neuro ICU, we had a CNS patient, history of multiple myeloma and has CNS lesions that we recently picked up. But lungs is not that common and we don't see it that much. So I don't know if audience has any comments or have they seen any of these cases. Not that I had seen, no, no, no. He had plasma cell infiltration in the liver, not in the spleen, but there was not evidence of extra medullary hematopoiesis occurring there. It was just in the lungs that we found, which was very interesting. Okay, thanks so much. Thank you. Moving on to the next. So what comes to mind when you think about placenta and lungs? So we have Dr. Jose Ariguna. Got it? All right, please. Hello, everyone. Thank you for joining this session. This presentation is called What Comes to Mind When You Think About Placenta and Lung. My name is Jose Ariguna. I'm a PGY-2 internal medicine resident at the University of Texas Health Science Center in San Antonio. I have nothing to disclose. Today, I'm going to talk about a case of unilateral bullous emphysema secondary to placental transmogrification of the lung. So just a little introduction, emphysema falls under the umbrella of COPD along with chronic bronchitis. It is the abnormal permanent enlargement of lung air spaces with the destruction of their walls. Most common cause of emphysema is smoking. And the second most common is alpha 1 antitrypsin deficiency. A subcategory of emphysema is bullous emphysema, which is air filled spaces greater than 1 centimeter called bully. And as you can see in image B, that shows a CT lung of normal lung parenchyma. And then in image C, it shows a CT of the lungs with emphysema. As you can see, the enlargement of the air spaces with the destruction of the walls. So going on to the case presentation, we have a 17-year-old female with past medical history of exercise-induced asthma presented to the clinic with pleuritic chest pain and shortness of breath during a road trip from Colorado. She did not have any fevers, chills, night sweats. Recent infections and prior similar symptoms. She had no smoking history. Family history was unremarkable. And she had no childhood history of viral bronchiolitis and pneumonitis. Going on to the objective data, she was hemodynamically stable, sitting well in room air, physical exam. Lungs were clear bilaterally. And there was no significant labs. A CTP was done, which was negative for PE. But it did show something interesting, which I'm going to show on the next slide. So this is the imaging of the case. As you can see in image A, the chest X-ray, pretty normal left lung. But if you can see in the lower right lung, lower lobe, you can see a curvy linear opacity. And at that time, it was described as atalexis. And then after that, the CTP was done in image B. And you can see a pretty normal left lung. But in the right lower lobe, you see an air-filled space, 11.6 centimeters in diameter. Bully, as well as multiple bully around that and some emphysema changes in the upper right lung field. So this is the patient's clinical course. Throughout the next few months, patient's pleuritic chest pain and shortness of breath worsened. And at that time, right-sided bulectomy and lung resection was performed. The biopsy pathology showed nonspecific but compatible features, suggestive of a disease called James-Sawyer syndrome, which is unilateral hyperlucency of one lung usually acquired when a patient has childhood viral bronchiolitis or pneumonitis. After this bulectomy, patient's symptoms recurred, continued having shortness of breath, continued having pleuritic chest pain, and now had multiple pneumonias, which required ED visits, as well as some exacerbation of bronchiectasis. So at that time, the next decision was she got a right-sided pneumonectomy, and then biopsy on that lung tissue did confirm a diagnosis of placental transmogrification of the lung, which I'll now just call PTL, given the long name. So image A just shows a CT of the lungs, classical bronchiectasis with widened airways. And then on image B, that's the histology picture of PTL, which I'll describe further in the next slide. So a little bit about PTL. It's a very rare disease, only fewer than 40 cases in the literature. It is most common in males 24 to 44 years of age. And as of our knowledge, this is the first case in a female teenager. The basic definition of PTL is a benign unilateral bolus or cystic pulmonary lesion. Currently, the pathology is unclear. However, there is a few hypotheses going around. Histology, it is a histological variant of unilateral bolus emphysema. And under the microscope, it looks as placental villus-like papillary structures covered with epithelial cells, hence the name placental transmogrification of the lung. As you can see on image A, that is a histology picture of third trimester placenta. You can see the villus-like placental structures encased in epithelial cells. Very similarly, image B shows the histology of PTL, again, with those similar villus-like papillary structures with the enclosed epithelial cells. So how does PTL usually present? It's usually asymptomatic with incidental findings of a pulmonary nodule or a mediastinal mass. This is important because PTL is also associated with pulmonary hematomas, as well as adenocarcinoma of the lung. However, it could also present much like this patient case with shortness of breath, pleurotic chest pain, and if one of the bully bursts, can cause or cause endometrial thorax. So going on to treatments, the main thing here is that there's no clear medical guidelines for long-term management of PTL. Most of the management is done through surgery. Goal is to preserve as much lung tissue as possible in the minimally invasive way. So image A shows the VAT surgery, which is video-assisted thoracic surgery, which most of these surgeries are done. And then the surgical options for surgery include bulectomy, which is seen in image B. And then you can go into submaectomy, lobectomy, and pneumonectomy. Most cases are cured with surgery. So in conclusion, one should suspect PTL with unilateral bullous emphysema with no classic risk factors such as smoking. To manage this disease, it's important to have a multidisciplinary team with primary care physicians, pulmonologists, and cardiothoracic surgeons. As mentioned before, in terms of the surgery, it's important to preserve as much lung tissue as possible. And throughout all the literature review I did, most literature reviews say that the most important thing like for further studies is like to figure out like long-term management for this PTL case, given the age of the patient as well. Thank you. Wow, these are all fascinating cases. So you said this is never reported in the literature? So there's like 40 or fewer cases. This is, from what I know, the first one in a female teenager. Okay. I've never seen it, anyone, in any. Okay. So from a clinical point of view, when, obviously it's rare, so you're not going to think normally, but when would you start thinking about it, like in your clinical practice, like? So I think initially, so there was two send-outs in pathology in the first one when they did the bulectomy. It was nonspecific, that like biopsy, that's why they first thought it was the Sawyer-James syndrome. And then they redid it after the numectomy, and that one did confirm the PTL. Before that, no one really had any idea what was going on. We just called it like a cystic, or less, lung disease. Yeah. I mean, we see some of these type of patients, young patients, that cystic or bullous disease, and they just are there, you know, like they're managed. But at what point do you start thinking about all these, you know, fancy, rare things? Yeah, I think, I don't know the answer, but, you know. All right. Thank you. All right. So next is Dr. Ibrahim Khatem, a rare case of pulmonary angiosarcoma. Please. All right. Hello, everyone. My name is Ibrahim, and I'm going to talk about an interesting case of a patient who presented to us with shortness of breath. I have nothing to disclose. All right. So our patient is a 55-year-old female who is an ex-smoker, has not really any past medical history. She was referred to the ED by her primary care provider for abnormal CT chest findings. She's been complaining of some dyspnea and exertion and dry cough for about six months prior to presentation to the ED. She didn't have any chest pain, any palpitations, any orthopnea or PND, or any lower extremity swelling. Didn't have any recent history of trouble or surgeries, but she did endure some weight loss and decreased appetite. On physical examination, the patient was comfortable, completely hemodynamically stable, except for that she required four liters of oxygen to maintain normal saturation. Her lung auscultation, cardiac auscultation, and lower extremity examination were completely unremarkable. Initial laboratory workup that we obtained included a complete blood count, some inflammatory markers, and CMP was unremarkable as well. And this is the CT chest that she presented with from her primary care provider, and it showed bilateral pulmonary arteries filling defects extending to the segmental, subsegmental, and distal branches. At this point, the patient was started on a heparin drip for concerns of PE, and she was admitted to our medical step doctor for further evaluation. She underwent an echocardiogram, and that showed no right heart strain, but more importantly, it showed multiple masses and possibly thrombi, and inferior vena cava, right atrium, and in the pulmonary artery. And she underwent a DVT study, lower extremity DVT study that was negative. At this point, the way she presented, the current state of her symptoms, the lack of DVT or right heart strain, we started suspecting that these filling defects are masses rather than thrombi. And she underwent a PET scan that showed increased metabolic activity of these masses. And from there, she underwent a CT-guided transthoracic biopsy of the pulmonary artery with interventional radiology, and pathology is shown here. This is an itch and e-stain slide that showed really most of the biopsy was necrotic tissue, but the viable areas showed ovoid and spindle cells with increased chromatin to cytoplasm ratio, and more importantly, immunohistochemical staining for vimentin was positive, but it was negative for other endothelial, epithelial, and lymphoma markers. And the final diagnosis from pathology was that this is a high-grade sarcoma, most consistent with intima sarcoma of the pulmonary artery. Our patient at this point had a staging walk-up with a CT of the abdomen pelvis that showed a sclerotic bone lesion in the pelvis concerning for metastasis. Given the extent of her disease, she was deemed to be not fit for surgery, and she was starting a palliative chemotherapy with ephosphamide and doxorubicin. So what is intima sarcoma of the pulmonary artery? It's basically a malignant, a very rare malignancy with an incidence of up to 0.03% that arises from the intima layer of large blood vessels and mainly the pulmonary artery. Most patients are going to present with nonspecific signs and symptoms, mainly shortness of breath, dyspnea and exertion or cough. Some patients can present with fevers, cachexia, night sweats, decreased appetite, and patients with locally invasive disease can present with hemoptysis or dysphonia. Patients with advanced disease can present with signs of hypertension, right-sided heart failure like lower extremity edema, and congestive hepatopathy. The main thing for the diagnosis is really differentiating venous thromboembolism from malignancy and ISPA, and that delay of that differentiation can delay the diagnosis. So that really happens through good history and finding the curiosity of symptoms. Then CTA, chest, PET scan, echocardiography, and the gold standard is biopsy. This is a table from the Korean Journal of Radiology, showing some different CT findings that can differentiate between ISPA and venous thromboembolism, including high attenuation, bulging contour of the tumor, and some other findings. But biopsy is the gold standard. It's mainly obtained through surgical resection, and that is mainly obtained through anamnectomy. Most of these patients are going to have the representation will be inoperable, and biopsy can be obtained just like our patient with a CT-guided biopsy, a trans-thoracic biopsy, transbronchial biopsies, or EBAS, and some case reports report biopsy via endovascular approach. And eventually, the most important thing in pathology is immunohistochemical staining that is positive for vimentin. Desmond and SMA expression is variable. And epithelial, endothelial, and lymphoma markers are usually negative. In terms of treatment and prognosis, this is really a poor prognosis disease. Most patients without treatment don't survive more than a couple of months. But with a combination treatment with surgery and chemotherapy, they can survive up to 25 months as well. Patients with inoperable tumors can, in some case reports, underwent endarterectomy to reduce the burden of the tumor and reduce the pulmonary hypertension. These are my resources, and thank you. Thank you. Very nicely done. So any questions out there? Pulmonary angiosarcoma, that's out there. We've seen it. And so the thought I have is, and I don't know what you folks thought about it when you were dealing with this case. So in previously, before we were doing thrombectomies for pulmonary embolism, we put patient on IV heparin and anticoagulation, and they go home or whatever. But nowadays, something like this, like you saw, if you are thinking on the CAT scan, that's a large clot burden. This patient could end up in the CAT lab or with IR and undergo a thrombectomy. So what are the implications in that situation? Of doing a thrombectomy on such a patient? Yes. I'm not really sure. I don't think we really thought about referring the patient to VAR at that point, given the current state of her symptoms, the features of the CAT scan, like the bulging tumor that she had. From the moment that we admitted her to step down, the concern that was that this is a mass, not a thrombus. So we pursued with getting the PET scan and the medicine as she woke up. So I think that's the point that, realizing the CAT scan findings of sarcoma, because you can differentiate it, at least you can get a suspicion that this is maybe not just a straightforward blood clot, but more than that. I agree. Good. All right. Thank you very much. Thank you. So moving along, coming to our last case. So diffuse idiopathic pulmonary cell hyperplasia diagnosed by a navigational bronchoscopy. Dr. Kimi Agenji. Hi, everyone. Thanks for being here. Eight minutes till lunch. So I'll be, I'm Kimi. I'm one of the Palmicret Fellows at MSK. I'll be presenting a case seen with Dr. Mohit Chawla on a DIPNIC diagnosed by robotic bronchoscopy. We have no disclosures. So our patient is a 49-year-old woman who was referred to our Inferential Pulmonary Clinic for biopsy for increasing diffuse small nodules over the course of six months. She had a dry cough and worsening shortness of breath and exertion that was partially improved with the use of a fluticasone inhaler. She had no known history of asthma, frequent bronchitis, or pneumonias. She had no wheezing, no fevers, chills, night sweats, weight changes, and the rest of her review of systems was negative. She's a never smoker. Well, she was born in Indian Group in New York City. She had a negative PPD about a year ago, no toxin or animal exposures, and she works as a preschool teacher. In terms of her physical exam, it was overall normal except for desaturation from 96 to 91% on ambulation. Her labs before coming to clinic were overall normal. She had a cardiac workup that was normal, and she had a CTA chest that was negative for PE, and she also had a PET scan that was done that showed no hypermenopaulic lesions. We had her complete PFTs, which showed no overt obstruction in the setting of mild extra thoracic restriction, and interestingly, her FEF 25 to 75 was low and responsive to bronchodilators, and she also had evidence of air trapping. Her CT chest showed many small solid nodules that were all centered around bronchioles. The sizes of the nodules were all subcentimeter, and the largest one was eight millimeters. There was evidence of subtle air trapping on this inspiratory CT. She didn't have any mediastinal lymphadenopathy, no pleural effusions, and no liver or adrenal lesions. I'll go through the differential diagnosis we had for this patient based on the information we had so far. So just to kind of summarize her case, she's a young female never smoker who presented with chronic dyspnea on insertion with mild desaturation, small airways disease, and many small nodules in the bronchovascular distribution associated with air trapping on imaging. So her presentation overall is one of bronchiolitis. The types of bronchiolitis that fit her history were potentially infectious, follicular, or constrictive bronchiolitis, which has many different causes. She didn't have any exposures that would fit hypersensitivity pneumonitis, and she's a non-smoker, making respiratory bronchiolitis unlikely. She had no evidence of bronchiectasis on her CT, and she didn't really fit the profile of the very rare condition of panbronchiolitis. Other causes of obstructive airways disease that didn't really fit her story were chronic severe asthma, COPD, excessive dynamic, airway collapse, and tracheomalacia. So she underwent robotic bronchoscopy, and she got biopsies of nodule and valingula, and then in the left lower lobe as well. So this view on the left here is the navigation that was done with the robot, and then the cone beam CT on the right in order to get to the target nodule, and this is showing the left lower lobe nodule, which was about eight millimeters by six millimeters, and then she had a BAL that was also done of the left lower lobe. So for her BAL results, her cytology was overall negative for malignancy, and all of her infectious workup was also negative, and then we had her do some serum workup, which was also negative, and that included chromogranin A, IgG subclasses, a full rheumatologic workup, HP panel, and the rest of her serum infectious workup was also negative. So this is the surgical pathology of the left lower lobe nodule, and this was the biopsy that was diagnostic. On the low-power image, you can see a piece of cartilage, so you can see that this nodule's actually very much centered around the airway, and on the high-power image, you can see there are hyperplastic neuroendocrine cells. Her immunostains were diffusely positive for TTF1 and chromogranin, and her KI67 immunostain showed a very low proliferation of less than 1%. Chromogranin comes from the secretory vesicles of neuroendocrine cells, and TTF1 actually normally exists within bronchioles, though we use it as a marker of lung adenocarcinoma. KI67 is a marker of cell proliferation. It tends to be fairly low in reactive neuroendocrine cell hyperplasia, slightly higher in DIPNIC, and even higher in small cell lung cancer. So her diagnosis overall was DIPNIC along with a slow-growing neuroendocrine tumor. Given the small size of the sample taken, the pathology was actually called nodular neuroendocrine cell proliferation. So we looked at the biopsied nodule on the CT scan, and it was larger than five millimeters, and it was contiguous with the bronchiole on imaging. So this nodule that was biopsied was diagnosed as a slow-growing neuroendocrine tumor. Given that she had many other small nodules, signs of small airways disease, this was thought to be progression of a nodule from an underlying diagnosis of DIPNIC. So neuroendocrine cell hyperplasia, it's one of many conditions we see in pulmonary where there's a clear correlation between the patient's story, their imaging, and pathology. Pulmonary neuroendocrine cells make up less than 1% of all cells in adult airways, and they're thought to act as, to regulate airway tone as airway chemoreceptors. These cells can proliferate as a reaction to a trigger, whether from inhaled substances or from underlying chronic infection or asthma and many other causes. DIPNIC is a premalignant condition without any known trigger. The neuroendocrine cells can be hyperplastic all over, so they tend to be associated with symptoms of shortness of breath, cough, and sometimes wheezing. Hyperplasia that is patchy is more characteristic of reactive neuroendocrine cell hyperplasia, and symptoms depend on how diffuse that patchiness is, so to speak. Focal proliferation or hyperplasia tends to be more associated with carcinoid tumors, which tend to be asymptomatic. DIPNIC is ultimately a histologic diagnosis, so to paraphrase the WHO definition, it's essentially more neuroendocrine cells where they're supposed to be inside the airway, and then if there's invasion of the basement membrane that's less than five millimeters, it's called a tumorlet, and if it's greater than five millimeters, it's called a tumor. So what specific things do we learn from this case? So right now there's some debate as to what's the best biopsy method for cases like this, or whether biopsy is always needed. The historical gold standard is surgical lung biopsy, but this case shows that you can use robotic bronchoscopy to navigate and biopsy these small nodules, but given that the sample size might be very small, you have to integrate the patient's story, their imaging, their PFTs, and then the pathology as well to come up with a full diagnosis. And just in terms of what's happening with our patient, she continues to follow with thoracic oncology, and her symptoms are actually well-controlled with consistent use of her steroid inhaler. Those are my references, thank you. Thank you.

patient

diffuse small nodules

shortness of breath

CT scans

biopsies

DIPNEC

steroid inhaler

diagnosis