Case Report of Succesful Multidisiplinary Treatment for Patient with Metastatic Lung Cancer and Relapsed/Refractory Chronic Lymphatic Leukemia/Small Lymphocytic Lymphoma

Introduction: Croatian National Cancer Registry of Croatian Institute for Public Health reported that in year 2020 lung cancer was the second most common cancer site diagnosed in men with 16% and the third most common in women with 10% incidence among all cancer sites. Unfortunatelly lung cancer has the highest mortality in both men and women. Haematological malignancies had 7% share in all malignancies in both male and female cances cases. In 2020 190 newly diagnosed cases of lymphatic leukemia in men and 128 cases in women were reporeted, meaning 1.5 and 1.2% of all malignancies, respectively. Chronic lymphatic leukemia (CLL) is an advanced age disease and incidence increases with age. Impaired immunity, T and B cell dysfunction in CLL, chromosomal aberations, long-term immunosuppressive therapy and genetic factors can all cause secondary malignancies. Co- occurence of solid tumors and CLL is very rare. Although patiens with CLL have an increased risk of developing second primary malignancies including lung carcinoma, the data about their clinical outcomes are lacking. Parekh et al. retrospectively analyzed patients with simultaneous CLL and lung carcinoma over a 20-year period, and they found that ~2% of patients with CLL actually developed lung carcinoma. The authors claimed that up to 38% of patients will also develop a third neoplasm more likely of the skin (melanoma and basal cell carcinoma), larynx (laryngeal carcinoma) or colon. Currently there are no specific guidelines for concurrent CLL and non-small cell lung carcinoma (NSCLC) treatment. Usually, when the tumors are diagnosed simultaneously, treatment is based to target the most aggressive malignancy, as the clinical outcomes depend on the response of the tumor with the poorest prognosis. For this reason, a multidisciplinary approach is mandatory. Case report: A patient with history of coronary heart disease, myocardial infarction and paroxysmal atrial fibrillation was diagnosed in 2019 (at the age of 71) with B chronic lymphocytic leukemia with bulky tumor (inguinal lymph nodes 8x5 cm), stage B according to Binet, intermediate risk. He was treated with 6 cycles of chemoimmunotherapy (rituximab/cyclofosfamid/fludarabine). In 10/2019 remission was confirmed, but MSCT described tumor in the posterior segment of upper right lung lobe measuring 20x17 mm and bilateral metastases up to 11 mm. Bronchoscopy and biopsy were performed, and EGFR neg, ALK neg, ROS 1 neg, PD-L1>50% adenocarcinoma was confirmed. He was referred to Clinical Hospital Center Osijek where monotherapy with pembrolizumab in a standard dose of 200 mg intravenously was started in 01/2020. Partial remission was confirmed in October 2020. Immunotherapy was discontinued due to development of pneumonitis, dysphagia and severe weight loss (20kg), but without radiologically confirmed disease progression. At that time he was referred to our hospital for further treatment. Gastroscopy has shown erosive gastritis with active duodenal ulcus, Forrest III. Supportive therapy and proton pump inhibitor were introduced. After complete regression of pneumonitis, improvement of general condition and resolution of dysphagia, no signs of lung cancer progression were found and pembrolizumab was reintroduced in 12/2021. Hypothyroidism was diagnosed in 01/2021 and levothyroxine replacement ther apy was started. In 03/2021 he underwent surgical removal of basal cell carcinoma of skin on the right temporal region with lobe reconstruction. From 02/2021, when pembrolizumab was reintroduced, regression in tumor size was continously confirmed with complete recovery of general condition. He was hospitalized for COVID 19 infection in 09/2021, and due to complications pembrolizumab was discontinued till 11/2021. Lung cancer immunotherapy proceeded till 11/2022, when Multidisciplinary team decided to finish pembrolizumab because of CLL relapse. CLL was in remission till August 2022 when due to B symptoms, lymphcytosis, anemia and generalized lymphadenopathy, hematological workup including biopsy of cervical lymph node was performed and CLL/SLL relapse was confirmed. Initially chlorambucil was introduced, but disease was refractory. Based on cytogenetic test results (IGHV unmutated, negative TP53) and due to cardiovascular comorbidity (contraindication for BTK inhibitors) venetoclax and rituximab were started in 01/2023. After just 1 cycle of treatment normal blood count as well as regression of B symptoms and peripheral lymphadenopathy occured, indicating the probability of complete disease remission. In our patient with metastatic lung adenocarcinoma excellent disease control is achieved during 41 month of treatment in first line setting. Furthermore, relapsed/refractory CLL/SLL is currently in confirmed remission. Conclusion(s): Successful treatment of patients with multiple primary malignancies is based on multidisciplinarity, early recognition and management of side effects, treatment of comorbidities with the aim of prolonging life, controlling symptoms of disease and preserving quality of life.

Is histogram analysis useful in the diagnosis of COVID-19 patients?

Aim: In this study, we aimed to show the contribution of the chest computed tomography (CT)-based histogram analysis method, which will enable us to make quick decisions for patients who are clinically suspected of having COVID-19 infection and whose diagnoses cannot be confirmed by polymerase chain reaction (PCR) tests. Material(s) and Method(s): A total of 84 patients, 40 in the PCR-positive group (age range: 17-90 years) and 44 in the PCR-negative group (age range: 15-75 years), were included in the study. A total of 154 lesions with ground-glass density, 78 in the PCR-positive group and 76 in the PCR-negative group, were detected in these patients' thorax CT scans. The region of interest was placed on the ground-glass opacities from the images and numerical data were obtained by histogram analysis. Numerical data were uploaded to the MATLAB program. Result(s): The localizations of ground-glass densities in the CT findings of patients with probable and definite COVID-19 diagnoses were similar;74.7% of the ground-glass areas in both groups showed peripheral distribution. Lesions were frequently observed in right lungs and lower lobes. In histogram analysis, standard deviation, variance, size %L, size %M, and kurtosis values were higher in the PCR-positive than the PCR-negative group. When receiver operating characteristic curve analysis was performed for standard deviation values, the area under the curve was 0.640, and when the threshold value was selected as 123.4821, the two groups could be differentiated with 62.8% sensitivity and 61.8% specificity. Discussion(s): The use of histogram-based tissue analysis, which is a subdivision of artificial intelligence, for clinically highly suspicious patients increases the diagnostic accuracy of CT. Therefore, performing CT analysis with the histogram method will significantly aid healthcare professionals, especially in clinics where rapid decisions are required, such as in emergency services.Copyright © 2022, Derman Medical Publishing. All rights reserved.

Airway remodeling in COVID-19 lungs

Besides parenchymal changes that have been described extensively in COVID-19, bronchiectasis is also reported but detailed characterization of airway changes is lacking. Hence, we aimed to quantify the number of visible airways and their diameters in end-stage COVID-19 lungs. Explanted right lungs, obtained after lung transplantation (n=2) or autopsy (n=1) (65.3+/-26.7 days after symptom onset), were inflated to total lung capacity, frozen and scanned with whole lung microCT (155 mum). Airways were segmented using Mimics Innovation Suite (Materialise, Belgium) and airway count and diameter were assessed using Neuronstudio. Three discarded donor lungs were used as controls. Number of visible airways increased in COVID-19 lungs compared to controls (fig.1a) potentially caused by airway remodeling and bronchiectasis (fig.1b, red arrows) due to fibrotic rearrangement (fig. 1b). Small airway count (diameter 0-2 mm) in generation (G) 1-11 was lower in COVID-19 patients compared to controls, with a shift of small airways from lower generations (G1-11) to higher generations (G12-27) in COVID-19 patients. Simultaneously, airways with a diameter > 2 mm were increased in all generations in COVID-19 (present until G21 compared to G13 in controls). This study shows that COVID-19 causes a remodeling of the (small) airways, leading to an increase of visible airways and diameter of large and small airways, similar to that seen in idiopathic pulmonary fibrosis due to traction bronchiectasis. (Figure Presented).

Clinical features of 153 patients with COVID-19 in Chongqing municipality.

Objective: To analyze the clinical features of patients with COVID-19 in Chongqing Municipality. Method(s): The clinical data, laboratory tests and chest imaging findings of 153 patients COVID-19 admitted in Chongqing Public Health Medical Center from January 26 to February 5, 2020 were retrospectively reviewed. According to the relevant diagnostic criteria, patients were divided into non-severe group (n=132) and severe group (n=21). The correlation between serum index changes and disease severity was analyzed. Result(s): The proportion of patients with underlying diabetes or chronic respiratory diseases in severe group was significantly higher than that in non-severe group (chi2=11.04 and 6.94, P<0.05). The proportion of symptom-free patients in non-severe group was significantly higher than that in severe group (chi2=4.09, P<0.05). The symptoms of fever, fatigue and muscle soreness in the severe group were more common than those in the non-severe group (chi2=4.40, 14.42 and 22.67, P<0.05). Among the concomitant symptoms, the proportion of cough and shortness of breath in the severe group was higher than that in the non-severe group (chi2=8.46 and 4.80, P<0.05). C-reactive protein and D-Dimer levels were higher in the severe group than those in the non-severe group (Z=-4.39 and -1.96, P<0.05), and the number of CD3+ T lymphocyte cells, CD4+ T lymphocyte cells and CD8+ T lymphocyte cells in the severe group was lower than that in the non-severe group (Z=27.25, 20.60 and 17.36, P<0.05). Compared with the non-severe group, both lungs and the right lung lower lobe were more susceptible to be involved(chi2=9.7123.61, P<0.05). Conclusion(s): There are significant differences in underlying diseases, clinical symptoms, imaging manifestations and laboratory findings between severe and non-severe patients with COVID-19.Copyright © 2020 by the Chinese Medical Association.

The diagnosis of spontaneous secondary tension pneumothorax following apparentrecovery from COVID19 pneumonitis

Background: Coronavirus disease 19 has been associated with a plethora of different manifestations of systems affected (including pulmonary, gastro-intestinal, and thrombotic disease) and time to presentation of complications. Pneumothorax has been established as a complication in the literature. However, tension pneumothorax remains a rare presentation with higher mortality. We report a case of secondary tension pneumothorax in a patient following apparent recovery from COVID-19 pneumonitis. Case presentation: Eight days after resolution of COVID-19 pneumonitis symptoms a 51-year-old Caucasian man with no pre-existing pulmonary disease was brought into the emergency department following 48 hours of progressive shortness of breath. Further clinical assessment revealed reduced breath sounds in the right lung, BP was 116/95 mmHg and jugular venous pressure was not elevated. Chest x-ray showed right-sided tension pneumothorax with mediastinal shift. Insertion of a chest drain led to rapid resolution of symptoms and the patient was discharged following full re-expansion of the lung. Conclusion(s): The period of recovery from COVID-19 is variable. Clinicians should consider tension pneumothorax as a possible complication of COVID-19 pneumonitis in patients presenting with type 1 respiratory failure, even after resolution of pneumonitis symptoms and a considerable time period following initial contraction of COVID-19.

Legionella Myocarditis Masquerading as Dual Chamber Cardiac Arrhythmia

Background Legionella pneumonia is a rare cause of myocarditis. Case 64-y.o male with CAD and PCI to LAD, DM and HTN presented to ER with mental status changes. On exam he was febrile and hypoxic.Presenting rhythm was Afib with frequent bouts of sustained and non-sustained stable posteroseptal VT treated with amiodarone and mexilitene. With right lung infiltrate on CXR and elevated WBC count, antibiotics were initiated for pneumonia. SARS COV-2 Ag and Influenza A & B was negative. Urine Ag for legionella was positive and was promptly treated with Levofloxacin. Coronary angiogram prior to discharge showed non-obstructive CAD. Decision-making Legionnaires' disease with myocarditis was suspected. Patient underwent CMR with late gadolinium enhancement (LGE) and Rest 82Rb perfusion and 18F-FDG PET/CT with high-fat dietary preparation scan for evaluation of legionella myocarditis. CMR revealed LVEF of 46%, with LGE and PET findings as described in the Figure. He was initiated on solumedrol for ongoing inflammation after completion of antibiotic therapy for Legionella pneumonia. Conclusion Our case highlights a systematic approach to differential diagnosis and use of multimodality imaging in legionella myocarditis presenting with dual chamber arrhythmia. There was good correlation between LGE inflammation/scar location and origin of VT, as well as active inflammation demonstrated by FDG PET imaging. The patient was successfully treated with antibiotics, steroids and anti-arrhythmic drugs. [Formula presented]Copyright © 2023 American College of Cardiology Foundation

Not all shortness of breath and throat pain is COVID

Case Report: A previously, healthy 18-year-old female presents to a Pediatric Emergency Medicine Department with shortness of breath, fever, and worsening throat and abdominal pain for 3 days. She had a sick contact, a teacher that tested positive for COVID-19 2 weeks prior to presentation. She denies runny/stuffy nose, cough, loss of taste/smell, or rashes/lesions. She denies any significant past medical history including allergies, as well as any history of smoking or any illicit drug use. Upon arrival to the ED, the patient was noted to be tachycardic, hypotensive and febrile. There were no desaturations. Initial physical examination revealed a generally uncomfortable female that was alert and oriented, with noted tenderness over the right anterior neck region, diffuse cervical lymphadenopathy, and painful neck range of motion. Her pharynx was noted to be erythematous without exudates or any unilateral tonsillar swelling. In the ED patient received IV fluid resuscitation and was started on norepinephrine drip, broad spectrum antibiotics. Initial lab workup revealed an anion gap metabolic acidosis, likely secondary to uremia or lactic acidosis from poor perfusion in setting of sepsis and hypovolemia. BUN and creatinine were elevated, likely due to an acute kidney injury (AKI) secondary to hypovolemia. The patient was also found to have an elevated LDH, fibrinogen, and mild elevation of AST. D-Dimer was elevated at 29 000. Covid PCR, Rapid Strep, and respiratory PCR panel were negative. Her chest X-ray (CXR) was negative and ECG showed sinus tachycardia. Given the patient's history of throat and neck pain with shortness of breath, in the setting of a septic picture, a CT scan of neck, chest, abdomen was ordered prior to transferring the patient to the PICU. CT scan of the chest revealed small patches of consolidation with ground glass opacities in the right lung apex, as well as an nearly occlusive, acute thrombosis of the anterior right facial vein. The patient's initial blood cultures grew gram negative bacilli which later were revealed to be Fusobacterium necrophorum. These findings are consistent with Lemierre's syndrome. The patient was treated in the PICU on vasopressors, heparin anticoagulation, and antibiotics for 6 days and discharged with a course of Augmentin. Lemierre's syndrome is an infectious thrombophlebitis of the internal jugular vein. First described by Andre Lemierre in 1936, it begins as a bacterial pharyngitis, generally developing into a peritonsillar abscess or other deep space neck infection with progressive erosion into the internal jugular vein. Diagnostic criteria for Lemierre's syndrome includes radiographically evidence of thrombophlebitis of the internal vein and positive blood cultures. CT and MRI can help make the diagnosis, but are not always required. Treatment is prompt intravenous antibiotics with beta-lactamase penicillins, metronidazole, clindamycin, and third generation cephalosporins. [Figure presented] Copyright © 2023 Southern Society for Clinical Investigation.

Community-acquired Methicillin-resistant Staphylococcus aureus Pneumonia and Parapneumonic Effusion in a Male Patient with Diabetes: A Case Report

Methicillin-resistant Staphylococcus aureus (MRSA) infections are a primary health concern. They are commonly differentiated as hospital-acquired methicillin-resistant Staphylococcus aureus (HA-MRSA) and community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) infections, based on their epidemiology, susceptibility findings, and molecular typing patterns. Therefore, appropriate contact precautions and isolation measures should be implemented. CA-MRSA mostly causes skin and soft-tissue infections, but the probability and incidence of it causing sepsis and invasive infections have increased dramatically in recent years. In this study, we report a case of CA-MRSA pneumonia with pan-pneumonic effusion in a 59-year-old male diabetic patient with preexisting comorbidities such as diabetic ketoacidosis and non-ST elevated myocardial infarction. The early reporting of the organism's identity and its antimicrobial susceptibility, as well as timely initiation of antibiotic therapy, aided in the successful management and cure of the patient.

CLOSE-TO-EFFORTLESS BREATHING PARADIGM MAPS GLOBAL AND REGIONAL LUNG FUNCTION USING ELECTRICAL IMPEDANCE TOMOGRAPHY CROSS SECTIONALLY AND LONGITUDINALLY

SESSION TITLE: Late Breaking Investigations From Pulmonary and Critical Care SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/18/2022 01:30 pm - 02:30 pm PURPOSE: Although spirometry is the standard lung functional test, it requires the patient to perform a series of maneuvers correctly, which is difficult for elderly, children, and patients with severe lung impairments. Furthermore, spirometry lacks regional assessment for detecting and monitoring subtle changes in lung diseases, e.g., chronic obstructive pulmonary disease (COPD) and potentially COVID-19. We aim to establish a home-based imaging system, portable electrical impedance tomography (EIT), that can detect lung function deterioration and monitor its recovery through a close-to-effortless breathing paradigm. METHODS: We developed a palm-sized EIT system and a novel guided breathing paradigm that consists of a periodic inhalation and exhalation at 12 breaths per minute. We validated them on healthy subjects (n=23) performing different breathing efforts (deep vs shallow), then on patients with ILD (n=2), COPD (n=8), asthma (n=4) and bronchiectasis (n=4) against healthy (n=8) cross-sectionally, and last monitored a COVID-19 discharged subject with two age- and gender-matched healthy controls longitudinally. We further applied machine learning to distinguish between healthy and patients, and calculated its sensitivity and specificity. RESULTS: We detected higher amplitude during deep breathing compared to shallow (p < 0.001) in healthy subjects, with right lung having more activated voxels and higher total amplitude than the left lung (p < 0.001), likely due to the position of the heart. Cross-sectionally, we observed lower amplitude in patients compared to healthy (p < 0.01), while coefficient of variation (CV) of the amplitude in the lungs is higher in patients (p < 0.05). Note that CV is a parameter reflecting inhomogeneity which is indicative of lung function deterioration. Longitudinally, the COVID-19 discharged subject had higher CV in the left lung (p < 0.001) which decreased across time (p < 0.01), suggesting a functional deterioration at the beginning followed by a recovery. Regional analysis further pin-pointed the potential deterioration and recovery was in the anterior left lung. Separately, despite the small sample size, the sensitivity and specificity for detecting patients using a machine learning classifier were 76% and 62%, respectively, and will likely increase with a larger sample. CONCLUSIONS: Home-based portable EIT with close-to-effortless guided breathing paradigm can map global and regional lung function deterioration and recovery cross-sectionally and longitudinally. More importantly, it can potentially be deployed as a screening tool for various lung diseases through the application of machine learning. CLINICAL IMPLICATIONS: Portable EIT with guided breathing paradigm enables lung function diagnostic screening and treatment monitoring at home, advancing telemedicine and lowering hospital burden. DISCLOSURES: no disclosure on file for Peng Cao;Owner/Founder relationship with Gense Technologies Ltd Please note: Since 2017 Added 06/06/2022 by Russell Chan, value=Ownership interest No relevant relationships by Wang Chun Kwok No relevant relationships by Wei-Ning Lee No relevant relationships by Terence Tam Employee relationship with Gense Technologies Please note: Setpember 2021 - Now Added 06/07/2022 by Adrien Touboul, value=Salary contractor relationship with Gense Technologies Ltd Please note: since Apr 2021 Added 06/06/2022 by Eddie Wong, value=Consulting fee Employee relationship with Gense Technologies Please note: since 2020 Added 06/06/2022 by Fedi Zouari, value=Royalty

PLEOMORPHIC CARCINOMA IN THE MIDST OF COVID

SESSION TITLE: Pathology Identifying Chest Infections Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Pleomorphic carcinoma is a subtype of sarcomatoid carcinomas that represents <1 % of all primary lung neoplasms. This case highlights a recent diagnosis of a patient with pleomorphic carcinoma in the midst of COVID-19 pneumonia. CASE PRESENTATION: A 75 year old female with a 180-pack year smoking history presented to the emergency department with dyspnea and chest discomfort. Vital signs significant for oxygen saturation at 93% on room air. The patient had been admitted to the hospital 7 months prior for acute hypoxemic respiratory failure due to COVID-19 pneumonia. At that point, computed tomography (CT) of the chest showed a right lower lobe 5.5 cm juxtapleural lesion measuring fluid attenuation by Hounsfield units without intralesional enhancement. The lesion was initially thought to be secondary to the patient's COVID-19 pneumonia and was not investigated further. The patient was subsequently lost to follow up. Seven months later the patient presented with worsening shortness of breath. Chest CT revealed large right complex pleural effusion with near complete lung collapse. The patient underwent pigtail catheter placement with partial re-expansion of the lung. Pleural fluid analysis showed an exudative effusion with no malignant cells on cytology. Follow-up CT imaging showed a large mass-like area in the right mid and lower hemithorax. Video assisted thorascopic surgery (VATS) decortication and thoracotomy revealed a right lower lobe abscess and empyema. Pathology samples collected during procedure showed malignant cells of sarcamatoid features found in right lung and intraparenchymal lymph nodes. Histology and immunostaining showed a tumor composed of a component of poorly differentiated adenocarcinoma and more than 10% spindle/pleomorphic cells. Immunostaining showed the poorly differentiated adenocarcinoma component was positive for moc 31, Ber-EP4, cytokeratin AE1/AE3, CAM 5.2, lack TTF-1 and p40. The spindle/pleomorphic component was negative for cytokeratins. DISCUSSION: Pulmonary pleomorphic carcinoma (PC) is a rare, poorly differentiated non-small cell lung cancer (NSCLC) that contains at least 10% spindle and/or giant cells or a carcinoma consisting only of spindle and giant cells. PC has poor response to conventional treatments for NSCLC and subsequently poor 5 year survival. It more common in men and smokers. COVID-19 causes a variety of pulmonary radiographic manifestations, including nodules and mass-like consolidations. Superimposed bacterial infections are also common. Our case, however, highlights the importance of serial radiographic monitoring and, when indicated, tissue sampling to rule out alternative explanations for abnormal CT findings. CONCLUSIONS: Appropriate screening and careful follow up of suspicious lung lesions is vital to ensure prompt diagnosis and treatment of lung malignancy. Reference #1: WHO Classification of Tumours Editorial Board. Thoracic Tumours. In: WHO Classification of Tumours,Earke 5th ed, IARC Publications, 2021. Vol 5. Reference #2: Ito K, Oizumi S, Fukumoto S, Harada M, Ishida T, Fujita Y, Harada T, Kojima T, Yokouchi H, Nishimura M;Hokkaido Lung Cancer Clinical Study Group. Clinical characteristics of pleomorphic carcinoma of the lung. Lung Cancer. 2010 May;68(2):204-10. doi: 10.1016/j.lungcan.2009.06.002. Epub 2009 Jul 3. PMID: 19577320. Reference #3: Maneenil K, Xue Z, Liu M, Boland J, Wu F, Stoddard SM, Molina J, Yang P. Sarcomatoid Carcinoma of the Lung: The Mayo Clinic Experience in 127 Patients. Clin Lung Cancer. 2018 May;19(3):e323-e333. doi: 10.1016/j.cllc.2017.12.008. Epub 2017 Dec 21. PMID: 29454534. DISCLOSURES: No relevant relationships by Rachel Earle No relevant relationships by Samantha Gillenwater No relevant relationships by Miquel Gonzalez No relevant relationships by Sikandar Khan No relevant relationships by Christopher Lau no disclosure submitted for Jinesh Mehta;

MANIFESTATIONS OF SCIMITAR SYNDROME IN AN ADULT

SESSION TITLE: Anatomical Cardiovascular Disease Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Scimitar Syndrome is a rare congenital disorder characterized by partial or complete anomalous pulmonary venous drainage to the inferior vena cava, right lung hypoplasia and displacement of the cardiac structures into the right hemi-thorax (1) The name Scimitar Syndrome is derived from the resemblance of the anomalous pulmonary vein to a curved Middle Eastern sword known as a scimitar (1). Some patients experience no symptoms while others may have severe pulmonary hypertension and dyspnea (2) (3). Cases may be discovered in infancy although some patients are not diagnosed until early adulthood. Here we present a case of a woman who was discovered to have symptomatic scimitar syndrome in her teenage years and opted to forego correction. CASE PRESENTATION: Patient was a 38 year old female with past medical history of Scimitar Syndrome who presented to the pulmonary clinic after a short hospitalization with mild COVID-19. Prior to contracting COVID-19 she had experienced worsening dyspnea over one year and now becomes short of breath after walking one half block. Open surgical correction was offered at diagnosis but was not pursued due to patient preference. A CT Angiogram of the chest was performed while she was hospitalized which showed an anomalous right pulmonary vein draining to the hepatic IVC, minimal bilateral patchy opacities and displacement of the heart into the right hemi-thorax. Echocardiography showed normal right ventricular size and function and did not show evidence of pulmonary hypertension. She was referred to the adult congenital heart disease clinic and continued to have severe dyspnea and fatigue. 6 months later, repeat echocardiography and cardiac MRI demonstrated borderline dilation of the right ventricle and atrium but no echocardiographic evidence of pulmonary hypertension. The patient remains hesitant to undergo invasive procedures or interventions. She has been offered a right heart catheterization to better characterize her volume status and obtain direct measurement of her pulmonary artery pressures. DISCUSSION: Although generally discovered in infancy and childhood, Scimitar syndrome may not be discovered until adulthood. Various treatments are available for correction of scimitar syndrome including open surgical approaches with direct implantation of the scimitar vein into the left atrium, trans-catheter occlusion of aorto-pulmonary collaterals and re-routing of the anomalous vein into the left atrium via endoscopic graft placement (4) (6) (7) (8). If left uncorrected worsening left to right shunting and pulmonary hypertension may occur (5). Our patient has developed borderline right ventricular dilation and has experienced severe functional limitation. A right heart catheterization is indicated to determine her pulmonary artery pressures. This case illustrates the potential consequences of deferring early treatment for Scimitar Syndrome CONCLUSIONS:. Reference #1: Frydrychowicz A, Landgraf B, Wieben O, François CJ. Images in Cardiovascular Medicine. Scimitar syndrome: added value by isotropic flow-sensitive four-dimensional magnetic resonance imaging with PC-VIPR (phase-contrast vastly undersampled isotropic projection reconstruction). Circulation. 2010 Jun 15;121(23):e434-6. doi: 10.1161/CIRCULATIONAHA.109.931857. PMID: 20547935 Reference #2: Abdullah A. Alghamdi, Mansour Al-Mutairi, Fahad Alhabshan, Scimitar syndrome: restoration of native pulmonary venous connection, European Heart Journal Supplements, Volume 16, Issue suppl_B, November 2014, Pages B41–B43, https://doi.org/10.1093/eurheartj/suu025 Reference #3: Khan A, Ring NJ, Hughes PD. Scimitar syndrome (congenital pulmonary venolobar syndrome). Postgrad Med J. 2005 Apr;81(954):216. doi: 10.1136/pgmj.2004.027813. PMID: 15811882;PMCID: PMC1743239 DISCLOSURES: No relevant relationships by John Prudenti No relevant relationships by Anthony Smith

BENIGN PLEURAL SCHWANNOMA WITH PLEURAL EFFUSIONS AND NECROTIZING PNEUMONIA

SESSION TITLE: Invasion of the Pleura SESSION TYPE: Case Reports PRESENTED ON: 10/18/2022 11:15 am - 12:15 pm INTRODUCTION: Schwannoma is a well circumscribed encapsulated solitary neoplasm arising from myelin producing cells of peripheral nerve sheaths. Pleural schwannomas represent only 1-2% of thoracic tumors and rarely present with pleural effusion. To our knowledge only six cases of benign pleural schwannoma have presented with a pleural effusion to date. We present a rare case of a pleural schwannoma with bilateral serosanguinous pleural effusions complicated by necrotizing pneumonia. CASE PRESENTATION: 54 year old smoking male with no past medical history was transferred from an outside hospital after two weeks of worsening acute hypoxemic respiratory failure while being treated for necrotizing pneumonia, right sided loculated pleural effusion, and a right paramediastinal mass. His only presenting symptom was worsening dyspnea for three days. Upon arrival to our hospital, the patient was on maximal ventilator settings with two right sided chest tubes draining blood tinged pleural fluid. CTA of the chest showed a large cavitary consolidation in the right upper lobe with destruction of the lung parenchyma. Additionally, there was an intrapleural heterogenous mass in the posterior aspect of the right lung apex which abut the mediastinum measuring 9.7 x 7.5 x 10.3 cm. He was treated with zosyn for positive sputum cultures growing beta hemolytic strep group F. Patient underwent a flexible bronchoscopy with EBUS-TBNA of mediastinal lymphnodes and lung mass which was non-diagnostic. A CT guided biopsy revealed a spindle cell neoplasm with a Ki-67 of 10-20%. Immunohistochemical analysis demonstrated positive staining of the tumor cells for S-100 protein. The final pathological diagnosis was benign schwannoma. He underwent a tracheostomy and PEG and was sent to a rehab center with outpatient follow-up with cardiothoracic surgery for tumor removal. DISCUSSION: Pleural schwannomas are slow growing, rarely progress to malignancy, and are often located in the posterior mediastinum. Patients are usually asymptomatic but can present with symptoms associated with obstructive pneumonia. It is very rare for a benign pleural schwannoma to present with a pleural effusion. Literature review has revealed only six cases of benign schwannoma presenting with a pleural effusion, all of which were blood stained. Spontaneous tumor hemorrhage or cyst rupture has been a theory of etiology for the effusions. Prognostically, once the pleural schwannomas are surgically resected there is minimal chance of recurrence. CONCLUSIONS: Our case represents a benign pleural schwannoma that caused extrinsic compression on the right upper lobe bronchus leading to a necrotizing pneumonia along with bilateral serosanguinous pleural effusions. A pleural schwannoma should be considered in the differential diagnosis of intrathoracic tumors even when presenting with pleural effusions. Reference #1: Shoaib D, Zahir M, Khan S, et al. Difficulty Breathing or Just a Case of the Nerves? Incidental Finding of Primary Pleural Schwannoma in a Covid-19 Survivor. Cureus. 2021. 13(8): e17511. Reference #2: Bibby A, Daly R, Internullo E, et al. Benign Pleural Schwannoma Presenting with a Large, Blood Stained Pleural Effusion. Thorax. 2018. 73:497-498. Reference #3: Nosrati R, Annissian D, Ramezani F, et al. Benign schwannoma of posterior mediastinum accompanied by blood pleural effusion misdiagnosed as solitary fibrous tumor: A Case report. Casplan J Intern Med. 2019. 10:468-471. DISCLOSURES: No relevant relationships by Brittany Bass No relevant relationships by Oleg Epelbaum No relevant relationships by Theresa Henson No relevant relationships by Yasmin Leigh No relevant relationships by Ester Sherman No relevant relationships by Sally Ziatabar

IDENTIFYING A NOVEL SURFACTANT PROTEIN MUTATION IN A FAMILY WITH PULMONARY FIBROSIS

SESSION TITLE: Rare Genetic Mutations and Anatomical Variants SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 12:25 pm - 01:25 pm INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a fatal disease affecting older adults that results in progressive scarring of the lung parenchyma. Familial IPF (FPF), defined by disease in two or more first-degree relatives, is estimated to occur in 2–20% of all IPF cases and can present with varying phenotypes which may be difficult to diagnose. Inherited gene variation as well as environmental factors predispose a patient to disease development. Additionally, rare genetic variants in the genes encoding surfactant A (SFTPA1, and SFTPA2) that affect alveolar stability and endoplasmic reticulum stress have been reported in less than 1% of FPF cases. Understanding these genetic variants is essential in the diagnosis and management of patients with FPF. CASE PRESENTATION: A 47-year-old Hispanic male with a history of COVID-19 one year ago (not requiring hospitalization) presented to the hospital for a two-day history of subjective fever and shortness of breath. He was hypoxic requiring oxygen via high flow nasal cannula. He was admitted four months ago for shortness of breath and treated for pneumonia. Since then, he has had chronic dyspnea with exertion. Computed tomography of the chest showed extensive ground glass opacities, worse in the right lung, with basilar and upper lobe honeycombing, and air bronchograms in the bilateral lower lobes. Family history was significant for a mother, maternal aunt, maternal grandfather, and maternal cousin who all died from pulmonary fibrosis. His maternal cousin was treated at our facility, in which genetic sequencing revealed a mutation in SFTPA2, c.697T>C. Our patient was found to have the same genetic mutation. DISCUSSION: The genetic basis of IPF remains poorly understood. Prior studies suggest only 20-30% of FPF cases harbor an identifiable causative genetic variant. Rare variants in two biologic pathways contribute to the known heritability of FPF including pathologic variants in surfactant related genes which cause improper protein trafficking leading to endoplasmic reticulum stress, defects in autophagy, and type II alveolar cell toxicity. SFTPA1 and SFTPA2 variants have been associated with FPF and lung adenocarcinoma in a small number of families and there are few reported cases. While currently the SFTPA2, c.697T>C mutation, previously reported by our group in 2016, is considered a variant of unknown significance, its occurrence in two relatives with serious progressive interstitial lung diseases suggests that it is indeed pathogenic. CONCLUSIONS: Gene sequencing should be considered for all patients with a family history of pulmonary fibrosis as identification of a rare genetic variant may offer guidance to diagnosis, prognostication, and risk stratification when considering lung transplantation as well as identify additional relatives who may be affected by IPF. Reference #1: Kropski JA, Young LR, Cogan JD, et al. Genetic Evaluation and Testing of Patients and Families with Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2017;195(11):1423-1428. doi:10.1164/rccm.201609-1820PP Reference #2: Wang Y, Kuan PJ, Xing C, Cronkhite JT, Torres F, Rosenblatt RL, DiMaio JM, Kinch LN, Grishin NV, Garcia CK. Genetic defects in surfactant protein A2 are associated with pulmonary fibrosis and lung cancer. Am J Hum Genet. 2009 Jan;84(1):52-9. doi: 10.1016/j.ajhg.2008.11.010. Epub 2008 Dec 18. PMID: 19100526;PMCID: PMC2668050. Reference #3: Pulmonary Fibrosis Due to a Novel Surfactant Protein Mutation R.A. Arciniegas Flores, I.A. Vital, K. Medepalli, D. DeMarzo, M.K. Glassberg Csete, R.A. Alvarez. https://doi.org/10.1164/ajrccm-conference.2019.199.1_Meetings.A5437 DISCLOSURES: No relevant relationships by Roger Alvarez No relevant relationships by Eduardo Lopez Gonzalez No relevant relationships by Anita Singh

A UNIQUE CASE OF SEVERE PULMONARY NOCARDIOSIS PRESENTING WITH ARDS AND CARDIOGENIC SHOCK

SESSION TITLE: Shock and Sepsis in the ICU Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Nocardiosis is a rare bacterial infection, which frequently affects immunocompromised patients. It can present as an acute, subacute, or chronic pulmonary infection with non-specific symptoms, such as fever, cough, dyspnea, weight loss, and hemoptysis. CASE PRESENTATION: A 34-year-old female with a history of chronic granulomatous disease and hidradenitis suppurativa on adalimumab presented to the ED with fever, shortness of breath, and productive cough of 2 days. Her vitals were T 101F, BP 66/48, HR 148, RR 42, and SPO2 94% on room air. On exam, she was cachectic, with bilateral crackles and rales in the right lung base. Extremities were cold, with trace pitting edema was present on bilateral lower extremities. COVID-19 PCR was negative. Despite fluid resuscitation, she remained hypotensive and was started on norepinephrine. Blood cultures were collected, and broad-spectrum antibiotics and an antifungal agent were initiated. Chest CT demonstrated bilateral multifocal consolidation with surrounding ground-glass opacities and complete consolidation of the right lower lobe. Due to worsening respiratory distress and tachypnea, and lack of improvement with non-invasive ventilation, she was intubated, placed on mechanical ventilation, and admitted to the Medical ICU. On hospital day 1, due to the patient's immunosuppression, unresolving shock, and radiographic findings, a bronchoscopy with bronchoalveolar lavage (BAL) was performed. On hospital day 2, a transthoracic echocardiogram showed LV ejection fraction of 20-25% with severe global hypokinesis of the LV. ACS workup had been unremarkable, with mildly elevated troponin and no ischemic changes on EKG. She was initiated on cardiac inotropes. On hospital day 3, BAL culture revealed Nocardia cyriacigeorgica. TMP-SMX and ceftriaxone were started for severe pulmonary nocardiosis. On hospital day 11, she was liberated from mechanical ventilation, and by hospital day 14, she was weaned off all pressors and inotropes. Approximately 4 weeks after admission, repeat TTE showed recovery of LV ejection fraction (55-60%) and she was discharged with a prolonged course of TMP-SMX and IV ceftriaxone, with duration to be determined at outpatient infectious disease follow-up. DISCUSSION: We discuss a unique case of severe pulmonary nocardiosis, presenting with ARDS and cardiogenic shock. To the best of our knowledge, this is the first case of a patient with pulmonary nocardiosis presenting with stress cardiomyopathy reported in the literature. While the pathophysiology is not well understood, theorized mechanisms include catecholamine excess, coronary artery spasm, microvascular dysfunction. CONCLUSIONS: This case highlights the need for a broad differential diagnosis in patients presenting with ARDS and cardiogenic shock and illustrates the value of clinical bronchoscopy in patients with unique presenting features. Reference #1: Lerner PI. Nocardiosis. Clin Infect Dis. 1996 Jun;22(6):891-903;quiz 904-5. doi: 10.1093/clinids/22.6.891. PMID: 8783685. Reference #2: Wittstein IS, Thiemann DR, Lima JA, Baughman KL, Schulman SP, Gerstenblith G, Wu KC, Rade JJ, Bivalacqua TJ, Champion HC. Neurohumoral features of myocardial stunning due to sudden emotional stress. N Engl J Med. 2005 Feb 10;352(6):539-48. doi: 10.1056/NEJMoa043046. PMID: 15703419. Reference #3: Park JH, Kang SJ, Song JK, Kim HK, Lim CM, Kang DH, Koh Y. Left ventricular apical ballooning due to severe physical stress in patients admitted to the medical ICU. Chest. 2005 Jul;128(1):296-302. doi: 10.1378/chest.128.1.296. PMID: 16002949. DISCLOSURES: no disclosure on file for D. Clark Files;No relevant relationships by Nisha Patel No relevant relationships by Meehir Shah

Pulmonary benign metastasizing leiomyoma (PBML) - A rare cause of lung tumor

Introduction: Benign metastasizing leiomyoma (BML) is a rare disorder that usually occur in premenopausal women with uterine leiomyoma. The commonest metastasis site is the lungs. Diagnosis and treatment are challenging and there is no standardized treatment for BML. Case Description: We report a case of a 66-year-old menopausal lady who presented to Sarawak General Hospital in October 2021 for Covid-19 infection, with incidental finding of pulmonary benign metastasizing leiomyoma (PBML). She experienced one-year history of weight loss. Chest X-ray on admission revealed a solitary right lung nodule (2 x 2cm). CT thorax revealed a right perihilar lesion with multiple smaller lung nodules. Tuberculosis was excluded. The histopathology result of lung biopsy surprisingly showed spindle cell neoplasm with smooth muscle differentiation. There was no palpable abdominal mass. Ultrasound showed irregular endometrial mass about 3.9 x2 cm. Repeated CT thorax and abdomen in April 2022 showed the lung lesion has enlarged to 4 x 4 cm. Patient is planned for a hysterectomy and bilateral salpingo-oophorectomy following a multidisciplinary meeting among Respiratory Physician, Oncologist and Gynae-oncologist. Discussion: Benign metastasizing leiomyoma is rare and not well understood. It usually occurs in premenopausal women with history of myomectomy or hysterectomy. Only few cases have been reported among postmenopausal women without prior history of leiomyoma. Lung biopsy is crucial in excluding serious causes such as malignancy and achieving the correct diagnosis. As there is no established treatment and monitoring guideline, treatment should be individualized and guided by a multidisciplinary team.

Proteinuria in Thin Basement Membrane Nephropathy: A Rare Finding

Hydrothorax occurs in approximately 2% of patients on peritoneal dialysis caused by migration of fluid from the peritoneal cavity into the pleural space via pleuroperitoneal fistulas. These diaphragmatic defects are usually congenital and right-sided, explaining the predominance of right-sided effusion.. Thoracocentesis with biochemical analysis of pleural fluid reveals a transudate with a very high glucose concentration. In uncertain cases, or when there is a clinical need to demonstrate the anatomy of the communication, an imaging approach such as peritoneal scintigraphy is required. 66-year-old Hispanic female with past medical history significant for end stage kidney disease on peritoneal dialysis for past 5 months, hypertension ,cerebrovascular accident with no residual deficit, and recent exposure to COVID19 presented with fever, shortness of breath, left eye gaze abnormality and + COVID PCR. She had been having issues with meeting ultrafiltration goals outpatient. On examination she has decreased breath sounds at right lung base, Abdomen non-tender with PD catheter in place with clean dressing, no pedal edema. Laboratory findings were consistent with a transudative effusion;no organisms were cultured and no malignant cells were visualized. CT failed to identify dispersal of contrast material into the right hemithorax. A nuclear isotope scan was subsequently done. Following administration of technetium 99m via the PD catheter, a high volume of radioactive dialysate was detected entering the right hemithorax. No tracer activity was seen in the left hemithorax. PD was stopped and switched to intermittent hemodialysis.Unfortunately she succumbed to covid 19 pneumonia and died few days later. 50% of the cases, a conservative approach allows reinstitution of CAPD Conservative approach with temporary cessation of peritoneal dialysis remains the first-line treatment. 1-4 months has been shown to be adequate cessation time and restarting with low volume PD. If conservative approach fails, Invasive approach with video-assisted thoracoscopic pleurodesis or diaphragmatic repair or both allows most of them to continue with CAPD

A Case of Hydrothorax Complicating Peritoneal Dialysis

Hydrothorax occurs in approximately 2% of patients on peritoneal dialysis caused by migration of fluid from the peritoneal cavity into the pleural space via pleuroperitoneal fistulas. These diaphragmatic defects are usually congenital and right-sided, explaining the predominance of right-sided effusion.. Thoracocentesis with biochemical analysis of pleural fluid reveals a transudate with a very high glucose concentration. In uncertain cases, or when there is a clinical need to demonstrate the anatomy of the communication, an imaging approach such as peritoneal scintigraphy is required. 66-year-old Hispanic female with past medical history significant for end stage kidney disease on peritoneal dialysis for past 5 months, hypertension ,cerebrovascular accident with no residual deficit, and recent exposure to COVID19 presented with fever, shortness of breath, left eye gaze abnormality and + COVID PCR. She had been having issues with meeting ultrafiltration goals outpatient. On examination she has decreased breath sounds at right lung base, Abdomen non-tender with PD catheter in place with clean dressing, no pedal edema. Laboratory findings were consistent with a transudative effusion;no organisms were cultured and no malignant cells were visualized. CT failed to identify dispersal of contrast material into the right hemithorax. A nuclear isotope scan was subsequently done. Following administration of technetium 99m via the PD catheter, a high volume of radioactive dialysate was detected entering the right hemithorax. No tracer activity was seen in the left hemithorax. PD was stopped and switched to intermittent hemodialysis.Unfortunately she succumbed to covid 19 pneumonia and died few days later. 50% of the cases, a conservative approach allows reinstitution of CAPD Conservative approach with temporary cessation of peritoneal dialysis remains the first-line treatment. 1-4 months has been shown to be adequate cessation time and restarting with low volume PD. If conservative approach fails, Invasive approach with video-assisted thoracoscopic pleurodesis or diaphragmatic repair or both allows most of them to continue with CAPD

ATYPICAL PRESENTATION OF LEGIONNAIRES DISEASE WITH DIVERTICULITIS IN IMMUNOSUPPRESSED PATIENT

CASE: A 69-year-old male smoker with stage 3b prostate cancer managed with abiraterone and prednisone, prior severe COVID-19 pneumonia requiring mechanical ventilation, and history of perforated sigmoid diverticulitis presented with 3 days of anorexia, watery diarrhea, and left lower abdominal pain. Two weeks earlier he developed a mild dry cough without fever, dyspnea, or chest pain. There were no sick contacts or recent travel. He was afebrile, and initial routine chemistries and a complete blood count were unremarkable. An abdomino-pelvic CT revealed acute diverticulitis of the distal descending and sigmoid colon. A consolidation at the right lung base was also incidentally noted. Follow up imaging confirmed a multifocal pneumonia on chest Xray. Legionella antigen was detected in the urine. Metronidazole and levofloxacin were initiated with clinical improvement and the patient was discharged home to complete a 10-day course of antibiotics IMPACT/DISCUSSION: Legionella bacteria are gram negative organisms found widespread in soil and bodies of water including lakes, streams, and artificial reservoirs. Transmission is via inhalation of aerosols and a high innoculum is typically needed to cause infection. Host risk factors for infection include older age, impaired cellular immunity, smoking, male sex, and medical co-morbidities such as diabetes mellitus, renal, lung and cardiovascular disease. The two most commonly known syndromes associated with Legionella infection are Legionnaire's disease, a pneumonia occurring typically in the late summer or early autumn months (as in our patient), and Pontiac fever, an acute self- limited febrile illness. The mortality rate for hospitalized Legionnaire's is up to 10%. Extra-pulmonary manifestations are rare and can include skin and soft tissue infections, septic arthritis, endocarditis, myocarditis, peritonitis, pyelonephritis, meningitis, brain abscesses, and surgical site infections. The diagnosis of extra-pulmonary disease requires detection of Legionella at the affected site by culture or polymerase chain reaction. In the absence of a known local Legionella outbreak, our patient's age, sex, smoking status, and underlying immune suppression most likely increased his risk for this sporadic infection. We postulate that the acute diarrhea associated with Legionnaire's disease may have triggered inflammation of his diverticula or the acute diverticulitis was an extra-pulmonary manifestation. To our knowledge, we are the first to report a case of Legionnaire's disease presenting as acute diverticulitis. CONCLUSION: Legionnaire's is a typical disease with many atypical and extra-pulmonary presentations. We present a case of Legionnaire's disease masquerading as acute diverticulitis and urge timely consideration and testing for Legionella in at-risk patients presenting with predominantly GI symptoms and subtle or no respiratory complaints, as it can be life-saving.

Postpneumonectomy Empyema Following SARS-CoV-2 Infection in a Patient with Metastatic Lung Cancer

We present a case of a male patient who underwent right-sided pneumonectomy due to central low-grade squamous cell carcinoma of the right lung, chemo-, radio-, and immunotherapy. However, the disease progressed. In addition the patient got SARSCoV-2 pneumonia in the left lung, followed by postpneumonectomy empyema. Right-sided uniportal video-assisted thoracoscopy, debridement, and definitive drainage of the pleural cavity were performed.

Multidrug-resistant Tuberculosis and COVID-19 Coinfection with Diabetes Mellitus in Medan, Indonesia: A Case Report

BACKGROUND: Coronavirus disease 2019 (COVID-19) has distracted the global health system due to significant morbidity and mortality. There are increasing mortality rates related to the existence of comorbidities. Due to immunologic conditions, other infectious diseases, such as multidrug-resistant tuberculosis (MDR-TB), might coinfect with COVID-19. We describe a case of MDR-TB with diabetes mellitus and critical COVID-19 patient with fatal outcome. CASE REPORT: A 60-year-old man was admitted to our hospital with shortness of breath for 2 days. A history of recurrent shortness of breath has had developed for about 7 months. Room air oxygen saturation was at 66%. RT-PCR SARS-CoV-2 nasopharynx swab result was positive. The chest X-ray series result showed destroyed left lung with increasing infiltrate in the lower right lung. The patient was diagnosed with pulmonary MDR-TB based on GeneXpert and LPA (Line Probe Assay) test 6 months prior and also has had history of diabetes mellitus for 7 years. Then, the patient was diagnosed with COVID-19, pulmonary MDR-TB, and diabetes mellitus. MDR-TB regimen, anti-diabetic medication, and management of COVID-19 were carried out. On the 6th day, the patient’s condition worsened to the point, where he needed intubation. The patient eventually passed away. CONCLUSION: The treatment outcome was highly related to the severity of COVID-19 symptoms and complications of comorbidities when patients are admitted to the hospital. The early screening and treatment of COVID-19 are important to prevent deteriorating clinical conditions caused by comorbidities.