Left Lower Lobe Research Articles

Surviving Multiple Cardiac Arrests Secondary to Catastrophic Sero-Negative Antiphospholipid Syndrome: A Case Report

Antiphospholipid syndrome (APS) is an autoimmune disorder which predisposes to venous and arterial thrombosis due to the autoantibodies targeting phospholipids in cell membranes contributing to a pro-thrombotic state. The appropriately named catastrophic anti-phospholipid syndrome (CAPS) is distinguished from classical APS by the rapid involvement of 3+ organs, evidence of areas of small vessel occlusions, and significantly elevated anti-phospholipid titers (Cervera, 2018). To further complicate the matter, there is a growing body of evidence of the existence of sero-negative APS (SNAPS) that are negative for the classical lupus anticoagulant, anticardiolipin antibody (aCL), and anti-beta2 glycoprotein-I but may be positive for non-criteria APS antibodies. Presented below is a patient with SNAPS who responded to the therapy for CAPS despite negative classical autoantibodies. A 34 year old man with a past medical history of chronic alcohol use presented to the ED with a two day history of worsening shortness of breath. The patient was found to have an spO2 of 45-50% on room air and was quickly transitioned to a non-rebreather and subsequently BIPAP. Initial CBC was significant for WBC 22.1, Hg 19.3, Hct 58.5, MCV 100.8, and platelets of 166 consistent with neutrophilic leukocytosis and macrocytic polycythemia. D-dimer >20 was suggestive recent/on-going coagulation and/or fibrinolysis. Due to worsening respiratory failure, he was intubated and admitted to the ICU. While on 3 vasopressors he became bradycardic and a code blue was called for pulseless electrical activity (PEA). After 3 rounds of CPR he achieved return of spontaneous circulation (ROSC) and was taken for urgent CT imaging. After arriving in the radiology department he went into cardiac arrest for two rounds of CPR before achieving ROSC and being scanned and returning to the ICU. Polycythemia tests were drawn which revealed elevated erythropoietin of 52.1 (2.6-18.5) and ultimately negative JAK2. Chest CTA was significant for a PE with a heavy clot burden involving the entire right lung and the left upper and lower lobes. CT C/A/P showed findings of bilateral lower lobe pneumonia, severe gastritis, pancolitis, proctitis, pancreatitis with a 4.6cm pseudocyst, and fatty liver with congestive enhancement. Tissue-type plasminogen activator (tPA) bolus and infusion was initiated based on the imaging findings with the third cardiac arrest occurring 7 minutes later early in the morning. Repeat CBC revealed an Hgb/Hct of 22.2 g/dL and 71.5%, respectively, with a significant drop in platelets down to 103 with occasional large platelets consistent with peripheral destruction of platelets was noted on peripheral blood smear (PBS). Therapeutic phlebotomy of 300mL was subsequently performed with two additional cardiac arrests within 2 hours of each other before vascular surgery was able to perform thrombectomies of the involved pulmonary arteries and initiate catheter-directed thrombolysis. Platelet count dropped while on heparin to 25,000 so a HIT panel was ordered and he was transitioned to argatroban. At this point hematology was consulted who was concerned for CAPS and ordered aPL (anti-phospholipid) tests along with an appropriate panel of labs to rule out alternative causes. Peripheral blood smear did not reveal fragmentation of RBC to suspect TTP. Tests for ACL IgG/IgM and anti-beta2GPI IgG/IgM antibodies, COVID, HIV, ADAMTS deficiency, hereditary thrombophilia, and ANA were negative. High-dose steroids and daily plasma exchanges via vascath was initiated and within 12 hours there was significant improvement. He was extubated and off pressors in 24 hours with improved platelets. Lupus anticoagulant test results were reported after discharge with dVVT mix and PTT LA mix both being prolonged, however, repeat lupus anticoagulant 3 months later was negative. Given the potentially catastrophic effects of a missed CAPS diagnosis, a high level of clinical suspicion is needed as plasma exchange can quickly alleviate an otherwise disastrous condition. Further research into non-criteria APS autoantibodies (IgA)may be able to help guide future SNAPS diagnoses.

Hold your Horses! An Uncommon Cause of Bacteremia in HCT Recipients

Abstract Background Diagnosis and management of bloodstream infections due to uncommon microorganisms in immunocompromised hosts (ICH) are challenging. Removing central venous catheters (CVC) and combination antimicrobial therapy may be necessary. Case A 2-year-old female with Mucopolysaccharidosis type I on day +125 after matched unrelated donor allogenic transplant presented with 2 days of decreased activity. Two days prior, she had completed 4 weeks of ciprofloxacin and clarithromycin for treatment of Mycobacterium cosmeticum catheter-related bacteremia (CRBSI). The CVC was removed. The patient lived in a rural area but had no direct animal exposure, except for an indoor vaccinated dog. Household contacts were healthy and immunized. The patient was febrile and tachycardic with no evidence of infection around CVL or G-tube. Laboratory results showed pancytopenia and elevated C-reactive protein Blood cultures grew diphtheroid gram-positive bacilli (GPB) and gram-positive cocci (GPC) in clusters, the later identified as S. epidermidis. The GPB could not be identified from the liquid media and was sent to an outside lab. The report was corrected to GPC after growing on solid medium culture. The patient was started on meropenem and vancomycin and the CVC was removed. The GPB was identified as Rhodococcus equi. A chest CT showed new ground glass nodules in the left lower lobe. The patient defervesced and was discharged on a 10-day course of oral linezolid for S. epidermidis, clarithromycin and levofloxacin. She completed one month of clarithromycin and levofloxacin. Chest CT at end of treatment confirmed resolution. Discussion and conclusions Rhodococcus equi is a pleomorphic gram-positive bacteria that presents as cocci in solid media and as a diphtheroid gram-positive rod in liquid media. Horses are the primary hosts, but most herbivores are fecal carriers. Transmission occurs through inhalation of dust from contaminated soil. R.equi frequently presents as pulmonary infection with cavitated nodules. Over half of ICH develop secondary bacteremia. Extrapulmonary infection secondary to dissemination of pulmonary disease can occur but isolated extrapulmonary disease is rare. R.equi can be easily dismissed as a contaminant requiring a high level of suspicion and communication with lab personnel. When isolated from an extrapulmonary location, chest imagining is essential to guide treatment. Treatment should combine two antibiotics, at least one of them with intracellular activity, with fluoroquinolones and macrolides among the preferred options. For device-related infections, removal is recommended. Treatment duration for uncomplicated infections is 10 to 14 days, but cavitary lesions require longer treatment based on the resolution of lesions

Pulmonary Vein Occlusion Requiring Lobectomy after Radiofrequency Catheter Ablation for Atrial Fibrillation: A Case Report and Review of the Literature

Pulmonary vein stenosis is a potential complication after radiofrequency ablation for atrial fibrillation. We present an unusual case of this complication that progressed to vein occlusion and required lobectomy and review the literature. A 54-year-old man presented with persistent chest pain, dry cough, and hemoptysis. Seven months before he underwent radiofrequency catheter ablation for atrial fibrillation. Chest computed tomography showed a narrowing of the left lower pulmonary vein after the procedure. The patient was treated conservatively. On the presentation, a chest computed tomography scan showed total pulmonary vein occlusion. A quantitative ventilation/perfusion scan revealed no perfusion to the left lower lobe. A balloon angioplasty was performed, however unsuccessfully. The left lower lobectomy was performed. Six years after the lobectomy the patient has neither cardiac nor pulmonary symptoms. Pulmonary vein occlusion after radiofrequency ablation for atrial fibrillation leading to lung resection is still a possible severe complication.

Thoracoscopic sublobar resection for congenital bronchial atresia in adults: a report of three cases

BackgroundCongenital bronchial atresia (CBA) is a rare bronchial abnormality. Adult patients with CBA generally do not show any symptoms. Although surgical resection is the first-line treatment, the indication for the surgical intervention is controversial, especially in mildly symptomatic or asymptomatic patients. Recently, thoracoscopic surgery is the commonly preferred approach to treat this condition.Case presentationCase 1: A woman in her 20s suffered from repeating episodes of fever and cough due to left CBA with the interruption of B9 and B10. Uniportal thoracoscopic left lower lobe wedge resection was performed, following which the symptoms disappeared. Case 2: A woman in her 40s presented to the clinic with dyspnea on exertion, and the computed tomography image revealed the absence of B2. Multiportal thoracoscopic right S2 segmentectomy was performed on her, after which the symptoms of dyspnea improved. Case 3: A woman in her 40s visited the hospital with symptoms of intermittent cough, sputum, and slight fever for 7 months. The examinations identified the absence of B6a. Right S6 segmentectomy and S2 wedge resection were carried out by multiportal thoracoscopic approach. The patient is now asymptomatic.ConclusionsBased on our experiences in the three adult CBA surgery cases, the three-dimensional image reconstruction system is very useful for diagnosis and surgical decisions with regard to this disease. Even for CBA patients with subtle symptoms, early surgical treatments may be beneficial from the point of view of minimally invasive interventions, and sublobar resection will be sufficient for treatments and preservation of respiratory function.

Abstract 18707: Concomitant Occurrence of Takotsubo Cardiomyopathy and Posterior Reversible Encephalopathy Syndrome: An Uncommon Encounter With Shared Pathophysiology

CASE PRESENTATION: A 61-year-old African American woman presented in an unconscious state after a seizure. She was febrile, tachycardic, tachypneic alongside elevated lactate, leukocyte counts, and procalcitonin levels. Initial high-sensitivity troponin I was notably raised at 4,217 ng/l, with ekg revealing a new onset left bundle branch block (LBBB). Patient was intubated and needed pressor support. Echocardiogram revealed hypokinesis in the basal-mid left ventricle, with a significantly decreased ejection fraction (EF) of 40%. Chest imaging showed a consolidation in the left lower lobe, and Hemophilus influenzae was identified in the sputum culture. A subsequent lumbar puncture yielded elevated leukocytes and proteins, suggesting a secondary meningitic process. DISCUSSION: She was started on antibiotic therapy for pneumonia and meningitis. LV hypokinesis was thought to be due to Takotsubo cardiomyopathy (TTC) or myocardial infarction. Although uncommon LBBB can be seen in TTC. Her clinical presentation, echocardiogram findings further points her LBBB and troponinemia towards TTC than Myocardial infarction. Patient was managed conservatively. Neuroimaging later on revealed restricted diffusion in bilateral occipital and parietal lobes, consistent with Posterior Reversible Encephalopathy Syndrome (PRES). A delayed coronary angiogram after clinical stabilization ruled out obstructive atherosclerotic disease. Weakness and ataxia improved over the course. EF at discharge was 80%.This case illustrates the possibility of concomitant TTS and PRES, conditions hypothesized to arise following intense physical or emotional stress, in this case, likely triggered by pneumonia and meningitis. This clinical scenario underscores the importance of awareness and understanding of these two entities and their potential for simultaneous presentation, providing valuable insights for clinical decision-making in such complex cases.

Unusual Finding in a Patient of COVID-19 Associated Pulmonary Aspergillosis: A Case Report

Invasive Fungal Infections have posed a difficult challenge in the recovery of people infected with COVID-19. COVID-19-associated pulmonary aspergillosis (CAPA) has been described and found in about 30 % of ICU patients worldwide. Here we present an unusual microscopic finding in a case of CAPA in an ICU patient which was helpful in initiating early definite therapy. A 56-year-old gentleman presented with complaints of fever and shortness of breath and subsequently tested positive for COVID. Post admission, his respiratory distress worsened and his condition deteriorated. A provisional diagnosis of COVID pneumonia with acute respiratory distress syndrome (ARDS) was established based on chest radiographic finding of left lower lobe consolidation, increased pulmonary infiltrates in bilateral lung fields with evidence of pleural effusion. Pleural Aspirate obtained via ultrasound guided thoracocentesis revealed branched hyaline septate hyphae along with structures which were composed of elongated vesicle with one layer of phialides covering two-thirds of the vesicle and bearing globose conidia on KOH direct mount. Aspergillus flavus grew on culture, which was later confirmed by MALDI TOF VITEK MS. Patient was diagnosed with Proven Invasive Pulmonary Aspergillosis with COVID-19 and voriconazole was started. Patient successfully recovered and was discharged. Identifying the Aspergillus species directly on the basis of KOH Mount, helped in decreasing the turnaround time, in early initiation of definite therapy and possibly contributed to the favorable outcome. CAPA is a potentially life-threatening complication in patients with severe COVID-19, thus a timely diagnosis and treatment becomes crucial in the management. Keywords: CAPA, COVID-19, Aspergillus

Alveolar Adenoma: A 20-Year Experience at a Western Balkan University Hospital and a Literature Review.

Alveolar adenoma is a rare benign lung tumor. We report 5 patients within a 20-year period diagnosed with this rare tumor and its clinical and histopathological features, in order to emphasize its significance in differential diagnosis of other lung tumors. Alveolar adenoma was more frequently diagnosed in female patients (80%). The patients ranged in age from 52 to 68. Eighty percent of the patients had symptoms like dyspnea, and shoulder or back pain. Three patients underwent a video-assisted thoracoscopic surgery, while 2 patients underwent a thoracotomy in order to remove the tumor. The tumor was localized within the right upper lobe, right lower lobe, and left lower lobe in 40%, 40%, and 20% of patients, respectively. The tumor size ranged from 14 to 30 mm. A frozen section finding of all 5 tumors reported that the lesion was benign. In all analyzed tumors, immunohistochemical analysis showed positive expression of TTF-1 and pankeratin in surface epithelial cells, and Vimentin positivity and TTF-1 and pankeratin negativity in round stromal cells. Alveolar adenoma should be carefully considered in the differential diagnosis of solitary pulmonary nodules. Complete surgical removal of the lesion is widely used in its primary management.

Pathologically confirmed spontaneous regression of small cell lung cancer after computed tomography-guided percutaneous transthoracic needle biopsy followed by surgery

BackgroundSpontaneous regression of malignant tumors is a rare phenomenon, especially in primary lung cancer. The underlying mechanisms remain unclear, but they may often involve immunological mechanisms.Case presentationIn January 2020, a 78-year-old female underwent examination during follow-up of interstitial pneumonia. Chest X-ray and computed tomography (CT) scan revealed a 1.2 × 1.2 cm nodule in the left lower lobe. Based on CT-guided percutaneous transthoracic needle biopsy (PTNB), it was diagnosed as small cell lung cancer (SCLC). Immunohistochemical staining showed that tumor cells were positive for CD56, synaptophysin, and chromogranin A. Twenty-three days after the CT-guided PTNB, repeat CT scan showed that the tumor size regressed to 0.6 × 0.6 cm. The tumor showed positive uptake in fluorodeoxyglucose (FDG) positron emission tomography (PET)–CT. The maximum standardized uptake value of the nodule was 2.24. PET–CT and enhanced magnetic resonance imaging of the brain showed no distant or lymph node metastasis. The patient’s preoperative disease was diagnosed as cT1aN0M0, stageIA1, SCLC. In March 2020, she underwent left lower lobectomy and mediastinal lymph node dissection. Pathological examination of the resected specimen showed that the small tumor cells were dense with a high nucleus to cytoplasm ratio, and the morphological diagnosis was small cell carcinoma. The resected tumor size regressed to 0.05 × 0.02 cm, and no lymph node metastasis was observed. Because it was extremely small, immunohistochemical staining could not be conducted. Active fibrosis and inflammation were present around the tumor. Finally, the patient was pathologically diagnosed as SCLC pT1miN0M0, stage IA1. The patient is alive without recurrence 23 months after surgery with no adjuvant therapy.ConclusionsWe present a rare surgical case of pathologically confirmed spontaneous regression of SCLC after CT-guided PTNB. Although spontaneous regression is extremely rare, we should recognize this phenomenon.

Dysanapsis is not associated with exertional dyspnoea in healthy male and female never-smokers aged 40 years and older.

In healthy adults, airway-to-lung (i.e., dysanapsis) ratio is lower and dyspnoea during exercise at a given minute ventilation (V̇E) is higher in females than in males. We investigated the relationship between dysanapsis and sex on exertional dyspnoea in healthy adults. We hypothesized that females would have a smaller airway-to-lung ratio than males and that exertional dyspnoea would be associated with airway-to-lung ratio in males and females. We analyzed data from n=100 healthy never-smokers aged≥40 years enrolled in the Canadian Cohort Obstructive Lung Disease (CanCOLD) study who underwent pulmonary function testing, a chest computed tomography scan, and cardiopulmonary exercise testing. The luminal area of the trachea, right main bronchus, left main bronchus, right upper lobe, bronchus intermedius, left upper lobe, and left lower lobe were 22%-37% smaller (all p<0.001) and the airway-to-lung ratio (i.e., average large conducting airway diameter relative to total lung capacity) was lower in females than in males (0.609±0.070vs. 0.674±0.082; p<0.001). During exercise, there was a significant effect of V̇E, sex, and their interaction on dyspnoea (all p<0.05), indicating that dyspnoea increased as a function of V̇E to a greater extent in females than in males. However, after adjusting for age and total lung capacity, there were no significant associations between airway-to-lung ratio and measures of exertional dyspnoea, regardless of sex (all r<0.34; all p>0.05). Our findings suggest that sex differences in airway size do not contribute to sex differences in exertional dyspnoea.

Clinical and Genetic Characterization of Patients with Primary Ciliary Dyskinesia in Southwest Saudi Arabia: A Cross Sectional Study.

Primary ciliary dyskinesia (PCD, MIM 244400) is an inherited ciliopathy disorder characterized by recurrent sinopulmonary infections, subfertility, and laterality defects. The true incidence of PCD in Saudi Arabia is not known, but it is likely underdiagnosed due to the high prevalence of consanguineous marriages. In this study, we aim to study the clinical and genetic characteristics of PCD patients in the southwestern region of Saudi Arabia to provide guidance to clinicians and researchers studying PCD. This was a cross-sectional study conducted between 2019 and 2023 in Abha Maternity and Children's Hospital. Twenty-eight patients with clinically diagnosed PCD were recruited. The diagnosis of PCD was confirmed via whole-exome sequencing. A total of 28 patients from 20 families were identified and recruited for this study. The median age of patients was 7.5 years (IQR = 3, 13 years). The people of different sexes were evenly distributed, and 18 patients (64%) had neonatal respiratory distress (NRD). The median age of diagnosis was 5.5 years (IQR = 2, 11 years), while the age when the first symptoms appeared was 3 months old (IQR = 1, 6 months). The prevalence of a chronic wet cough, chronic rhinosinusitis, ear infections were 100% (n = 28), 78.6% (n = 22), and 67.9% (19), respectively. The most common gene in our study was DNAH5, which represented 17.9% (five out of twenty-eight) of the cases. Furthermore, the remaining pathogenic variants included: 14.3% with RSPH9 in four individuals (three families), 14.3% with DNAI2 in four individuals (two families), and 10.7% with LRRC56 in three individuals (one family). The most common findings on the chest CT scans were consolidation (seen in all patients), mucus plugging (seen in 95%), and bronchiectasis (seen in 77%). In the patients with bronchiectasis, the most commonly affected lobes were the right lower lobe (88%) and left lower lobe (76%). The patients with PCD and situs inversus were more likely to experience NRD than the patients with PCD and situs solitus. The median PICADAR score in the patients with PCD and situs inversus (median: 11.5; Q1: 10-Q3: 12.5) was significantly higher compared to those with PCD and situs solitus (median: 7.5; Q1: 5.8-Q3: 8) (U = 10.5; p < 0.001). This study provides preliminary data on the clinical and genetic characteristics of PCD patients in the southwestern region of Saudi Arabia. We found that DNAH5 and RSPH9 genes were the most common genes among the studied population. Furthermore, PCD should be considered for each child with early NRD and laterality defects, and further confirmatory tests are recommended. These findings also highlight the need for greater awareness of the disease in daily clinical practice to facilitate early diagnosis and avoid irreversible lung damage.

SAT535 ANKRD26-RET Fusion Positive Metastatic Papillary Thyroid Carcinoma - Advanced Presentation of a Rare Mutation

Abstract Disclosure: H. Hashmi: None. A. Ankireddypalli: None. L. Burmeister: None. A. Nelson: None. K. Amin: None. Background: While rearranged during transfection (RET) protooncogene mutations have been commonly associated with thyroid malignancy, ankyrin repeat domain containing protein 26 (ANKRD26) has been primarily linked to hematologic malignancy. Exon 29 of ANKRD26 (Chromosome [Chr] 10p12.1) has been shown to fuse with exon 12 of RET (Chr 10q11.21), resulting in a genetic fusion mutation ANKRD26:RET. This is a rare genetic mutation in papillary thyroid cancer with only one previously reported case. We report the first case in the US of this novel genetic mutation with a unique clinical presentation. Clinical Presentation: A 48-year-old, male patient presented with lower back pain radiating to the lower extremities of one year duration. MRI lumbar spine revealed an irregular mass lesion in the L1 vertebral body with extraosseous soft tissue extension, L1 compression fracture and extension into the T12 body. CT CAP revealed a 4.8 x 3.6 cm, heterogenous right thyroid lobe with tracheal compression and sub centimeter pulmonary nodules in the left lower and right middle lobes. Biopsy of the L1 paravertebral mass was diagnostic for metastatic thyroid carcinoma of a follicular pattern. Molecular testing with a next generation sequencing fusion profile assay was positive for ANKRD26:RET gene rearrangement. Thyroid ultrasound was notable for a solid, hypoechoic, 3.2 x 3.1 x 2.9 cm right lobe nodule with echogenic foci and level 4 lymph nodes with loss of reniform shape and fatty hilum. FNA of the nodule and right level 4 lymph node was suspicious for follicular neoplasm. The patient was admitted for acute cord compression necessitating extensive laminectomy, vertebral reconstruction and resection of the L1 paraspinal tumor. Pathology was positive for metastatic, follicular variant, papillary thyroid carcinoma. Total thyroidectomy, central and right modified radical neck dissection was performed. Pathology demonstrated a 4.2 cm papillary carcinoma, follicular type with tall cell variant; confined to the thyroid. Extensive angioinvasion (≥ 4 vessels) and lymphatic invasion was present. 10/32 positive lymph nodes. TNM grading was T3aN1bM1. Summary: The only other case of ANKRD26-RET fusion positive papillary thyroid cancer in the literature was reported by Staubitz et al in Germany in 2019. They reported a case of a 78-year-old male with follicular variant papillary thyroid carcinoma (pT3,pN0,M0). To our knowledge, our case is the second worldwide and the first to be reported in the United States of this rare fusion protein positive thyroid cancer. Such an aggressive presentation with distant osseous metastases has to our knowledge not been previously described. Conclusion: The clinical and therapeutic implications of this rare genetic mutation are not currently well elucidated. We present this case with the intent to expand the existing literature and increase recognition of this rare genetic mutation. Presentation Date: Saturday, June 17, 2023

SAT560 A Case of Medullary Thyroid Carcinoma with Increasing Carcinoembryonic Antigen and Stable Calcitonin Levels

Abstract Disclosure: K.N. Youssef: None. L. Salej: None. A. Gavrila-Filip: None. Background: Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor arising from the parafollicular or C cells of the thyroid gland that secretes different tumor markers, including calcitonin (CT) and carcinoembryonic antigen (CEA). CT is only produced by parafollicular cells, while CEA is a non-specific marker produced in various normal tissues, such as epithelial cells of the gastrointestinal tract, lung, breast, and prostate. Different benign and malignant conditions are associated with elevated serum CEA levels. Clinical case: A 69 years-old active smoker male with a history of nasal squamous cell carcinoma (SCC) status post resection in the remote past and bilateral pulmonary nodules underwent total thyroidectomy for a thyroid nodule with FNA positive for medullary carcinoma. Surgical pathology revealed a unifocal 1.5 cm medullary carcinoma with clean surgical margins and no lymphadenopathy. Pre-operative studies included an elevated serum CT of 388 pg/mL (normal ≤ 10 pg/mL), normal serum calcium and PTH levels and a normal 24-hour urinary test for pheochromocytoma. Family history was irrelevant, and the RET proto-oncogene test was negative. Initial post-operative serum CT levels were undetectable and CEA levels were normal (0-4 ng/mL). CT levels increased slowly over the next five years and then they remained stable in the range of 32 to 45 pg/mL. Serum CEA started to rise progressively 7 years after the thyroid surgery up to 6.5 pg/mL. Follow-up neck ultrasounds showed no local recurrence or lymphadenopathy. An extensive evaluation was performed to search for possible MTC metastases and other etiologies for the elevated CEA levels. Bone scan was negative. Chest CT scans revealed enlarging small bilateral pulmonary nodules; stable calcified mediastinal lymph nodes were noted, consistent with chronic granulomatous disease. PET-CT scan showed pulmonary nodules, too small to characterize. The patient underwent a left lower pulmonary lobe robotic-assisted wedge resection. Surgical pathology revealed keratinizing SCC, though to represent primary lung SCC versus metastases from his prior nasal SCC. Upper endoscopy and colonoscopy with biopsy were negative for malignancy. On further workup, the patient was diagnosed with prostate adenocarcinoma and underwent surgery followed by external beam radiation with hormonal therapy. Serum CEA levels normalized one year after the prostate cancer treatment. Serum CT levels remained stable. Conclusion: In patients with MTC who show increasing serum CEA and stable CT levels during follow-up after the initial treatment, it is important to exclude other etiologies before concluding that this change is related to a progression of the medullary cancer. Reference: Carcinoembryonic Antigen. Kankanala VL, Mukkamalla SKR.2022 Jan 26. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing. Presentation Date: Saturday, June 17, 2023

THU012 Incidental Pituitary Adenoma In A Patient With Ectopic ACTH Secreting Cushing Syndrome - A Diagnostic Conundrum

Abstract Disclosure: M.S. Nali: None. R.R. Yatavelli: None. Background: Cushing’s syndrome (CS) is a rare condition with an estimated incidence of 2-3 cases/million. Endogenous CS can be divided into ACTH -dependent (80%) and ACTH Independent (20%) with Ectopic Cushing’s syndrome (ECS) accounting for 10-20% of all cases of ACTH-dependent CS. Herein we present a case with indolent onset ACTH-dependent CS who on initial work-up showed a pituitary microadenoma raising the suspicion for Cushing disease, but the source of excess ACTH was found to be from a pulmonary neuroendocrine tumor. Clinical Case: A 44-yr-old female with history of type 1 DM, Obesity, BMI 43.60, Sleep apnea was evaluated elsewhere for suspected Cushing’s due to increased insulin requirements and progressive weight gain. Outside labs showed cortisol of 4.5 ug/dl on 1 mg DST &amp; 8 mg DST cortisol was 3 ug/dl. MRI of the pituitary showed a 3.5x 2.8x 1.8 mm microadenoma &amp; she was initially presumed to have Cushing’s disease secondary to pituitary microadenoma. The patient was seen by neurosurgery who referred to endocrine for localization of cortisol excess by IPSS. Clinically she had moon facies, hirsutism, dorsocervical fat pad, violaceous striae on abdomen. On further laboratory evaluation her ACTH: 88 (0-46) and random cortisol 14 ug/dl with normal rest of pituitary hormones. Her 24-hour urine free Cortisol was 45ug/d (3.5 - 45) and late-night salivary cortisol: 74 &amp;&amp;lt;50 (Ref. range &amp;lt;100 ng/dl) on couple of nights, respectively. Due to non-suppressed cortisol on high dose DST, a CT chest was obtained which showed a 2cms non calcified nodule in the left lower lobe of lung. Transbronchial biopsy was performed, cytopathology showed well differentiated neuroendocrine tumor, likely carcinoid and stained strongly positive for ACTH confirming ectopic ACTH producing tumor. Gallium-68 Dotatate Scan showed abnormal increased uptake in the known left lung nodule. The patient was recommended for surgical removal of neuroendocrine tumor, but she could not tolerate anesthesia and had to undergo stereotactic body radiation therapy (SBRT) for the lung lesion. Repeat ACTH levels 2 months post SBRT showed the ACTH levels were elevated and lesion was still persistent on imaging, however on discussion with rad oncology, the neuroendocrine tumors tend to take longer to show response on imaging and plan is to repeat imaging and biochemical work up in couple of months. Conclusion: The diagnosis of CS and recognizing the source of hormonal excess poses significant challenges Pituitary adenomas are incidentally discovered in up to 10% of the normal population &amp; IPSS is advised for patients with ACTH-dependent Cushing's syndrome and an adenoma smaller than 6 mm, however in patients with equivocal findings, it is important to look for other sources of ACTH prior to any invasive procedure. The source of ectopic ACTH secretion should be established after diagnosis because the excision of an ACTH-producing tumor can be curative. Presentation: Thursday, June 15, 2023

SAT174 Doege-Potter Syndrome In A Patient With Solitary Fibrous Tumor Of The Lung

Abstract Disclosure: N. Mon: None. S.S. Krishnasamy: None. Doege-Potter Syndrome is a non-islet cell tumor hypoglycemia (NICTH) due to a solitary fibrous tumor (SFT) with overexpression of Insulin-like growth factor 2 (IGF2). We report a case of refractory hypoglycemia in a patient with SFT of the lung. Case presentation: A 67-year-old homeless male with hypertension, chronic smoker presented with altered mental status after his blood glucose was found 34mg/dl by EMS. His confusion improved after D50W. He could not recall the event but he reported that he missed his dinner prior to the event. He denied taking any antidiabetic medications. He was diagnosed with left lung solitary fibrous tumor without metastases a week ago after he presented to ER with dyspnea, cough and wheezing. CT chest reported a 21x15.6x18.7 cm lesion with mass effect on the left hemidiaphragm, mediastinum shift to the right and occluded left lower lobe bronchi. Core needle biopsy of the mass revealed SFT with necrosis. He was discharged with Medrol dosepak 4mg daily for presumptive treatment of COPD exacerbation and a plan for elective surgery. While in the ER his BG improved to 136mg/dl after D50W. Few hours later BG dropped to 42mg/dl and D10W infusion was started. Physical exam was unremarkable except bilateral pedal edema. Work up showed BG 42mg/dl, Insulin 3.3 (2.6-24.9 uIU/mL), C peptide 0.39 (1.1-4.4ng/mL), Proinsulin 4.7 (&amp;lt;18.8pmol/L), beta-hydroxybutyrate &amp;lt;0.05 (0.02-0.27mmol/l), 8AM cortisol 10.7 (7.0-25.0 ug/dl), albumin 3.2mg/dl, HbA1c 5.8%, IGF1 53ng/mL (34-245ng/mL), IGF2 203ng/mL (267-616ng/mL) with a IGF2:IGF1 ratio of 3.8. In our patient, the IGF2:IGF1 ratio was not &amp;gt;10 since his IGF 2 was suppressed by the recent steroid use. The rest of the clinical and biochemical features strongly suggested NICTH. His preoperative evaluation showed new onset HFrEF with volume overload. He underwent staged percutaneous coronary interventions with stents placement. He was given hydrocortisone 50mg q6H then to q8H during hospitalization and discharged home with hydrocortisone 40mg TID. He underwent radical resection of lung tumor after optimization of his cardiac condition. He was on stress dose of steroid during surgery. Since he was on high dose steroid for &amp;gt; 3months, hydrocortisone dose was tapered slowly after the surgery. Final biopsy reported solitary fibrous tumor extending to parenchymal staple line. Tumor cells are positive for CD34 and STAT6, and negative for desmin, SMA, and SOX10. He recovered uneventfully and was discharged home with prednisone 5mg daily and follow up in endocrine clinic. Conclusion: NICTH is a rare paraneoplastic syndrome characterized by low levels of insulin, C-peptide, proinsulin, and beta-hydroxybutyrate. A ratio of IGF-2/IGF-1 more than 10 confirms NICTH; however, normal ratio cannot exclude the diagnosis and factors such as low IGFBP 3 or steroid therapy for other conditions need to be considered. Surgery is the mainstay of treatment. Presentation: Saturday, June 17, 2023

Embolization via the brachial artery of an anomalous systemic artery supplying the left lower lung lobe: a case description.

Embolization via the brachial artery of an anomalous systemic artery supplying the left lower lung lobe: a case description.

Surgeon’s negligence. Descendent aorta blunt injury while dividing the left-lower lobe bronchus during left-lower lobectomy.

Surgeon’s negligence. Descendent aorta blunt injury while dividing the left-lower lobe bronchus during left-lower lobectomy.

Deep-Inspiration Breath-Hold Stereotactic Body Radiation Therapy by Combining Spirometer-Guided Breath-Hold and a Real-Time Tumor Tracking System: A Novel Approach

There are several methods used against respiratory motion (RM). Expiratory breath-hold (BH) is considered more stable and reproducible than inspiratory BH; therefore, BH with spirometry is often used for expiration. The real-time tumor tracking radiotherapy (RTRT) system is a highly effective method for reducing the margin of RM. This system ambushes and irradiates tumors during the expiratory phase when tumors move slowly. Although these methods usually involve expiration, it is advantageous to expand the lungs with inspiration to reduce the risk of adverse events. Here, we developed a new approach of performing stereotactic body radiation therapy (SBRT) under deep-inspiration BH (DIBH) by combining these two methods. Lung tumors with respiratory motion ≥ 1 cm were included. Three or four fiducial markers were placed near the tumor via bronchoscopy. DIBH CT (CT-IN) was performed under the guidance of spirometer. The PTV was obtained by adding a 5-mm margin to the GTV delineated on CT-IN. The prescribed dose was 42 Gy in four fractions for the D95 of the PTV. An error of 2.0 mm around the planned position of the fiducial marker on CT-IN was permitted along each orthogonal axis as a gating box. In preparation for cases in which the reproducibility of DIBH is low and treatment cannot be performed, light expiration BH CT (CT-EX) was also performed, and a radiotherapy plan was prepared for the conventional RTRT system so that it could be switched at any time. Lung volumes and doses (mean dose, V20 Gy, V10 Gy, and V5 Gy) on CT-EX and CT-IN were compared. Five patients underwent SBRT with DIBH, and all completed the planned irradiation course. The median treatment time per fraction was 27.86 min (range, 25.5-40.6). Four tumors were located in the left lower lobe and one in the right lower lobe. The median volume of PTV was 12.4 (range, 5.2-26.2) mL. The lung volumes and doses on CT-EX and CT-IN are shown in the Table. The lung volume on CT-IN was 1.6 times larger than that on CT-EX. The PTV-to-lung ratio on CT-IN was significantly lower than that on CT-EX. V20 Gy and V10 Gy on CT-IN were significantly lower than those on CT-EX. SBRT with DIBH was achieved by combining the spirometer and RTRT system. This can help to eliminate concerns about reproducibility and high-speed tumor movement during inspiration, which are weaknesses of spirometer-guided breath-hold and the RTRT system, respectively, while ensuring the accuracy of the RTRT system. DIBH SBRT is a promising method that can reduce lung dose.

Carrier State of Chronic Granulomatous Disease Presented at Middle Age: A Case Report

Introduction: Chronic granulomatous disease (CGD) is the most common inherited defect of phagocytes. Although most female carriers of X-linked CGD have been considered to be unaffected, they may have similar problems to those of CGD patients. This study suggests that the CGD carrier state might be more complex than it was previously appreciated. Case Presentation: A sixty-year-old woman visited our hospital in June 2021 due to pneumonia and pleural effusion. Chest computed tomography scan revealed left lower lobe pneumonia and mediastinal lymphadenopathies. In July 2021, her symptoms (high-grade fever, chills, and hemoptysis) were initially attributed to a hydatid cyst. Therefore, she underwent a lobectomy, resulting in purulent drainage on the excision site on the skin, refractory to local and systemic treatments. The refractory and recurrent nature of the lesions led to the immunological evaluation, which was completely normal with a 100% nitroblue tetrazolium (NBT) and a dihydrorhodamine (DHR) of 278 (normal &gt; 100). Further DHR123 flow cytometry investigations with multiple stimulants revealed a carrier state of CGD, which was relevant to her history of chronic complications. Conclusions: Adult patients with unusual manifestations suggestive of neutrophil function defects in adulthood should be evaluated for the CGD carrier state. In these cases, NBT alone may miss the diagnosis of CGD. In such cases, DHR testing with multiple stimulants may establish a robust diagnosis.

Endotracheal tube, by the venturi effect, reduces the efficacy of increasing inlet pressure in improving pendelluft.

In mechanically ventilated severe acute respiratory distress syndrome patients, spontaneous inspiratory effort generates more negative pressure in the dorsal lung than in the ventral lung. The airflow caused by this pressure difference is called pendelluft, which is a possible mechanisms of patient self-inflicted lung injury. This study aimed to use computer simulation to understand how the endotracheal tube and insufficient ventilatory support contribute to pendelluft. We established two models. In the invasive model, an endotracheal tube was connected to the tracheobronchial tree with 34 outlets grouped into six locations: the right and left upper, lower, and middle lobes. In the non-invasive model, the upper airway, including the glottis, was connected to the tracheobronchial tree. To recreate the inspiratory effort of acute respiratory distress syndrome patients, the lower lobe pressure was set at -13 cmH2O, while the upper and middle lobe pressure was set at -6.4 cmH2O. The inlet pressure was set from 10 to 30 cmH2O to recreate ventilatory support. Using the finite volume method, the total flow rates through each model and toward each lobe were calculated. The invasive model had half the total flow rate of the non-invasive model (1.92 L/s versus 3.73 L/s under 10 cmH2O, respectively). More pendelluft (gas flow into the model from the outlets) was observed in the invasive model than in the non-invasive model. The inlet pressure increase from 10 to 30 cmH2O decreased pendelluft by 11% and 29% in the invasive and non-invasive models, respectively. In the invasive model, a faster jet flowed from the tip of the endotracheal tube toward the lower lobes, consequently entraining gas from the upper and middle lobes. Increasing ventilatory support intensifies the jet from the endotracheal tube, causing a venturi effect at the bifurcation in the tracheobronchial tree. Clinically acceptable ventilatory support cannot completely prevent pendelluft.

Living-donor lobar lung transplantation

Living-donor lobar lung transplantation (LDLLT) is indicated for critically ill patients who would not survive the waiting period in the case of severe brain-dead donor shortage. It is essential to confirm that potential donors are willing to donate without applying psychological pressure from others. In standard LDLLT, the right and left lower lobes donated by 2 healthy donors are implanted into the recipient under cardiopulmonary support. LDLLT can be applied to various lung diseases including restrictive, obstructive, infectious, and vascular lung diseases in both adult and pediatric patients if size matching is acceptable. Functional size matching by measuring donor pulmonary function and anatomical size matching by 3-dimensional computed tomography volumetry are very useful. When 2 donors with ideal size matching are not available, various transplant procedures, such as single lobe, segmental, recipient lobe-sparing, and inverted lobar transplants are valuable options. There seems to be immunological advantages in LDLLT as compared to cadaveric lung transplantation (CLT). Unilateral chronic allograft dysfunction is a unique manifestation after bilateral LDLLT, which may contribute to better prognosis. The growth of adult lung graft implanted into growing pediatric recipients is suggested by radiologic evaluation. Although only 2 lobes are implanted, postoperative pulmonary function is equivalent between LDLLT and CLT. The long-term outcome after LDLLT is similar to or better than that after CLT. The author has performed 164 LDLLTs resulting in 71.6% survival rate at 10years. All living-donors returned to their previous life styles. Because of possible serious morbidity in donors, LDLLT should be applied only for critically ill patients.