Extrathoracic Involvement Research Articles

Comparison of 18F-FDG-PET/CT scans in patients diagnosed with sarcoidosis and tuberculosis

Positron emission tomography/computed tomography (PET/CT) is widely used to differentiate benign and malignant lesions. However, increased fluorodeoxyglucose (FDG) uptake may occur under certain benign conditions, leading to potential false positives in malignancy assessments. Tuberculosis and sarcoidosis are 2 conditions that can exhibit FDG uptake, presenting with both lymph node and extrathoracic involvement, alongside pulmonary manifestations. This study aimed to explore the utility of PET/CT in distinguishing between thoracic and extrathoracic involvement in patients with sarcoidosis and tuberculosis. A retrospective analysis was conducted on patients diagnosed with sarcoidosis or tuberculosis, either pathologically or microbiologically, who underwent 18F-FDG-PET/CT as part of their diagnostic process. This study evaluated demographic data, PET/CT findings, involvement sites, and maximum standardized uptake values (SUVmax) in patients with tuberculosis and sarcoidosis. PET/CT images of 62 patients (44 with tuberculosis and 18 with sarcoidosis) were analyzed. The median patient age was 55 years. Lymph node involvement in the cervical, abdominal, retro-pancreatic, inguinal, and extrathoracic regions was significantly more prevalent in patients with sarcoidosis than in those with tuberculosis (50% vs 20.5%, P = .0031; 27.8% vs 4.5%, P = .018; 22.2% vs 0%, P = .005; 27.8% vs 6.8%, P = .039; and 66.7% vs 27.3%, P = .009, respectively). No statistically significant difference was found in SUV values between patients with tuberculosis and those with sarcoidosis with regard to mediastinal lymph node, extrathoracic lymph node, lung, and bone PET/CT involvement. In conclusion, 18F-FDG-PET/CT imaging does not appear to be a reliable method for differentiating sarcoidosis and tuberculosis from malignant lesions, as it is not feasible to distinguish between sarcoidosis and tuberculosis solely based on 18F-FDG-PET/CT SUVmax values. Nevertheless, the increased prevalence of extrathoracic lymph node involvement in sarcoidosis compared to tuberculosis may offer valuable insights for clinicians in differential diagnosis.

Characteristics of Extra Thoracic Sarcoidosis: A Retrospective Study from Morocco

Background: Sarcoidosis is a multisystem granulomatous disorder with diverse extra thoracic manifestations requiring systematic evaluation. Objectives: To analyse the characteristics and outcomes of extra thoracic manifestations in sarcoidosis patients at Mohamed V Military Hospital, Rabat. Methods: Retrospective study of 54 sarcoidosis patients from January 2021 to January 2024. Clinical, biological, radiological, and therapeutic data were analysed. Results: Extra thoracic involvement occurred in 16 patients (30%), predominantly female (sex ratio 1/3), mean age 41±7 years. Type II radiographic sarcoidosis was most common (50%). Main manifestations were ocular (16.6%), peripheral lymph nodes (9.25%), and cutaneous (7.4%). Biological findings included elevated ACE (20.4%), leukopenia with lymphopenia (7.4%), and hypercalcemia (3.7%), Treatment comprised corticosteroids (75%), immunosuppressants (12.5%), and antimalarial drugs (12.5%). Conclusion: Extra thoracic sarcoidosis presents diverse manifestations requiring vigilant screening. Treatment strategies primarily rely on corticosteroids, with favourable outcomes in most cases.

Prognostic Factors and Clinical Outcomes of Surgical Treatment of Major Thoracic Trauma.

Major thoracic trauma represents a life-threatening condition, requiring a prompt multidisciplinary approach and appropriate pathways for effective recovery. While acute morbidity and mortality are well-known outcomes in thoracic-traumatized patients, long-term quality of life in patients surviving surgical treatment has not been widely investigated before. Between November 2016 and November 2023, thirty-two consecutive patients were operated on because of thoracic trauma. Age, sex, comorbidities, location and extent of thoracic trauma, Injury Severity Score (ISS), Abbreviated Injury Scale (AIS), Organ Injury Scale (OIS), intra and extrathoracic organ involvement, mechanism of injury, type of surgical procedure, postoperative complications, ICU and total length of stay, immediate clinical outcomes and long-term quality of life-by using the EQ-5D-3L scale and Numeric Rate Pain Score (NPRS)-were collected for each patient Results: Results indicated no significant difference in EQOL.5D3L among patients with thoracic trauma based on AIS (p = 0.55), but a significant difference was observed in relation to ISS (p = 0.000011). ISS is correlated with the EQOL.5D3L questionnaire on long-term quality of life, representing the best prognostic factor-in terms of long-term quality of life-in patients surviving major thoracic trauma surgical treatment.

Presentation, diagnosis, management and outcome of complicated hydatid cyst of the lung

Introduction: Introduction: The complicated hydatid cyst of the lung is defined as any cyst ruptured in the bronchi tree or in the pleural cavity, infected, and rarely in the pericardial cavity. The aim of this study was to evaluate the problems of complicated pulmonary hydatid cyst, including presentation, diagnosis choice of surgical methods. and complication of delay in surgical treatment of pulmonary hydatid cysts. Methods: Between 2016 and 2021, 192 operations operated for pulmonary hydatid cysts, 42 of these patients were complicated hydatid cyst. Presentations were cough, chest pain and dyspnea.Diagnosis tools were CXR and CT-scan of chest. surgical approach was a posterolateral thoracotomy and anterolateral thoracotomyin all patients; a phrenotomy in eight patients and one-stage bilateral thoracotomy in six patients. Results: In 18 patients (67.5%), there were single hydatid cysts; whereas 4 patients (32.5%) had multiple cysts.12 patients had bilateral cyst and 8 had both lung and liver and others organs, 6 patients had preoperative hydatid cyst history. Iatrogenic rupture of an intact hydatid cyst occurred in 4 patients. Extrathoracic involvement was apparent in 6 patients (27%). Intrathoracic but extrapulmonary involvement was apparent in 4 patients (16%). Conclusion: Complicated hydatid cyst may present with different clinical and radiologically manifestations as a primary lung tumor , pleural effusion ,empyema hydropneumothorax. In patients with suspicious lung masses in endemic area or history of a hydatid cyst in patients a complicated pulmonary hydatid cyst may be in differential diagnosis.

Prognostic value of hypermetabolic bone sarcoidosis observed by 18F-fluorodeoxyglucose positron emission tomography.

Sarcoidosis is a multisystemic granulomatosis diagnosed mainly in young adults. 18F-fluorodeoxyglucose (18F-FDG) PET/CT is useful in sarcoidosis cases to search for a biopsiable site or assess disease activity.18F-FDG PET/CT can reveal bone hypermetabolism in sarcoidosis patients, even in the absence of osteoarticular symptoms. The aim of this study was to describe metabolic bone involvement in sarcoidosis patients and to evaluate its prognostic impact. This was an observational, comparative, retrospective, monocentric study. Inclusion criteria were a confirmed diagnosis of sarcoidosis according to the World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) criteria and at least one 18F-FDG PET/CT scan during follow-up. Metabolic bone involvement of sarcoidosis was defined as focal bone hypermetabolism with no argument for a differential diagnosis of bone 18F-FDG uptake. Patients with and without bone involvement were compared. Among the 175 included patients, 32 (18%) had metabolic bone involvement of sarcoidosis. The metabolic bone involvement was mainly axial and mostly without bone abnormalities on CT. Metabolic bone involvement was associated with intrathoracic and extrathoracic lymph node involvement and with a greater number of organs involved. Patients with metabolic bone involvement more frequently received corticosteroids, methotrexate and TNF-α inhibitors and a greater number of treatments. Relapse of sarcoidosis occurred sooner in patients with metabolic bone involvement. These results suggest that metabolic bone involvement is associated with more diffuse and more severe sarcoidosis.

Liquid biopsy in clinical outcomes and detection of T790M mutation in metastatic non-small cell lung cancer after progression to EGFR-TKI.

Liquid biopsy (LB) is used to detect epidermal growth factor receptor (EGFR) mutations in non-small cell lung cancer (NSCLC) and has been demonstrated to have prognostic and predictive value. To associate the rates of EGFR and T790M mutations detected by LB during disease progression after first- or second-generation EGFR-TKIs with clinical characteristics and survival outcomes. From January 2018 to December 2021, 295 patients with advanced EGFR mutant (EGFRm) NSCLC treated with first- or second-generation EGFR-TKIs were retrospectively analyzed. LB was collected at the time of progression. The frequency of EGFRT790M mutations, overall survival (OS), and the clinical characteristics associated with LB positivity were determined. The prevalence of EGFRT790M mutation detected using LB was 44%. In patients with negative vs. positive LB, the median OS was 45.0 months vs. 25.0 months (p= 0.0001), respectively. Patients with a T790M mutation receiving osimertinib had a median OS of 44 months (95% CI [33.05-54.99]). Clinical characteristics associated with positive LB at progression extra-thoracic involvement, > 3 metastatic sites, and bone metastases. Our findings showed that LB positivity was associated with worse survival outcomes and specific clinical characteristics. This study also confirmed the feasibility and detection rate of T790M mutation in a Latin American population.

A 10-year experience of hydatid cysts of lung in association with other extrathoracic system involvement in a tertiary care hospital in Kolkata, India

Background: The metacystode of tapeworm Echinococcus causes hydatid disease. Infection in man is mainly caused by Echinococcus granulosus. Surgical intervention to treat complicated hydatid cyst (HC) is different from uncomplicated HC. Aims and Objectives: The present study was performed with an aim to review 82 cases of pulmonary HCs and associated HCs in other locations. We analyze and evaluate our experience in the surgical treatment and complication. Materials and Methods: In a 10-year period, from August 1, 2005, to July 31, 2015; 82 patients were operated on in our hospital for HCs in pulmonary as well as other associated extrapulmonary locations. The diagnosis was established on the basis of clinical features and imaging studies supplemented by serological test. Surgical approach consisted of a posterolateral thoracotomy in all cases of thoracic HCs. For cysts in extrathoracic locations, either laparotomy or local excisions were performed. Results: Radical resections [lobectomy] were done in four cases, and conservative operations in the remaining. Post-operative complications occurred in 12 cases (14.63 %). We had two recurrences, three cases of prolonged air leak with bronchopleural fistula, two had atelectasis, and five wound infections. At 6 months follow-up, 97.56% of our patients were free of any recurrence. Conclusion: HCs of the lung are best treated with surgery. Even when associated with HCs in other locations in the body, surgery is considered the treatment of choice. When supplemented with long-term perioperative Albendazole therapy, the chance of recurrence is practically negligible.

Unusual presentation of lepidic adenocarcinoma in a healthy female

BackgroundLepidic adenocarcinoma represents a histologic pattern of non-small cell lung cancer that characteristically arises in the lung periphery with tracking alongside pre-existing alveolar walls. Noninvasive and invasive variants of lepidic adenocarcinoma are dependent on parenchymal destruction, vascular, or pleural invasion. The lepidic-predominant lung malignancies are collectively recognized as slow growing with rare metastasis and excellent prognosis. The World Health Organization classification of lung malignancies depends on molecular and histopathological findings. CT findings most commonly include ground-glass characteristics, commonly mistaken for inflammatory or infectious etiology. These tumors are generally surgically resectable and associated with better survival given infrequent nodal and extrathoracic involvement. Rarely these tumors present with diffuse pneumonic-type involvement associated with worse outcomes despite lack of nodal and distant metastases.Case presentationA 63-year-old Caucasian athletic immunocompetent female presented with 2 months of progressive shortness of breath, fatigue, loss of appetite and 15 pound weight loss. History was only notable for well controlled essential hypertension and hypothyroidism. Contrast computed tomography angiogram and positron emission tomography revealed diffuse hypermetabolic interstitial and airspace abnormalities of the lungs without lymphadenopathy (or distant involvement) in addition to right hydropneumothorax and left pleural effusion. Baseline laboratory testing was unremarkable, and extensive bacterial and fungal testing returned negative. Bronchoscopy and video-assisted thoracoscopic surgery was subsequently performed with pleural fluid cytology, lung and pleural biopsies returning positive for lepidic adenocarcinoma with 2% programmed death ligand 1 expression and genomic testing positive for PTEN gene deletion. Prior to treatment, the patient perished on day 15 of admission.ConclusionWe present a rare case of lepidic predominant adenocarcinoma with extensive bilateral aerogenous spread in the context of no lymphovascular invasion in a healthy, low risk patient. This case presentation may add to the body of knowledge regarding the different behavior patterns of lepidic predominant adenocarcinomas.

Sarcoidosis

Sarcoidosis is a granulomatous systemic disease of unknown etiology most commonly affecting the lungs and thoracic lymph nodes. The diagnosis is based on typical clinical radiologic appearance and histology with evidence of noncaseating epithelioid cell granulomas without central necrosis. In the acute form, Löfgren's syndrome, histologic confirmation may not be necessary. Approximately half of patients may develop a chronic form, and extrathoracic organ involvement should be investigated during the course. Indications for therapy are based on functional limitations, marked organ-related or systemic symptoms, and life-threatening organ manifestations (cardiac, central nervous system, renal, and ocular sarcoidosis). To date, there is no approved drug therapy for sarcoidosis. Administration of immunosuppressants such as glucocorticosteroids and as add-on or sequential, methotrexate, azathioprine or mycophenolate mofetil is recommended in the currently published international guideline.

Pediatric primary mediastinal lymphoma – a descriptive study of a single cancer center of Bangladesh

Lymphomas involving the mediastinum occur in a wide age range and represent heterogeneous histological subtypes with various clinical symptoms and complex radiological findings. However, this cross sectional study that describes the clinical, pathological and radiological features of Bangladeshi pediatric patients aged less than 18 years. The study conducted in National Institute of Cancer Research and Hospital with 71 primary pediatric mediastinal masses, diagnosed between 2014 and 2018 and evaluated at enrollment or admission in the department of Pediatric Hematology and Oncology (PHO). The presence of extra thoracic lymph node involvement was excluded from the study. All patients were underwent computerized tomography (CT) or ultra sound (USG) guided needle biopsy. Only diagnosed cases of lymphoma (N-38) with mediastinal mass on chest radiography or CT scan were taken for analysis. Mediastinal lymphoma was 38 in number which, 16.30% of total (N-233) pediatric lymphoma of PHO. The median age of the patients was 11.43 years with mostly (68.42%) in 10-17 years age group. Males and females were equal in number. Common symptoms of the patients were fever, cough, dyspnea, chest pain. Most common side involved by tumors were anterior mediastinum 68.42% (N-26) followed by middle mediastinum 50% (N-19), Posterior mediastinum 10.53% (N-4) and superior mediastinum 7.9% (N-3). More than one mediastinal anatomical side involvement was in 34.21% (N-13) cases. Tissue biopsy revealed non Hodgkin Lymphoma were 86.84% (N-33), Hodgkin Diseases 10.53% (N-4) and T-lymphoblastic lymphoma (LBL) 2.63% (N-1). All tumors were malignant with 73.68% metastasis. Pediatric mediastinal mass is a troublesome condition for doctors during emergency management at presentation, for interventional radiologists during tissue biopsy, pathologists during histopathological examination and oncologists during treatment planning. A few publications narrated the pediatric mediastinal oncological conditions but in Bangladesh no such study has conducted before addressing the pediatric mediastinal lymphoma. So we conducted this study to show the importance of development of infrastructure to manage these type of tumors successfully.

CATAMENIAL CONUNDRUM: A CASE OF PULMONARY EMBOLISM IN A PATIENT WITH CATAMENIAL PNEUMOTHORAX

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Endometriosis is a common condition affecting about 10% of reproductive-age women, in which ectopic endometrial tissue is found outside of the uterine cavity [1]. While it most commonly affects the pelvis, rarely endometrial tissue can also be found in the chest, often presenting as catamenial pneumothorax [2]. There have also recently been reports of systemic effects of endometriosis such as supporting a pro-inflammatory and prothrombotic state, increasing the risk for deep vein thrombosis (DVT) and pulmonary embolism (PE) [3]. CASE PRESENTATION: A 32 year old woman with endometriosis presented to the hospital with several weeks of pleuritic chest pain, nausea, vomiting, and inability to tolerate oral intake. Several months prior, she was diagnosed with thoracic endometriosis after a hospitalization for chest pain and discovery of bilateral pneumothoraces and pleural effusions, necessitating bilateral chest tube placement. Her consequent outpatient course was complicated by an unprovoked DVT, for which she was started on apixaban. She was admitted to the hospital the next month for worsening dyspnea and underwent computed tomography angiography of the chest and was found to have a left lower lobe subsegmental pulmonary embolism and loculated bilateral pneumothoraces, leading to video-assisted thoracoscopic surgery with decortication, pleural biopsy, and chemical pleurodesis for catamenial pneumothorax, with improvement in symptoms (Image 1). Anticoagulation (AC) was switched from oral apixaban to enoxaparin injections 1.5mg/kg daily, with a plan for clinic follow up for further AC management. DISCUSSION: Extrathoracic involvement is a rare and challenging presentation of endometriosis, often necessitating invasive interventions, and associated with recurrence rates of up to 14% (4). Our patient presented the challenge of requiring AC for her PE while managing bleeding risk in the setting of endometriosis, all while being unable to tolerate oral medications due to nausea and vomiting associated with endometriosis. In such patients, injectable, quickly reversible options for AC such as enoxaparin should be considered. CONCLUSIONS: There have been several studies suggesting that endometriosis supports a prothrombotic state, making it imperative to remember this dual challenge when treating endometriosis patients, and to have a low threshold for suspecting PE in any patient with persistent chest discomfort even after treatment of catamenial pneumothorax. Management should be individualized, with attention to the effect of related systemic complications such as nausea and vomiting precluding oral medication success to prevent potentially devastating consequences. REFERENCE #1: Shafrir AL, Farland LV, Shah DK, et al. Risk for and consequences of endometriosis: A critical epidemiologic review. Best Pract Res Clin Obstet Gynaecol. 2018;51:1-15. REFERENCE #2: Mecha E, Makunja R, Maoga JB, et al. The Importance of Stromal Endometriosis in Thoracic Endometriosis. Cells. 2021;10(1):180. Published 2021 Jan 18. REFERENCE #3: Ding D, Liu X, Guo SW. Further Evidence for Hypercoagulability in Women With Ovarian Endometriomas. Reprod Sci. 2018;25(11):1540-1548. DISCLOSURES: No relevant relationships by Teressa Ju, source=Web Response No relevant relationships by Gina Villani, source=Web Response No relevant relationships by Angelina Voronina, source=Web Response

Results of Inter-B-NHL Ritux 2010 - Phase II Study of DA-EPOCH-R for Children and Adolescents with Primary Mediastinal Large B-Cell Lymphoma (PMLBL) on Behalf of European Intergroup for Childhood Non Hodgkin's Lymphoma (EICNHL) and Children's Oncology Group (COG)

Abstract Background Dose Adjusted EPOCH with Rituximab (DA-EPOCH-R) has been shown to deliver excellent survival for adults with PMLBL (Dunleavy K et al. NEJM 2013; 368:1408-16). We evaluated this regimen in a prospective international trial of children and adolescents with this disease. Methods The international phase II trial (NCT01516567) sponsored by Gustave Roussy and COG, evaluated the event-free-survival (EFS) following DA-EPOCH-R in patients with PMLBL aged Results Between April 2012 and April 2016, 47 eligible patients (35 from Europe and 12 from COG) were recruited across 7 countries. Twenty-seven were female and the median age was 15 years. Thirty-two percent of patients had lactate dehydrogenase (LDH)> 2xNormal, 66% had mediastinal mass >10cm and 16% were Stage IV by Ann Arbor classification. Pathological review is ongoing (15/47 cases already reviewed). Due to a protocol error 7 patients received only half dose predniso(lo)ne in DA-EPOCH-R and on the recommendation of the Independent Data Monitoring Committee the additional 7 patients were recruited. The primary analysis was restricted to the 40 patients treated according to the full predniso(lo)ne dosing. Sensitivity analyses were conducted on the whole group of 47 patients. The median follow up was 27 months (range 9.9- 53 months) Forty percent of patients (17/47) received a COP prior to the first course of DA-EPOCH-R. The protocol was well tolerated and 47% (22/47) of patients reached dose level 4 or higher. Febrile neutropenia was seen in 11% (30/281) of courses. One patient experienced left ventricular systolic dysfunction (grade 2) at dose level 6. Eighty-five percent (39/46) of patients achieved complete remission (CR) or CR (unconfirmed). There were 13 events (11 relapse/refractory or progression and 2 second malignancies). Isolated CNS relapse occurred in 2 patients. The observed 2-year EFS among the 40 patients who received full dose prednisolone of 69% (95%CI 52%-82%) did not differ from the historical rate (p=0.71). A similar result was seen in the sensitivity analysis of all 47 patients (EFS 72% (95% CI 57%-84%), p=0.68). The 2-year overall survival rate for all 47 patients was 82% (95%CI 67-91%). An analysis of prognostic factors for the whole group showed that the presence of extrathoracic involvement was associated with associated with inferior outcome in multivariate analysis (HR 5.62[1.49-21.2], p=0.011). Conclusion DA-EPOCH-R did not improve the EFS compared to a historical control in this international study of children and adolescents with PMLBL in contrast to the published adult series. Further studies are required to determine the optimum therapy for children and adolescents with this lymphoma. Disclosures Bollard: Cellectis: Membership on an entity's Board of Directors or advisory committees; Neximmune: Consultancy, Membership on an entity's Board of Directors or advisory committees; Torque: Membership on an entity's Board of Directors or advisory committees.

Unsupervised Clustering Reveals Sarcoidosis Phenotypes Marked by a Reduction in Lymphocytes Relate to Increased Inflammatory Activity on 18FDG-PET/CT.

Introduction: Sarcoidosis is a T-helper cell mediated disease characterized by granulomatous inflammation. We posited that unsupervised clustering of various features in sarcoidosis would establish phenotypes associated with inflammatory activity measured by 18FDG-PET/CT. Our goal was to identify unique features capable of distinguishing clusters and subsequently examine the relationship with FDG avidity to substantiate their potential use as markers for sarcoidosis inflammation.Methods: We performed a retrospective study of a diverse, but primarily African American, cohort of 58 subjects with biopsy proven sarcoidosis followed at the University of Illinois Bernie Mac Sarcoidosis Center and Center for Lung Health who underwent 18FDG-PET/CT scan. Demographic, therapeutic, radiographic, and laboratory data were utilized in unsupervised cluster analysis to identify sarcoidosis phenotypes. The association between clusters, their defining features, and quantitative measurements on 18FDG-PET/CT was determined. The relevance of these features as markers of 18FDG-PET/CT inflammatory activity was also investigated.Results: Clustering determined three distinct phenotypes: (1) a predominantly African American cluster with chronic, quiescent disease, (2) a predominantly African American cluster with elevated conventional inflammatory markers, advanced pulmonary disease and extrathoracic involvement, and (3) a predominantly Caucasian cluster characterized by reduced lymphocyte counts and acute disease. In contrast to the chronic quiescent cluster, Clusters 2 and 3 were defined by significantly greater FDG avidity on 18FDG-PET/CT. Despite similarly increased inflammatory activity on 18FDG-PET/CT, Clusters 2, and 3 differed with regards to extrathoracic FDG avidity and circulating lymphocyte profiles, specifically CD4+ T-cells. Notably, absolute lymphocyte counts and CD4+ T-cell counts were found to predict 18FDG-PET/CT inflammatory activity by receiver operating curve analysis with a 69.2 and 73.42% area under the curve, respectively.Conclusions: Utilizing cluster analysis, three distinct phenotypes of sarcoidosis were identified with significant variation in race, disease chronicity, and serologic markers of inflammation. These phenotypes displayed varying levels of circulating inflammatory cells. Additionally, reduction in lymphocytes, specifically CD4+ T-cells, was significantly related to activity on 18FDG-PET/CT. Though future studies are warranted, these findings suggest that peripheral lymphocyte counts may be considered a determinant of sarcoidosis phenotypes and an indicator of active inflammation on 18FDG-PET/CT.

Sarcoidosis in Jordan: A Study of the Clinical Phenotype and Disease Outcome.

This study aims to evaluate the clinical phenotypic features of sarcoidosis in a single-center academic hospital in Jordan. A retrospective file review was performed at an academic medical center in Jordan that included all patients diagnosed with sarcoidosis between January 2000 and December 2018. A total of 150 patients with sarcoidosis (38 males, 112 females; mean age 47.8±11.7 years; range, 17 to 79 years) were evaluated. Clinical data extracted from the files included the sex of the patient, the age at time of diagnosis, diagnosis date, the season during which the diagnosis was established, and smoking history. Biopsy histopathology, spirometry, nerve conduction, echocardiography, and imaging reports including plain radiographs, ultrasonographic, magnetic resonance and computed tomography reports were reviewed. Data including laboratory values, medication usage, clinical outcomes, and morbidity/mortality were collected. Pulmonary function tests including spirometry and lung volumes along with the diffusing capacity for carbon monoxide were reviewed for the presence of restriction, obstruction or reduction in the diffusion capacity of carbon monoxide. Identification of extra-thoracic organ involvement was determined in each patient in accordance with the criteria suggested by the updated World Association of Sarcoidosis and Other Granulomatous Disorders. A total of 77.3% of the patients were diagnosed by biopsy. One case of Lofgren's syndrome was identified. Of the patients, 18.0% had isolated pulmonary sarcoidosis, 75.3% had pulmonary and extra-pulmonary sarcoidosis and 6.7% had isolated extra-pulmonary sarcoidosis while 81.3% had respiratory symptoms, mostly shortness of breath and cough. Extra-thoracic organ involvement mostly involved the musculoskeletal system (33%) followed by the skin (20%). Female patients had more extra-thoracic involvement but the sex difference was only statistically significant for cutaneous involvement. Of the patients, 84% received treatment while 20% had disease remission during the first two years after diagnosis and 70% required treatment beyond two years after diagnosis. Various sarcoidosis clinical phenotypes are seen among Jordanian patients. Jordanian females are more affected by the disease and have more extra-thoracic involvement compared to male patients. A large number of the study patients received treatment.

Clinical Characteristics of Sarcoidosis in Asian Population: A 14-year Single Center Retrospective Cohort Study from Thailand.

Background:Little is known about epidemiology and clinical characteristics of sarcoidosis in Asian population.Objectives:This study aimed to examine the epidemiology and clinical characteristics of Thai patients with sarcoidosis, using databases of a tertiary care medical center.Methods:Potential cases of sarcoidosis were identified from two sources, the medical record-linkage system and the pathology database of Siriraj Hospital, Mahidol University in Bangkok, Thailand. Patients with ICD-10-CM codes for sarcoidosis were identified and retrieved from the medical record-linkage system from 2005 to 2018. Patients with histopathology positive for non-caseating granuloma were identified and retrieved from the pathology database from the same time period. All potential cases underwent individual medical record review to confirm the diagnosis of sarcoidosis which required compatible clinical pictures supported by presence of non-caseating granuloma, radiographic evidence of intrathoracic sarcoidosis and exclusion of other granulomatous diseases.Results:From 2005 to 2018, 89 confirmed cases of sarcoidosis were identified. 80.9% of them were female and mean age at diagnosis was 46.8 years (standard deviation (SD) 13.9 years). The majority of patients had intrathoracic disease (81 cases; 91.0%) but less than half had respiratory symptoms (34 cases; 41.9%). Extrathoracic disease was common in this cohort that pulmonary sarcoidosis was accompanied by extrathoracic involvement in 53 patients (65.4%). Sarcoid uveitis was the most common extrathoracic disease (35 cases; 39.3%), followed by cutaneous sarcoidosis (24 cases; 26.9%), extrathoracic lymphadenopathy (18 cases; 22.5%) and sarcoid arthropathy (4 cases; 4.5%).Conclusion:The current study examined clinical characteristics of sarcoidosis in an Asian population and found high prevalence of uveitis and marked female predominance. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (4): e2020011)

Primary pulmonary extranodal NK/T cell lymphoma of an elderly adult: a case report and literature review

Extranodal natural killer/T-cell lymphoma (ENKTCL), nasal type, is rare and aggressive and often involves the nose, nasopharynx, and upper aerodigestive tract. The non-nasal type can affect the skin, salivary glands, gut, testes, brain, salivary glands, and other sites. Primary ENKTCL of the lung is rare. Here, we report a 68-year-old non-smoking female who presented with fever, dry cough, and night sweats. The chest image showed lung consolidation in the right lower lung field. Pulmonary biopsy showed diffuse abnormal lymphocyte infiltrate in the necrotic exudate. Immunohistochemical data indicated that the tumour cells were positive for CD56, granzyme B, CD3, and TIA. Using in situ hybridization, Epstein–Barr virus-encoded ribonucleic acid (EBER) was detected. There was no evidence to indicate extrathoracic lymphoma involvement. Primary pulmonary ENKTCL was therefore diagnosed. The patient underwent chemotherapy using the P-GEMOX regimen (pegaspargase, gemcitabine, and oxaliplatin) and is still alive.

PULMONARY VEIN THROMBOSIS VISUALIZED BY ENDOBRONCHIAL ULTRASOUND (EBUS): A RARE FINDING

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Endobronchial ultrasound (EBUS) offers a minimally invasive tool to allow for visualization and histopathological diagnosis of mediastinal and hilar lymph nodes or masses. Simultaneously, it allows for visualization of major vasculature surrounding the tracheobronchial tree such as the pulmonary veins and arteries. Limited report exists regarding pulmonary vein thrombosis (PVT) visualization with EBUS with only one case being found after conducting a PubMed search. We are presenting an additional case of PVT incidentally diagnosed by chest computed tomography (CT) and later visualized by EBUS. CASE PRESENTATION: A 66-year-old female with a history of 30 pack-year smoking and COPD stage IVB, was found to have a sub-centimeter left lower lobe (LLL) nodule on lung cancer screening. CT chest with contrast was performed revealing new right and left hilar lymphadenopathy without extra-thoracic involvement. Due to claustrophobia PET scan was not tolerated. Repeat CT with contrast revealed that the presumed left hilar mass was actually an occlusive pulmonary vein thrombosis in the superior pulmonary vein. Few weeks later patient was admitted to the hospital with worsening fatigue and right side pleuritic chest pain. Exam was unremarkable. She was managed with therapeutic anticoagulation and antibiotics for possible infectious thrombosis. EBUS-transbronchial needle aspiration of station R10 revealed small cell lung carcinoma. Incidentally the pulmonary vein thrombosis visualized by EBUS (Figure1&2) and confirmed with Doppler mode was comparable with CT images. Chest CT repeated after 6 weeks showed minimal change in the PVT and the patient was continued on therapeutic enoxaparin without complication. DISCUSSION: PVT is a rare and fatal condition with non-specific presenting symptoms making diagnosis a challenge. There have been several case reports on PE diagnosis by EBUS, but to the best of our knowledge there has been only one other case report of PVT visualized on EBUS. No guidelines exist for the diagnosis of PE and PVT with EBUS and much of literature are the case reports only. We are presenting this case to demonstrate that EBUS could be an option to diagnose or confirm PVT when other modalities are contraindicated (e.g. CT pulmonary angiography in the presence of renal impairment or contrast allergy). Although EBUS visualization of PVT may not be applicable to the general population due to cost and invasiveness it could be considered as an alternative diagnostic modality in the right sub-population. CONCLUSIONS: PVT is a rare condition which could prove fatal if diagnosed late. Due to its nebulous presentation, diagnosis can be challenging. EBUS could be considered for the diagnosis or confirmation of PVT in the right sub-population. Reference #1: MacEachern P, Dang B, Stather D, Tremblay A. Tumor invasion into pulmonary vessels viewed by endobronchial ultrasound. J Bronchol 2008; 15:206–207. Reference #2: Swartz MA, Gillespie CT. Pulmonary emboli detected by endobronchial ultrasound. Am J Respir Crit Care Med 2011; 183:1569. Reference #3: Mohammad R. Al-Ajam et al. Endobronchial Ultrasound. When to Venture into the Vasculature. ll AnnalsATS Issues. Vol. 10, No. 4 | Aug 01, 2013. DISCLOSURES: No relevant relationships by Muhammad Bilal, source=Web Response No relevant relationships by Cynthia Callahan, source=Web Response No relevant relationships by Ameet Kumar, source=Web Response No relevant relationships by Benedicta Nnodum, source=Web Response

A RARE MALIGNANCY

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Ewing's sarcoma is a malignant neoplasm which arises from the skeletal bones. It most commonly affects children and young adults with a female preponderance. Ewing’s sarcoma of the rib is a rare enity as long bones are usually affected . CASE PRESENTATION: A 24 year-old male presented with complaints of shortness of breath , cough and left chest pain for 1 month. He also had episodes of seizures since last 3 years. He had multiple soft cutaneous nodules and macular patches over chest , back and upper limbs. There was bulging of left anterior chest wall with shifting of trachea as well as apex beat to right. There was increased vocal fremitus as well dull percussion note on left. There were reduced breath sounds with increased vocal resonance on left. Routine blood investigations were within normal limits. Chest X -ray revealed a homogeneously white left hemithorax with shifting of mediastinum to the right. Pleural fluid cytology had features suggestive of small round cell tumor. CECT thorax revealed a large heterogeneous mass in the left hemithorax with shifting of mediastinum to right and lytic destruction of left 6 th rib. There was minimal left pleural effusion with nodular pleural deposits. USG guided trucut biopsy from the left lung mass revealed small cell neuroendocrine carcinoma. The tumour was CD 99, Synaptophysin, CD 56 , FLI-1 - positive and negative for CK, CK7, TTF-1 and chromogranin. Dermatology opinion favoured the skin nodules to be neurofibromatosis. So a final diagnosis of Ewing’s sarcoma left sixth rib with Neurofibromatosis and seizure disorder was made. The patient received Vincristine , Adriamycine and Cyclophosphamide (VAC),alternating with Ifosfamide and Etoposide as well as was planned for radiotherapy DISCUSSION: Ewing sarcoma is an uncommon disease, which usually arises from the skeletal bones. It constitutes approximately 10 % of all primary malignant bone tumours. Only 40 % of such cases are seen in flat bones like innominate, ribs, scapula and sterum #1.A characteristic chromosomal translocation, (11; 22) (q24; q12) can be detected in 90% of cases .Patients with Ewing's Sarcoma of the ribs present with breathlessness, weight loss and painful chest wall mass. #2 pleural effusion as in our case, is an unusual finding #3 . The tumour has propensity to spread inwards towards the thoracic cavity and thus,may manifest as an extra pleural mass.our case unique in a wayas it had several other associated finding like neurofibromatosis,seizure disorder as well as skin lesion although such association are not commonly reported CONCLUSIONS: This case showcases the significance of IHC an important tool in diagnosis of lung malignancies and lays stress on excluding extrathoracic involvement in all such cases. Reference #1: MirraJM. Bone tumors: clinical, radiologic, and pathologic correlations. Philadelphia: Lea&Febiger, 1989; 1087-1117. Reference #2: Gladish GW, Sabloff BM, Munden RF, Truong MT, Erasmus JJ, Chasen MH. Primary thoracic sarcomas. Radiographics 2002 May-Jun;22(3):621-637 Reference #3: Wolf G, Aigner RM, Schwarz T. Massive pleural effusion with dyspnea in a 17-year-old boy as the first sign of ewing sarcoma. J Pediatr Hematol Oncol 2002 Jun-Jul;24(5):420 DISCLOSURES: No relevant relationships by UMER AHMED SYED, source=Web Response

Systemic phenotype of sarcoidosis associated with radiological stages. Analysis of 1230 patients

To analyze the association between Scadding radiological stages of sarcoidosis at diagnosis and the disease phenotype (epidemiology, clinical presentation and extrathoracic involvement) in one of the largest cohorts of patients with sarcoidosis reported from southern Europe. The SARCOGEAS-Study Group includes a multicenter database of consecutive patients diagnosed with sarcoidosis according to the WASOG 1999 criteria. Extrathoracic disease at diagnosis was defined according to the 2014 instrument and the clusters proposed by Schupp et al. RESULTS: We analyzed 1230 patients (712 female, mean age 47 yrs.) who showed the following Scadding radiologic stages at diagnosis: stage 0 (n = 98), stage I (n = 395), stage II (n = 500), stage III (n = 195) and stage IV (n = 42). Women were overrepresented in patients presenting with extrathoracic/extrapulmonary disease, while the diagnosis was made at younger ages in patients presenting with BHL, and at older ages in those presenting with pulmonary fibrosis (q values <0.05). Multivariable adjusted analysis showed that patients presenting with pulmonary involvement (especially those with stages II and III) had a lower frequency of concomitant systemic involvement in some specific extrathoracic clusters (cutaneous-adenopathic/musculoskeletal, ENT and neuro-ocular/OCCC) but a higher frequency for others (hepatosplenic), in comparison with patients with extrapulmonary involvement (stages 0 and I). The presence of either BHL or fibrotic lesions did not influence the systemic phenotype of patients with pulmonary involvement. The key determinant associated with a differentiated systemic phenotype of sarcoidosis at diagnosis was interstitial pulmonary involvement rather than the individual Scadding radiological stage.

Osseous sarcoidosis: A multicenter retrospective case-control study of 48 patients.

To describe the clinical presentation, distribution of lesions, treatment, and outcomes of osseous sarcoidosis. A French retrospective multicenter study of patients with biopsy-proven sarcoidosis analyzed patients with 1) a biopsy-proven granuloma without caseous necrosis, and either 2) osseous clinical manifestations, or 3) abnormal osseous imaging. Sarcoidosis patients with osseous involvement (cases) were compared with 264 age- and sex-matched sarcoidosis patients with no osseous manifestations (controls). In the osseous sarcoidosis group (n=88), forty-two (48%) patients had osseous-related symptoms involving the axial (69%) and/or appendicular (58%) skeleton. On imaging, the most commonly affected bones were in the spine (52%), pelvis (42%), hands (22%) and femur (19%). Compared with controls, cases had higher rates of mediastinal (93% vs. 47%) and extra-thoracic lymph node involvement (66% vs. 21%), pulmonary (90% vs. 65%) and cutaneous involvement (44% vs. 23%) (all P<0.0001), and hypercalcemia (8.5% vs. 2%, P=0.014). Spleen/liver and gastrointestinal involvement were less frequent in the osseous sarcoidosis group (29% vs. 45%, and 1% vs. 17%, respectively, P<0.0001). Response rates to with glucocorticoids alone, glucocorticoids plus methotrexate or glucocorticoids plus hydroxychloroquine were 23/44 (52%), 9/13 (69%) and 4/6 (67%), respectively. In patients with osseous sarcoidosis the spine and pelvis were the most commonly affected bones. Compared with controls, cases with osseous sarcoidosis have higher rates of thoracic and extra-thoracic lymph node involvement, pulmonary and cutaneous involvement, and hypercalcemia. Most patients with osseous sarcoidosis had a good response to glucocorticoids in combination with methotrexate or hydroxychloroquine.