Intrathoracic Malignancies Research Articles

Yield and Complications of Endobronchial Ultrasound Using the Expect Endobronchial Ultrasound Needle

Background: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) stands as the gold standard for sampling the mediastinum and possesses the capability to detect a diverse range of disease processes. The EBUS needle industry has been experiencing rapid advancement, characterized by numerous companies either enhancing existing needles or introducing innovative ones. The majority of EBUS studies to date have predominantly utilized the OlympusTM Vizishot needles, which are constructed from stainless steel. In this paper, we focus on the evaluation of a cobalt chromium needle, namely the ExpectTM EBUS needle, with a specific emphasis on its diagnostic efficacy and any associated complications. It is important to note that our investigation is conducted independently, and we do not provide a comparative analysis with other needle types available in the market. Methods: This is an institutional review board-approved retrospective analysis of all patients who have undergone an EBUS-TBNA lymph node sampling using the ExpectTM needle between August 2016 and September 2017 at the IU Health University Hospital. Comparisons of clinical characteristics by complications, diagnosis, needle gauge, and lymph node size were performed using chi-square test and Fisher’s exact test. Results: 75% of the 102 included patients had their procedures done with the 22-gauge needle which were majorly performed in the setting of suspected intrathoracic malignancy followed by sarcoidosis and lymphoma. 99% of the patients had no complications after their procedures which were almost all diagnostic with two cases of bronchoscope damage. Mutational analysis was successful with both the 22 and 25-Gauge needles. Conclusion: In this paper, we demonstrate that the ExpectTM 22 and 25-gauge needles are safe and effective when used for EBUS-TBNAs through the OlympusTM EBUS bronchoscope for the evaluation of intrathoracic lymphadenopathy.

Persistent Left Superior Vena Cava: A Case Report and Review of the Literature

The persistence of the left superior vena cava (LSVC) is a rare and benign congenital malformation. It is often asymptomatic, and its discovery is usually incidental in the majority of cases. This venous malformation was identified incidentally in a 60-year-old woman during thoracic multi-detector computed tomography (MDCT), which was performed with the suspicion of intra-thoracic malignancy. This is associated with abnormal venous return and an atrial septal communication.

Recommendations for Post-Operative RadioTherapy After Complete Resection of Thymoma—a French DELPHI Consensus Initiative

IntroductionThymomas are rare intrathoracic malignancies that can relapse after surgery. Whether or not Post-Operative RadioTherapy (PORT) should be delivered after surgery remains a major issue. RADIORYTHMIC is an ongoing, multicenter, randomized phase 3 trial addressing this question in patients with completely R0 resected Masaoka-Koga stage IIb/III thymoma. Experts in the field met to develop recommendations for PORT. MethodsA scientific committee from the RYTHMIC network identified key issues regarding the modalities of PORT in completely resected thymoma. A DELPHI method was used to question 24 national experts, with 115 questions regarding the following: (1) imaging techniques, (2) clinical target volume (CTV) and margins, (3) dose constraints to organs at risk, (4) dose and fractionation, and (5) follow-up and records. Consensus was defined when opinions reached more than or equal to 80% agreement. ResultsWe established the following recommendations: preoperative contrast-enhanced computed tomography (CT) scan is recommended (94% agreement); optimization of radiation delivery includes either a four-dimensional CT-based planning (82% agreement), a breath-holding inspiration breath-hold–based planning, or daily control CT imaging (81% agreement); imaging fusion based on cardiovascular structures of preoperative and planning CT scan is recommended (82% agreement); right coronary and left anterior descending coronary arteries should be delineated as cardiac substructures (88% agreement); rotational RCMI/volumetric modulated arc therapy is recommended (88% agreement); total dose is 50 Gy (81% agreement) with 1.8 to 2 Gy per fraction (94% agreement); cardiac evaluation and follow-up for patients with history of cardiovascular disease are recommended (88% agreement) with electrocardiogram and evaluation of left ventricular ejection fraction at 5 years and 10 years. ConclusionThis is the first consensus for PORT in thymoma. Implementation will help to harmonize practices.

COVID-19 vaccination outcomes in patients with a solid malignancy: Insights from extensive real-world data and propensity score matched analyses

This nationwide, multicentric, retrospective analysis of 1,126,946 COVID-19 cases (March 2020-June 2022) aims to elucidate the impact of COVID-19 vaccination on mortality in patients with a sole solid malignancy. Using data from the national digital medical record repository, outcomes were compared among fully vaccinated and nonvaccinated cohorts, factoring in gender, virus type, age, vaccination status, vaccine type, and cancer type. Logistic regression calculated odds ratios (OR) and their significance. Among 6,050 patients with both cancer and comorbidities, 1,797 had only solid malignancy. Vaccinated individuals in this group had reduced mortality rates, especially those >63 years (OR 0.169 [95% confidence interval [CI] 0.090-0.317]; P<.001). Lower deaths were observed in non-ICU (OR 0.193 [95% CI 0.097-0.382]; P<.001) and ICU cases (OR 0.224 [95% CI 0.077-0.646]; P=.003), with both vaccine types. No statistically significant benefits were observed against delta and omicron variants. Intrathoracic malignancies (OR 0.376 [95% CI 0.146-0.971]; P=.043) and palliative treatment (OR 0.384 [95% CI 0.192-0.766]; P=.006) showed vaccination benefits. Logistic regression revealed a higher fatal risk in nonvaccinated males >63. Propensity score matching supported these outcomes. Patients with sole solid malignancies face elevated COVID-19 mortality risk, particularly without active cytostatic therapy, with advanced disease on palliative treatment, or intrathoracic malignancies.

Cancer and Atrial Fibrillation Comorbidities Among 25 Million Citizens in Shanghai, China: Medical Insurance Database Study.

With population aging, the prevalence of both cancer and atrial fibrillation (AF) have increased. However, there is scarce epidemiological data concerning the comorbid state of cancer and AF in low- and middle-income countries, including China. We aimed to evaluate the site-, sex-, and age-specific profiles of cancer and AF comorbidities in Chinese populations. Data from the Shanghai Municipal Health Commission database between 2015 and 2020 were screened, covering all medical records of Shanghai residents with medical insurance. Site-specific cancer profiles were evaluated for the population with AF relative to the age- and sex-adjusted population of residents without AF. The sex distribution and peak age of cancer diagnosis were also assessed. A total of 25,964,447 adult patients were screened. Among them, 22,185 patients presented cancers comorbid with AF (median 77, IQR 67-82 years of age; men: n=13,631, 61.44%), while 839,864 presented cancers without AF (median 67, IQR 57-72 years of age; men: n=419,020, 49.89%), thus yielding a higher cancer prevalence among residents with AF (8.27%) than among those without AF (6.05%; P<.001). In the population with AF, the most prevalent cancer type was lung cancer, followed by colorectal, male genital organ, stomach, breast, liver, bladder, thyroid, leukemia, and esophageal cancers. AF was associated with an average of nearly 1.4-fold (prevalence ratio [PR] 1.37, 95% CI 1.35-1.38) increased prevalence of cancer after adjusting for age and sex. For site-specific analyses, an increased prevalence of cancer in the population with AF was observed in 20 of 21 cancer sites. This increased prevalence was most prominent for nonsolid tumors, including multiple myeloma (PR 2.56, 95% CI 2.28-2.87), leukemia (PR 1.73, 95% CI 1.57-1.90), and non-Hodgkin lymphoma (PR 1.59, 95% CI 1.43-1.77); intrathoracic malignancies, including mediastinum (PR 2.34, 95% CI 1.89-2.90), lung (PR 1.64, 95% CI 1.59-1.69), and esophageal cancers (PR 1.41, 95% CI 1.28-1.56); bone and soft tissue neoplasms (PR 1.56, 95% CI 1.37-1.77); and kidney cancer (PR 1.53, 95% CI 1.36-1.72). Cancer prevalence in the population with AF relative to that in the population without AF was higher in men than in women in 14 of 18 cancer sites, and female predominance was only observed for thyroid cancer. The peak age of index cancer diagnosis was lower in the population with AF (age group: 70-74 years) than in that without AF (age group: 75-79 years), especially for specific cancer types, including thyroid, central nervous system, mediastinum, esophageal, bladder, and biliary cancers. Patients with AF are associated with increased prevalence, heightened male predominance, and younger peak age of cancer. Further studies are needed to determine whether early screening of specific cancers is cost-effective and beneficial for patients with AF.

Malignant Pleural Mesothelioma and Radiotherapy: Lung Toxicity Results of an Interim Analysis in Prospective Pilot Trial

Malignant Pleural Mesothelioma (MPM) is a rare but aggressive intrathoracic malignancy with an overall poor prognosis. Neoadjuvant chemotherapy, surgery and then radiotherapy is the standard of care in early-stage disease. Our study aim is to evaluate the feasibility and toxicity of the novel adjuvant RT in accelerated hypofractionation. We report the lung toxicity preliminary results of the first patients entreated. Starting in 2017, 29 MPM patients were enrolled in the trial (IRST trial 163). All patients were treated with accelerated hypofractionated radiotherapy using a helical 3-D CRT and IMRT system and intensity-modulated arc therapy. We conducted a prospective mono-institutional clinical trial enrolling cyto-histological proven, MPM patients. The major exclusion criteria were: previous thorax radiotherapy, contralateral mediastinum involvement (N3) and/or M1, interstitial pneumopathy, active pneumonitis, and fissural disease. The prescription dose was 30 Gy in five daily fractions, while an inhomogeneous dose escalation to 40 Gy was prescribed based solely upon the presence of gross residual tumor. All patients were treated in a 3-D CRT and IMRT system. Patients underwent functional lung study before to start radiotherapy treatment, 2 months and 6 months after the end of the treatment. The organs at risk dose-volume histograms were converted to a 2-Gy equivalent dose, and we closely adhered to the dose constraints of the literature data. We in particular analyzed lung toxicity of the first 20 patients. No G3/G4 lung toxicity was found. We reported 65.0% G1 pneumonitis and 10% G2 as acute lung toxicity. The majority of the G1 patients had only mild symptoms and pneumonitis was described only for radiologic features with no need for medical therapy. Other respiratory toxicities were G1-G2 cough in 50% of the patients; G1 dyspnea occurred in 65% of the patients and G2 in the 15%. The total lung mean dose was 18 Gy (range 13 Gy-23 Gy), and the contralateral lung mean dose was 2 Gy (range 1 Gy-2 Gy). The median value of Forced Expired Volume in one second (FEV1) recorded was 75.5% (range: 46%-137%) before the treatment was started, the median value of Forced Vital Capacity (FVC) at baseline was 74 (range 46-123) with progressive decreasing values through time. DLCO has also been reported with a progressive decrease over time after radiotherapy treatment. No case of respiratory failure was reported after treatment. Treatment of the intact lung with pleural intensity-modulated arc irradiation is a novel treatment strategy that appears to be safe, feasible, and without a high grade of lung toxicity. More investigations are mandatory. The protocol is ongoing.

Cytological, cell block and immunohistochemical analysis of spectrum of malignant pleural effusion with special reference to non-small cell lung carcinoma: A hospital based study of North East India

Malignant pleural effusions (MPE) can result from primary malignancies of the pleura or from underlying intrathoracic or extrathoracic malignancies that reach the pleural space by hematogenous, lymphatic, or contiguous spread. Conventional smear and cell block (CB) technique is one of the oldest methods for the evaluation of body cavity fluids. The principal advantage of cell block over conventional smears are an additional yield of malignant cells and obtaining multiple sections for ancillary tests such as immunohistochemistry (IHC). This study is a hospital-based cross-sectional study which was conducted for a period of 14 months from June 2021 to July 2022 in a tertiary care centre of North East India. Among 277 pleural effusion specimens 11.91% cases were positive for malignancy.Lung carcinoma was the most common cause of malignant pleural effusion. Non-small cell lung carcinoma comprised majority of lung carcinoma cases. Adenocarcinoma was the most common subtype of NSCLC with female preponderance. Cytological diagnosis had a Sensitivity of 76.19%, specificity of 100% andAccuracy of 96.5%. Immunohistochemistry of lung carcinoma in cell block preparation revealed 77.77% cases of adenocarcinoma of lung were positive for TTF-1(P&amp;#60;0.018). Immuno-expression of P63 and P40 showed positive expression in both the cases of squamous cell carcinoma. Conventional smear and cell block method with application of immunohistochemistry will help us in accurate diagnosis of primary site of the tumour aiding early treatment especially in resources restricted hospitals.

Unusual Spread of Extranodal Malignant Lymphoma to the Lung and Nodal to the Mediastinum; How do we know?

Background:Lymphoma is a malignancy that occurs primarily in the lymph nodes. Lymphoma divided into 2 major parts, namely: Non-Hodgkins Lymphoma (LNH) and Hodgkins Lymphoma (LH). About 85% of these malignancies are LNH. Epidemiological studies have found that LH is rare in children under 5 years of age and relatively rare in adults, but is the most common cancer among children aged 15-19 years. Computed Tomography Scan(CT-Scan) is the most frequently used examination, because it is effective for diagnosing and showing the stage of Malignant Lymphoma. Lymphoma intrathoracic malignancy usually occurs with a non-specific clinical picture, very often misdiagnosed in some cases in the field. Method: This study is a descriptive type with a retrospective approach. In 33 patients who underwent thoracic CT scan with contrast which had histopathological proven Malignant Lymphoma. Assess the characteristics of Malignant Lymphoma based on gender, age, intrathoracic location (lungs and mediastinum) and characteristics that will appear on the chest CT scan. Results: Based on the study, a total sample of 30 patients was obtained, with 23 samples of mediastinal malignant lymphoma patients, and 10 samples of patients with lung malignant lymphoma. The characteristics of the mass in lung lymphoma with spiculated edges, tend to be in the superior lobe, with the most component of the lesion being solid with necrosis with contrast, an increase in attenuation of >24 HU. There are other dominant characteristics, namely the presence of lymph node enlargement elsewhere, presence of nodules in the lungs, pleural effusion and consolidation. In malignant mediastinal lymphoma, the characteristic mass itself is lobulated, with a predominance of a solid component within it and is usually located in the anteromedius mediastinum. Conclusion:Lung Malignant Lymphoma Patients are predominantly aged 60-69 years and Malignant Lymphoma in the mediastinum is 20-29 years. With 70% of mediastinal malignant lymphoma samples. In the lungs, the dominant lesion is in the form of a mass with an atelectatic component and most of them also describe multiple nodules in the lung with the dominant lesion in the superior lobe, other features: the presence of multiple lymph nodes elsewhere and pleural effusion. In the mediastinum: the most common location is in the anteromedius mediastinum with the most common lesion in the form of a mass, the presence of nodules in the lungs is also found in mediastinal malignant lymphoma.

Intraoperative near-infrared molecular imaging for diagnostic thoracoscopy in difficult clinical scenario.

Precise diagnosis in intrathoracic malignancies is paramount for adequate treatment planning. Standard approach is histologic analysis from targeted biopsy obtained with different invasive procedures. Rarely, in difficult clinical scenarios, even gold standard diagnostic procedures can be ineffective in obtaining a satisfying result. Procedural developments and technological improvements applied to the chosen technique can be helpful to deal with such situation. We present two clinical cases of suspected intrathoracic malignancy in which repeated unsuccessful diagnostic procedures had already been attempted. We adopted a protocol based on intraoperative fluorescence during diagnostic thoracoscopy to increase diagnostic efficacy. In both cases we obtained a precise pathological diagnosis.

Left brachiocephalic vein–right atrial bypass in superior vena cava syndrome

Superior vena cava syndrome (SVCS) is a condition with total or partial stenosis in the superior vena cava (SVC) as a result of intrathoracic malignancies or microtrauma caused by central venous catheterisation. Various invasive and surgical interventions are performed to provide venous drainage in these patients whose head and neck venous blood flow is impaired. In this case report, we report on a bypass performed with a synthetic graft between the left brachiocephalic vein and the right atrium in a patient with SVCS.

Respiratory Emergency in Hospitalized patient with Intrathoracic Malignancy at H. Adam Malik General Hospital

Background: Increased number of intrathoracic malignancy cases (Lung tumor, Mediastinum tumor, Secondary lung cancer and Chest wall tumor) contribute in increased complications of respiratory emergencies such as superior cava vein yyndrome (scvs), massive malignant pleura effusion (mpe), central airway obstruction (cao), massive hemoptysis, lung thromboembolism and pneumothorax. This study aims to look at the proportions, outcomes, and factors that influence respiratory emergency in intrathoracic malignancy cases.Methods: This was a retrospective case series of inpatient respiratory emergencies in patients intrathoracic malignancy at H. Adam Malik Central General Hospital - Medan from 1 May 2011 to 30 April 2016.Results: From 690 subjects intrathoracic malignancy, there were 137 (19.8%) patients with respiratory emergencies and mostly caused by lung tumors (83.9%). Massive MPE was is the most common emergency of 45 cases (6.5%) and massive haemoptysis of at least 4 cases (0.6%). The risk of death was higher in patients with respiratory emergencies. Mediastinum and location of tumor contribute in respiratory emergency appearance with respective OR of 3.9 and 1.5 (p value &lt;0.005)Conclusions: Increased of mortality rate in patients with respiratory emergency and MPE massive is the most cases. Type of the malignancy and Right lung tumor contribute of respiratory emergency in intrathoracic malignancy cases.

Treatment strategies for thymic carcinoma in a real-life setting. Insights from the RYTHMIC network

BackgroundThymic carcinomas are aggressive and difficult to treat a subset of thymic epithelial tumours that represent a heterogeneous group of rare intrathoracic malignancies. The treatment strategy of thymic carcinomas is based on whether surgical resection may be achieved, which represents the most significant favourable prognostic factor on survival. For this study, we took advantage of the unique prospective Réseau tumeurs THYMiques et Cancer (RYTHMIC) database to describe baseline characteristics, analyse treatment strategies in light of existing guidelines and provide landmark patient outcomes data with regards to response and survival of patients in a real-life clinical practice setting. MethodsInclusion criteria for this analysis were the following: (1) histologically-confirmed thymic carcinomas – excluding neuroendocrine tumours-after pathological review by the RYTHMIC pathology panel, (2) discussion of the case at the RYTHMIC multidisciplinary tumour board, (3) at least one active treatment modality. ResultsA total of 213 patients were analysed. Overall, 60 (28%) patients were considered as surgical candidates upfront, 91 (43%) patients received primary chemotherapy, and 62 (29%) patients received exclusive chemotherapy. Median overall survival (OS) was 49.2 months (IC95%: 34.8–63.6); OS was significantly longer in patients with a lower stage at diagnosis (p < 0.001), who were operated on upfront, as opposed to patients who received primary or exclusive chemotherapy (p < 0.001). Surgery, conducted upfront or after primary chemotherapy, was significantly associated with more prolonged OS (p < 0.001); complete resection and postoperative radiotherapy were also predictors of better outcome (p = 0.018 and p = 0.051, respectively). ConclusionsOur cohort is the first to analyse in-depth outcomes and treatment strategies in a prospective cohort of consecutive patients with thymic carcinoma. While we confirm the major prognostic impact of surgery, our data highlight the need for optimised multidisciplinary management and innovative therapies as the survival of patients remains limited.

COVID-19-Vaccination Prioritization in Patients with Cancer

In patients with cancer, SARS-CoV-2 (COVID-19) can contribute to increasing morbidity and mortality and decreased survival was found in patients with hematological and intrathoracic malignancies, poor performance status, comorbidities, and increased age. Patients with hematological malignancies who were treated with stem cell transplantation and anti-CD-20 antibody demonstrated lower rates of seroconversion, compared to COVID-19-infected-cancer patients. Patients with hematological malignancies might have substantially compromised B-cell and T-cell responses.

Pleuropulmonary Blastoma (PPB) in Child with DICER1 Mutation: The First Case Report in the State of Qatar

Pleuropulmonary blastoma (PPB) is a rare intrathoracic malignancy, which arises from the lung parenchyma and/or pleura. PPB has strong genetic association with mutations in DICER1 gene. Despite being rare, PPB is the most common lung tumor in children below 6 years of age. International registry of the disease has a total of 350 cases worldwide. We report the first case of PPB in the state of Qatar, which presented as a large cystic lung lesion. The patient was first thought to have benign congenital pulmonary airway malformation (CPAM) based on chest X-ray findings. The diagnosis of PPB was suspected based on chest CT scan findings and was confirmed after surgical resection of the cystic mass. The case highlights the need to consider PPB in the differential diagnosis of cystic lung lesions in children and the need for further radiological imaging (i.e., CT scan), genetic testing, and/or excisional biopsy to confirm the diagnosis.

Prioritization of Covid-19 Vaccination in Patients with Cancer

In patients with cancer, SARS-CoV-2 (COVID-19) can contribute to increasing morbidity and mortality and decreased survival was found in patients with hematological and intrathoracic malignancies, poor performance status, comorbidities, and increased age. Patients with hematological malignancies who were treated with stem cell transplantation and anti-CD-20 antibody demonstrated lower rates of seroconversion, compared to COVID-19-infected-cancer patients. Patients with hematological malignancies might have substantially compromised B-cell and T-cell responses.

Early In Vivo Detection of Radiation-Induced Cardiotoxicity With Hyperpolarized C-13 Pyruvate Magnetic Resonance Spectroscopy

Radiation therapy is commonly used for treatment of patients with intrathoracic malignancies, such as lung cancer, esophageal cancer, and lymphoma. Radiation-Induced Heart Disease (RIHD) is a major source of morbidity and mortality in patients receiving thoracic radiation. Therefore, there is an acute need for development of non-invasive approaches for detection of RIHD at a stage that offers potential for early intervention and reversibility. Cardiac mitochondrial dysfunction is a hallmark of radiation-induced cardiac injury. During aerobic respiration, pyruvate enters the tricarboxylic acid cycle and is metabolized into bicarbonate in mitochondria. We hypothesized that radiation-induced mitochondrial dysfunction results in decreased conversion of pyruvate to bicarbonate in the mitochondria and increased conversion to lactate in cytosol. We sought to non-invasively assess radiation-induced changes in mitochondrial myocardial metabolism by tracking the fate of hyperpolarized (HP) C-13 pyruvate utilization using Magnetic Resonance Spectroscopy (MRS).Sprague-Dawley rats (n = 10) underwent baseline echocardiography and HP C-13 pyruvate MRS. Rats in the cardiac radiation group (n = 5) underwent image-guided cardiac radiation with cone-beam CT, to a total dose of 40 Gy in 5 fractions. All rats underwent repeat echocardiography and hyperpolarized C-13 pyruvate MRS one week later.For the first time, we have demonstrated feasibility of employing HP C-13 pyruvate MRS for detecting radiation-induced myocardial mitochondrial metabolic changes in a pre-clinical rat model. In the cardiac radiation group, C-13 pyruvate MRS demonstrated a statistically significant decrease in cardiac bicarbonate-to-lactate ratio compared to pre-radiation baseline (P = 0.02, one-tailed paired t-test), suggesting increased metabolism of pyruvate into lactate in the cytoplasm (at the expense of metabolism into bicarbonate in the mitochondria) due to mitochondrial dysfunction. No significant decrease in this ratio was observed in the non-irradiated, age matched controls (P = 0.90). No significant changes in left ventricular ejection fraction or global longitudinal strain were observed in either the cardiac irradiation or control group of rats at this time point.Radiation-induced myocardial mitochondrial dysfunction is an early event and can be detected in vivo by hyperpolarized C-13 pyruvate MRS within 1 week after radiation, and prior to onset of echocardiographic changes. Due to its non-invasive nature, this technology has the potential to serve as a platform for building radiation-focused cardio-oncology programs for early detection and mitigation of radiation-induced cardiac injury in hundreds of thousands of patients receiving thoracic radiation annually.

P62.09 A Prospective Cohort Evaluation of the Sensitivity and Specificity of the Chest X-Ray for the Detection of Lung Cancer in Symptomatic Adults

The chest radiograph (CXR) plays a pivotal role in the triage of symptomatic individuals with potential lung malignancy. In the United Kingdom the National Institute of Clinical Excellence recommends CXR as an initial investigation in those aged over 40 with specified symptoms or in the presence of one symptom and smoking history. Urgent specialist evaluation is advised if the CXR demonstrates an abnormality suggestive of malignancy. The evidence regarding the effectiveness of the CXR in this role is incomplete and based upon the retrospective evaluation of populations identified with lung cancer. The design of these studies prevents the evaluation of correctly excluded cases and an estimation of the test specificity. This prospective cohort study aimed to establish the accuracy of the CXR in the investigation of malignancy amongst symptomatic adults over the age of 50 for intrathoracic malignancies Secondary aims were to evaluate how a history of smoking alters the test characteristics, the role of CXR follow-up, and whether the reported presence of abnormalities for which no follow-up is advised predict the presence of malignancy. The study population was a cohort of consecutive “self-request” CXR studies prospectively collated between January 2011 and October 2016 as part of the National Awareness and Early Diagnosis Initiative. The initiative combined an early lung cancer diagnosis awareness campaign with a policy of open access to a CXR. Individuals were able to obtain a CXR providing they met eligibility criteria. The criteria were symptoms persisting for at least 3 weeks (cough, fatigue, shortness of breath, chest pain, loss of appetite or loss of weight) and age over 50. Patient’s self-reported paper questionnaires collected during the study period formed the cohort for this study, with patient subsequently cross referenced with the regional cancer registry to identify intrathoracic malignancies. The CXR report was retrieved and coded using a novel definition of a positive test as a CXR resulting in investigation with CT. Studies were positive if the report documented an abnormality with a recommendation which directly (or indirectly after a CXR or clinical follow-up) led to investigation with CT. Studies which did not result in investigation with CT were considered negative. 8,948 CXR outcomes were evaluated. 496 positive studies led to a diagnosis of 80 patients with Non-Small Cell Lung Cancer (NSCLC) amongst 101 primary intrathoracic malignancies. Within two-years, a cumulative total of 133 NSCLC amongst 168 primary intrathoracic malignancies were observed. The sensitivity and specificity for NSCLC were 76% (95%CI 68-84) and 95% (95%CI 95-96) within 1-year and 60% (95%CI 52-69) and 95% (95%CI 95-96) within 2-years. The 2-yr positive and negative likelihood ratios were 12.8 and 0.4. The results did not differ for NSCLC compared to all primary malignancies. A positive test strongly increases the probability of malignancy whereas a negative test moderately reduces the risk. A quarter of NSCLC will have a negative CXR in the year prior to diagnosis. The findings allow the risk of malignancy following a negative test to be estimated.

A CASE REPORT OF HYPERTROPHIC OSTEOARTHROPATHY LEADING TO DIAGNOSIS OF PULMONARY EPITHELIOID HEMANGIOENDOTHELIOMA

TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: Hypertrophic Osteoarthropathy (HOA) is a condition characterized by abnormal proliferation of skin and osseous structures of extremities. Periostitis is the imaging hallmark of HOA and most commonly manifests symmetrically along the shaft of tubular bones. Incidence of HOA in patients with intrathoracic malignancy is 5-10%. CASE PRESENTATION: A 49-year-old male presented to his primary care physician for bilateral knee pain with associated swelling. After failing conservative management, bilateral knee x-rays were performed which revealed a thin periosteal reaction in both femoral shafts, prepatellar soft tissue swelling and small bilateral joint effusions.The radiologist recommended a chest x-ray for further evaluation, which showed subtle soft tissue fullness at the right lung base. Chest CT was then performed revealing a 4.2 x 2.2 x 4.7 cm spiculated soft tissue lung mass of the right lower lobe with extension into the mediastinum and right hilar, paratracheal and subcarinal lymph nodes.The patient underwent bronchoscopy with FNA of the right lower lobe mass and station 7 and 11 lymph nodes. Highly atypical cells suspicious for malignancy were noted, but analysis was limited by scant material. IR guided biopsy was subsequently performed revealing patchy proliferation of epithelioid cells with ample eosinophilic cytoplasm, occasional cytoplasmic vacuoles and necrosis. Immunohistochemically, the tumor cells stained positive for ERG, CD31, and CD24. The cytomorphologic and immunohistochemical features support the diagnosis of epithelioid hemangioendothelioma (EHE). DISCUSSION: This case illustrates EHE's heterogeneous presentations. The primary lesion of EHE has been documented in numerous locations throughout the body, but most commonly arises in the liver, lungs, and bones. HOA as the presenting sign in pulmonary EHE is not well documented in the literature and periosteal reaction is considered rare. Digital clubbing was not present in this case and the only known location of periosteal reaction was the femoral shafts. While the mechanism of EHE is not well understood, it is believed that angiogenic stimulators, such as vascular endothelial growth factor (VEGF), may act as promoters of endothelial cell proliferation.The proposed pathogenesis of HOA has been linked to hypersecretion of vasoactive agents such as VEGF, platelet derived growth factor (PDGF), and prostaglandin E2 (PGE2). The proposed pathogenesis of secondary HOA has been linked to hypersecretion of vasoactive agents such as VEGF, PDGF, and PGE2. These agents are released both systemically in response to hypoxemia and by the tumor itself. CONCLUSIONS: Pulmonary Epithelioid Hemangioendothelioma can also present with Hypertrophic Osteoarthropathy.Bone X- ray can lead to the diagnosis of Hypertrophic Osteoarthropathy if careful evaluation is done. Periostitis is frequently a sign of pulmonary and systemic pathology. REFERENCE #1: Chakraborty, Rebanta K. "Secondary Hypertrophic Osteoarthropathy." StatPearls [Internet]., U.S. National Library of Medicine, 1 Mar. 2021, www.ncbi.nlm.nih.gov/books/NBK513342/. REFERENCE #2: Sardaro, Angela, et al. "Epithelioid Hemangioendothelioma: an Overview and Update on a Rare Vascular Tumor." Oncology Reviews, PAGEPress Publications, Pavia, Italy, 13 Oct. 2014, www.ncbi.nlm.nih.gov/pmc/articles/PMC4419652/. REFERENCE #3: Silveira LH;Martínez-Lavín M;Pineda C;Fonseca MC;Navarro C;Nava A; "Vascular Endothelial Growth Factor and Hypertrophic Osteoarthropathy." Clinical and Experimental Rheumatology, U.S. National Library of Medicine, pubmed.ncbi.nlm.nih.gov/10728444/. DISCLOSURES: No relevant relationships by Julia Lawinger, source=Web Response No relevant relationships by Raul Mendoza-Ayala, source=Web Response

PRIMARY INTRATHORACIC SYNOVIAL SARCOMA

TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: Synovial sarcomas (SS) are a rare group of malignancies, seen in 0.17 per 100,000 persons, accounting for 7% of all soft tissue sarcomas. These malignant tumors most commonly present as a tumor of the extremities. While they have a high propensity for metastasis to bone and lung, primary intrathoracic malignancy is rare. We present a case of a patient who presented with acute onset shortness of breath, diagnosed with primary synovial sarcoma of the lung. CASE PRESENTATION: A 22-year-old male with no past significant medical history presented with a four-week history of right upper quadrant abdominal pain and acute onset chest pain radiating to his shoulder with associated dyspnea. On arrival to emergency department, patient was tachycardia, otherwise vitally stable. Labs were notable for leukocytosis. Chest x-ray showed a moderate right-sided pleural effusion with associated opacity of the lower hemithorax. A chest tube was placed however no fluid was evacuated. A CT of the chest was obtained which showed a right lower lobe hemithorax, a right lower lobe pleural-based nodule measuring 2x2 x 1.6 cm, and a lesion within the right hemithorax measuring 20x17.9x19.3 cm. The patient was taken for video-assisted thoracoscopic surgery for surgical exploration and chest tube placement. Patient was found to have large tumor invading whole right chest with compression on mediastinal structures including the heart, lung, and IVC. Biopsies were taken. Patient developed acute respiratory distress and obstructive shock and was intubated and placed on vasopressor support. Preliminary pathology was revealing of synovial sarcoma. Operative findings revealed a large right pleural and posterior mediastinal mass invading the right lower lobe through the diaphragm into the peritoneal space and liver capsule. He underwent thoracotomy with excision and resection of the thoracoabdominal and mediastinal tumor, total pulmonary decortication, wedge resection of right lower and middle lobe, and reconstruction of diaphragm. The patient was successfully extubated three days post operatively and soon after discharged home. Post-operative CT showed no definite evidence of gross residual disease. The patient received 3 cycles of doxorubicin/isofosfamide following discharge. DISCUSSION: Reports of primary thoracic SS are rare. Diagnosis is often delayed because of the benign radiographic appearance and indolent nature of these tumors; sequelae of disease often do not manifest until tumor burden is great enough to cause mass effect. Though standard of care is chemotherapy, the patient in this case required emergency palliative resection due to cardiovascular compromise. CONCLUSIONS: Though soft tissue sarcomas account for less than 0.1% of all malignant thoracic neoplasms, it should be considered in the differential for thoracic malignancies as delay in diagnosis could be consequential to patient outcome. REFERENCE #1: Javad Parvizi, Gregory K. Kim, Chapter 218 - Synovial Sarcoma, Editor(s): Javad Parvizi, Gregory K. Kim, High Yield Orthopaedics, W.B. Saunders, 2010, Pages 455-456, ISBN 9781416002369, https://doi.org/10.1016/B978-1-4160-0236-9.00230-3. REFERENCE #2: Nedra Joseph, Samantha St. Laurent, Jeanenne Joy Nelson, Shan Zheng, and Heide Stirnadel-Farrant. Journal of Clinical Oncology 2019 37:15_suppl, e22535-e22535 DISCLOSURES: No relevant relationships by Neiha Kidwai, source=Web Response

Hypofractionated radiation therapy as palliative management for symptomatic and local control of advanced thoracic malignancies.

Radiation therapy plays an important role for symptom palliation for intrathoracic malignancies ineligible for curative-intent therapy. Limited data exists regarding the role of stereotactic body radiation therapy (SBRT) versus conformal radiation in intrathoracic tumors for palliation. We report the efficacy of hypofractionated RT (or palliative SBRT) in the symptom management and durable control of lung and non-lung intrathoracic tumors. We performed a retrospective review of ninety-two thoracic lesions across 76 patients who completed palliative SBRT with doses ranging 25-50 Gy in 5-10 fractions between 2009 and 2019. Symptoms (cough, chest pain, hemoptysis, shortness of breath) were assessed at consult and 1-6 months follow-up. Local control was evaluated using follow-up CT imaging via RECIST criteria. Descriptive statistics were used to evaluate symptom palliation and Kaplan-Meier method to analyze local control. Of primary lung (Cohort P) lesions, 40% showed stable symptoms, 30% never developed symptoms, and 19% showed symptom relief. CT imaging 1-6 months post-SBRT showed 91% with partial response (PR) or stable disease (SD) in Cohort P and 87% with PR or SD in metastatic (Cohort M) lesions. In patients with initial PR/SD, local control until death was achieved in 71% of Cohort P and 84% of Cohort M. Of our symptomatic patients (Cohort S), 98% showed no symptom progression post-radiotherapy. All patients with hemoptysis at presentation achieved hemostasis post-radiotherapy. Palliative SBRT has the advantage of higher biologic dose without protracted course for patients with limited prognosis. Patients showed significant symptom palliation and long-term local control. Palliative SBRT represents a reasonable treatment modality for incurable thoracic malignancies.