Primary mediastinal Large B-cell Lymphoma

Primary mediastinal large B-cell lymphoma (PMBCL) is a separate entity in the WHO classification based on clinico-pathologic features and a distinct molecular signature which overlaps with nodular sclerosis classical Hodgkin lymphoma (NScHL). Molecular classifiers can distinguish PMBCL from diffuse large B-cell lymphoma (DLBCL) using RNA derived from paraffin-embedded tissue and are integral to future studies. However, given that ~5% of DLBCL can have a 'molecular' PMBCL phenotype in the absence of mediastinal involvement, clinical information will remain critical for diagnosis. Studies over the last 10-20 years have elucidated the biologic hallmarks of PMBCL which are reminiscent of cHL, including the importance of JAK-STAT and NFKB signaling pathways as well as an immune evasion phenotype through multiple converging genetic aberrations. The outcome of PMBCL has improved in the modern rituximab era, however controversies remain whether there is a single standard treatment for all patients and when to integrate radiotherapy. Regardless of the frontline therapy, refractory disease can occur in up to 10% of patients and correlates with poor outcome. With emerging data supporting high efficacy of PD1 inhibitors in PMBCL, studies are underway integrating them into the up-front setting.

Spontaneous Regression of Metastatic Renal Cell Carcinoma after SARS-CoV-2 Infection: A Report of Two Cases

Spontaneous regression of metastatic renal cell carcinoma (mRCC) is a rare event, often associated with an activation of innate immunity by various triggers. SARS-CoV-2 infection induces a strong inflammatory response in some patients and a cytokine storm is one of the main causes of severe morbidity and mortality associated with the virus. Here, we describe two cases of patients with histologically and radiologically proven mRCC whose treatment was delayed due to COVID-19 and who experienced spontaneous tumour regression following the infection. Both patients reported here had predominantly pulmonary and mediastinal involvement and underwent nephrectomy. The interval between the diagnosis of COVID-19 and the detection of tumour regression was 3 and 4 months, respectively. Although approved vaccines and other measures are clearly the best way to prevent COVID-19-associated morbidity and mortality in cancer patients, we hypothesize that innate immunity activation by the infection can contribute to tumour regression in special circumstances.

Deep neck infections with and without mediastinal involvement: treatment and outcome in 218 patients

Purpose Infections of the deep neck, although becoming scarcer due to the widespread use of antibiotics, still represent a dangerous and possibly deadly disease, especially when descending into the mediastinum. Due to the different specialities involved in the treatment and the heterogenous presentation of the disease, therapeutic standard is still controversial. This study analyzes treatment and outcome in these patients based on a large retrospective review and proposes a therapeutic algorithm. Methods The cases of 218 adult patients treated with deep neck abscesses over a 10-year period at a tertiary university hospital were analyzed retrospectively. Clinical, radiological, microbiological and laboratory findings were compared between patients with and without mediastinal involvement. Results Forty-five patients (20.64%) presented with abscess formation descending into the mediastinum. Those patients had significantly (all items p < 0.0001) higher rates of surgical interventions (4.27 vs. 1.11) and tracheotomies (82% vs. 3.4%), higher markers of inflammation (CRP 26.09 vs. 10.41 mg/dl), required more CT-scans (3.58 vs. 0.85), longer hospitalization (39.78 vs 9.79 days) and more frequently needed a change in antibiotic therapy (44.44% vs. 6.40%). Multi-resistant pathogens were found in 6.67% vs. 1.16%. Overall mortality rate was low with 1.83%. Conclusion Despite of the high percentage of mediastinal involvement in the present patient collective, the proposed therapeutic algorithm resulted in a low mortality rate. Frequent CT-scans, regular planned surgical revisions with local drainage and lavage, as well as an early tracheotomy seem to be most beneficial regarding the outcome.

Using predictive models to determine the presence of non-small cell lung cancer metastasis to N2 and N3 regions.

e20560 Background: Accurate assessment of non-small cell lung cancer (NSCLC) mediastinal involvement is key to developing treatment plans and determining prognosis. To date, there is no reliable imaging-based means to determine the presence or absence of mediastinal involvement. Current computed tomogram (CT) and fluorodeoxyglucose-positron emission tomography/ computed tomogram (PET-CT) technologies provide numerous derived automated variables have not been sufficiently evaluated to determine the presence of metastasis to the mediastinum. We have developed predictive models to determine the presence or lack of metastatic NSCLC in N2 and N3 regions. Methods: Consecutive patients from 2012-2017 with biopsy-proven NSCLC who had CT and PET-CT, as well as biopsy of the mediastinum had their images reread by a team of blinded specialty radiologists and nuclear medicine specialists. Patients with no mediastinal malignancy on biopsy were followed for 6 months from the initial evaluation to confirm lack of mediastinal malignancy.278 regions (N2 and N3) from 139 patients were included. Logistic regression models were used to build a baseline model, as well as models with additional nodal station maximum standard uptake valuve (SUVm) measurements (SUVm, SUVm-SUVmeanbloodpool and SUV lymph node/tumor (LN/T)) for N2 and N3 regions, respectively. When nodal station SUVm was not measured, SUVmeanbloodpool was used. The SUVm within each region was used. Stepwise selection was used to select variables in the baseline model. Cross-validated ROC curve and area under the curve (AUC) were reported. All analyses were done in SAS 9.4 (SAS Institute, Inc., Cary, NC). Results: 40/139 N2 regions had malignancy, 4/139 N3 regions had malignancy. Baseline models for N2 regions selected lung laterality (OR right vs left: 4.84 (1.79, 13.05)) and nodal station short-axis diameter > 1 cm (OR yes vs no: 5.49 (1.71, 17.54)) while no variables were selected for the baseline model for N3 regions due to lack of statistical power. We used the same variables for the N3 baseline model. Conclusions: We have identified models that use a more advanced analysis of predicting the presence or absence of metastatic NSCLC in both N2 and N3 regions with respect to the primary lesion. All models perform better with SUVm related measurements. From this information, we are developing a clinical application to provide practitioners a better means of assessing the presence of mediastinal involvement of NSCLC. [Table: see text]

Invasive Mediastinal Mucormycosis with Pulmonary and Cardiac Involvement in an Adult with Chronic Granulomatous Disease: Case Report and Review of the Literature

Mucormycosis is a rare fungal infection that often causes rhinocerebral disease. However, there have been rare cases of mediastinal involvement. These patients remain a therapeutic challenge and mortality in this group is very high. We report a case of mediastinal mucormycosis with invasion of the heart and right lung in a patient with chronic granulomatous disease (CGD) and also review the available literature on mediastinal mucormycosis.

Cardiac lipoblastoma of the pulmonary valve: A rare tumour in an unusual location

Lipoblastoma is a rare, benign, fatty tissue tumour that occurs in infancy and early childhood. Intrathoracic and mediastinal involvement of this tumour is rare, and an intracardiac location is even rarer. In this study, we report the case of a 15-month-old girl who presented with a tumour in the pulmonary valve. A complete resection of the tumour was undertaken, with a histopathology report confirming the diagnosis of lipoblastoma.

The Outcomes of Multi-Disciplinary Treatment of Esophageal Cancer in Vajira Hospital

Background: Esophageal cancer is one of the most fatal and difficult-to-treat cancer. Multi-modality management is the key to success of improving outcomes, however, which modality is the most proper is difficult to determine. Objective: To evaluate the overall survival (OS) of patients with early or locally-advanced (E/LA) esophageal carcinoma treated in Vajira Hospital. The outcomes of the multi-modality management among patients with E/LA diseases were evaluated. Materials and Methods: The retrospective analyses of esophageal carcinoma patients who attended at Vajira Hospital between January 1, 2012 and December 31, 2016 were performed. Results: There were 86 patients with complete medical records. The median age was 60.5 years (IQR 52 to 66). Sixty-five patients (75.6%) presented with E/LA diseases. Most of the patients had primary site at thoracic part of esophagus (58 patients, 67.4%) and had squamous cell carcinoma histology (84 patients, 97.7%). Tri-modality treatment including neoadjuvant chemoradiation and esophagectomy for clinically fitted patients without evidence of mediastinal involvement and non-regional lymph node metastasis resulted in the best survival outcome [28.56 months (IQR 10.64 to 46.47)]. The OS of patients with E/LA disease was only 9.15 months (IQR 4.49 to 23.02). Male patients, non-cervical site, and non-surgical treatment were associated with the worse OS. Conclusion: The outcomes of patients with esophageal carcinoma treated in a real-world practice is still not impressive. Tri-modality management would be the best paradigm; however, it is suitable for well-selected patients. Keywords: Esophageal cancer, Multi-modality treatment, Real-world practice

Uveitis as an Open Window to Systemic Inflammatory Diseases

Spondyloarthritis (Spa), Behçet’s disease (BD) and sarcoidosis are major systemic inflammatory diseases worldwide. They are all multisystem pathologies and share a possible ocular involvement, especially uveitis. We hereby describe selected cases who were referred by ophthalmologists to our internal medicine department for unexplained uveitis. Physical examination and/or the use of laboratory and imaging investigations allowed to make a diagnosis of a systemic inflammatory disease in a large proportion of patients. In our tertiary referral center, 75 patients have been diagnosed with Spa (n = 20), BD (n = 9), or sarcoidosis (n = 46) in the last two years. There was a significant delay in the diagnosis of Spa-associated uveitis. Screening strategies using Human Leukocyte Antigen (HLA)-B27 determination and sacroiliac magnetic resonance imaging in patients suffering from chronic low back pain and/or psoriasis helped in the diagnosis. BD’s uveitis affects young people from both sexes and all origins and usually presents with panuveitis and retinal vasculitis. The high proportion of sarcoidosis in our population is explained by the use of chest computed tomography (CT) and 18F-fluorodeoxyglucose positron emission tomography CT that helped to identify smaller hilar or mediastinal involvement and allowed to further investigate those patients, especially in the elderly. Our results confirm how in these sight- and potentially life-threatening diseases a prompt diagnosis is mandatory and benefits from a multidisciplinary approach.

Quantifying and reporting cardiac findings in imaging of COVID-19 patients

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection continues to be a public health emergency and a pandemic of international concern. As of April 31st, the reported cases of COVID-19 are three million in 186 countries. Reported case fatality has crossed 200 thousand among which more than fifty thousand has been in the USA. Most patients present with symptoms of fever, cough, and shortness of breath following exposure to other COVID-19 patients. Respiratory manifestations predominate in patients with mild, moderate, severe illness. Imaging of patients with COVID-19 consistently reports various pulmonary parenchymal involvement. In this article we wanted to reinforce and review the various reported imaging patterns of cardiac and mediastinal involvement in COVID-19 patients. Among patients with COVID 19 who underwent various imaging of chest various cardiac findings including pericardial effusion, myocarditis, cardiomegaly has been reported. Most of these findings have been consistently reported in patients with significant acute myocardial injury, and fulminant myocarditis. Acute biventricular dysfunction has also been reported with subsequent improvement of the same following clinical improvement. Details of cardiac MRI is rather limited. In a patient with clinical presentation of acute myocarditis, biventricular myocardial interstitial edema, diffuse biventricular hypokinesia, increased ventricular wall thickness, and severe LV dysfunction has been reported. Among patients with significant clinical improvement in LV structure and function has also been documented. With increasing number of clinical cases, future imaging studies will be instrumental in identifying the various cardiac manifestations, and their relation to clinical outcome.