Pulmonary blastoma: a comprehensive overview of a rare entity

Introduction: Pulmonary blastoma is a rare malignancy, accounting for less than 0.5% of primary lung tumors. It belongs to the group of pulmonary sarcomatoid carcinomas, and it is typically characterized by a biphasic pattern of an epithelial and a mesenchymal component. Only a few hundred cases have been reported worldwide. The aim of this study is to review and critically assess the literature regarding pulmonary blastoma.Material and methods: A narrative literature review of PubMed database from the inception of the database up to January 2021, limited to the English language, was conducted, using combinations of the following keywords: “pulmonary blastoma”, “biphasic pulmonary blastoma”, “sarcomatoid carcinoma”.Results: Pulmonary blastoma is composed of an epithelial and a mesenchymal malignant component. Regarding pathogenesis, the origin of the biphasic cell population remains elusive. Characteristic immunohistochemical stains are supportive of diagnosis.Clinically, the symptomatology is non-specific, while 40% of the cases are asymptomatic. It is diagnosed at a younger agecompared to other types of lung cancer, and it is often non-metastatic at diagnosis allowing for surgical treatment. Data on management and survival are scarce and mainly come from isolated cases. Advances on targeted therapy may provide novel treatment options. Given the rarity of the cases, multicenter collaboration is needed in order to establish therapeutic guidelines

Thromboembolic Disease in Patients With Cancer and COVID-19: Risk Factors, Prevention and Practical Thromboprophylaxis Recommendations–State-of-the-Art.

Cancer and COVID-19 are both well-established risk factors predisposing to thrombosis. Both disease entities are correlated with increased incidence of venous thrombotic events through multifaceted pathogenic mechanisms involving the interaction of cancer cells or SARS-CoV2 on the one hand and the coagulation system and endothelial cells on the other hand. Thromboprophylaxis is recommended for hospitalized patients with active cancer and high-risk outpatients with cancer receiving anticancer treatment. Universal thromboprophylaxis with a high prophylactic dose of low molecular weight heparins (LMWH) or therapeutic dose in select patients, is currentlyindicated for hospitalized patients with COVID-19. Also, prophylactic anticoagulation is recommended for outpatients with COVID-19 at high risk for thrombosis or disease worsening. However, whether there is an additive risk of thrombosis when a patient with cancer is infected with SARS-CoV2 remains unclear In the current review, we summarize and critically discuss the literature regarding the epidemiology of thrombotic events in patients with cancer and concomitant COVID-19, the thrombotic risk assessment, and the recommendations on thromboprophylaxis for this subgroup of patients. Current data do not support an additive thrombotic risk for patients with cancer and COVID-19. Of note, patients with cancer have less access to intensive care unit care, a setting associated with high thrombotic risk. Based on current evidence, patients with cancer and COVID-19 should be assessed with well-established risk assessment models for medically ill patients and receive thromboprophylaxis, preferentially with LMWH, according to existing recommendations. Prospective trials on well-characterized populations do not exist

The prevalence of rheumatic diseases in central Greece: a population survey

<p>Abstract</p> <p>Background</p> <p>Rheumatic diseases are a major health and financial burden for societies. The prevalence of rheumatic diseases may change over time, and therefore, we sought to estimate the prevalence of rheumatic diseases in an adult population of central Greece.</p> <p>Methods</p> <p>In this prospective cross-sectional population survey, a random sample of adult population was drawn from poll catalogues of a region in central Greece. A postal questionnaire was sent to 3,528 people for the presence of any rheumatic disease. All positive cases were further confirmed by clinical examination using the American College of Rheumatoloy criteria. Multiple regression analysis was used to assess risk factors for rheumatic diseases.</p> <p>Results</p> <p>The response rate was 48.3% (1,705 answers). Four hundred and twenty individuals (24.6%) had a rheumatic disease. The prevalence of rheumatoid arthritis was 0.58% (95% confidence interval [CI], 0.32-0.87), of psoriatic arthritis was 0.35% (95% CI, 0.33-1.13), of ankylosing spondylitis was 0.29% (95% CI, 0.28-0.94), of primary Sjögren's syndrome was 0.23% (95% CI, 0.22-0.75) and of systemic lupus erythematosus was 0.11% (95% CI, 0.11-0.37). One individual had systemic sclerosis (prevalence, 0.058%), 1 individual had dermatomyositis (prevalence, 0.058%; 95% CI, 0.05-0.18), 2 individuals had vasculitis (prevalence 0.11%; 95% CI, 0.11-0.37), 81 individuals had gout (prevalence, 4.75%; 95% CI, 4.41-5.13), and 304 individuals had osteoarthritis (OA) (prevalence 17.82%; 95% CI, 16.50-19.34). Gout was associated with male gender, diabetes mellitus, and hypertension, and OA was associated with age, female gender, and hypertension.</p> <p>Conclusions</p> <p>Rheumatic diseases are common in central Greece, affecting nearly a quarter of adult population. OA and gout are the most common joint disorders.</p

COVID-19 symptoms at hospital admission vary with age and sex: results from the ISARIC prospective multinational observational study

Background: The ISARIC prospective multinational observational study is the largest cohort of hospitalized patients with COVID-19. We present relationships of age, sex, and nationality to presenting symptoms. Methods: International, prospective observational study of 60 109 hospitalized symptomatic patients with laboratory-confirmed COVID-19 recruited from 43 countries between 30 January and 3 August 2020. Logistic regression was performed to evaluate relationships of age and sex to published COVID-19 case definitions and the most commonly reported symptoms. Results: ‘Typical’ symptoms of fever (69%), cough (68%) and shortness of breath (66%) were the most commonly reported. 92% of patients experienced at least one of these. Prevalence of typical symptoms was greatest in 30- to 60-year-olds (respectively 80, 79, 69%; at least one 95%). They were reported less frequently in children (≤ 18 years: 69, 48, 23; 85%), older adults (≥ 70 years: 61, 62, 65; 90%), and women (66, 66, 64; 90%; vs. men 71, 70, 67; 93%, each P &lt; 0.001). The most common atypical presentations under 60 years of age were nausea and vomiting and abdominal pain, and over 60 years was confusion. Regression models showed significant differences in symptoms with sex, age and country. Interpretation: This international collaboration has allowed us to report reliable symptom data from the largest cohort of patients admitted to hospital with COVID-19. Adults over 60 and children admitted to hospital with COVID-19 are less likely to present with typical symptoms. Nausea and vomiting are common atypical presentations under 30 years. Confusion is a frequent atypical presentation of COVID-19 in adults over 60 years. Women are less likely to experience typical symptoms than men

Ρευματικές εκδηλώσεις ως ανεπιθύμητες ενέργειες σε ασθενείς με καρκίνο που λαμβάνουν θεραπεία με αναστολείς των σημείων ελέγχου (Immune checkpoint inhibitor)- Βιβλιογραφική ανασκόπηση-

Η ανοσοθεραπεία με αναστολείς των σημείων ελέγχου αποτελεί μια νέα επιλογή για ασθενείς με καρκίνο. Μετά την πρώτη δεκαετία χρησιμοποίησής τους, φαίνεται να βελτιώνουν τη συνολική πρόγνωση και αρκετοί ασθενείς παρουσιάζουν καλύτερη ανταπόκριση σε σχέση με άλλες αντικαρκινικές θεραπείες. Παρόλα αυτά, η εφαρμογή της δημιούργησε νέες προκλήσεις σχετικά με τη βελτίωση των φαρμάκων, την επιλογή των κατάλληλων ασθενών, αλλά και την αντιμετώπιση των εμφανιζόμενων ανεπιθύμητων ενεργειών. Αυτές οι ανεπιθύμητες ενέργειες είναι αποτέλεσμα άτοπης και εκσεσημασμένης φλεγμονώδους αντίδρασης, αφορούν όλα σχεδόν τα συστήματα του οργανισμού και η εμφάνισή τους είναι ετερογενής και απρόβλεπτη. Οι ρευματικές ανεπιθύμητες ενέργειες εμφανίζονται λιγότερο συχνά και συνήθως είναι ηπιότερες, με αποτέλεσμα να μην έχουν μελετηθεί επαρκώς. Από τα βιβλιογραφικά δεδομένα φαίνεται να έχουν κάποια ιδιαίτερα χαρακτηριστικά σε σχέση με τις ανεπιθύμητες ενέργειες που προκύπτουν από άλλα συστήματα. Το χρονικό φάσμα της εκδήλωσής τους είναι ασαφές, επιβαρύνουν σημαντικά την ποιότητα ζωής του ασθενή με καρκίνο, ενώ συχνά εμμένουν για μεγάλο χρονικό διάστημα μετά τη διακοπή της ανοσοθεραπείας. Καθώς δεν υπάρχουν σαφείς οδηγίες για την αναγνώριση και την αντιμετώπισή τους, ο Ρευματολόγος καλείται να τις ξεχωρίσει από τις αναγνωρισμένες ρευματικές παθήσεις και, σε συνεργασία με τον Ογκολόγο, να χορηγήσει την κατάλληλη αντιρευματική θεραπεία, ώστε να συνεχιστεί απρόσκοπτα η ανοσοθεραπεία. Με την ολοένα αυξανόμενη ανάπτυξη των αναστολέων των σημείων ελέγχου, καθίσταται απαραίτητη η παρακολούθηση της σύγχρονης βιβλιογραφίας, η κατάρτιση διαγνωστικών και θεραπευτικών αλγορίθμων για τη διαχείριση των ασθενών αυτών, η διεπιστημονική αντιμετώπισή τους, καθώς και η διεξαγωγή νέων μελετών για την απάντηση των αναδυόμενων ερωτημάτων που καλούνται να απαντήσουν οι κλινικοί ιατροί.Immunotherapy with immune checkpoint inhibitors is a new, revolutionary, efficient therapeutic choice for cancer patients. After one decade of application, it seems to improve the overall prognosis and the response to therapy compared to other anticancer regimens. However, there are new challenges emerging concerning its further improvement, the proper choice of suitable patients, and the management of the new type of adverse events. Those adverse events are a result of an off-target activation of the immune system, affect nearly all organ systems, and they remain heterogenous and unpredictable. Rheumatic immune-related adverse events are less common and usually mild. As a result, they have not been studied thoroughly. Moreover, their clinical manifestation presents some distinct features. The time of onset is unpredictable, they influence the quality of life of cancer patients, and they tend to persist long after the cessation of immunotherapy. There are no specific recommendations concerning the diagnosis and management; thus, rheumatologists must be well equipped to discriminate them from the known rheumatic diseases and in collaboration with the oncologists to make decisions for the induction of immunosuppressive therapy and for the safe continuation of immunotherapy. With the rapid development and clinical application of the immune checkpoint inhibitors, it is crucial to follow the modern literature and structure diagnostic and therapeutic algorithms and recommendations. The upcoming years will be needed multidisciplinary approach for the management of immune related adverse events and new clinical studies to deal with the emerging questions and challenges from cancer immunotherapy

A case of trastuzumab-induced dermatomyositis

Human epidermal growth factor receptor 2 (HER-2) is a checkpoint, controlling cell proliferation and differentiation. Trastuzumab, a humanized monoclonal antibody directed against HER-2, is nowadays standard treatment for breast cancer patients whose tumors express HER-2. It is generally well tolerated, with a small number of patients developing mild adverse reactions. Dermatomyositis is a rare adverse event of trastuzumab therapy not well described in the literature. We herein present a case of a patient treated for hormone-sensitive invasive ductal carcinoma, who presented with symptoms of proximal muscle weakness, arthralgias, skin rash, and generalized fatigue. The symptoms started after the sixth cycle of trastuzumab and progressively deteriorated. The patient&apos;s medical and family history was unremarkable. Disease progression as a possible cause of dermatomyositis had been ruled out, and laboratory evaluation revealed moderate elevation of serum muscle proteins and acute-phase reactants. Trastuzumab treatment was discontinued, and 3 months later, the patient was free of symptoms without any further intervention

Pulmonary Blastoma: A Comprehensive Overview of A Rare Entity

Introduction: Pulmonary blastoma is a rare malignancy, accounting for less than 0.5% of primary lung tumors. It belongs to the group of pulmonary sarcomatoid carcinomas, and it is typically characterized by a biphasic pattern of an epithelial and a mesenchymal component. Only a few hundred cases have been reported worldwide. The aim of this study is to review and critically assess the literature regarding pulmonary blastoma. Material and methods: A narrative literature review of PubMed database from the inception of the database up to January 2021, limited to the English language, was conducted, using combinations of the following keywords: “pulmonary blastoma”, “biphasic pulmonary blastoma”, “sarcomatoid carcinoma”. Results: Pulmonary blastoma is composed of an epithelial and a mesenchymal malignant component. Regarding pathogenesis, the origin of the biphasic cell population remains elusive. Characteristic immunohistochemical stains are supportive of diagnosis.Clinically, the symptomatology is non-specific, while 40% of the cases are asymptomatic. It is diagnosed at a younger agecompared to other types of lung cancer, and it is often non-metastatic at diagnosis allowing for surgical treatment. Data on management and survival are scarce and mainly come from isolated cases. Advances on targeted therapy may provide novel treatment options. Given the rarity of the cases, multicenter collaboration is needed in order to establish therapeutic Guideliness

Systematic review with meta-analysis: COVID-19 outcomes in patients receiving anti-TNF treatments

Background Accumulating evidence suggests a beneficial effective of tumour necrosis factor-alpha (TNF-alpha) inhibitors on the outcomes of COVID-19 disease, which, however is not validated by all studies. Aims To perform a systematic review and meta-analysis of existing reports to investigate the impact of anti-TNF treatments on the clinical outcomes of COVID-19 patients. Methods A systematic search at PubMed and SCOPUS databases using specific keywords was performed. All reports of COVID-19 outcomes for patients receiving anti-TNF therapy by September 2021 were included. Pooled effect measures were calculated using a random-effects model. The Newcastle Ottawa Scale for observational studies was used to assess bias. Studies that were not eligible for meta-analysis were described qualitatively. Results In total, 84 studies were included in the systematic review, and 35 were included in the meta-analysis. Patients receiving anti-TNF treatment, compared to non-anti-TNF, among COVID-19 cases had a lower probability of hospitalisation (eight studies, 2555 patients, pooled OR = 0.53, 95% CI: 0.42-0.67, I-2 = 0) and severe disease defined as intensive care unit admission or death (two studies, 1823 patients, pooled OR = 0.63, 95% CI: 0.41-0.96, I-2 = 0), after adjustment for validated predictors of adverse disease outcomes. No difference was found for the risk for hospitalisation due to COVID-19 in populations without COVID-19 for patients receiving anti-TNF treatment compared to non-anti-TNF (three studies, 5 994 958 participants, pooled risk ratio = 0.97, 95% CI: 0.68-1.39, I-2 = 20) adjusted for age, sex and comorbidities. Conclusions TNF-alpha inhibitors are associated with a lower probability of hospitalisation and severe COVID-19 when compared to any other treatment for an underlying inflammatory disease

Vaccine-Related Autoimmune Hepatitis: Emerging Association with SARS-CoV-2 Vaccination or Coincidence?

Background: There is an increasing number of liver injury cases resembling autoimmune hepatitis (AIH) following SARS-CoV-2 vaccination; however, an association has not yet been established. Methods/Materials: A literature review was performed to identify articles regarding the association of AIH with vaccination, emphasizing on SARS-CoV-2 vaccines, and the proposed mechanisms. We then performed a literature search for AIH-like cases following SARS-CoV-2 vaccination, and we evaluated the included cases for AIH diagnosis using simplified diagnostic criteria (SDC), and for vaccination causality using the Naranjo score for adverse drug reactions. Results: We identified 51 AIH-like cases following SARS-CoV-2 vaccination. Forty cases (80%) were characterized as &ldquo;probable&rdquo;, &ldquo;at least probable&rdquo;, or &ldquo;definite&rdquo; for AIH diagnosis according to SDC. Forty cases (78.4%) were characterized as &ldquo;probable&rdquo;, four (7.8%) as &ldquo;possible&rdquo;, and three (5.8%) as &ldquo;definite&rdquo; for vaccine-related AIH according to the Naranjo score. Conclusion: SARS-CoV-2 vaccine-related AIH carries several phenotypes and, although most cases resolve, immunosuppressive therapy seems to be necessary. Early diagnosis is mandatory and should be considered in any patient with acute or chronic hepatitis after SARS-CoV-2 vaccination, especially in those with pre-existing liver disease

Cardiac Injury in COVID-19: A Systematic Review of Relevant Meta-Analyses

Background: Cardiac injury (CI) is not a rare condition among hospitalized patients with coronavirus disease 2019 (COVID-19). Its prognostic value has been extensively reported through the literature, mainly in the context of observational studies. An impressive number of relevant meta-analyses has been conducted. These meta-analyses present similar and consistent results; yet interesting methodological issues emerge. Methods: A systematic literature search was conducted aiming to identify all relevant meta-analyses on (i) the incidence, and (ii) the prognostic value of CI among hospitalized patients with COVID-19. Results: Among 118 articles initially retrieved, 73 fulfilled the inclusion criteria and were included in the systematic review. Various criteria were used for CI definition mainly based on elevated cardiac biomarkers levels. The most frequently used biomarker was troponin. 30 meta-analyses reported the pooled incidence of CI in hospitalized patients with COVID-19 that varies from 5% to 37%. 32 meta-analyses reported on the association of CI with COVID-19 infection severity, with only 6 of them failing to show a statistically significant association. Finally, 46 meta-analyses investigated the association of CI with mortality and showed that patients with COVID-19 with CI had increased risk for worse prognosis. Four meta-analyses reported pooled adjusted hazard ratios for death in patients with COVID-19 and CI vs those without CI ranging from 1.5 to 3. Conclusions: The impact of CI on the prognosis of hospitalized patients with COVID-19 has gained great interest during the pandemic. Methodological issues such as the inclusion of not peer-reviewed studies, the inclusion of potentially overlapping populations or the inclusion of studies with unadjusted analyses for confounders should be taken into consideration. Despite these limitations, the adverse prognosis of patients with COVID-19 and CI has been consistently demonstrated