Addition of long-acting beta2 agonists or long-acting muscarinic antagonists versus doubling the dose of inhaled corticosteroids (ICS) in adolescents and adults with uncontrolled asthma with medium dose ICS: a systematic review and network meta-analysis.

BACKGROUND: Inhaled corticosteroids (ICS) are the mainstay treatment for persistent asthma. Escalating treatment is required when asthma is not controlled with ICS therapy alone, which would include, but is not limited to, adding a long-acting beta2-agonist (LABA) or a long-acting muscarinic antagonist (LAMA) or doubling the dose of ICS. OBJECTIVES: To assess the efficacy and safety of adding a LABA or LAMA to ICS therapy versus doubling the dose of ICS in adolescents and adults whose asthma is not well controlled on medium-dose (MD)-ICS using a network meta-analysis (NMA), and to provide a ranking of these treatments according to their efficacy and safety. SEARCH METHODS: We searched the Cochrane Airways Trials Register, CENTRAL, MEDLINE, Embase, Global Health, ClinicalTrials.gov, and the World Health Organization ICTRP for pre-registered randomised controlled trials (RCTs) from January 2008 to 19 December 2022. SELECTION CRITERIA: We searched for studies including adolescents and adults with uncontrolled asthma who had been treated with or were eligible for MD-ICS, comparing it to high-dose (HD)-ICS, ICS/LAMA, or ICS/LABA. We excluded cluster- and cross-over RCTs. Studies were of at least 12 weeks duration. DATA COLLECTION AND ANALYSIS: We conducted a systematic review and network meta-analysis according to a previously published protocol. We used Cochrane's Screen4ME workflow to assess search results. We used Grading of Recommendations Assessment, Development and Evaluation (GRADE) to assess the certainty of evidence. The primary outcome is asthma exacerbations (moderate and severe). MAIN RESULTS: We included 38,276 participants from 35 studies (median duration 24 weeks (range 12 to 78); mean age 44.1; 38% male; 69% white; mean forced expiratory volume in one second 2.1 litres and 68% of predicted). MD- and HD-ICS/LABA likely reduce and MD-ICS/LAMA possibly reduces moderate to severe asthma exacerbations compared to MD-ICS (hazard ratio (HR) 0.70, 95% credible interval (CrI) 0.59 to 0.82; moderate certainty; HR 0.59, 95% CrI 0.46 to 0.76; moderate certainty; and HR 0.56, 95% CrI 0.38 to 0.82; low certainty, respectively), whereas HD-ICS probably does not (HR 0.94, 95% CrI 0.70 to 1.24; moderate certainty). There is no clear evidence to suggest that any combination therapy or HD-ICS reduces severe asthma exacerbations compared to MD-ICS (low to moderate certainty). This study suggests no clinically meaningful differences in the symptom or quality of life score between dual combinations and monotherapy (low to high certainty). MD- and HD-ICS/LABA increase or likely increase the odds of Asthma Control Questionnaire (ACQ) responders at 6 and 12 months compared to MD-ICS (odds ratio (OR) 1.47, 95% CrI 1.23 to 1.76; high certainty; and OR 1.59, 95% CrI 1.31 to 1.94; high certainty at 6 months; and OR 1.61, 95% CrI 1.22 to 2.13; moderate certainty and OR 1.55, 95% CrI 1.20 to 2.00; high certainty at 12 months, respectively). MD-ICS/LAMA probably increases the odds of ACQ responders at 6 months (OR 1.32, 95% CrI 1.11 to 1.57; moderate certainty). No data were available at 12 months. There is no clear evidence to suggest that HD-ICS increases the odds of ACQ responders or improves the symptom or qualify of life score compared to MD-ICS (very low to high certainty). There is no evidence to suggest that ICS/LABA or ICS/LAMA reduces asthma-related or all-cause serious adverse events (SAEs) compared to MD-ICS (very low to high certainty). HD-ICS results in or likely results in little or no difference in the included safety outcomes compared to MD-ICS as well as HD-ICS/LABA compared to MD-ICS/LABA. The pairwise meta-analysis shows that MD-ICS/LAMA likely reduces all-cause adverse events (AEs) and results in a slight reduction in treatment discontinuation due to AEs compared to MD-ICS (risk ratio (RR) 0.86, 95% confidence interval (CI) 0.77 to 0.96; 4 studies, 2238 participants; moderate certainty; and RR 0.51, 95% CI 0.26 to 0.99; 4 studies, 2239 participants; absolute risk reduction 10 fewer per 1000 participants; moderate certainty, respectively). The NMA evidence is in agreement with the pairwise evidence on treatment discontinuation due to AEs, but very uncertain on all-cause AEs, due to imprecision and heterogeneity. AUTHORS' CONCLUSIONS: The review findings suggest that MD- or HD-ICS/LABA and MD-ICS/LAMA reduce moderate to severe asthma exacerbations and increase the odds of ACQ responders compared to MD-ICS whereas HD-ICS probably does not. The evidence is generally stronger for MD- and HD-ICS/LABA than for MD-ICS/LAMA primarily due to a larger evidence base. There is no evidence to suggest that ICS/LABA, ICS/LAMA, or HD-ICS/LABA reduces severe asthma exacerbations or SAEs compared to MD-ICS. MD-ICS/LAMA likely reduces all-cause AEs and results in a slight reduction in treatment discontinuation due to AEs compared to MD-ICS. The above findings may assist in deciding on a treatment option during the stepwise approach of asthma management. Longer-term safety of higher than medium-dose ICS needs to be addressed in phase 4 or observational studies given that the median duration of included studies was six months.

Effectiveness and tolerability of dual and triple combination inhaler therapies compared with each other and varying doses of inhaled corticosteroids in adolescents and adults with asthma: a systematic review and network meta-analysis.

BACKGROUND: Current guidelines recommend a higher-dose inhaled corticosteroids (ICS) or adding a long-acting muscarinic antagonist (LAMA) when asthma is not controlled with medium-dose (MD) ICS/long-acting beta2-agonist (LABA) combination therapy. OBJECTIVES: To assess the effectiveness and safety of dual (ICS/LABA) and triple therapies (ICS/LABA/LAMA) compared with each other and with varying doses of ICS in adolescents and adults with uncontrolled asthma. SEARCH METHODS: We searched multiple databases for pre-registered randomised controlled trials (RCTs) of at least 12 weeks of study duration from 2008 to 18 February 2022. SELECTION CRITERIA: We searched studies, including adolescents and adults with uncontrolled asthma who had been treated with, or were eligible for, MD-ICS/LABA, comparing dual and triple therapies. We excluded cluster- and cross-over RCTs. DATA COLLECTION AND ANALYSIS: We conducted a systematic review and network meta-analysis according to the previously published protocol. We used Cochrane's Screen4ME workflow to assess search results and Grading of Recommendations Assessment, Development and Evaluation (GRADE) to assess the certainty of evidence. The primary outcome was steroid-requiring asthma exacerbations and asthma-related hospitalisations (moderate to severe and severe exacerbations). MAIN RESULTS: We included 17,161 patients with uncontrolled asthma from 17 studies (median duration 26 weeks; mean age 49.1 years; male 40%; white 81%; mean forced expiratory volume in 1 second (MEF 1)1.9 litres and 61% predicted). The quality of included studies was generally good except for some outcomes in a few studies due to high attrition rates. Medium-dose (MD) and high-dose (HD) triple therapies reduce steroid-requiring asthma exacerbations (hazard ratio (HR) 0.84 [95% credible interval (CrI) 0.71 to 0.99] and 0.69 [0.58 to 0.82], respectively) (high-certainty evidence), but not asthma-related hospitalisations, compared to MD-ICS/LABA. High-dose triple therapy likely reduces steroid-requiring asthma exacerbations compared to MD triple therapy (HR 0.83 [95% CrI 0.69 to 0.996], [moderate certainty]). Subgroup analyses suggest the reduction in steroid-requiring exacerbations associated with triple therapies may be only for those with a history of asthma exacerbations in the previous year but not for those without. High-dose triple therapy, but not MD triple, results in a reduction in all-cause adverse events (AEs) and likely reduces dropouts due to AEs compared to MD-ICS/LABA (odds ratio (OR) 0.79 [95% CrI 0.69 to 0.90], [high certainty] and 0.50 [95% CrI 0.30 to 0.84], [moderate certainty], respectively). Triple therapy results in little to no difference in all-cause or asthma-related serious adverse events (SAEs) compared to dual therapy (high certainty). The evidence suggests triple therapy results in little or no clinically important difference in symptoms or quality of life compared to dual therapy considering the minimal clinically important differences (MCIDs) and HD-ICS/LABA is unlikely to result in any significant benefit or harm compared to MD-ICS/LABA. AUTHORS' CONCLUSIONS: Medium-dose and HD triple therapies reduce steroid-requiring asthma exacerbations, but not asthma-related hospitalisations, compared to MD-ICS/LABA especially in those with a history of asthma exacerbations in the previous year. High-dose triple therapy is likely superior to MD triple therapy in reducing steroid-requiring asthma exacerbations. Triple therapy is unlikely to result in clinically meaningful improvement in symptoms or quality of life compared to dual therapy considering the MCIDs. High-dose triple therapy, but not MD triple, results in a reduction in all-cause AEs and likely reduces dropouts due to AEs compared to MD-ICS/LABA. Triple therapy results in little to no difference in all-cause or asthma-related SAEs compared to dual therapy. HD-ICS/LABA is unlikely to result in any significant benefit or harm compared to MD-ICS/LABA, although long-term safety of higher rather than MD- ICS remains to be demonstrated given the median duration of included studies was six months. The above findings may assist deciding on a treatment option when asthma is not controlled with MD-ICS/LABA.

Understanding the Knowledge Gap and Assessing Comfort Level among Healthcare Professionals Who Provide Inhaler Education.

Inhaled medications play a pivotal role in the management of COPD and asthma. Provider knowledge and ability to teach various devices is paramount as poor inhaler technique directly correlates with worse disease control. The goal of our survey was to assess the knowledge and comfort level with various inhaled devices among providers involved in patient inhaler education. We constructed a 20-question survey consisting of a five-question Likert scale-based comfort assessment and a 15-question multiple-choice inhaler knowledge test that was distributed both internally and nationwide. Groups surveyed included internal medicine residents, family medicine residents, pulmonary fellows, respiratory therapists, nursing staff, and pharmacists. A total of 557 providers responded to the survey. The overall correct response rate among all respondents was only 47%. There was no significant difference between correct response rates among prescribers (internal medicine residents, family medicine residents, and pulmonary fellows) and non-prescribers (respiratory therapists, nursing staff, and pharmacists), 47% and 47%, respectively (p = 0.6919). However, respiratory therapists had the overall highest correct response rate of 85%. Over 72% of respondents indicated that they educate patients on inhaler technique as part of their clinical duties. Furthermore, the correct response rates for various inhaler devices varied with 55% among metered dose inhalers, 52% among dry powder inhalers, and 34% among soft-mist inhalers. Our study reveals that there is a continued need for education on the subject of inhaler devices among providers given their overall poor knowledge, particularly in an era of fast-changing inhaler devices. We continue without knowing what we teach.

Patient Perception of Vaping in the Midst of the Electronic Cigarette and Vaping Product Associated Lung Injury (EVALI) Epidemic.

PURPOSE OF STUDY: EVALI has reached epidemic proportions. With the increasing prevalence, new information has come to light regarding the safety of electronic cigarettes. We investigate the impact of the recent EVALI epidemic on patient perception surrounding vaping. METHODS: An anonymous survey was developed to evaluate patient perception regarding vaping. The survey was administered to adult patients at various clinics at University of Missouri. A total of 287 survey responses were analyzed. RESULTS: In all, 81.5% of respondents reported that e-cigarettes are unsafe, 73.2% did not feel that the absence of THC oils or vitamin E acetate made e-cigarettes safer, and 55.1% felt that e-cigarettes are less safe than traditional cigarettes. A majority (76%) felt that e-cigarettes/vaping products should be more regulated and pose a public health concern. Most surprising, 67.6% of participants answered that they knew somebody who was negatively affected by EVALI. CONCLUSION: The survey responses suggest that the current EVALI epidemic is changing the public's perception and the narrative surrounding safety of e-cigarettes and vaping products.

Increased Incidence, Morbidity, and Mortality in Human Coronavirus NL63 Associated with ACE Inhibitor Therapy and Implication in SARS-CoV-2 (COVID-19).

BACKGROUND: The endemic human coronavirus NL63 strain (HCoV-NL63) employs angiotensin-converting enzyme 2 (ACE-2) receptors on cell surfaces to infect hosts in the same manner as SARS-CoV and the novel SARS-CoV-2. It has been proposed that patients on angiotensin-converting enzyme inhibitor (ACE-I) and angiotensin receptor blockers (ARB) therapy infected with SARS-CoV-2 have a higher mortality rate due to over-expression of ACE-2 receptors. AIM: We sought to evaluate the impact of ACE-I/ARB on infectivity of various endemic coronavirus strains, hypothesizing that rates of ACE-I use among patients with HCoV-NL63 would be higher compared to other endemic coronavirus strains that do not utilize the ACE-2 receptor. DESIGN/METHODS: A retrospective cohort study was designed to evaluate a total 466 subjects with a positive respiratory pathogens panel for one of the endemic coronavirus strains. Rate of ACE-I/ARB use among each coronavirus strain and clinical outcomes from the 88 HCoV-NL63 positive subjects was collected. RESULTS: Analysis revealed a higher rate of ACE-I (p=0.006) use among the HCoV-NL63 positives compared to the other three endemic coronavirus strains. The rate of invasive mechanical ventilation (p=0.007) and 90-day mortality (p=0.045) among HCoV-NL63 positives on ACE-I therapy was higher compared to those HCoV-NL63 positives not on ACE-I therapy. CONCLUSION: Concurrent therapy with an ACE-I was associated with an increased rate and severity of infection with the HCoV-NL63. This association was not found in infected patients on concurrent ARB therapy. These findings support the importance of further evaluation in patients on these therapies who are infected with the novel coronavirus SARS-CoV-2.

The WHO 2022 Classification of Renal Neoplasms (5th Edition): Salient Updates.

The first categorization for renal tumours was made by the WHO in 1981 and included only renal cell carcinoma (RCC). After that, classification was continuously altered over five decades. The WHO 2022 Classification of Urinary and Male Genital Tumours 2022 (5th edition) is molecular-driven and contains major revisions compared to the earlier classification from 2016. This revised edition divided renal tumours into four major broad categories: clear cell renal tumours, papillary renal cell tumours, oncocytic and chromophobe renal tumours, and collecting duct tumours. 'Other renal tumours' and 'molecularly defined renal carcinomas' are two other categories that were also included. Transcription factor binding to IGHM enhancer 3 (TFE3)-rearranged, TFEB-altered, elongin C (ELOC)-mutated (formerly TCEB1)-mutated, fumarate hydratase (FH)-deficient, succinate dehydrogenase (SDH)-deficient, anaplastic lymphoma kinase (ALK)-rearranged, and SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1)-deficient renal cell carcinomas are molecularly defined entities. Eosinophilic vacuolated tumours and low-grade oncocytic tumours are classified as emerging entities. Molecularly characterized renal tumours include those with SMARCB1 deficiencies, TFE3 rearrangements, TFEB alterations, ALK rearrangements, ELOC mutations, etc. Thyroid-like follicular carcinoma, eosinophilic vacuolated tumour, and low-grade oncocytic tumour are a few emerging entities of renal tumours. Improved therapy targets for each kidney tumour can be achieved using immunohistochemistry (IHC) and molecular definition updates. This study aims to highlight new developments in the WHO 2022 categorization of renal tumours with regard to diagnostic, morphological, molecular, IHC, clinical, and prognostic updates.

Role of Imaging Spectrum Along With Other Diagnostic Modalities in Rhino-Orbital-Cerebral Mucormycosis (ROCM).

OBJECTIVES: Rhino-orbito-cerebral mucormycosis (ROCM), a rare angio-invasive fungal infection, had become a major outbreak during the second wave of the coronavirus disease (COVID-19) pandemic in India, with over 28,000 reported cases. The purpose of this study was to describe the imaging spectrum of ROCM, which may prove useful in prompt diagnosis, considering its grave prognosis in populations with a high load of immunosuppressed patients (e.g., COVID-19, HIV-AIDS, etc.). MATERIAL AND METHODS: Evaluation of the clinical data and imaging of patients with symptoms suspicious of mucormycosis of the craniofacial region was done. The diagnosis was made using computed tomography (CT) or magnetic resonance (MR) imaging, a biopsy, and culture. The data analysis was done using descriptive statistical methods. RESULTS: The sample group consisted of a total of 36 patients ranging from 33 years to 75 years of age, out of which 31 (86.11%) were male and five (13.8%) were female. A total of 30 (83.33%) patients had a positive correlation with COVID-19 infection, and 29 (80.55%) patients had a positive correlation with diabetes. The major presenting complaints were facial pain and swelling (20 patients; 55.55%). The intracranial spread was seen in 14 (38.88%) patients. Our study demonstrated a mortality rate of 38.88% (14 patients). CONCLUSION: ROCM, once considered to occur predominantly in diabetics, is increasingly being seen in other immunosuppressive patients, such as COVID-19. CT and MR imaging help provide an early diagnosis in conjunction with pathologic and microbiological correlations. Immediate correction of immunosuppression with the initiation of amphotericin B therapy combined with extensive and diligent surgical debridement of the diseased tissue is required.

WHO 2022 updates on follicular cell and c-cell derived thyroid neoplasm.

The latest edition of the WHO Classification of thyroid tumors was released in 2022 and incorporates novel concepts vital to patient management. Thyroid follicular nodular disease is a term used to collectively represent a wide variety of benign and non-neoplastic lesions, including both clonal and non-clonal proliferations that manifest clinically as multinodular goiter. Thyroid neoplasms develop from follicular cells and can be either benign, low-risk, or malignant. To avoid classifying all lesions under 1 cm in diameter as low-risk illnesses, the new classification method highlights the need for subtyping papillary thyroid cancer based on histomorphologic indicators rather than tumor size. Formerly known as the cribriform-morular variety of papillary thyroid carcinoma, this tumor is now more commonly referred to by its more accurate name, cribriform-morular thyroid carcinoma. Its histogenesis is unknown. Similar to the traditional definition of 'poorly differentiated thyroid carcinoma' according to the Turin criteria, the newly defined 'differentiated high-grade thyroid carcinoma' encompasses papillary thyroid cancer, follicular thyroid carcinomas, and oncocytic carcinomas with high-grade characteristics linked to worse prognosis. The squamous cell subtype of anaplastic thyroid cancer has also recently been characterized as a distinct morphologic pattern. In this article, we will discuss the latest revision to the World Health Organization's classification system for thyroid cancer.

Thiazolidinediones: Recent Development in Analytical Methodologies.

The instrumental analytical methods that have been developed and utilized for the determination of thiazolidinedione in bulk medications, formulations and biological fluids have been reviewed after an in-depth analysis of the literature published in a variety of analytical and pharmaceutical chemistry-related journals. The approaches covered by this research, which covers the years 2001-2022, include complex methods for analysis, chromatographic techniques and spectrometric analytical procedures. The mobile phase, flow rate, sample matrix, wavelength and other factors identified in the literature were just a few of the parameters used to evaluate thiazolidinediones. The present review focuses on the published analytical techniques for thiazolidinedione analysis that have been previously identified in the literature. The specified outcomes followed extensive learning, and the most recent advances in analytical methods for the identification of pioglitazone, pioglitazone HCl, rosiglitazone, rosiglitazone maleate and lobeglitazone were reviewed. Additionally, this article briefly discusses features of analytical discovery on thiazolidinediones, which will enable readers to access all discoveries in one place with precise outcomes.

Perspective Toward Complementary & Alternative Medicines in the Prevention of COVID-19 Infection.

Background: Across the globe, people are seeking integrative and holistic measures to prevent coronavirus (COVID-19) infection in the form of complementary and alternative medicines (CAM) with or without conventional medicines. This study was done to know the extent of CAM use for COVID-19 prophylaxis and to know beliefs and attitudes of people related to CAM use in India. Methodology: A pretested and prevalidated questionnaire was circulated on social media. Participants, who completed the online form and gave voluntary consent, were included. The questionnaire included demographic details and questions related to CAM use, preferences with reasons, preparations used, perceived role of CAM in prevention, immunity boosting and side effects, sources of information, etc. Results: Out of 514 responses, 495 were analyzed. 47.07% of respondents were males and 52.93% were females. 66.9% were using CAM for COVID-19 prophylaxis. The association between age, gender, and profession with CAM use was statistically significant (P < 0.05). 41.1% reported CAM use in the past. 36.6% of CAM users were taking "Kadha" and 33% were using ayurvedic medicines. Other frequently used CAM preparations were chyavanprash, giloy, tulsi, ginger, pepper, cloves, honey, sudarshanghanvati, arsenic-30, lemon juice, cinnamon, steam inhalation, ashwagandha, swasarivati, coronil, and warm saline water gargles. 46.9% of the CAM users were on self-medication and 52.3% preferred CAM over allopathy. Conclusion: Complementary and alternative medicine utilization for COVID-19 prophylaxis is widespread and self-medication is prevalent. As no specific cure is available in conventional systems, people believe in traditional medicines more than conventional, yet confusion exists. There is a need of increasing awareness regarding side effects, drug-drug interactions, and self-medication.

A novel case of left atrial and right lung mass turned out to be unconventional metastasis of uterine leiomyosarcoma with a review of literature.

Uterine leiomyosarcoma (ULMS) is a rare malignancy arising from the myometrial smooth muscle wall, and cardiac metastases are extremely rare. Metastasis to the heart is a very unusual finding, and atrial metastasis is even rarer. Here, we report a case of a 45 year old woman who presented with dyspnea and pleural effusion and had a significant history of hysterectomy done for ULMS. Magnetic resonance imaging revealed a left atrial mass, which was resected and revealed to be a metastasis of leiomyosarcoma on histopathology and immunohistochemistry. Metastatic ULMS may rarely present as a left atrial mass with acute clinical presentation. Detailed clinical history and accurate diagnosis are vital for further management.

A case of acute non-typeable Hemophilus influenza infective endocarditis in a patient with hypocomplementemia.

Hemophilus influenzae is a gram-negative bacteria responsible for significant cases of invasive infections, especially in the pediatric population and in immunosuppressed adult patients. Before vaccination, most cases were frequently caused by capsulated or typeable variants. Due to the absence of effective vaccination against the nontypeable variant, it is now responsible for most invasive infections. Predisposing risk factors in adults include asplenia, hypocomplementemia, cancer, human immunodeficiency virus infection, and chronic cardiopulmonary disease. Immunity to the nontypeable variants causing disease is perplexing and not yet wholly described as they are genetically diverse. Infective endocarditis (IE) is a cardiac infection with devastating consequences if not detected earlier and treated appropriately. Gram-positive bacteria are the primary cause of IE overall, followed by gram-negative bacteria. Hemophilus species belong to the HACEK group of gram-negative bacteria responsible for causing IE in the pediatric population more than in adults. Hemophilus species, especially the nontypeable variant, is a rare cause of IE in adults. Here we present a case of IE due to Nontypeable Hemophilus influenzae in a 49-year-old caucasian male with hypocomplementemia.

Prevalence and Patterns of Nodal Metastasis Among Oral Cavity Cancer Patients: A Prospective Observational Study.

Introduction: Lip and oral cavity cancer remains the most common cancer among Indian males and third most common cause of cancer-related deaths in India. Cervical lymph node metastasis is one of the most important prognostic factors in oral cavity cancer. The aim of present study was to estimate the prevalence of nodal involvement and analyse patterns of nodal metastasis in oral cavity cancer patients. Methods: This prospective observational study was conducted between January 2019 and June 2020. Patients of oral cavity squamous cell carcinoma undergoing surgery with simultaneous neck dissection were included. Data pertaining to the clinical profile, treatment and histology details were collected and analysed. Results: A total of 63 patients were included in present study. Out of 63 patients, unilateral neck dissection was performed in 47 (75%), while 16 (25%) underwent bilateral neck dissection. Overall clinical and pathological nodal positivity rates were 75% and 52%, respectively. Level I (48%) followed by IIa (20%) were most commonly involved stations. Combined involvement of level IV and V was seen in only 5%, and no skip metastasis was reported in level IV and V. Conclusion: Level I and II are the most commonly involved stations in oral cavity squamous cell carcinoma (SCC). Oral SCC has a predictable nodal spread pattern with no skip metastasis to level IV/V noted in present study. There is a need for good quality randomised control trials to optimise the treatment protocols in clinically node-positive patients with respect to level IIB and V dissection.

Benign Chondroid Syringoma on Chin: A Case Report and Literature Review.

Chondroid syringoma is a cutaneous adnexal tumor originating from sweat glands origin. It is rare in occurrence and usually benign, having an incidence of 0.01 to 0.098%. As these tumors are uncommon, their diagnosis is missed many times and are misdiagnosed. Hence in any case of facial skin swelling increasing slowly in size, this entity should be kept in mind as one of the possibilities and differential diagnosis. Histopathological examination of the excision biopsy gives the definitive confirmatory diagnosis. Surgically excising the swelling locally along with a surrounding normal tissue cuff is the standard treatment given which prevents recurrence. Hereby we present a 35-year-old case of facial chondroid syringoma having a focal component of eccrine hidrocystoma, keratinous cyst as well as syringocystadenoma papilliferum on the chin that was clinically suspected to be an epidermoid cyst or mucocele.

Gliomatosis Peritonei and Its Relation to Teratoma: Role of Imaging and Histological Aspects.

Gliomatosis peritonei (GP) is a rare disease, usually associated with immature ovarian teratoma. GP may be rarely associated with mature ovarian teratoma. GP is composed of mature glial tissue elements, which histopathological examination can further confirm. Benign glial implants usually involve the omentum, peritoneum and lymph nodes. Many benign and malignant peritoneal diseases may mimic GP on clinical examination. GP may be confused with peritoneal carcinomatosis on computed tomography (CT) scan. A microscopic examination from peritoneal mass biopsy helps to rule out differential diagnosis. GP consists of mature glial tissue and is regarded as grade 0 according to the WHO grading of immature teratoma (IT). GP corresponds to a good prognosis with occasional cases showing malignant evolution.

A rare case of ovarian gonadoblastoma flourishing into malignant mixed germ cell tumour with review of literature.

Gonadoblastoma is an extremely rare neoplasm of the ovary showing admixture of germ cells and sex cord cells. It may be associated with gonadal dysgenesis. Gonadoblastoma cells may give rise to individual germ cell tumours or mixed germ cell tumours with variable tumour components. Very few cases of ovarian gonadoblastoma admixed with malignant germ cell tumours have been recorded worldwide. Because of the rareness of the tumour, a component of gonadoblastoma might be overlooked on microscopic examination. Here we report a rare case of ovarian gonadoblastoma giving rise to an admixture of immature teratoma and dysgerminoma. We discuss microscopic features, immunohistochemistry findings and review of literature.

The Implication of New Developments in Hemophilia Treatment on Its Laboratory Evaluation.

Laboratories monitor hemophilia replacement therapy by specific coagulation factor measurement before and after the infusion of human-derived or recombinant factors. Bypassing agents are now used for patients with inhibitors. Recently, modified long-acting coagulation factors have been introduced, for which discrepant results may be expected when the measurement is performed with one-stage clotting or chromogenic assays. Currently, novel drugs not based on coagulation factors are being developed and further tested in clinical studies. These drugs do require new methods, and therefore, laboratory evaluation of hemophilia will undergo dramatic changes in the near future. Accordingly, present laboratory methods for monitoring, which include one-stage clotting or chromogenic assays, used to measure either factor VIII (FVIII) or factor IX (FIX), will not be sufficient. A thrombin generation test (TGT) or thromboelastometry may be used to monitor bypassing agents. For measuring modified long-acting coagulation factors, chromogenic assays will be probably more suitable than one-stage clotting assays. Novel drugs that are not based on coagulation factors, such as emicizumab, fitusiran, or concizumab, will require alternative methods.

Hand and Foot Glomus Tumors: Significance of MRI Diagnosis Followed by Histopathological Assessment.

Introduction Glomus tumors are benign lesions with hamartomatous proliferation in the neuromyoarterial glomus bodies. Glomus tumors are small, reddish, painful blue nodules usually underneath the fingernail. Objectives This study is mainly focusing on magnetic resonance imaging (MRI) findings of glomus tumors on T1, T2, short inversion time inversion recovery (STIR), and post-gadolinium images. Further study of clinical and histopathological findings to support the radiological diagnosis. Material & methods The retrospective study included an elaborate study of MRI imaging findings of 24 cases of glomus tumors of the hand and leg at a tertiary care center in Udaipur. Patients with imaging findings confirmed on histopathology were included in the study. MRI study was conducted using a 3-T MR unit and a high-spatial-resolution module. Results A total of 24 cases of glomus tumors were diagnosed during the six years between January 2015 and November 2020. Out of 24 patients, 14 were female and 10 were male. The most common site of involvement was a hand, followed by a foot. All 24 cases showed isointense to hyperintense lesions on T2-weighted images with a conspicuous hyperintense lesion on STIR images. Further histopathological examination confirmed the diagnosis showing nests of monomorphic tumor nuclei arranged in a perivascular pattern. Conclusion Glomus tumors can present with variable pain. A high index of suspicion is needed for diagnosis. Contrast MRI has a significant role in the diagnosis of glomus tumors. The clinical and histopathological picture further confirms the diagnosis. MRI imaging is further supportive to differentiate postoperative fibrosis from residual or recurrent tumors.

A Rare Case of Endolymphatic Sac Tumour: Clinical, Radiological, Pathological and Immunohistochemical Findings with Review of Literature.

Endolymphatic sac tumour (ELST) is a rare low grade malignant epithelial tumour of the petrous temporal bone, thought to arise from papillary epithelium of the endolymphatic sac. They may occur sporadically or in association with Von-Hippel Lindau disease. ELST is extremely rare neoplasm with benign histopathological appearance and clinically destructive behaviour. Because of the rarity of this tumour, it can easily be confused with other tumours such as paraganglioma, middle ear adenoma, metastatic carcinomas or choroid plexus papilloma. We report here a rare case of ELST with review of literature and discuss the differentiating features of ELST from its mimickers, showing a papillary configuration.

A Rare Case of Pulmonary Embolism in a Patient With Interrupted Inferior Vena Cava and Polysplenia.

Interrupted inferior vena cava (IVC) with azygos continuation is one of the anomalies of the inferior vena cava (AIVCs) where venous drainage of the lower extremities is accomplished through a dilated azygos system and is usually accompanied by other congenital malformations such as polysplenia. AIVCs are more common in patients younger than 40 presenting with deep venous thrombosis (DVT). However, pulmonary embolism (PE) in association with AIVCs remains underreported. In this article, we describe a rare case of a 23-year-old male who presented with syncope secondary to sub-massive pulmonary embolism in the setting of an interrupted vena cava draining directly into the azygous vein.