Non-IgG4-Related Fibrosing Mediastinitis Diagnosed on Core Needle Biopsy and Treated with Steroids: A Case Study and Review of the Differential Diagnoses.

OBJECTIVES: This study aimed to investigate the histological characteristics and treatment efficacy of non-immunoglobulin G4-related fibrosing mediastinitis and discuss differential diagnoses for this rare entity. METHODS: We present a case study of non-immunoglobulin G4-related fibrosing mediastinitis diagnosed on core biopsy and treated with steroids. A total of four 18-gauge core needle biopsy specimens were obtained for surgical pathology. Analysis of the patient's medical history, radiological characteristics of fibrosing mediastinitis, histological features, immunohistochemistry results, the differential diagnosis and treatment efficacy of different types of fibrosing mediastinitis was performed. RESULTS: This report describes a unique presentation of fibrosing mediastinitis (syncope and weight loss) that was concerning for malignancy. Histological, laboratory and radiographical studies confirmed the diagnosis of non-immunoglobulin G4-related fibrosing mediastinitis. The patient received corticosteroid treatment which showed marked improvement after 1 month of treatment. CONCLUSIONS: Fibrosing mediastinitis is an extremely uncommon entity with unknown pathogenesis, and it is more important to rule out malignancy and infection than to delineate between fibrosing mediastinitis and IgG4-related disease. In doing this, we may reasonably initiate a trial of corticosteroids which may prove beneficial, as in this patient. More studies on the pathogenesis of fibrosing mediastinitis are necessary to guide better directed treatments.

Diagnostic Challenges and Long-term Outcomes of Neuroendocrine Carcinoma of the Cervix: A Case Series.

Neuroendocrine carcinomas (NECs) of the cervix are rare, aggressive malignancies that are challenging to diagnose and treat. They are high-grade lesions that often share features with poorly differentiated adenocarcinoma and squamous cell carcinoma. NECs are classified into large-cell or small-cell subtypes but can often have a mixed appearance or occur concurrently with a squamous or adenocarcinoma. Diagnosis is dependent on tissue sampling, histomorphology, and immunohistochemistry. Eight cases of NEC were retrieved from the Department of Pathology at our institution from 2008 to 2022. Tumor slides were reviewed and evaluated by 2 independent pathologists. Seven of 8 patients tested positive for neuroendocrine markers, including CD56, synaptophysin, and chromogranin. We discuss the diagnostic challenges, review the histopathology, and describe the treatment courses and clinical outcomes. This case series reveals that traditional markers, such as p16, p63, and p40, may be focally positive in NEC and should not be considered a confirmation of squamous cell carcinoma. Patient outcomes can be affected by delays in diagnosis, misdiagnosis, and inadequate treatment when NEC is not considered in the initial differential diagnosis.

Seronegative Autoimmune Hepatitis: A Rare Manifestation of COVID-19.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus responsible for the coronavirus pandemic in 2019, commonly causes hepatic dysfunction. Liver injury ranges from mildly elevated liver enzymes to fulminant liver failure. Interestingly, there are cases that suggest a relationship between autoimmune hepatitis (AIH) in patients who either contracted coronavirus disease in 2019 (COVID-19) or were vaccinated against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We present a case of a 39-year-old female without a significant past medical history who presented with two weeks of jaundice, abdominal pain, nausea, and diarrhea. She had significantly elevated liver enzymes and conjugated hyperbilirubinemia. She also tested positive for SARS-CoV-2 but denied any respiratory symptoms; her vaccination status was up to date. She denied taking hepatotoxic agents, and the workup was negative for acute viral hepatitis. The F-actin antibody level was 22 units, but serum immunoglobulin (IgG), anti-nuclear (ANA), anti-smooth muscle, anti-mitochondrial, anti-liver/kidney microsomal-1, anti-soluble liver antigen, and anti-neutrophil cytoplasmic antibodies levels were not elevated. Computerized tomography of the abdomen and pelvis revealed hepatic hemangiomas. Eventually, a liver biopsy was performed, and histology showed active lymphoplasmacytic hepatitis with prominent regenerative changes and areas of confluent necrosis. The histologic findings, along with the patient's clinical course, were suggestive of autoimmune hepatitis. The patient was started on systemic steroids with an improvement of abdominal pain and jaundice, as well as an improvement of her liver chemical profile. She was discharged with plans for hepatology clinic follow-up. Here, we present a rare case of seronegative AIH in a patient with a recent COVID-19 infection and discuss the potential underlying mechanism. We call for further investigation into the relationship between autoimmune dysfunction and COVID-19, as well as the pathophysiology behind it. Analyzing how the virus causes autoimmune dysfunction may allow clinicians to more effectively treat patients suffering from sequelae of COVID-19 infection, and it is important not to exclude autoimmune hepatitis from the differential based on the initial autoimmune workup.

Paratesticular Ewing's sarcoma.

This case report follows a 23-year-old man who presented with a painful right scrotal mass which was found to be a paratesticular vascular solid mass on ultrasound, and after uncomplicated orchiectomy, was revealed to be a high-grade extraskeletal Ewing's sarcoma. Diagnosis leading up to the orchiectomy was primarily clinical with only ultrasound used in identification and characterization of the paratesticular mass. Paratesticular masses are more commonly benign, and ultrasound is the first modality, with computed tomography and magnetic resonance imaging providing more definitive findings. We discuss imaging findings and histopathology of this rare tumor with an uncommon presentation.

Pediatric combined hepatocellular-cholangiocarcinoma (cHCC-CC) with neuroendocrine features: distinguishing genetic alterations detected by chromosomal microarray.

BACKGROUND: Liver tumors exhibiting hepatocellular, cholangiocarcinoma, and neuroendocrine features are extremely rare, with only five cases reported in the literature. CASE PRESENTATION: We present an unusual case of a combined hepatocellular-cholangiocarcinoma (cHCC-CC) with neuroendocrine features in a pediatric patient. A 16-year-old presented with abdominal pain and a 21.0 cm mass in the right hepatic lobe with extension into the left lobe. Histology showed a poorly differentiated tumor with a solid, tubuloglandular, and microcystic architecture. Immunohistochemistry results were negative for hepatic markers, positive for markers of biliary differentiation, and positive for neuroendocrine differentiation. The neoplasm was reviewed at several institutions with differing diagnoses. Single nucleotide polymorphism (SNP) chromosomal microarray (CMA) showed large deletions within chromosomes 6q and 13q in both the hepatocellular-like areas and the cholangiocarcinoma-like areas, with additional large deletions in the cholangiocarcinoma-like areas, supporting origin from hepatocellular carcinoma. The final diagnosis was a cHCC-CC with neuroendocrine features. CONCLUSIONS: Diagnosis of cHCC-CCs relies predominately on histomorphology, as per the 2018 International Consensus Group on the nomenclature of cHCC-CC. These findings in this case support that the pathological classification of these lesions be based on molecular data, which could better direct treatment. Further classification of cHCC-CCs and determination of their clinicopathological relevance will require more interobserver consistency and continued molecular profiling of these lesions.

Ewing's sarcoma of the male external genitalia: a case report and review of the literature.

BACKGROUND: Ewing's sarcoma (ES) within the genitourinary tract are relatively unheard of and those within the external male genitalia are even rarer. To our knowledge, this is the first known case of primary ES within the paratesticular region in an adult. CASE PRESENTATION: We present a case of a 24-year-old man with a right sided testicular mass on examination that was initially characterized as an adenomatoid tumor on ultrasound. After the patient was lost to follow up over the course of 9 months, the testicular mass grew significantly and was excised with pathology revealing primary paratesticular Ewing's sarcoma. This rare case emphasizes the importance of elucidating between the broad differentials of paratesticular masses, including the rare presentation of primary ES and adds a review of the literature of ES in the external male genitalia. CONCLUSIONS: Rare differentials such as this case should be considered in patients with paratesticular masses. Further diagnostic and management algorithms for extraosseous Ewing Sarcoma, particularly in the adult population, are warranted.

Metastatic Basal Cell Carcinoma: An Unexpected Diagnosis in a Young Patient With Back Pain.

ABSTRACT: Basal cell carcinoma (BCC) portends a notoriously favorable prognosis in most patients with morbidity limited to localized destruction and recurrence. Metastatic BCC (mBCC) is an unexpected outcome affecting less than 1% of patients with a known primary lesion and predominantly involves regional lymph nodes. Reports of isolated bone involvement and spinal cord compression are rare. In the cases we identified in the literature, patients presented with massive primary lesions on the trunk that had been present for years and that were often still present at the time of diagnosis. Additionally, histology of distant metastatic lesions typically reveals aggressive subtypes. Herein, we report a case of mBCC in a patient with a history of BCC involving the cheek; the lesion was excised more than 10 years ago. He was referred to our institution for acutely worsening back pain and multifocal neurologic deficits. Clinical symptoms and radiographic findings demonstrated isolated bone involvement, with multiple lytic bone lesions and spinal cord compression noted on imaging studies. Biopsy revealed nests of small basaloid cells with peripheral palisading and immunohistochemical staining consistent with the unexpected diagnosis of mBCC, nodular subtype. Our case illustrates that a historically resected primary lesion may cause distant metastasis after a decade and that nonaggressive subtypes can also be implicated. We also provide insight into the potential pathogenesis of this manifestation.

Endomyocardial biopsy facilitates diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA): a case report.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small vessel leukocytoclastic vasculitis that affects multiple organs and is often associated with anti-neutrophil cytoplasmic antibody (ANCA). EGPA presenting with cardiac involvement is often ANCA-negative, difficult to diagnose, and often fatal. The treatment and prognosis and can be quite different for other conditions included in the differential diagnosis. Imaging modalities have proven unreliable, and the skin is the most commonly biopsied site for histological diagnosis. CASE SUMMARY: We report a case of a 55-year-old Hispanic man who presented with a non-ST-elevated myocardial infarction, reduced ejection fraction heart failure, and hypereosinophilia. The patient's clinical history also included poorly controlled asthma and sinonasal polyps. Despite ANCA titers within the normal range and a skin biopsy lacking evidence of EPGA, high clinical suspicion prompted an endomyocardial biopsy on day nine from hospital admission which facilitated the diagnosis of ANCA-negative EGPA-induced cardiomyopathy. Six months of follow-up revealed that therapeutic response, as measured by the cardiac ejection fraction, directly correlated with medication compliance. CONCLUSION: Endomyocardial biopsy aids in the diagnosis of EGPA and initiates use of appropriate therapy.

Parkinson's disease increases the risk of perioperative complications after total knee arthroplasty: a nationwide database study.

PURPOSE: Therefore, the purpose of this study was to evaluate the short-term perioperative outcomes of PD patients who underwent total knee arthroplasty (TKA). We specifically evaluated: (1) perioperative surgical and medical complications; (2) lengths of stay (LOS); and (3) total hospital charges. METHODS: The Nationwide Inpatient Sample was used to identify PD patients who underwent TKA between 2002 and 2013. To control for potential confounders, PD TKA and non-PD TKA patients were propensity score matched (1:3) based on age, sex, ethnicity, Charlson Comorbidity Index, and insurance type. A total of 31,979 PD and 95,596 non-PD TKA patients were included. RESULTS: PD patients had a 44% higher risk of suffering from any complication (OR 1.44; 95% CI 1.35-1.54), a 45% increased risk for any medical complication (OR 1.45; 95% CI 1.36-1.55), and a 9% higher risk for any surgical complication (OR 1.09; 95% CI 0.84-1.41). Compared to the matched cohort, PD patients had a mean LOS that was 6.5% longer (95% CI 5.46-7.54) and mean total hospital charges that were 3.05% higher (95% CI 1.99-4.11). CONCLUSIONS: PD patients are more likely to have postoperative complications, longer LOS, and higher costs after TKA than non-PD TKA patients. Since many of these complications can be prevented, a team-based multi-specialty patient optimization is needed. LEVEL OF EVIDENCE: Level III, therapeutic study.

A Comparison of Relative Value Units in Primary versus Revision Total Ankle Arthroplasty.

INTRODUCTION: To determine the effort required to provide a service, the United States Medicare uses Relative Value Units (RVUs). Consequently, higher RVUs are assigned to the procedures or services that require more effort, which ultimately means the physician will be properly compensated for the additional effort required. In total ankle arthroplasty (TAA), revision cases usually are more technically challenging and require more effort than primary TAA. Therefore, the purpose of this study was to compare the: 1) RVUs; 2) length-of-surgery; 3) RVU per unit of time between primary and revision total ankle arthroplasty; and 4) the individualized idealized surgeon annual cost difference analysis. MATERIALS AND METHODS: We utilized the American College of Surgeons, National Surgical Quality Improvement Program database from 2008 to 2015 to identify patients who underwent either a primary Current Procedural Terminology [CPT]: 27702) or revision (CPT: 27703) TAA. There were a total of 653 patients, 586 of which underwent a primary, and 67 who underwent a revision, TAA. The mean RVUs, length of surgery (in minutes), and RVU per minute, were calculated. Dollar amount per minute, per case, per day, and per year, to find an individualized idealized surgeon annual cost difference, were also calculated. An analysis of variance was used to compare variables between primary and revision TAA. A p-value of less than 0.05 was used to determine statistical significance. RESULTS: The mean RVU was significantly higher in revision versus primary TAA (16.93 vs. 14.41, p=0.001). However, there was no significant difference in the mean lengths of surgery between primary and revision TAA (160 vs. 157 minutes, p=0.613). Additionally, the mean RVU per minute was significantly higher in revision versus primary TAA (0.13 vs. 0.10, p=0.001). CONCLUSION: Based on the results of this study, it appears that revision TAA cases are appropriately assigned a higher RVU per minute for performing them as they require more effort and are more challenging compared to the primary TAA. Furthermore, not only did the revision cases have lower mean lengths of surgery, but they also maintained a higher RVU per minute. Therefore, orthopaedists can use this information to further help them yield the best potential practice design.

Demographics of Tennis-Related Injuries that Presented to Emergency Departments in the United States.

INTRODUCTION: Tennis injuries are not uncommon, and efforts have been made to reduce the risk of these injuries. There are a number of different factors that have been shown to influence injury rates of tennis players, in particular patient-related risks. Therefore, the purpose of this study was to investigate the epidemiology of tennis-related injuries. Specifically, we evaluated: 1) demographics; 2) incidence and trends of injuries; and 3) incidence and trends of body parts that were injured. MATERIALS AND METHODS: This study utilized the National Electronic Injury Surveillance System (NEISS) database to collect all tennis-related sprains/strains that occurred between January 1, 2010 and December 31, 2016. Patients were stratified into four groups based on their age: 13 years and younger, 14 to 29 years, 30 to 54 years, and 55 years and older. Race was reported as white, black, Hispanic, Asian, Native Hawaiian, American Indian, Multiracial, and not stated. The various types of tennis-related injuries that occurred, and the different body parts that were affected were identified. RESULTS: There were a total of 150,747 tennis-related injuries that occurred during the study period. Thirty-nine percent of all injuries occurred in patients aged 55 years and older, 25% in patients between the ages of 14 and 29 years, 24% occurred between the ages of 30 and 54 years, and 12% occurred in patients aged 13 years and younger. In terms of gender, 56% were men and 44% were women. In terms of race, injuries most commonly occurred in patients who were white (48%). The most common overall injury pattern was sprains/strains, which represented 32% of all reported injuries, followed by fractures (15%) and contusions (11%). The most commonly injured body parts were the ankle (13%), wrist (9.4%), upper-trunk (8.5%), knee (7.8%), and lower-trunk (6.7%). Over the study period, there was a significant decrease in ankle injuries (R2=0.691, p=0.021) and a significant increase in upper-trunk injuries (R2=0.695, p=0.020). CONCLUSION: The present study provided important insight regarding tennis-related injuries in terms of demographics, injury types, and injury patterns. This information is paramount for the future treatment of all tennis-related injuries and for the implementation of prevention strategies for those injuries which are most prevalent.