Methodist Le Bonheur Healthcare

Background: Historical trials of adjunctive dexamethasone for management of neurologic symptoms in malignant spinal cord compression (MSCC) utilized high doses of dexamethasone for 3days then tapered over an additional 12days. There is limited evidence on the current practice of adjunctive dexamethasone. Objective: The purpose of this study was to evaluate various dosing strategies of dexamethasone in MSCC. Methods: This was a retrospective, single-site, analysis of hospitalized patients presenting with MSCC and received dexamethasone. Patients were grouped into 2 cohorts; an acute course (AC, ≤3days) and a prolonged course (PC, >3days), based on length of high-dose dexamethasone therapy (16mg/day). Primary outcome was cumulative dexamethasone dose between groups. Other additional outcomes evaluated safety and efficacy. Results: A total of 26 patients were included, with 14 patients in the AC group and 12 in the PC group. The median cumulative dexamethasone dose was significantly lower in the AC group compared to the PC group (117mg vs 165mg, P < 0.001). Time to IV-to-PO conversion was significantly delayed in the PC group (6days vs 1day, P < 0.001). There were no significant differences in additional outcomes. Conclusion: The findings of this study provide insights into consideration of early tapering strategies to decrease dexamethasone exposure and lessen the risk of adverse effects. Despite the retrospective nature of this small study, the findings of this study highlight the need for more standardized guidance to optimal dose, duration, and tapering strategy for dexamethasone use in patients with MSCC.

DEK::AFF2 squamous cell carcinoma is a recently recognized entity characterized by a non-viral-related non-keratinizing squamous cell carcinoma with chromosomal rearrangement between DEK on 6p22.3 and AFF2 on Xq28. This neoplasm is associated with an aggressive clinical behavior, particularly regarding local recurrences and distant metastases even with its deceptively bland histomorphology. In this article, we report on 2 patients with DEK::AFF2 squamous cell carcinomas, which were only diagnosed at the time of recurrence. Patient #1 was originally misdiagnosed with inverted papilloma. This occurred 3 years previously, and at second presentation the tumor was recurrent in the nasal cavity and paranasal sinuses. Patient #2 was identified at second recurrence of a "nasopharyngeal carcinoma," initially diagnosed in the nasopharynx 5 years earlier, with a subsequent recurrence in the middle ear 2 years later, involving the mastoid process. The immunophenotypic and molecular work-up, as well as the management of these patients, are discussed. In our review of the English-language literature on DEK::AFF2 squamous cell carcinomas, we identified 41 patients, including our 2 patients. Of these, 26 had information on their clinical course. Sixty-nine percent of patients reported a history of locoregional recurrence, and 19% reported a history of distant metastases, with an average follow-up duration of 36 months after multimodality therapies. Given our improved understanding of DEK::AFF2 squamous cell carcinoma as an aggressive tumor, it is crucial to differentiate this neoplasm from other potential histomorphologic mimickers to ensure early and accurate diagnosis.

This case study focuses on a 49-year-old woman several years after a right transplant nephrectomy for delayed renal hemorrhage. The patient had an incidental finding of a right external iliac artery pseudoaneurysm measuring 8.1 cm at its largest dimension. Of note, the patient had undergone cardiac catheterization several months prior with right common femoral artery access. This study aimed to implement endovascular repair of posttransplant nephrectomy iliac artery pseudoaneurysms as a less invasive treatment option as compared with open repair in suitable candidates. Percutaneous access of the right common femoral artery with the deployment of a 9 × 50 mm covered stent within the right external iliac artery with the successful exclusion of the pseudoaneurysm. Postoperative course was uncomplicated, and the patient was discharged home on dual antiplatelet therapy. The patient's symptoms completely resolved after the intervention. She underwent follow-up surveillance scans, which redemonstrated a patent external iliac stent and excluded pseudoaneurysm. Endovascular repair with a covered stent for posttransplant nephrectomy iliac artery pseudoaneurysms is a safe, minimally invasive, and valuable treatment option in the absence of underlying infection.

The pathologic assessment of colorectal carcinoma specimens plays a crucial role in the therapeutic management of patients and disease prognostication. The TNM staging system is used globally and is a critical component of colorectal carcinoma pathology reporting. However, our experience informs us that there are significant variations in the assignment of the TNM stage, both between pathologists and between hospital centers. We identify several potential reasons for this, among them suboptimal gross and microscopic assessment of colorectal resection specimens and, later, nonuniformity in applying criteria set forth in pathologic TNM staging guidelines. In addition, some defining characteristics of the staging system remain poorly defined. We aim to enlist those issues with potential remedies to improve reproducibility and, therefore, multidisciplinary discussion.

Background: Transthyretin cardiac amyloidosis (ATTR) is a life-threatening, debilitating disease caused by abnormal formation and deposit of transthyretin (TTR) protein in multiple tissues, including myocardial extracellular matrix. It can be challenging to diagnose due to the myriad of presenting signs and symptoms. Additionally, numerous TTR mutations exist in certain ethnicities. Interestingly, our patient was discovered to have a very rare Gly67Ala TTR mutation typically not found in individuals of Asian descent. Recent advances in cardiovascular imaging techniques have allowed for earlier recognition and, therefore, management of this disease. Although incurable, there are now new, emerging treatments that are available for symptom control of cardiac amyloidosis, making early diagnosis vital for these patients, specifically their quality of life. Case summary: We outline a case of a 50-year-old Asian female who was initially hospitalized for nausea and vomiting and persistent orthostatic hypotension. She underwent a multitude of laboratory and imaging tests, resulting in a diagnosis of cardiac amyloidosis, which was confirmed to be due to a rare TTR mutation via genetic testing. Conclusions: Our objective is to describe various TTR mutations, existing diagnostic imaging modalities, and available treatments, as well as highlight the importance of early screening and awareness of cardiac amyloidosis, allowing for quicker diagnosis and treatment of this disease.

Introduction: When status epilepticus (SE) persists despite emergent and urgent treatment with antiepileptic drugs (AED), it is referred to as refractory SE (RSE). Guidelines recommend quickly escalating therapy by either repeating initial agents, choosing an alternative AED, or even initiating continuous intravenous (CIV) anesthetic agents. Adherence to a standardized treatment protocol for general treatment of SE has been associated with better seizure control and shorter intensive care unit and hospital length of stay, but no literature exists evaluating protocols aimed at treating patients for RSE with CIV anesthetic agents. Due to the lack of literature in this area, we aimed to determine the benefits of implementing a standard protocol for use of CIV anesthetic agents in the treatment of RSE. Methods: A retrospective analysis of adult patients admitted to the Methodist University Hospital (MUH) with a diagnosis of RSE was conducted from January 1, 2011 – July 31, 2021. In 2018, MUH began using a standard protocol for treatment for RSE. Therefore, patients were grouped based on whether they received treatment prior to or after implementation of the protocol. Discharge disposition, duration of hospitalization, time to seizure termination, and rates of adverse effects were evaluated. Results: A total of 64 patients were included, with 16 (20%) pre-implementation and 48 (80%) post-implementation. Patients included were predominantly female (61%), had a history of seizures (62.5%), and a mean age of 59 years. For the primary outcome, the percent of patients with in-hospital death or discharge to hospice was numerically lower in the post-implementation group (25% vs 16.7%, p=0.48). Patients who received treatment through the protocol also had a numerically shorter time to seizure termination (21 vs 26.7 hours, p=0.19). Duration of hospitalization (13.8 vs 14.4 hours, p=0.93) was similar between groups. Conclusions: Although there was no statistical difference in the primary outcome between groups, there was a nearly 10% improvement in survival, as well as reduced time to seizure termination, through implementation of an RSE protocol. Further research is needed to determine the overall impact of similar protocols amongst a larger cohort of patients.

Introduction: Apixaban is currently the only oral direct factor Xa inhibitor approved for treatment and prevention of venous thromboembolism (VTE) in patients on hemodialysis. Exclusion of dialysis patients from major clinical trials results in prescriber uncertainty regarding the optimal dose of apixaban for VTE treatment in this population. This study sought to characterize the variance in apixaban prescribing patterns for thrombotic indications other than atrial fibrillation. Methods: This retrospective, multi-center, descriptive study analyzed apixaban dosing patterns for hospitalized chronic dialysis patients with history of thrombosis. The primary outcome was incidence of deviation from manufacturer recommendations for dosing, assessed for either a new start or receipt prior to hospitalization. Secondary outcomes included observation of recurrent thrombotic and bleeding event rates during subsequent hospitalizations. Patients were analyzed into subgroups according to type of thrombotic indication for treatment. Data are reported with descriptive statistics. Results: A total of 101 patients were included. Deviations in recommended dosing were observed in 53 of 80 (66.2%) patients receiving apixaban for treatment of acute or chronic thrombosis. Of 44 patients started on apixaban during hospitalization for the indication of acute VTE, a dose deviation was observed in 79.5% of patients. Rates of rehospitalization for recurrent thrombotic events and bleeding were 11.8% and 9.9%, respectively. Conclusion: Variation in apixaban prescribing practices for the treatment of VTE in dialysis patients is common, suggesting an urgent need for prospective studies and updated dosing guidance to optimize safety with apixaban use in this population.

Background: This study aims to describe COVID-19–related clinical outcomes after immunotherapies (ICIs) for cancer patients. Methods: In this meta-analysis, we searched databases to collect data that addressed outcomes after immunotherapies (ICIs) during the COVID-19 pandemic. The primary endpoint was COVID-19–related mortality. Secondary endpoints included COVID-related hospital readmission, emergency room (ER) visits, opportunistic infections, respiratory complications, need for ventilation, and thrombo-embolic events. Pooled event rates (PERs) were calculated and a meta-regression analysis was performed. Results: A total of 262 studies were identified. Twenty-two studies with a total of forty-four patients were eligible. The PER of COVID-19–related mortality was 39.73%, while PERs of COVID-19–related ER visits, COVID-19–related pulmonary complications, and COVID-19–related ventilator needs were 40.75%, 40.41%, and 34.92%, respectively. The PER of opportunistic infections was 34.92%. The PERs of the use of antivirals, antibiotics, steroids, prophylactic anticoagulants, and convalescent plasma were 62.12%, 57.12%, 51.36%, 41.90%, and 26.48%, respectively. There was a trend toward an association between previous respiratory diseases and COVID-19–related mortality. Conclusion: The rates of COVID-19–related mortality, ER visits, pulmonary complications, need for a ventilator, and opportunistic infections are still high after ICIs during the COVID-19 pandemic. There was a trend toward an association between previous respiratory diseases and COVID-19–related mortality.

Pineoblastomas are rare, malignant pineal parenchymal tumors encountered predominantly in pediatric patients. They are distinct from primitive neuroectodermal tumors (PNET) at other sites in that they exhibit photosensory differentiation including Flexner–Wintersteiner rosettes and fleu-rettes. Diagnosis can be challenging since they share morphologic and immunohistochemical fea-tures with other embryonal tumors and the developing pineal gland. Pineal anlage tumor is a rare variant of pineoblastoma defined by divergent neuroepithelial and ectomesenchymal differentia-tion without an endodermal component. To date the five published cases of anlage tumors be-haved aggressively. We describe a case series which includes one patient with pineal anlage tu-mor and the clinical, radiological and pathological characteristics of pediatric pineoblastomas.