Sclerosing epithelioid fibrosarcoma associated with WRN gene variant presenting as chronic dyspnea and pathologic cervical fracture: a case report and review of the literature.

BACKGROUND: Sclerosing epithelioid fibrosarcoma is an aggressive sarcoma subtype with poor prognosis and limited response to conventional chemotherapy regimens. Diagnosis can be difficult owing to its variable presentation, and cases of sclerosing epithelioid fibrosarcoma are rare. Sclerosing epithelioid fibrosarcoma typically affects middle-aged individuals, with studies inconsistently citing gender predominance. Sclerosing epithelioid fibrosarcoma typically arises from the bones and soft tissues and often has local recurrence after resection and late metastases. Immunohistochemical staining typically is positive for mucin-4. Werner syndrome is due to an autosomal recessive mutation in the WRN gene and predisposes patients to malignancy. CASE PRESENTATION: A 37-year-old Caucasian female presented to the emergency department with 4 months of dyspnea and back pain. She had been treated for pneumonia but had persistent symptoms. A chest, abdomen, and pelvis computed tomography showed near-complete right upper lobe collapse and consolidation, mediastinal lymphadenopathy, lytic spinal lesions, and a single 15-mm hypodense liver nodule. The patient underwent a transthoracic right upper lobe biopsy, bronchoscopy, endobronchial ultrasound with transbronchial lymph node sampling, and bronchoalveolar lavage of the right upper lobe. The bronchoalveolar lavage cytology was positive for malignant cells compatible with poorly differentiated non-small cell carcinoma; however, the cell block materials were insufficient to run immunostains for further investigation of the bronchoalveolar lavage results. Consequently, the patient also underwent a liver biopsy of the liver nodule, which later confirmed a diagnosis of sclerosing epithelioid fibrosarcoma. Next-generation sequencing revealed a variant of unknown significance in the WRN gene. She was subsequently started on doxorubicin. CONCLUSION: Sclerosing epithelioid fibrosarcoma is a very rare entity, only cited approximately 100 times in literature to date. Physicians should be aware of this disease entity and consider it in their differential diagnosis. Though pulmonary involvement has been described in the context of sclerosing epithelioid fibrosarcoma, this malignancy may affect many organ systems, warranting extensive investigation. Through our diagnostic workup, we suggest a possible link between sclerosing epithelioid fibrosarcoma and the WRN gene. Further study is needed to advance our understanding of sclerosing epithelioid fibrosarcoma and its clinical associations as it is an exceedingly rare diagnosis.

Acute Respiratory Distress Syndrome: A Rare Manifestation of Rhinovirus Infection.

Human rhinovirus (HRV) is a common cause of respiratory infections. HRV-related lower respiratory tract infections, including community-acquired pneumonia, are seldom seen in the clinical setting, and progression to acute respiratory distress syndrome (ARDS) is even rarer. We report on a case of a young immunosuppressed host who presented to the hospital for respiratory distress. She was diagnosed with HRV-related pneumonia, which rapidly progressed to ARDS based on clinical examination. After orotracheal intubation and mechanical ventilation with a low tidal volume strategy, she made a rapid recovery. This case highlights the importance of understanding that HRV may be an etiology of community-acquired pneumonia in immunosuppressed hosts and that ARDS may be a complication of this infection. Rapid recognition and clinical suspicion are important to the care of these patients, as ARDS has a high mortality rate.

Disseminated Histoplasmosis in a Patient With Acquired Immunodeficiency Syndrome in a Non-Endemic Region (California).

Histoplasmosis is caused by infection with Histoplasma capsulatum (H. capsulatum). Progressive disseminated histoplasmosis is a more severe form of histoplasmosis and is seldom diagnosed in non-endemic regions of the world owing to the fungus's geographical distribution. In the United States (USA), Histoplasma capsulatum is classically known to be endemic to the Mississippi and Ohio River valleys, and cases in non-endemic areas, such as the southwest USA, are exceedingly rare. Patients with acquired immunodeficiency syndrome (AIDS) are at risk for infection with H. capsulatum, and failure to recognize and treat histoplasmosis may be devastating to patients. In non-endemic regions, the proposed mechanism for disseminated histoplasmosis in AIDS patients is reactivation of a previous infection. Here, we present the case of a young male patient who presented to a southern California hospital with diarrhea, was diagnosed with AIDS, and developed acute hypoxic respiratory failure. Chest imaging revealed diffuse reticulonodular opacities, and histoplasmosis was confirmed by urine and serologic examination. He was subsequently treated with liposomal amphotericin B and safely discharged from the hospital with oral itraconazole therapy. This case contributes to the current limited body of literature citing histoplasmosis infections in California, and clinicians should consider histoplasmosis as a differential diagnosis in non-endemic regions.

A Rare Case of Coccidioidomycosis Meningitis.

Disseminated coccidioidomycosis is a fungal disease endemic to the Southwest United States as well as South and Central America. This dimorphic fungus typically manifests as pulmonary infection; however, there are very rare instances of extrapulmonary disseminated disease especially in immunocompromised hosts. Here, we present a 46-year-old immunocompromised patient with a recent diagnosis of pulmonary coccidioidomycosis that initially presented with acute respiratory failure and was found to have coccidioidomycosis meningitis. This case highlights that despite early and adequate treatment of a known pulmonary coccidioidomycosis infection, dissemination of the disease can still ensue and should be considered in cases of acute encephalopathy.

Marijuana-Induced Lung Injury: A Case Report and a Review of the Literature.

Marijuana is a commonly abused illicit substance around the world, and lung injury related to its use has seldom been cited in the literature. Most cases describe marijuana-induced lung injury via vaping and the use of butane hash oil; however, no cases, to our knowledge, have associated lung injury related to marijuana smoke in the form of rolled "blunts" or cigarettes. We describe the case of a patient who presented to the hospital due to chest computed tomography findings demonstrating diffuse bilateral opacifications without signs of systemic inflammatory response syndrome. Bronchoscopy with bronchoalveolar lavage and sputum cultures failed to identify an infectious etiology, and serologies were negative for autoimmune etiologies. We aim to contribute to the limited body of literature describing marijuana-induced lung injury.

Left-Sided Transudative Chylothorax With Concomitant Chylous Ascites in the Setting of Liver Cirrhosis.

Most chylothoraces are caused by trauma and malignancy, and pleural fluid analysis typically demonstrates an exudative effusion. Transudative chylothorax is a rare manifestation and has only been cited in case reports in the current literature. Here, we present the case of a 59-year-old male with a history of liver cirrhosis secondary to alcohol abuse, chronic kidney disease stage 3a, and hypertension who presented with a left-sided pleural effusion and abdominal ascites. A thoracentesis and abdominal paracentesis were performed, and fluid analyses demonstrated a transudative chylothorax with concomitant chylous ascites. In this review, we aim to highlight a rare case of transudative chylothorax and discuss the pathogenesis and management of this condition.

Correlation Between HbA1c Levels and Mortality Rates in Hospitalized COVID-19 Patients.

Objective Throughout the coronavirus disease 2019 (COVID-19) pandemic, multiple factors have been associated with poor prognosis for those infected by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) virus. Age, obesity, and medical comorbidities have been linked to poor outcomes, including admission to the intensive care unit (ICU), acute renal failure, stroke, myocardial infarction (MI), mechanical ventilation, and even death for hospitalized COVID-19 patients. Although diabetes mellitus (DM) has also been included in this set of medical comorbidities, there have been inconsistencies in the currently available body of literature, suggesting that mortality rates may or may not be correlated to elevated glycosylated hemoglobin (HbA1c) levels. This study aims to determine whether there is a correlation or trend between a range of HbA1c values and in-hospital mortality among patients admitted to the hospital with a COVID-19 diagnosis. Materials and methods This study was a retrospective review of electronic medical records at Arrowhead Regional Medical Center in Colton, CA. Any patient above the age of 18 admitted to the hospital during a predetermined time frame, with either a positive COVID-19 PCR test on admission or during their hospital stay, was included in the study. These medical charts were reviewed for HbA1c values during admission or within three months prior to admission. In-hospital mortality was then recorded for each medical record with an available HbA1c value. Hospital discharge summaries were used to delineate comorbidities, including chronic kidney disease, cerebrovascular disease, coronary artery disease, congestive heart failure, cancer history, or history of deep vein thrombosis/pulmonary embolism among the patients included in the study. Average HbA1c values were recorded for the mortality and non-mortality groups, and their statistical significance was calculated. Results In this retrospective study, HbA1c levels were compared to mortality rates among adult patients admitted to the hospital with a concurrent COVID-19 diagnosis. From the analysis conducted, those with higher HbA1c levels did not have an increased rate of in-hospital mortality, and those with lower HbA1c levels did not have a decreased rate of in-hospital mortality. Comorbidity data as a confounding factor was also reviewed and excluded from the final analysis. The SARS-CoV-2 vaccine was also excluded as a confounder in this study by selecting a specific time frame for data collection. Based on our results, we propose that HbA1c levels likely have little to minimal correlation with mortality rates among hospitalized COVID-19 patients. Conclusion In this study, we show that HbA1c levels, regardless of concurrent comorbidities, are unlikely to be correlated to mortality rates among hospitalized COVID-19 patients. HbA1c levels should not be used as a marker for whether these patients should be admitted to the hospital for further inpatient management or discharged from the emergency department.

Intrapleural Tissue Plasminogen Activator and Dornase Alfa Administration for a Multiloculated Recurrent Malignant Pleural Effusion: A Case Report.

Malignant pleural effusions (MPEs) can often be very difficult to manage despite conservative interventions including thoracentesis and indwelling pleural catheter placement. These effusions can be septated and loculated, leading to complexities in drainage and symptomatic relief for patients. As such, physicians have experimented with the use of tissue plasminogen activator (t-PA) and dornase alfa (DNase) in attempts to drain complex malignant pleural effusions. Although the use of t-PA and DNase has been well studied in the context of empyema, the literature is limited in regards to the use of these medications in MPEs. Here, we present the case of a patient with a history of metastatic lung adenocarcinoma complicated by recurrent MPEs. Bedside ultrasonography revealed a septated fluid pocket in the pleural space of the right hemithorax. An indwelling pleural catheter (IPC) was placed with minimal symptomatic relief. The decision was made to administer t-PA and DNase through the IPC, resulting in the resolution of symptoms and radiographic findings. This case highlights the potential benefit of using t-PA and DNase to help drain complex malignant pleural effusions.

A Case of Chronic Aspiration Bronchiolitis.

Chronic aspiration bronchiolitis or diffuse aspiration bronchiolitis is a rare disease characterized by chronic inflammation of the bronchioles due to recurrent aspiration of foreign material. To date, there are limited data on chronic aspiration bronchiolitis because it is often present subclinically or remains undiscovered until autopsy. Additionally, time to diagnosis is often prolonged and includes invasive workup prior to definitive diagnosis. Here, we present a case of lung disease attributed to chronic aspiration after a thorough workup resulted in histopathology consistent with a primary diagnosis of aspiration bronchiolitis. Radiographic and pathologic specimens also demonstrated features of usual interstitial pneumonia adding to the complexity of pathology that can be seen with aspiration diseases.

West Nile Neuroinvasive Disease Treated With High-Dose Corticosteroids.

West Nile neuroinvasive disease, which includes encephalitis, meningitis, or acute flaccid paralysis, is one of the two most common manifestations of West Nile virus (WNV). According to many national agencies, since 1999, WNV has been one of the most common causes of epidemic viral encephalitis in the United States, especially in the state of California, and it will likely remain an important cause of neurological disease for years to come. To date, the mainstay of treatment for West Nile neuroinvasive disease has been supportive care with no data to support the routine use of any agents. Here, we present a unique case of West Nile encephalitis in a 61-year-old male who was successfully treated with a five-day course of high-dose corticosteroids. Although this rapid improvement could be a mere coincidence, it facilitates the need for further trials to determine if high-dose corticosteroids and other drugs may benefit patients in the treatment of West Nile neuroinvasive disease.

Diffuse Alveolar Hemorrhage in the Setting of Cytarabine Therapy in a Critically Ill Patient.

Diffuse alveolar hemorrhage (DAH) is a potentially life-threatening pulmonary condition characterized by hypoxemia with progression to respiratory failure, rapid onset of dyspnea, and blood loss anemia. While hemoptysis may be present and corroborates the diagnosis, it is absent in about half of the cases, resulting in a diagnostic challenge with variable presenting symptoms. Imaging findings on chest x-ray or computed tomography (CT) scans are also non-specific, often showing diffuse bilateral alveolar opacities. Because DAH is an under-recognized diagnosis, physicians should maintain a degree of clinical suspicion for DAH in patients with unexplained airspace opacities and no signs of an infectious etiology. This is especially important in higher-risk populations such as patients with hematological malignancies, who have a propensity for thrombocytopenia and coagulopathy compounded by the use of anticoagulants. Patients with hematological malignancies, namely acute myeloid leukemia (AML), are also at risk for drug-induced DAH due to the use of cytotoxic medications like cytarabine. Here, we present the case of a 48-year-old male with a past medical history of AML and myeloid sarcoma who developed shortness of breath after receiving cytarabine chemotherapy. Chest radiography revealed diffuse bilateral infiltrates. He was intubated and underwent flexible bronchoscopy, which resulted in a bloody effluent consistent with DAH. After ruling out infectious etiologies, we reached a final diagnosis of DAH and started the patient on corticosteroid therapy.

Tracheobronchitis Due to Invasive Aspergillus fumigatus in a Male With HIV With Minimal Imaging Findings.

Aspergillus tracheobronchitis (AT) is a rare manifestation of invasive aspergillosis. We present a case of tracheobronchitis caused by Aspergillus fumigatus in a 33-year-old male with neutropenia and known human immunodeficiency virus (HIV) infection with acquired immunodeficiency syndrome (AIDS). A 33-year-old male with HIV/AIDS presented to the hospital with symptoms of productive cough for over two months associated with subjective fevers, chills, and body aches. Computed tomography (CT) of the chest was significant for scattered sub-centimeter bilateral upper lobe nodules but otherwise normal. The patient underwent an extensive evaluation for his respiratory symptoms, including an initial sputum culture, which grew Haemophilus parainfluenzae in addition to preliminary fungal growth, prompting further evaluation with bronchoscopy. Bronchoscopy revealed diffuse adherent obstructive pseudomembranous plaques in the trachea and bilateral upper lobe segmental bronchi. Bronchoalveolar lavage cultures later grew Aspergillus fumigatus. Tracheobronchitis due to Aspergillus species is a rare cause of infection in patients with HIV. We believe that this case underscores the importance of further evaluation utilizing bronchoscopy in patients with AIDS who have respiratory symptoms despite mild abnormalities on chest CT. This approach can be used to rule out atypical endobronchial infections such as tracheobronchitis due to Aspergillus species.

Development of Arterial Thrombosis in a Patient on Therapeutic Anticoagulation for COVID-19.

The severe acute respiratory syndrome coronavirus-2 (SARS-CoV2) virus has been known to manifest various non-pulmonary complications in the affected patient, including both venous and less frequently, arterial thrombosis. Ongoing research is necessary to determine who might benefit from therapeutic anticoagulation as well as potentially develop an algorithm to predict thromboembolic events in this patient population. We present a case of a 65-year-old man with a past medical history of hypertension, diabetes type 2, and a previous cerebrovascular accident who was admitted with acute hypoxemic respiratory failure due to coronavirus disease 2019 (COVID-19). On admission to the hospital, the patient was initiated on therapeutic anticoagulation. Subsequently, he developed left lower limb ischemia. Imaging discovered arterial thrombosis in the bilateral deep femoral and left popliteal arteries. Consequently, the patient required catheter directed thrombolysis for partial reperfusion. Unfortunately, the patient succumbed to the complications of COVID-19. This case is notable in that it highlights the arterial thrombophilia associated with COVID-19 despite early intervention with therapeutic anticoagulation.