The imitation game: Pulmonary artery sarcoma masquerading as an acute pulmonary embolism.
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ABSTRACT:
SUBMITTER: Pupovac SS
PROVIDER: S-EPMC8691795 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease. The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.
| S-EPMC4189355 | biostudies-literature
Project description:Pulmonary artery sarcoma (PAS) is a rare and aggressive mesenchymal tumor that often mimics thromboembolic disease. Due to its rare and fatal nature, patients are often underdiagnosed or misdiagnosed. There is still no consensus regarding the diagnosis and treatment of PAS. We present a case of a 63 year old male misdiagnosed with pulmonary thromboembolism who received anticoagulant therapy. 18FDG positron emission tomography (PET) integrated with computed tomography (PET/CT) and subsequent surgery led to the final diagnosis of PAS. Whole exome sequencing of the tissue identified the genetic alterations profile of PAS: copy number variation (CNV) of KIT and mutations of TP53, PIK3CA, IL7R and ATR. Treated with chemotherapy followed by anlotinib, the patient's survival time was 8 months after firm diagnosis. To our knowledge, anlotinib used as a treatment for PAS has not been reported.
| S-EPMC6689559 | biostudies-literature
Project description:AimsThe non-invasive calculation of right ventricular (RV) haemodynamics as pulmonary artery (PA) capacitance (PAC) and pulmonary vascular resistance (PVR) have proved to be feasible, easy to perform, and of high prognostic value. We, therefore, evaluated whether baseline PAC and PVR could predict clinical outcomes for patients with acute pulmonary embolism (PE).Methods and resultsWe prospectively followed 373 patients [mean (standard deviation) age, 64.1 (14.9) years; 58.4% were men, and 27.9% had cancer] who had acute PE and transthoracic echocardiography within 1 day of diagnosis from 1 March 2013 through 30 June 2020. Pulmonary artery capacitance was calculated as left ventricular stroke volume/(PA systolic pressure - PA diastolic pressure). Pulmonary vascular resistance was calculated as (tricuspid regurgitant velocity/RV outflow tract velocity time integral) × 10 + 0.16. These two variables were calculated retrospectively from the values obtained with transthoracic echocardiography. Pulmonary artery capacitance was acquired in 99 (27%) patients and PVR in 65 (17%) patients. Univariable and bivariable logistic regression analyses, and receiver operating characteristic curves were used to evaluate the ability of these haemodynamic measurements to predict mortality up to 6 months. After using bivariable models to adjust individually for age, cancer, and pulmonary hypertension. Pulmonary vascular resistance was associated with all-cause mortality at 3 months [area under the curve (AUC) 0.75, 95% confidence interval (CI) 0.61-0.86; P = 0.01], and 6 months (AUC 0.81; 95% CI 0.69-0.91; P ≤ 0.03). Pulmonary artery capacitance was associated with all-cause mortality at 30 days (AUC 0.95; 95% CI 0.82-0.99; P < 0.001) and 3 months (AUC 0.84; 95% CI 0.65-0.99; P = 0.003).ConclusionNon-invasive measurement of RV haemodynamics could provide prognostic information of patients with acute PE. Pulmonary artery capacitance and PVR are potentially important predictors of all-cause mortality in these patients and should be explored in future studies.
| S-EPMC9242029 | biostudies-literature
Project description:Primary cardiac tumors are an incredibly rare finding. Cardiac myxomas are the most primary cardiac tumors that often occur within the left atrium. When left untreated, they pose a high risk of causing hemodynamic collapse by obstruction or can embolize and result in thromboembolic stroke. The presentation of cardiac myxoma varies greatly and can be associated with significant morbidity and mortality when undiagnosed. A careful physical examination and high degree of suspicion is crucial in early diagnosis and intervention. Our team presents a 46-year-old female patient with no significant past medical history that presented to the emergency department with a neurological deficit that was concerning for a transient ischemic attack. Initial laboratory workup and electrocardiogram was suggestive for pulmonary embolism; however, upon evaluation with imaging, the patient was found to have a 1.6 × 3.4 cm mass fixed to the mitral leaflet that was then confirmed on transthoracic echocardiography. Our patient was found to have non-obstructive coronary artery disease on cardiac catheterization and ultimately underwent successful mass resection by cardiothoracic surgery.
| S-EPMC8683111 | biostudies-literature
Project description:Acute pulmonary embolism (APE) is a common cause of acute cardiovascular failure and has a high morbidity and mortality rate. Inhibiting the excessive proliferation and migration of pulmonary artery smooth muscle cells (PASMCs) is a potential treatment strategy following an APE. Various microRNAs (miRNAs/miRs) have been shown to regulate cell proliferation, apoptosis and other physiological processes. However, the specific mechanisms underlying the action of multiple miRNAs are still not understood in APE. In the present study, the role of miR?106b?5p on APE was demonstrated in platelet?derived growth factor (PDGF)?induced PASMCs in vitro and in an APE?mouse model in vivo. The results showed that miR?106b?5p expression was downregulated in PDGF?induced PASMCs and APE mice, and NOR1 levels were upregulated. Proliferating cell nuclear antigen (PCNA) expression levels in cells and proliferation of PASMCs proliferation and migration were reduced following treatment with miR?106b?5p agomiR, and increased following treatment with miR?106b?5p antagomiR. miR?106b?5p targeted the 3' untranslated region of NOR?1 mRNA and reduced NOR1 expression. NOR1 overexpression reversed the effects of miR?106?5p on PDGF?induced PASMCs. The functional roles of miR?106b?5p in PDGF?induced PASMCs and an APE mouse?model, and the underlying molecular mechanisms were evaluated. AgomiR?106b?5p improved APE?induced mortality and pulmonary vascular proliferation in mice. These data suggest that miR?106?5p is a novel regulator of proliferation of PASMCs and of pulmonary vascular remodeling through PDGF?induced PASMCs in an APE mouse model via targeting NOR1. These results expand the understanding of the pathogenesis underlying APE and highlight potential novel therapeutic targets.
| S-EPMC7138273 | biostudies-literature
Project description:Primary pulmonary artery sarcoma (PAS) is an extremely rare malignant disorder that presents like pulmonary thromboembolism (PE). Primary osteogenic sarcoma in the pulmonary artery (PA) is even rarer and can produce osteoid or cartilaginous matrix. Few studies have described the radiographic characteristics of osteosarcoma of the PA. We there report a case of a 78-year-old male patient with osteosarcoma in the PA where the patient went through surgical treatment after careful multimodalityimaging assessment. The patient was admitted to our hospital with the nonspecific symptom of heart failure. Multimodality imaging showed the primary lesion adhering to the arterial wall but without invading into surrounding tissues. PET/CT showed signs of hypometabolic activity within the lumen of the main PA. Cardiac MRI showed preserved left ventricular systolic function. CT showed distinctive features of PA osteosarcoma (a slightly hyperdense mass with calcification in pulmonary trunk).
| S-EPMC8712784 | biostudies-literature
Project description:Impact statementAccumulating evidence suggests that vascular remodeling due to immoderate proliferation and migration of SMCs is a common process occurring in APE. In this work, we tried to find a breakthrough in the pathological mechanism to alleviate the prognosis of APE by improving SMCs proliferation and explored the effect of JANEX-1 on PDGF-induced proliferation-related molecules in PVSMCs and assessed the therapeutic potential of JAK3 for vascular remodeling in APE mice. We demonstrated that JANEX-1, blocking JAK3 expression or activity, reduced JAK3/STAT3 signaling pathway, VEGF expression and FAK activation, and PDGF-induced proliferation of PVSMCs. Moreover, JANEX-1 inhibited the thrombus-induced intimal hyperplasia and the expression of VEGF and FAK activation in neointimal SMCs of APE mice. The data are helpful to elucidate the pharmacological mechanism and potential therapeutic effect of JANEX-1 in APE.
| S-EPMC7441354 | biostudies-literature
Project description:Rats were given pulmonary embolism by i.v. injection of 25 micron polystyrene microspheres or 0.01% Tween20 solution as vehicle control Embolism of microspheres is irreversible and causes dose dependent pulmonary hypertension Keywords: time course and dose response
2009-09-01 | GSE13535 | GEO
Project description:BackgroundCoronavirus disease 2019 (COVID-19) disease is a highly prothrombotic state. Deep vein thrombosis (DVT) and pulmonary embolism (PE) are observed with increased incidence in patients infected with the severe acute respiratory syndrome coronavirus 2 virus.Case summaryA 57-year-old male patient with a recent COVID-19 infection complained of leg swelling shortly after his COVID ward discharge. A few days later he was hospitalized with acute massive PE and DVT of his left leg was diagnosed. In another facility, as the first line of treatment, the PE was managed with catheter-directed therapy (CDT) using thrombus defragmentation via 5F (French) Pigtail catheter and supraselective application of 40 mg alteplase. Following the procedure, in addition, 50 mg alteplase was also applied as a 1 hour systemic infusion. Despite the haemodynamic stabilization of the patient, he remained persistently symptomatic and tachycardic. Three days later-in our institution, a second computed tomography pulmoangiography revealed massive thrombotic masses mainly in the left pulmonary artery. Successful percutaneous thrombus aspiration was conducted. The procedure was uneventful with an immediate drop of systolic pulmonary artery pressure from 68 to 47 mmHg and relief of the patient's symptoms.DiscussionIn the era of the COVID-19 pandemic, physicians have to remain vigilant of its potential thrombotic complications, the most commonly observed being DVT and PE. We demonstrated the efficacy of percutaneous thrombus aspiration in a patient with acute COVID-19-associated PE, after initial CDT with thrombus defragmentation and high-dose tissue plasminogen activator was implemented with a suboptimal result.
| S-EPMC9214152 | biostudies-literature
Project description:BACKGROUND:Physicians treating acute pulmonary embolism (PE) are faced with difficult management decisions while specific guidance from recent guidelines may be absent. METHODS:Fourteen clinical dilemmas were identified by physicians and haematologists with specific interests in acute and chronic PE. Current evidence was reviewed and a practical approach suggested. RESULTS:Management dilemmas discussed include: sub-massive PE, PE following recent stroke or surgery, thrombolysis dosing and use in cardiac arrest, surgical or catheter-based therapy, failure to respond to initial thrombolysis, PE in pregnancy, right atrial thrombus, role of caval filter insertion, incidental and sub-segmental PE, differentiating acute from chronic PE, early discharge and novel oral anticoagulants. CONCLUSION:The suggested approaches are based on a review of the available evidence and guidelines and on our clinical experience. Management in an individual patient requires clinical assessment of risks and benefits and also depends on local availability of therapeutic interventions.
| S-EPMC3913120 | biostudies-literature